ketogenic diet guide
TRANSCRIPT
Edited by
Ketogenic Diet Guide ي تجویز رژیم کتوژنیک
Sepideh Tabarestani, MD Pediatric Neurologist
Parvaneh Karimzadeh, MD Pediatric Neurologist Zahra Babaei, Dietitian
Ketogenic Diet Guideي تجویز رژیم کتوژنیکراهنما
Edited By
Sepideh Tabarestani, MD Pediatric Neurologist
Parvaneh Karimzadeh, MD Pediatric Neurologist
Zahra Babaei, Dietitian Maryam Azizi, Dietitian
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Ketogenic Diet Guide
Sepideh Tabarestani, MD
Maryam Azizi, Dietitian
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3
Editor
Sepideh Tabarestani MD Pediatric Neurologist
Contributors
Parvaneh Karimzadeh MD Pediatric Neurologist
Professor of Pediatric Neurology
Zahra Babaie MSc Dietitian
Maryam Azizi MSc
Dietitian
Pediatric Neurology Department, Pediatric Neurology Research Center, Mofid Children’s Hospital, Shahid Beheshti University of
Medical Sciences,Tehran, Iran
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Ketogenic Diet Guide
ISBN: 978-605-9528-72-6 Publisher: 2019 İstanbul Medikal Sağlık ve Yayıncılık Hiz. Tic. Ltd. Şti. 34104, Çapa-İstanbul-Türkiye www.istanbultip.com.tr E-mail: [email protected] Publisher certificate no: 12643 Printed at: SAGE Yayıncılık Rek. Mat. San. Tic. Ltd. Şti. Kazım Karabekir Cad. Uğurlu İş Merkezi No: 97/27 İstiklal-Ankara-Türkiye Tel: 0312 341 00 02 www.bizimdijitalmatbaa.com E-mail: bilgi@ bizimdijitalmatbaa.com
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برنگذرد یشهبرتراند یننام خداوند جان و خرد کز به
شود به یم یمکتاب تقد ینا
آنها آموختم ینکه بر بال یکودکان يهمه This book is dedicated to all children from whom I learned. S.T.
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ACKNOWLEDGMENT I would like to express my sincere appreciation to all who helped me in publishing this book: Dr. Mohamad Ghofrani, Pediatric neurology department head, Shahid Beheshti University of Medical Sciences for giving us a chance to work as a team, Dr. Parvaneh Karimzadeh, my dear professor for her unwavering support and friendship, Dr. Beheshteh olang and her team at Mofid children’ hospital Nutrition department; for offering their assistance and resources, Nutricia team in Tehran and Turkey; special thanks to Ms. Mojdeh Zohari for her enthusiastic work and follow-ups, And finally Dr. Eric Kossoff for his kind advice and comments, beyond the borders.
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INTRODUCTION
The world of ketogenic diet therapies is expanding. Families and patients in all countries are asking their neurologists to help them start ketogenic diets to improve epilepsy symptoms as well as other conditions. As various cultures, religions, and beliefs have very different ways of diet and food types in their practices, books like this amazing one are very important. It is with great pleasure that this guide to ketogenic diet therapies for children and adults in Iran is published. Dr.Tabarestani and Dr.Karimzadeh, along with their colleague dietitian Zahra Babaei have written this book to help families adapt to this change in diet and it is a worthy addition to ketogenic diet books around the world. It includes the latest research, guidelines, protocols and even Iranian diet and food tradition to help make sure the diet is done correctly and safely. They are to be commended for this excellent work and I wish them (and you the reader) the best of luck on your ketogenic journey. Sincerely, Dr. Eric Kossoff Professor of Neurology and Pediatrics Director, Pediatric Ketogenic Diet Center Johns Hopkins University Baltimore, Maryland, USA
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This book intends to offer a simple and quick guide for pediatric neurologists and nutritionists who work in pediatric ketogenic diet team to: · Correctly apply Ketogenic Diet including right time and appropriate candidates, · Precisely implement the diet including an individualized approach, accurate monitoring in a sufficient
and consistent way · Managing side effects by considering vulnerability implications, predictions / timely actions · And finally, keep the children as safe as possible in this journey.
To be brief, some recommendations and diet calculations are summarized in tables, or shown by numbers in order they should be done or based on the level of importance. The comparisons are also summarized as tables to appear easily notable. All information is updated based on the recent recommendations of international ketogenic diet study group, 2018. Remember that the ketogenic diet is an anti-seizure medication so as other antiepileptic drugs should be prescribed under the supervision of a Ketogenic Diet team for correct application and to prevent any undesired complications.
فوق نظر تحت دیضد تشج با هاي نیا. شود زیتجو یککتوژن میرژ م ازین ماریضد تشنج؛ ب يداروها ریسا کینورولوژ ي نهیمعاو همچنین تشنج ها
در درمان تشنج هاي مقاوم به درمان توضیح انتخاب صحیح بیماران، )خلاصه شده در جداول مختلف
در حد امکان با در (با ذکر مثال و دستورات غذایی براي بیماران، کلینیکی توصیه شده
عملی بالینی و تغذیه اي و در دو فصل آخر نکات .فرم هاي لازم براي ارزیابی و پیگیري بیماران در آخر آورده شده است
به روز 2018 در سالیک کتوژن یمرژ
هايدارو سایرضد تشنج است و مانند يدارومیدر ت دهیموزش دآي هیمتخصص تغذ تخصص اعصاب کودکان وسا زیمانند تجو رایکاملا متفاوت است ز ها میرژتشنج ها تیوضعی و احتمال عوارض جانب نظر
در درمان تشنج هاي مقاوم به درمان توضیح یکاین کتاب ابتدا درباره ي جایگاه رژیم کتوژنخلاصه شده در جداول مختلف(قدم به قدم به صورت
با ذکر مثال و دستورات غذایی ویز و محاسبه ي انواع مختلف رژیمکلینیکی توصیه شدهپیگیري هاي لازم کلینیکی و پارا ،)نظر گرفتن غذاهاي ایرانی
و در دو فصل آخر نکات پیشگیري، تشخیص و درمان عوارض جانبی، فرم هاي لازم براي ارزیابی و پیگیري بیماران در آخر آورده شده است. ضروري شرح داده شده است
رژ یالملل ینانجمن ب يها یهکتاب براساس توص
9
پیشگفتاردارو کی کیکتوژن میرژ
تخصص اعصاب کودکان ورژ ریاز سا ییغذا میرژنظر از نظمم یابیارز به
. ددار يدوره ا
این کتاب ابتدا درباره ي جایگاه رژیم کتوژنبه صورتسپس می دهد وویز و محاسبه ي انواع مختلف رژیمچگونگی تج
نظر گرفتن غذاهاي ایرانیپیشگیري، تشخیص و درمان عوارض جانبی،
ضروري شرح داده شده استکتاب براساس توص ینضمنا اطلاعات ا
. شده است
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Ketogenic D
Ketogenic Diet Guide
11
uide
Chapters
Chapter 1: Individualized Ketogenic Diet The Ketogenic Diet (KD) History Different Methods Brief mechanism of action Why KD? Patient selection Summary of KD Indications General considerations Introduction for families AEDs discontinuation Supplementation Other Anti-seizure modalities Monitoring Important follow up, tips Visit intervals Urinary Ketosis Blood Beta Hydroxy-Butyric Acid (BHB)Side Effects Fluid Physical exam and blood work Medications Optional Investigations Chapter 2: Traditional KD Methods What is classic KD? Calorie Fluid Fat Protein Carbohydrate Diet Prescription Case: Calculation and Meal Plan Diet Initiation: Inpatient Monitoring during diet initiation Hypoglycaemia Gastrointestinal Symptoms Metabolic Acidosis Seizure Frequency Diet Initiation: Outpatient Diet Process Using Exchange List Common Problems In the first MonthHunger Thirst Lost seizure control Diet Discontinuation Important tips in all KD types Classic KD Discontinuation Medium-chain KD (MCT-KD) Important Tips Diet initiation
Chapters
Butyric Acid (BHB)
Common Problems In the first Month
MCT Calculation Case: Calculation and Meal Plan Diet Process Monitoring Discontinuation Exchange List Exchange list for Fat and Protein in CKD Exchange List for carbohydrates in MCT diet Chapter 3: KD for InfantsImportant Tips Patient Selection Baseline AssessmentCalorie Fat Protein Carbohydrate Fluid Diet Initiation Case: Calculation and MeDiet Process Monitoring and Follow upGrowth Side Effects Chapter 4: Modern KD methodsModified Atkins Diet (MAD)Important Tips Diet Initiation Diet Process Case: Diet and Meal PlanMonitoring Discontinuation Low Glycaemic Index Diet (LGID)Important Tips Diet Initiation Diet Process Case: Calculation and Meal PlanMonitoring During Illness Discontinuation Modified Ketogenic Diet (MKD)Complementary TablesCarbohydrate Contents of FoodsGlycaemic Index of Foods
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MCT Calculation Case: Calculation and
Exchange list for Fat and Protein in CKD
List for carbohydrates in MCT diet
: KD for Infants
Baseline Assessment
Case: Calculation and Meal plan
Monitoring and Follow up
: Modern KD methods Modified Atkins Diet (MAD)
Case: Diet and Meal Plan
Low Glycaemic Index Diet (LGID)
Case: Calculation and Meal Plan
Modified Ketogenic Diet (MKD) Complementary Tables Carbohydrate Contents of Foods Glycaemic Index of Foods
Chapters
Chapter 5: Side Effects management Side Effects Classification Managing side effects Gastrointestinal Metabolic Infection Renal Calculi Growth Cardiac Problems Bone health Long Term Side Effects Chapter 6: Which KD? How to choose diet type CKD candidates MCT candidates MAD candidates LGID candidates Switching diet Seizure control Tolerance / Compliance issue How to switch Chapter 7: Practical pointsCommon Medications Monitoring Ketosis and ComplianceManaging Illness NPO Situations Complementary information Food’s Value RDA Tables Forms
How to switch
Chapter 7: Practical points
Monitoring Ketosis and Compliance
Chapter 8: KD in Neurometabolic disorders Mechanism of action Important tips KD and GLUT1 deficiency KD and PDHD Chapter 9: KD in adultsImportant tips MAD Protocol Chapter 10: KD ProductsKD Commercial productsFormulas Modulars Carbohydrate Protein Lipid Sweeteners Calorie Free Water Carbohydrate Free MultivitaminsUseful Links Chapter 11: Iranian keto foods and recipes Foods with 4:1 ratio Kuku Soup Stew Snacks and deserts with 4:1 ratioFoods with 3:1 ratio Soup Stew Salad Snacks and deserts with 3:1 ratio
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Chapter 8: KD in Neurometabolic
KD and GLUT1 deficiency
Chapter 9: KD in adults
Chapter 10: KD Products KD Commercial products
Carbohydrate Free Multivitamins
Chapter 11: Iranian keto foods and
Snacks and deserts with 4:1 ratio
Snacks and deserts with 3:1 ratio
Chapter One
History: The classic Ketogenic Diet (KD) was first developed in the in children. Originally, it was a high fat, low carbohydrate diet administered after 48 hours of fasting by the gradual introducless attention after the introductionyears, its use was expanded worldwide with a variety of options. Current possibilities:
· Alternative methods of administration: MediumAtkins Diet (MAD) / Low Glycemic Index Diet (LGID)
· Different forms of KD products: oral prepared meals, the feedings, or total par
· Individualised diet plan
Recent guidelines have been updated based on the with a more liberal diet ratio, calories, Now, the Ketogenic diet is considered as an effective, acceptable, nontreatment all over the world, for refractory seizures in children and many other conditions such as Alzheimer’s disease or ALS. Evidence
Individualized Ketogenic Diet
The classic Ketogenic Diet (KD) was first developed in the early 1920s for refractory seizures in children. Originally, it was a high fat, low carbohydrate diet administered after 48 hours of
introduction of calories (Ketogenic meals) over 3 days. KD received introduction of new antiepileptic medications. However, in the last 15
years, its use was expanded worldwide with a variety of options.
methods of administration: Medium-chain KD (MCT
Atkins Diet (MAD) / Low Glycemic Index Diet (LGID) Different forms of KD products: oral prepared meals, the formulafeedings, or total par enteral nutrition.
et plan
Recent guidelines have been updated based on the child’s individual nutritional requirements liberal diet ratio, calories, fluids, and supplementations.
Now, the Ketogenic diet is considered as an effective, acceptable, non-pharmacologtreatment all over the world, for refractory seizures in children and many other conditions such as Alzheimer’s disease or ALS. Evidence-based KD applications are shown in
Individualized Ketogenic Diet
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1920s for refractory seizures in children. Originally, it was a high fat, low carbohydrate diet administered after 48 hours of
tion of calories (Ketogenic meals) over 3 days. KD received of new antiepileptic medications. However, in the last 15
chain KD (MCT-KD), Modified
formula for infants, tube
individual nutritional requirements
pharmacological treatment all over the world, for refractory seizures in children and many other conditions
based KD applications are shown in Table 1-1.
Chapter One
Table 1-1. Evidence-based applications of KD Proceedings of Ketogenic Diet Therapies Symposium, March 2015, Manhattan Beach, CA, USA
Level I Evidence
(studies with no control groups case studies, and anecdote)
- Epilepsy – medication resistant; pediatrics
- -
- - -
-
based applications of KD Proceedings of Ketogenic Diet Therapies Symposium, March 2015, Manhattan Beach, CA, USA
Level II Evidence
(prospective or retrospective studies)
(randomized controlled trials)
Dravet syndrome Epilepsy - medication
resistant; adults Infantile spasms Lennox-Gastaut syndrome Myoclonic-astatic epilepsy
(Doose syndrome) Tuberous sclerosis complex
- Adenylosuccinate lyase deficiency- Alpers-Huttenlocher syndrome- Alzheimer’s disease - Argininosuccinate lyase deficiency- Amyotrophic lateral sclerosis- Autism - Cancers - Diabetes mellitus- Dravet syndrome- Glycogenosis and glycogen storage
disorders - Glucose-1 transporter deficiency
syndrome (Glut- Lafora body disease- Landau-Kleffner Syndrome- Lennox Gastau- Metabolic Syndrome- Migraine - Mitochondrial disorders
I, II, IV - Mitochondrial encephalopathy w/
lactic acidosis & stroke; (MELAS)- Nonketotic - Obesity - Otahara syndrome- Parkinson’s disease- Phosphofructokinase deficiency- Polymerase gamma related disorders
(POLG) - Pyruvate dehydrogenase deficiency- Respiratory chain defects- Rett syndrome- Subacute sclerosing panencephalitis- Succinic semialdehyde
dehydrogenase deficiency- Traumatic brain injury
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Proceedings of Ketogenic Diet Therapies Symposium, March 2015, Manhattan Beach, CA, USA Level III Evidence
(randomized controlled trials)
Adenylosuccinate lyase deficiency Huttenlocher syndrome
Alzheimer’s disease – early onset Argininosuccinate lyase deficiency Amyotrophic lateral sclerosis
Diabetes mellitus Dravet syndrome
osis and glycogen storage
1 transporter deficiency syndrome (Glut-1DS) Lafora body disease
Kleffner Syndrome Lennox Gastau Metabolic Syndrome
Mitochondrial disorders – Complex
Mitochondrial encephalopathy w/ ic acidosis & stroke; (MELAS)
Nonketotic hyperglycinemia
Otahara syndrome Parkinson’s disease Phosphofructokinase deficiency Polymerase gamma related disorders
Pyruvate dehydrogenase deficiency Respiratory chain defects Rett syndrome
e sclerosing panencephalitis Succinic semialdehyde dehydrogenase deficiency Traumatic brain injury
Chapter One
Different methods of applying the KD There are different methods of KD diet; all have high facomponents with variable design and administration to make the diet more tolerable. These methods include:
· Classic Ketogenic Diet (KD); inpatient, outpatient and for infants.· Medium-chain Triglyceride KD (MCT· Modified Atkins Di· Low Glycaemic Index Diet (LGID)
Differences between components of each KD method are summarized in 1-2. They will be separately discussed in following chapters.
Table 1-2. Comparison of different KDs Regular Fat 30-35
Protein 20 (10-15) CHO 50 Ratio† 0.2-0.3/1
*10g at the start, † fat/non-fat ratio, ®, his diet is not ratio related, yet relies on MCT content in the
Mechanism of action in brief: There are three suggested arms:
1) Reduce glucose (glycolysis inhibition) · Increased leptin → glucose levels and inhibit AMPA receptor
excitation · Adenosine release → Adenosine receptors activation (A1R) →↑
channels → Hyper polarization· Decreased Brain-Derived Neurotrophic Factor (BDNF) and TrkB
↓ Seizures, epileptogenesis effects
2) High blood free fatty acids level (PUFAs)
· PUFAs → ↑ activity of braingeneration, neuronal dysfunction, and consequent neurodegeneration
· PUFAs → ↑ peroxisome proliferatorcytokine expression (that shows Improved seizure con
Different methods of applying the KD
There are different methods of KD diet; all have high fat and low carbohydrate components with variable design and administration to make the diet more tolerable. These methods include:
Classic Ketogenic Diet (KD); inpatient, outpatient and for infants.chain Triglyceride KD (MCT-KD)
Modified Atkins Diet (MAD) Low Glycaemic Index Diet (LGID)
Differences between components of each KD method are summarized in . They will be separately discussed in following chapters.
2. Comparison of different KDs LGID MAD MCT-KD
60 60-70 30-60 LCT:11-45
20-30 20-30 10 10 10 g* 15-19 1:1 1-2:1 3:1®
, his diet is not ratio related, yet relies on MCT content in the
Mechanism of action in brief:
There are three suggested arms:
Reduce glucose (glycolysis inhibition) ↓ → glucose levels and inhibit AMPA receptor-mediated synaptic
→ Adenosine receptors activation (A1R) →↑ opening ofHyper polarization in neurons and decreased neuronal excitability
Derived Neurotrophic Factor (BDNF) and TrkB ↓ Seizures, epileptogenesis effects
High blood free fatty acids level (PUFAs) → ↑ activity of brain-specific uncoupling proteins (UCPs)
generation, neuronal dysfunction, and consequent neurodegeneration→ ↑ peroxisome proliferator-activated receptor- α (PPAR α) → ↓IL
cytokine expression (that shows hyper excitability and seizure generation effects Improved seizure control
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t and low carbohydrate components with variable design and administration to make the diet more
Classic Ketogenic Diet (KD); inpatient, outpatient and for infants.
Differences between components of each KD method are summarized in Table
KD KD 85-90
(LCT) 6-8 2-4 3-4:1
, his diet is not ratio related, yet relies on MCT content in the diet.
mediated synaptic
opening of KATP creased neuronal excitability
Derived Neurotrophic Factor (BDNF) and TrkB signaling in brain →
specific uncoupling proteins (UCPs) → limit ROS generation, neuronal dysfunction, and consequent neurodegeneration
α (PPAR α) → ↓IL-1ꞵ and seizure generation effects →
Chapter One
· PUFAs → ↑mitochondrial biogenesis → enhance energy production and reserves → stabilized synaptic function
3) High blood ketone body level· KD provides: High fat / Low carbohydrate diet;
Switch body metabolism to use fatty acid as primary source instead of glucose fatty acid beta-oxidation → production of Acyl CoA in high amount → excess Acyl CoA shift to → high rate synthesis of 3 ketone bodies in the body (KBs)
· Anti-convulsant mechanism of Ketone Bodies (KBs)
KBs → passing through the bloodthe brain interstitial space, the glia and the neurons glucose consumption.
· Further changes induce by KBs include:· pH changes → acidosis → induce
neurotransmitter receptors· Increased inhibition and/or decreased excitation
balance favoring inhibition:o Increased GABA level by inhibiting glutamate decarboxylase.
§ GABA influx of negatively charged ions neuronal
o inactivation of VGLUT and inhibition of g§ KBs
of VGLUT. Vesicular Glutamate Transporters determine the of glutaminergic neurons
· Change in biologic monoamines: o Reduced dopamine and seroo Increased nor epinephrineo Increased adenosine level (efficacy depends on the presence of adenosine A1
receptors) · Antioxidant effects because of reducing reactive oxygen species
Why KD? Patient selection Almost 20-30% of children requiring treatments such as KD. In the past, KD had been used as the last therapeutic resource. Currently, the refractory(Anti-Epileptic Drug) tried with proper dose and duration.
→ ↑mitochondrial biogenesis → enhance energy production and reserves → stabilized synaptic function → improved seizure control
High blood ketone body level KD provides: High fat / Low carbohydrate diet;
witch body metabolism to use fatty acid as primary source instead of glucose → production of Acyl CoA in high amount → excess Acyl CoA shift
→ high rate synthesis of 3 ketone bodies in the body (KBs)
lsant mechanism of Ketone Bodies (KBs)
→ passing through the blood-brain → delivered by monocarboxylic acid transporters to the brain interstitial space, the glia and the neurons → being used as primary fuel/and reduced
changes induce by KBs include: → acidosis → induce behavioral alteration of ion channels and
neurotransmitter receptors Increased inhibition and/or decreased excitation → altering the inhibitory/excitatory
inhibition: GABA level by inhibiting glutamate decarboxylase. GABA → stimulation of chloride channel receptors → increasing the influx of negatively charged ions → changes membrane potentials → neuronal hyper polarization
inactivation of VGLUT and inhibition of glutamate neurotransmitter receptorsKBs → competitive inhibition of chloride channels which are activators of VGLUT. Vesicular Glutamate Transporters determine the of glutaminergic neurons
Change in biologic monoamines: Reduced dopamine and serotonin level in CSF
nor epinephrine level Increased adenosine level (efficacy depends on the presence of adenosine A1
because of reducing reactive oxygen species
Why KD? Patient selection
30% of children with epilepsy will experience medically refractory seizures requiring treatments such as KD. In the past, KD had been used as the last therapeutic
refractory seizure is defined as failure of two appropriate AEDs ug) tried with proper dose and duration.
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→ ↑mitochondrial biogenesis → enhance energy production and reserves →
witch body metabolism to use fatty acid as primary source instead of glucose → high rate of → production of Acyl CoA in high amount → excess Acyl CoA shift
→ delivered by monocarboxylic acid transporters to → being used as primary fuel/and reduced
alteration of ion channels and
→ altering the inhibitory/excitatory
GABA level by inhibiting glutamate decarboxylase. → stimulation of chloride channel receptors → increasing the
→ changes membrane potentials →
lutamate neurotransmitter receptors → competitive inhibition of chloride channels which are activators
of VGLUT. Vesicular Glutamate Transporters determine the function
Increased adenosine level (efficacy depends on the presence of adenosine A1
with epilepsy will experience medically refractory seizures requiring treatments such as KD. In the past, KD had been used as the last therapeutic
seizure is defined as failure of two appropriate AEDs
Chapter One
The chance of seizure control with the following AEDs is fairly low; around 30%. However, KD offers a 50% chance of seizure reduction, and 10% of seizure freedom. Besidresponder group, just a minority will have seizure recurrence after diet discontinuation which is usually after 2 years. KD was originally reported to be nonetheless; it is not specific for any parCommonly, it is used in refractory epilepsy and epileptic encephalopathy of childhood as the last resource. Recently, Dressler, A. et al. 2010 study showed, an important factor in response to KD is the shorter duration of epilepsy before diet AEDs. About 30% of children on AEDs will develop side effects. Some AEDs may negatively affect cognition and behavior in children with neurologic disorders, who may already have theseissues. Altogether, KD offers a promising seizure control rate, some cognitive and improvements, along with relatively lower side effects compared to AEDs. Considering, shorter refractory seizure period before diet initiation. Epilepsy, syndromes, and conditions in which KD has been reported effective are summarized in Table 1-3.
Table 1-3. Conditions in which KD has been effective
Glucose transporter protein 1 (GLUT-1) deficiency (SLC2A1 gene mutation)
Pyruvate dehydrogenase deficiency (PDHD) Infantile spasms Myoclonic-astatic epilepsy (Doose syndrome) Severe myoclonic epilepsy of infancy (Dravet
syndrome) Associated with Leigh’s syndrome Associated with lactic acidosis and cerebral dysgenesis
KD indication based on international KD study group 2018 is summarized in these Indications, KD has been consistently effective by more than 60seizure frequency reported at least by 3 papers from 2 KD therap Table 1-4 is practical and will improve decision making about diet initiation, we believe.
Table 1-4. KD indications (International KD study group recommendations, 2018)
Angelman syndrome Complex I mitochondrial disorders Dravet syndrome Epilepsy with myoclonic-atonic seizures (Doose
syndrome) Glucose transporter protein 1 (Glut-1) deficiency
syndrome (Glut1DS) Febrile infection-related epilepsy syndrome (FIRES)
The chance of seizure control with the following AEDs is fairly low; around 30%. However, % chance of seizure reduction, and 10% of seizure freedom. Besid
responder group, just a minority will have seizure recurrence after diet discontinuation which
KD was originally reported to be most effective in refractory myoclonic epilepsies nonetheless; it is not specific for any particular form of seizure or epilepsy syndrome. Commonly, it is used in refractory epilepsy and epileptic encephalopathy of childhood as the
resource. Recently, Dressler, A. et al. 2010 study showed, an important factor in response duration of epilepsy before diet initiation, therefore, receiving
About 30% of children on AEDs will develop side effects. Some AEDs may negatively affect in children with neurologic disorders, who may already have these
Altogether, KD offers a promising seizure control rate, some cognitive and improvements, along with relatively lower side effects compared to AEDs. Considering, shorter refractory seizure period before diet initiation.
conditions in which KD has been reported effective are summarized
3. Conditions in which KD has been effective 1) deficiency
HD)
astatic epilepsy (Doose syndrome) Severe myoclonic epilepsy of infancy (Dravet
Associated with lactic acidosis and cerebral dysgenesis
Succinic semialdehyde dehydrogenase (SSADH Deficiency)
Phosphofructokinase deficiency Mitochondrial respiratory chain complex defects Ketotic hypoglycemia Glycogenosis type V (McArdle disease Acquired epileptic aphasia (Landau Lafora body disease Subacute Sclerosing Panencephalitis
KD indication based on international KD study group 2018 is summarized in these Indications, KD has been consistently effective by more than 60-70% of the seizure frequency reported at least by 3 papers from 2 KD therapy centers
is practical and will improve decision making about diet initiation, we believe.
(International KD study group recommendations, 2018)
atonic seizures (Doose
1) deficiency
related epilepsy syndrome (FIRES)
Formula-fed (solely) children or infants Infantile spasms Ohtahara syndrome Pyruvate dehydrogenase deficiency (PDHD) Super-refractory status epilepticus Tuberous sclerosis complex
18
The chance of seizure control with the following AEDs is fairly low; around 30%. However, % chance of seizure reduction, and 10% of seizure freedom. Besides, in a
responder group, just a minority will have seizure recurrence after diet discontinuation which
effective in refractory myoclonic epilepsies ticular form of seizure or epilepsy syndrome.
Commonly, it is used in refractory epilepsy and epileptic encephalopathy of childhood as the resource. Recently, Dressler, A. et al. 2010 study showed, an important factor in response
, receiving fewer
About 30% of children on AEDs will develop side effects. Some AEDs may negatively affect in children with neurologic disorders, who may already have these
Altogether, KD offers a promising seizure control rate, some cognitive and behavioral improvements, along with relatively lower side effects compared to AEDs. Considering,
conditions in which KD has been reported effective are summarized
Succinic semialdehyde dehydrogenase (SSADH
Phosphofructokinase deficiency Mitochondrial respiratory chain complex defects
Glycogenosis type V (McArdle disease Acquired epileptic aphasia (Landau-Kleffner Syndrome)
Subacute Sclerosing Panencephalitis (SSPE)
KD indication based on international KD study group 2018 is summarized in Table 1-4. In 70% of the reduction in
centers.
is practical and will improve decision making about diet initiation, we believe.
fed (solely) children or infants
te dehydrogenase deficiency (PDHD) refractory status epilepticus
Chapter One
KD study group also suggested seven particular metabolic and epileptic syndromes in which KD should be strongly considered very early in therapy including glucose transporter type 1 deficiency syndrome (Glut1DS), pyruvate dehydrogenase deficiency (PDHD), epilepsy with myoclonic-atonic seizures, infantile spasms, tuberous sclerosis complex, children with gastrostomy tubes, and Dravet syndrome. There are some reports of KD efficacy in other epileptic conditions such as Lennoxsyndrome. In this syndrome KD can induce 50% seizure reduction and is not considered as an indication (according to the new recommendateven concurrent with VNS (Vagal Nerve Stimulation). In focal epilepsy, KD can be used to reduce seizure frequency and number of AEDs for a time period particularly before surgery. Efficacy was reported in hypothaand cortical dysplasia. However, recent improvements in lesion detection and surgery techniques may change this opinion. KD is the treatment of choice in two metabolic conditions: Glucose transporter protein 1 (GLUT 1) deficiency and Pyconditions such as phosphofructokinase and nonby KD. Please see chapter 8 for details. In infants, the infantile spasm is a common KD indication specificthe first line therapy (corticosteroids and vigabatrin). Please see chapter 3 for details in infants. Conversely, there are some metabolic conditions in which KD is contraindicated (These inborn errors of metabohas sign or symptoms of metabolic diseases. In some of these metabolic disorders, because of an enzymatic defect, the bodyenergy production so fasting and KD will be very dangerous resulting in coma and death. In porphyria fasting / KD leads to exacerbation of porphyria symptoms.
KD study group also suggested seven particular metabolic and epileptic syndromes in which onsidered very early in therapy including glucose transporter type 1
deficiency syndrome (Glut1DS), pyruvate dehydrogenase deficiency (PDHD), epilepsy with atonic seizures, infantile spasms, tuberous sclerosis complex, children with
ubes, and Dravet syndrome.
There are some reports of KD efficacy in other epileptic conditions such as Lennoxsyndrome. In this syndrome KD can induce 50% seizure reduction and is not considered as an indication (according to the new recommendation) however, KD can be used effectively
concurrent with VNS (Vagal Nerve Stimulation).
In focal epilepsy, KD can be used to reduce seizure frequency and number of AEDs for a time period particularly before surgery. Efficacy was reported in hypotha
However, recent improvements in lesion detection and surgery techniques may change this
of choice in two metabolic conditions: Glucose transporter protein 1 (GLUT 1) deficiency and Pyruvate dehydrogenase deficiency PDHD). Other metabolic conditions such as phosphofructokinase and non-ketotic hyper glycinaemia are also treated by KD. Please see chapter 8 for details.
spasm is a common KD indication specifically when it is refractory to the first line therapy (corticosteroids and vigabatrin). Please see chapter 3 for details in
Conversely, there are some metabolic conditions in which KD is contraindicated (These inborn errors of metabolism should be ruled out by proper tests especially if the child has sign or symptoms of metabolic diseases. In some of these metabolic disorders, because of
body is not able to produce ketone bodies or use them as a fuel for y production so fasting and KD will be very dangerous resulting in coma and death. In
porphyria fasting / KD leads to exacerbation of porphyria symptoms.
19
KD study group also suggested seven particular metabolic and epileptic syndromes in which onsidered very early in therapy including glucose transporter type 1
deficiency syndrome (Glut1DS), pyruvate dehydrogenase deficiency (PDHD), epilepsy with atonic seizures, infantile spasms, tuberous sclerosis complex, children with
There are some reports of KD efficacy in other epileptic conditions such as Lennox-Gastaut syndrome. In this syndrome KD can induce 50% seizure reduction and is not considered as
ion) however, KD can be used effectively
In focal epilepsy, KD can be used to reduce seizure frequency and number of AEDs for a time period particularly before surgery. Efficacy was reported in hypothalamic hamartoma
However, recent improvements in lesion detection and surgery techniques may change this
of choice in two metabolic conditions: Glucose transporter protein 1 ruvate dehydrogenase deficiency PDHD). Other metabolic
glycinaemia are also treated
ally when it is refractory to the first line therapy (corticosteroids and vigabatrin). Please see chapter 3 for details in
Conversely, there are some metabolic conditions in which KD is contraindicated (Table 1-5). lism should be ruled out by proper tests especially if the child
has sign or symptoms of metabolic diseases. In some of these metabolic disorders, because of is not able to produce ketone bodies or use them as a fuel for
y production so fasting and KD will be very dangerous resulting in coma and death. In
Chapter One
Table 1-5. Contraindications of KDAbsolute
Pyruvate carboxylase deficiency Organic acidurias A defect in Carnitine metabolism Carnitine deficiency (primary) Carnitine palmitoyltransferase (CPT) I or II deficiency Carnitine translocase deficiency Defects in fatty acid oxidation Short-chain acyl dehydrogenase deficiency (SCAD) Medium-chain acyl dehydrogenase deficiency (MCAD) Long-chain acyl dehydrogenase deficiency (LCAD) Medium-chain 3-hydroxyacyl CoA deficiency Long-chain 3-hydroxyacyl CoA deficiency Glutaric aciduria, type II Pyruvate dehydrogenase phosphate deficiency Selected mitochondrial disease Porphyria
Baseline evaluation and tests are also required for other issues such as kidney, liver or heart disorders that may be relative contraindications to KD. (assessment form (no. 7)
Table 1-6. Other systemic diseases that may be relative contraindications of KD
Cardiovascular Gastrointestinal Hyperlipidemia Arrhythmia Cardiomyopathy
Failure to thrive Dysphagia Unresolved GI
symptoms
In Iran, KD had been started almost 30 years ago for refractory seizures in Mofid Children’s Hospital with a similar Studies about KD efficacy in our centerthat also offers ketogenic diet in Tehran is Children’s Medical Centre at Tehran University of Medical Sciences with published researches.
5. Contraindications of KD Relative
(CPT) I or II deficiency
acyl dehydrogenase deficiency (SCAD)
chain acyl dehydrogenase deficiency (LCAD) CoA deficiency
CoA deficiency
ehydrogenase phosphate deficiency
Inability to maintain adequate nutrition Surgical focus identified by neuroimaging and video
EEG monitoring A parent or caregiver noncompliance Propofol concurrent use (risk of pro
syndrome may be higher)
Baseline evaluation and tests are also required for other issues such as kidney, liver or heart disorders that may be relative contraindications to KD. (Table 1-6). Please also see Pre
6. Other systemic diseases that may be relative contraindications of KDGastrointestinal Renal
Failure to thrive Dysphagia Unresolved GI symptoms
Renal calculi Osteopenia
n Iran, KD had been started almost 30 years ago for refractory seizures in Mofid similar rate of success as like other parts of the world.
Studies about KD efficacy in our center have been summarized in Table 1that also offers ketogenic diet in Tehran is Children’s Medical Centre at Tehran University of Medical Sciences with published researches.
20
elative Inability to maintain adequate nutrition Surgical focus identified by neuroimaging and video
or caregiver noncompliance Propofol concurrent use (risk of propofol infusion
Baseline evaluation and tests are also required for other issues such as kidney, liver or heart ). Please also see Pre-KD
6. Other systemic diseases that may be relative contraindications of KD Musculoskeletal
Osteopenia
n Iran, KD had been started almost 30 years ago for refractory seizures in Mofid rate of success as like other parts of the world.
1-7. Another center that also offers ketogenic diet in Tehran is Children’s Medical Centre at Tehran University of
Chapter One
† At 6 months. *At 8 weeks. °Number of patients, the results stayed the same at 6 months Summary of KD indications:· Medically intractable seizures (failure of at least two AEDs) · Poor tolerance or significant side effects from AEDs· Surgery candidates i.e., callosotomy; nonlesional, · Specific neurometabolic or neurologic syndromes.
And remember that time does matter to select and start the diet. KD is not considered the last treatment option anymore. General considerations for all patients before diet initiation 1) Complete physical examination and histo
in a chart. (Form no.5, 6, 9)
It is important to differentiate refractory seizure from nonappropriate AEDs for proper dose or period of time. 2) Complete GI history includes any food allergy/intolerance, reflux or any child personality trait that make diet challenging, should be obtained before diet initiation. Treatment of these
Table 1-7. Summary of studies in Iran overNo. of patient
Mean Age m.
Diettype
215 5.1 years
Classic
87 55±24 Classic
24 53±31 MAD
20 78±23 MAD
42 3.2-5.6 years
LGI
9 25±5
ClassicKetocal
† At 6 months. *At 8 weeks. °Number of patients, the results stayed the same at 6 months
Summary of KD indications: Medically intractable seizures (failure of at least two AEDs) Poor tolerance or significant side effects from AEDs Surgery candidates i.e., callosotomy; nonlesional, extra temporal resections
lic or neurologic syndromes.
And remember that time does matter to select and start the diet. KD is not considered the last
General considerations for all patients before diet initiation Complete physical examination and history; seizure type, frequency, etc. which is better to record in a chart. (Form no.5, 6, 9)
It is important to differentiate refractory seizure from non-epileptic seizure and/or not taking appropriate AEDs for proper dose or period of time.
I history includes any food allergy/intolerance, reflux or any child personality trait that make diet challenging, should be obtained before diet initiation. Treatment of these
7. Summary of studies in Iran over 30 years: 1988-2017 Diet type
↓50% in Seizure
Frequency 3rd m.
Seizure Free at 3rd m.
Studyduration
Classic 20%† 40%† 1988-1998 Retrospective
Classic 87% 22% 1999-2006Retrospective/
ProspectiveMAD 67% 21% 2004-2006
Prospective
MAD 70% 20% 2005-2007Prospective
LGI 78%* 16.6% 2009-2011Prospective
Classic Ketocal
4° 5° 2015-2017Prospective
21
† At 6 months. *At 8 weeks. °Number of patients, the results stayed the same at 6 months after diet.
resections
And remember that time does matter to select and start the diet. KD is not considered the last
General considerations for all patients before diet initiation ry; seizure type, frequency, etc. which is better to record
epileptic seizure and/or not taking
I history includes any food allergy/intolerance, reflux or any child personality trait that make diet challenging, should be obtained before diet initiation. Treatment of these
Study duration
Author
1998 Retrospective
Ghofrni et.al. J of Med council of
Iran, 2000 2006
Retrospective/ Prospective
Karimzadeh et al. Iran J Child Neurol,
2009 2006
Prospective Tonekaboni et al. Archive of Iran Medicine, 2010
2007 Prospective
Tonekaboni et al. Iran J Child Neurol,
2014 2011
Prospective Karimzadeh et al.
Seizure, 2014 2017
Prospective Karimzadeh et al.
Iran J Child Neurol, 2019
Chapter One
Pre-existing issues will improve ddifficulties are summarized in form no.3. Feeding assessment may be necessary and if the patient needs tube feeding, a combination of oral and enteral feeding is possible. 3) Evaluation of the family environment/size, collaboration,them to read, learn and prepare for diet initiation. See also
4) Full screening in view of nutritional status e.g. weight and height, BMI, ideal weight for height, any pre-existing Vitamin D deficiency due to AEDs, or probable metabolic conditions depending on history and physical examination. Recommended presummarized in Table 1-8. 5) The pre-KD checklist is summarized in form no. 7 and should be filleincluding metabolic workup as there is no neonatal screening program in Iran. MRI or EEG may be helpful to detect a possible focal Remember if no underlying evaluation should be completed before starting KD. 6) A thorough review of medications is necessary, and carbohydrate free products should substitute previous medications. ( 7) A food intake record of 3individualized diet planning. (Form No. 1) It helps to select a right type of KD for each child based on his/her food preferences. Although in some disorders such as there is no choice other than classic KD.
Table 1-8. Recommendations Pre KD(International KD study group recommendations, 2018)Counselling
Discuss seizure reduction, medication, and cognitive expectations
Identify potential psychosocial barriers to the use of KD
Review anticonvulsants and other medications for carbohydrate content
Make dose changes before diet initiation. Identify compounding pharmacy
Recommend family read parent-oriented KD information; books, internet
issues will improve diet tolerance. Questions regarding the possibility of feeding difficulties are summarized in form no.3. Feeding assessment may be necessary and if the
needs tube feeding, a combination of oral and enteral feeding is possible.
family environment/size, collaboration, and competence. Encouraging them to read, learn and prepare for diet initiation. See also counseling.
4) Full screening in view of nutritional status e.g. weight and height, BMI, ideal weight for isting Vitamin D deficiency due to AEDs, or probable metabolic conditions
depending on history and physical examination. Recommended pre-assessment steps are
checklist is summarized in form no. 7 and should be filled out for each patient including metabolic workup as there is no neonatal screening program in Iran. MRI or EEG may be helpful to detect a possible focal etiology and surgical intervention.
Remember if no underlying etiology is diagnosed for a patient’s epilepsy, an extensive evaluation should be completed before starting KD.
6) A thorough review of medications is necessary, and carbohydrate free products should substitute previous medications. (Form no. 9 AEDs History)
7) A food intake record of 3-5 days for each child is necessary; particularly important for diet planning. (Form No. 1) It helps to select a right type of KD for each child
based on his/her food preferences. Although in some disorders such as PDHDchoice other than classic KD.
. Recommendations Pre KD-assessment (International KD study group recommendations, 2018)
Nutritional evaluation Laboratory evaluation
Review anticonvulsants and other
Baseline weight, height, and ideal weight for stature
Body mass index (BMI) when appropriate
Head circumference in infants Nutrition intake history: 3-day
food intake record, food preferences, allergies, aversions, and intolerances.
Establish diet formulation: infant oral, enteral, or a combination
A decision on which diet to begin (MCT, classic, modified Atkins, or low glycaemic index)
Calculation of calories, fluid, and the ketogenic ratio (or percentage of MCT oil)
Establish nutritional supplementation products based on dietary reference intake
Complete blood count with platelets Electrolytes to include serum
bicarbonate, total protein, calcium, magnesium, phosphorus
Vit D level Serum liver and kidney tests (including
albumin, AST, ALT, blnitrogen, creatinine)
Fasting lipid profile (cholesterol, Triglyceride, LDL, HDL
Serum acylcarnitine profileUrinalysis
Anticonvulsant drug levels (if applicable)
Ancillary testing (optional) Renal ultrasound (If a
kidney stones) EEG MRI ECG (Echocardiogram) if the
heart disease
22
iet tolerance. Questions regarding the possibility of feeding difficulties are summarized in form no.3. Feeding assessment may be necessary and if the
needs tube feeding, a combination of oral and enteral feeding is possible.
competence. Encouraging
4) Full screening in view of nutritional status e.g. weight and height, BMI, ideal weight for isting Vitamin D deficiency due to AEDs, or probable metabolic conditions
assessment steps are
d out for each patient including metabolic workup as there is no neonatal screening program in Iran. MRI or EEG
and surgical intervention.
epilepsy, an extensive
6) A thorough review of medications is necessary, and carbohydrate free products should
ays for each child is necessary; particularly important for diet planning. (Form No. 1) It helps to select a right type of KD for each child
PDHD or in infants
Laboratory evaluation Complete blood count with platelets Electrolytes to include serum bicarbonate, total protein, calcium, magnesium, phosphorus Vit D level Serum liver and kidney tests (including albumin, AST, ALT, blood urea nitrogen, creatinine) Fasting lipid profile (cholesterol, Triglyceride, LDL, HDL-cholesterol) Serum acylcarnitine profile Urinalysis Anticonvulsant drug levels (if applicable) Ancillary testing (optional) Renal ultrasound (If a family history of idney stones)
ECG (Echocardiogram) if the history of heart disease
Chapter One
If there is no known etiologyamino acid, and other necessary investigations Introduction for families ( Ketogenic Diet should be discussed with patients and families as early as possible, not just before diet initiation, so they will have time to research and decide about it. This means that we should predict which patients would be refractory and probably will require KD. KD should be offered earlier in refractory seizures after the particularly in refractory epilepsy syndromes, not as a last option, depending on tunderlying etiology, patient and family complianceno.1 and 4 designed for parents) Consultation sessions should be offered by giving enough information e.g.; weighing foods, exchange list, low definitely helps you to choose which KD would be more appropriate Parents should have realistic expectations from the diet in terms of seizure control and discontinuation of anti-seizure medications. Theyinvolved in developing seizure control (at least 3 months). Potential side effects such as nausea, vomiting etc., and other challenges they may encounter as a caregiver should be fully described. The diet response rate should be disclosed; approximately onebe seizure free or almost seizure free, onethird will be non-responsive. Moreover, parents should be KD including cognitive and its prolonged effect on seizure control even after discontinuation. The KD team should ensure that parents understand the limitations and strict rules required for diet; for example, careful avoidance of carbohydrates, food limitations and its consequences for both child and parents in terms of expense or time that is required. Parents should also get a comprehensivepreparing and weighing meals/formula, cooking style, label reading, vitamin and mineral supplementation, fluid consumption goals, handling illness and side effects. This step may be arranged during hospital admission or in KD clinic. Different websites are available for families to learn how to and desserts for their children. In Iran, we don’t have a website for families recently, but we are in the half to design a website with all information particularly food recipthere soon. (https://www.charliefoundation.org/ (http://www.matthewsfriends.org)
etiology for refractory epilepsy consider: Urine organic acid, serum cessary investigations
Introduction for families (counseling)
Ketogenic Diet should be discussed with patients and families as early as possible, not just before diet initiation, so they will have time to research and decide about it. This means that
should predict which patients would be refractory and probably will require KD. KD should be offered earlier in refractory seizures after the failure of two or three AEDs, particularly in refractory epilepsy syndromes, not as a last option, depending on t
, patient and family compliance, and acceptance. (Please also see forms no.1 and 4 designed for parents)
Consultation sessions should be offered by giving enough information e.g.; foods, exchange list, low glycaemic foods depending on the diet type. Family
definitely helps you to choose which KD would be more appropriate
Parents should have realistic expectations from the diet in terms of seizure control and seizure medications. They should also be aware of the time period
involved in developing seizure control (at least 3 months). Potential side effects such as nausea, vomiting etc., and other challenges they may encounter as a caregiver should be fully
rate should be disclosed; approximately one–third of children on diet will be seizure free or almost seizure free, one-third will experience seizure reduction and one
responsive. Moreover, parents should be counseled about other potentialKD including cognitive and behavioral improvements, reducing AEDs dose and dosage, its prolonged effect on seizure control even after discontinuation.
The KD team should ensure that parents understand the limitations and strict rules required r diet; for example, careful avoidance of carbohydrates, food limitations and its
consequences for both child and parents in terms of expense or time that is required.
comprehensive education about identifying proper foods for dipreparing and weighing meals/formula, cooking style, label reading, vitamin and mineral supplementation, fluid consumption goals, handling illness and side effects. This step may be arranged during hospital admission or in KD clinic.
es are available for families to learn how to prepare/cookdesserts for their children. In Iran, we don’t have a website for families recently, but we
to design a website with all information particularly food recip
https://www.charliefoundation.org/)
(http://www.matthewsfriends.org)
23
for refractory epilepsy consider: Urine organic acid, serum
Ketogenic Diet should be discussed with patients and families as early as possible, not just before diet initiation, so they will have time to research and decide about it. This means that
should predict which patients would be refractory and probably will require KD. KD of two or three AEDs,
particularly in refractory epilepsy syndromes, not as a last option, depending on the acceptance. (Please also see forms
Consultation sessions should be offered by giving enough information e.g.; high-fat content, glycaemic foods depending on the diet type. Family
Parents should have realistic expectations from the diet in terms of seizure control and should also be aware of the time period
involved in developing seizure control (at least 3 months). Potential side effects such as nausea, vomiting etc., and other challenges they may encounter as a caregiver should be fully
third of children on diet will third will experience seizure reduction and one-
about other potentials of dose and dosage, and
The KD team should ensure that parents understand the limitations and strict rules required r diet; for example, careful avoidance of carbohydrates, food limitations and its
consequences for both child and parents in terms of expense or time that is required.
education about identifying proper foods for diet, preparing and weighing meals/formula, cooking style, label reading, vitamin and mineral supplementation, fluid consumption goals, handling illness and side effects. This step may be
prepare/cook keto meals, cookies, desserts for their children. In Iran, we don’t have a website for families recently, but we
to design a website with all information particularly food recipes. We will get
Chapter One
KD and AEDs discontinuationUsually, no change is suggested control, AEDs can be reduced very slowly. Reducing some medication like phenobarbital or benzodiazepines may come with higher risk of seizure exacerbation. Supplementations DRI recommendations for gender and age should be met for vitamins, minerals and trace elements. All KD types require supplementation. This has been explained in chapter 7. KD and other anti-seizure modalities Currently, there is no specific combination of KD and AEDs that wKD can be effective with the about better response when KD was added to VNS or Zonisamide regimen or less effectiveness of KD while receiving acidic conditions and there is a potential risk of achieving high levels in KD.Topiramate and zonisamidewhile a patient on KD requiring careful monitoring of bicarbonate level. Oraindicated in symptomatic cases involving lethargy or vomiting.Carnitine levels may drop earlier and be more significant with concurrent use of valproic acid and KD. This also requires monitoring and supplementation.MCT-KD is not recommenliver failure. KD monitoring Children on KD should have regular follow up visits by dietitians and Patients should be seen after one month and every 3months in phone calls with KD team are recommended. Infants should have more frequent visits; every 2-3 weeks. Follow-ups should then be spaced out to every 3 months depending on the child’s condition. In the first year, the blood tspecific concern or side effects. Some references suggest a blood test after one month, then every three months. Parents will have the contact information of the dietitian in order to speak about their concerns. Over the phone followdepending on the family preference. In the second year of KD, visit intervals can be increased depending on patient condition, and blood tests spaced out to every 6 months ifconcern. Table 1-8 and Table 1laboratory investigations with suggested intervals, respectively. However, the approach should always be individualized.
KD and AEDs discontinuation no change is suggested in the first month. After the first few months of seizure
control, AEDs can be reduced very slowly. Reducing some medication like phenobarbital or benzodiazepines may come with higher risk of seizure exacerbation.
r gender and age should be met for vitamins, minerals and trace elements. All KD types require supplementation. This has been explained in chapter 7.
seizure modalities
Currently, there is no specific combination of KD and AEDs that work better.the patient on VNS (Vagal nerve stimulation). There are reports
about better response when KD was added to VNS or Zonisamide regimen or less effectiveness of KD while receiving phenobarbital. Phenobarbital elimination gacidic conditions and there is a potential risk of achieving high levels in KD.
zonisamide; carbonate anhydrase inhibitor AEDs can exaggerate acidosis on KD requiring careful monitoring of bicarbonate level. Ora
indicated in symptomatic cases involving lethargy or vomiting. Carnitine levels may drop earlier and be more significant with concurrent use of valproic acid and KD. This also requires monitoring and supplementation.
KD is not recommended to commence in patients on valproic acid because of the risk of
Children on KD should have regular follow up visits by dietitians and pediatricPatients should be seen after one month and every 3months in the first year. Close contact and phone calls with KD team are recommended. Infants should have more frequent visits; every
should then be spaced out to every 3 months depending on the child’s condition. In the first year, the blood test is recommended every 3 months unless there is a
concern or side effects. Some references suggest a blood test after one month, then
Parents will have the contact information of the dietitian in order to speak about their ncerns. Over the phone follow-ups for cases from distant areas will be considered
depending on the family preference. In the second year of KD, visit intervals can be increased depending on patient condition, and blood tests spaced out to every 6 months if
Table 1-9 summarized different aspects of follow up visits and laboratory investigations with suggested intervals, respectively. However, the approach should always be individualized.
24
in the first month. After the first few months of seizure control, AEDs can be reduced very slowly. Reducing some medication like phenobarbital or
r gender and age should be met for vitamins, minerals and trace elements. All KD types require supplementation. This has been explained in chapter 7.
ork better. on VNS (Vagal nerve stimulation). There are reports
about better response when KD was added to VNS or Zonisamide regimen or less elimination gets slower in
acidic conditions and there is a potential risk of achieving high levels in KD. ; carbonate anhydrase inhibitor AEDs can exaggerate acidosis
on KD requiring careful monitoring of bicarbonate level. Oral bicarbonate is
Carnitine levels may drop earlier and be more significant with concurrent use of valproic acid
ded to commence in patients on valproic acid because of the risk of
pediatric neurologists. the first year. Close contact and
phone calls with KD team are recommended. Infants should have more frequent visits; every should then be spaced out to every 3 months depending on the child’s
est is recommended every 3 months unless there is a concern or side effects. Some references suggest a blood test after one month, then
Parents will have the contact information of the dietitian in order to speak about their ups for cases from distant areas will be considered
depending on the family preference. In the second year of KD, visit intervals can be increased depending on patient condition, and blood tests spaced out to every 6 months if there is no
summarized different aspects of follow up visits and laboratory investigations with suggested intervals, respectively. However, the approach
Chapter One
KD follow up (form no. 8) is designed for physicians who are visiting these patients to record details in appropriate order. Recommended investigations and interval are also summarized in form no. 10.
Table 1-9. Follow up visits* in KD clinicNutritional assessment (dietitian)
· Obtain height weight, ideal weight for stature, growth velocity, BMI
· Review appropriateness of diet prescription (calories, protein, and fluid)
· Review vitamin and mineral suppbased on dietary reference intake guidelines
· Assess compliance to therapy · Adjust therapy if necessary to improve
compliance and optimize seizure control
*Patients should be visited at least every 3 months for the first year of the KD. Important follow-up tips Visit intervals Children less than one year of age, cerebral palsy cases, infants or children with failure to thrive or any child who develops difficulties or illness during KD should have more frequent visits/phone calls Urinary ketosis Monitoring of urinary ketosis is important, daily moderate level and then 2-3 times per week by contact the KD team if ketosis is not achieved or maintained. This is very important in classic KDs and during the first months oconsidered during diet tuning (changing ratio for example) or illness. In LGI, ketones are not usually requested. Blood Beta-HydroxyButyrate (BHB)Serum BHB correlates with seizure control but ketosis is not compatible with seizure control, for example, positive urine ketones in case of seizure worsening and vice versa.
KD follow up (form no. 8) is designed for physicians who are visiting these patients to record details in appropriate order. Recommended investigations and interval are also summarized in
. Follow up visits* in KD clinic Medical evaluation (
Obtain height weight, ideal weight for stature,
Review appropriateness of diet prescription
Review vitamin and mineral supplementation based on dietary reference intake guidelines
Adjust therapy if necessary to improve compliance and optimize seizure control
- Complete Physical examination- Efficacy of the diet (is the KD meeting parental
expectations?) - Anticonvulsant reduction (if applicable)- Evaluation of KD side effect - Should the KD be continued?
*Patients should be visited at least every 3 months for the first year of the KD.
tips
year of age, cerebral palsy cases, infants or children with failure to thrive or any child who develops difficulties or illness during KD should have more frequent
Monitoring of urinary ketosis is important, daily checkup until achieving ketosis at a 3 times per week by Ketostix test-strips. Parents should know to
contact the KD team if ketosis is not achieved or maintained. This is very important in classic KDs and during the first months of MAD. After this period and stabilized ketosis, it should be considered during diet tuning (changing ratio for example) or illness. In LGI, ketones are not
utyrate (BHB) Serum BHB correlates with seizure control but can be reserved for conditions where urine ketosis is not compatible with seizure control, for example, positive urine ketones in case of seizure worsening and vice versa.
25
KD follow up (form no. 8) is designed for physicians who are visiting these patients to record details in appropriate order. Recommended investigations and interval are also summarized in
Medical evaluation (pediatric neurologist) Complete Physical examination Efficacy of the diet (is the KD meeting parental
Anticonvulsant reduction (if applicable) Evaluation of KD side effect Should the KD be continued?
year of age, cerebral palsy cases, infants or children with failure to thrive or any child who develops difficulties or illness during KD should have more frequent
until achieving ketosis at a strips. Parents should know to
contact the KD team if ketosis is not achieved or maintained. This is very important in classic f MAD. After this period and stabilized ketosis, it should be
considered during diet tuning (changing ratio for example) or illness. In LGI, ketones are not
can be reserved for conditions where urine ketosis is not compatible with seizure control, for example, positive urine ketones in case of
Chapter One
Side effects Check for side effects: Any warning sign and symptoms such as lethargy, vomiting etc. Lethargy can occur due to multiple reasons; for example, relative dehydration, ketosis, or metabolic acidosis in classic KD, less commonly in MAD and LGI. BUN can induce lethargy in the Fluid consumption Sufficient fluid intake is critical. A low carbohydrate diet is a foods. Dehydration risk is higher in conditions such as increased activity, febrile illness, warm temperature, and also in infants. This should be clearly explained to the families. Urine specific gravity is a helpful measure representing adequate hydratiofluid intake. (<1015 shows adequate hydration) Physical exam & blood work A complete physical examination and growth rate checkheight, BMI, also albumin and total protein level to make suis provided by KD. Fasting cholesterol and triglyceride levels should be monitored. They usually rise after diet initiation and require monitoring. Medications Carbohydrate free medications/supplementations are reqabout that. Any decision about AEDs dose is based on response to the diet and usually not considered in the first month. Appropriate Supplementations should be monitored. Optional investigations A renal ultrasound every 6-children with a family history of renal stones, or any previous kidney issues e.g. Infections. Echocardiography, bone mineral density evaluations (except for DEXA) or carotid ultrasounds may be considered in specific cases and are not routinely requested.
fects: Any warning sign and symptoms such as lethargy, vomiting etc. Lethargy can occur due to multiple reasons; for example, relative dehydration, ketosis, or metabolic acidosis in classic KD, less commonly in MAD and LGI.
induce lethargy in the LGI diet given high protein intake.
Sufficient fluid intake is critical.
A low carbohydrate diet is a diuretic in itself, and keto foods contain less fluid than normal foods. Dehydration risk is higher in conditions such as increased activity, febrile illness, warm temperature, and also in infants. This should be clearly explained to the families. Urine specific gravity is a helpful measure representing adequate hydration if there is concern about fluid intake. (<1015 shows adequate hydration)
Physical exam & blood work
A complete physical examination and growth rate check-up is necessary including weight and height, BMI, also albumin and total protein level to make sure that enough calorie and protein
Fasting cholesterol and triglyceride levels should be monitored. They usually rise after diet initiation and require monitoring.
Carbohydrate free medications/supplementations are required, and parents should be advised about that. Any decision about AEDs dose is based on response to the diet and usually not considered in the first month. Appropriate Supplementations should be monitored.
-12 months has been suggested: A baseline ultrasound reserved for history of renal stones, or any previous kidney issues e.g. Infections.
Echocardiography, bone mineral density evaluations (except for DEXA) or carotid ds may be considered in specific cases and are not routinely requested.
26
fects: Any warning sign and symptoms such as lethargy, nausea, and vomiting etc. Lethargy can occur due to multiple reasons; for example, relative dehydration, ketosis, or metabolic acidosis in classic KD, less commonly in MAD and LGI. The elevated
in itself, and keto foods contain less fluid than normal foods. Dehydration risk is higher in conditions such as increased activity, febrile illness, warm temperature, and also in infants. This should be clearly explained to the families. Urine
n if there is concern about
up is necessary including weight and re that enough calorie and protein
Fasting cholesterol and triglyceride levels should be monitored. They usually rise after diet
uired, and parents should be advised about that. Any decision about AEDs dose is based on response to the diet and usually not considered in the first month. Appropriate Supplementations should be monitored.
12 months has been suggested: A baseline ultrasound reserved for history of renal stones, or any previous kidney issues e.g. Infections.
Echocardiography, bone mineral density evaluations (except for DEXA) or carotid ds may be considered in specific cases and are not routinely requested.
Chapter One
At the first month some investigations are better (CBC), platelets, fasting lipid profile, glucose, calcium, sodium, potassium, and chloride, bicarbonate, creatinine, liver e
Table 1-10. Recommendations for blood investigations in KD monitoring (International KD study group recommendations, 2018)Investigation (Recommended)
· Full blood count including platelets· Electrolytes to include serum bicarbonate, total
protein, calcium Serum liver and kidney profile (including albumin, AST, ALT, blood urea nitrogen, creatinine)
· Vitamin D level · Fasting lipid profile · Free and total carnitine · Urinalysis · Selenium level
Investigation (Optional) · Serum beta-hydroxybutyrate (BHB) level· Urine calcium and creatinine · Zinc, copper levels · Renal ultrasound · ECG · Bone mineral density (DEXA scan) yearly, after 2
years on the KD
month some investigations are better requested including complete blood count (CBC), platelets, fasting lipid profile, glucose, calcium, sodium, potassium, and chloride,
enzymes.
. Recommendations for blood investigations in KD monitoring (International KD study group recommendations, 2018)
Frequency of monitoringFull blood count including platelets Electrolytes to include serum bicarbonate, total protein, calcium Serum liver and kidney profile (including albumin,
, ALT, blood urea nitrogen, creatinine) Vitamin D level
- Baseline, 3rd months then every 3 months, can be spaced out to every 6 months year and depending on the patient’s condition
hydroxybutyrate (BHB) level
an) yearly, after 2
27
including complete blood count (CBC), platelets, fasting lipid profile, glucose, calcium, sodium, potassium, and chloride,
Frequency of monitoring months then every 3 months, can be
ut to every 6 months -1 year in the second year and depending on the patient’s condition
Chapter One
· References · Bergqvist, A. G. C. (2012). Long
Research, 100(3), 261–6. · Chaffe H. Ketone monitoring and management of il· Dhamija, R., Eckert, S., &Wirrell, E. (2013). Ketogenic diet. The Canadian Journal of Neurological
Sciences. Le Journal CanadienDes Sciences Neurologiques, 40(2), 158· Dressler, A., Stöcklin, B., Reithofe
Long-term outcome and tolerability of the ketogenic diet in drugAustrian experience. Seizure, 19(7), 404
· Kossoff.,E Henry B.J., Cervenka M. C, Do6th edition, New York, Demos health, 2016.
· Ghazavi A, Tonekaboni SH, Karimzadeh P, Nikibakhsh AA, Khajeh A, Fayyazi A. The Ketogenic and Atkins Diets Effect on Intractable Epilepsy: A Comparison. 17.
· Karimzadeh P, Tabarestani S, Mahvelati F., Tonekaboni SH, Ghofrani M, Intractable seizure disorders efficacy of the classic ketogenic diet. Iran J Child Neurology Jan 2009
· Karimzadeh P., Sedighi M., Beheshti MGlycemic Index Treatment in pediatric refractory epilepsy: The first Middle East report, Seizure 23 570–572, 2014.
· Kossoff, E. H., Zupec-Kania, B. A., Auvin, S., BallabanBlackford, R., … Wirrell, E. C. (2018). Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group. Epilepsia Open, 3(2), 175–192.
· Kossoff, E. H. (2010). The ketogenic diet: An appropriate firstNeurotherapeutics, 10(6), 843–845.
· Kossoff, E. H., Zupec-Kania, B. A., & Rho, J. M. (2009). Ketogenic diets: an update for child neurologists. Journal of Child Neurology, 24(8), 979
· Kossoff, E. H. W., & Shields, W. D. (2014). Nonpharmacologic care for patients with Lennoxsyndrome: Ketogenic diets and vagus nerve stimulation. Epilepsia, 55(s4), 29
· Kossoff, E. H., Zupec-Kania, B. A., Amark, P. E., BallabanBlackford, R., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2), 304–17
· Larson, A. M., Pfeifer, H. H., & Thiele, E. A. (2012). Low glycemic index treatment for epilepsy in tuberous sclerosis complex. Epilepsy Research, 99(1
· Lima, P. A. de, Sampaio, L. P. de B., &Damasceno, N. R. T. (2014). a ketogenic diet in refractory epilepsy. Clinics (Sao Paulo, Brazil), 69(10), 699
· Liu, Y., & Wang, H.-S. (2013). MediumDrug-resistant Epilepsy and A Comparison with Other Ketogen
· Martikainen, M. H., Päivärinta, M., Jääskeläinen, S., &Majamaa, K. (2012). Successful treatment of POLG-related mitochondrial epilepsy with antiepileptic drugs and low glycaemic index diet. Epileptic Disorders, 14(4), 438–441.
· Miranda, M. J., Turner, Z., & Magrath, G. (2012). we be more liberal? Epilepsy Research, 100(3), 278
· Neal E. Monitoring and side effects in Dietary Treatment of Epilepsy.In Neal E. PrImplementation of Ketogenic Therapy. Wiley & Sons, 2012
Bergqvist, A. G. C. (2012). Long-term monitoring of the ketogenic diet: Do’s and Don’ts. Epilepsy
Chaffe H. Ketone monitoring and management of illness In Neal E.Dietary Treatment of Epilepsy: Dhamija, R., Eckert, S., &Wirrell, E. (2013). Ketogenic diet. The Canadian Journal of Neurological Sciences. Le Journal CanadienDes Sciences Neurologiques, 40(2), 158–67. Dressler, A., Stöcklin, B., Reithofer, E., Benninger, F., Freilinger, M., Hauser, E., … Feucht, M. (2010).
term outcome and tolerability of the ketogenic diet in drug-resistant childhood epilepsyAustrian experience. Seizure, 19(7), 404–408.
.,E Henry B.J., Cervenka M. C, Doerrer S., Turner Z. The Ketogenic and Modified Atkins Diet. New York, Demos health, 2016.
Ghazavi A, Tonekaboni SH, Karimzadeh P, Nikibakhsh AA, Khajeh A, Fayyazi A. The Ketogenic and Atkins Diets Effect on Intractable Epilepsy: A Comparison. Iran J Child Neurol. 2014 Summer;8(3): 12
Karimzadeh P, Tabarestani S, Mahvelati F., Tonekaboni SH, Ghofrani M, Intractable seizure disorders efficacy of the classic ketogenic diet. Iran J Child Neurology Jan 2009. Karimzadeh P., Sedighi M., Beheshti M., Azargashb E., Ghofrani M., AbdollaheGlycemic Index Treatment in pediatric refractory epilepsy: The first Middle East report, Seizure 23
Kania, B. A., Auvin, S., Ballaban-Gil, K. R., Christina Bergqvist,Blackford, R., … Wirrell, E. C. (2018). Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group.
192. . The ketogenic diet: An appropriate first-line therapy? Expert Review of
845. Kania, B. A., & Rho, J. M. (2009). Ketogenic diets: an update for child
neurologists. Journal of Child Neurology, 24(8), 979–988. Kossoff, E. H. W., & Shields, W. D. (2014). Nonpharmacologic care for patients with Lennoxsyndrome: Ketogenic diets and vagus nerve stimulation. Epilepsia, 55(s4), 29–33.
Kania, B. A., Amark, P. E., Ballaban-Gil, K. R., Christina Bergqvist, A. G., Blackford, R., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study
17. Larson, A. M., Pfeifer, H. H., & Thiele, E. A. (2012). Low glycemic index treatment for epilepsy in tuberous sclerosis complex. Epilepsy Research, 99(1–2), 180–2. Lima, P. A. de, Sampaio, L. P. de B., &Damasceno, N. R. T. (2014). Neurobiochemical mechaa ketogenic diet in refractory epilepsy. Clinics (Sao Paulo, Brazil), 69(10), 699–705.
S. (2013). Medium-chain Triglyceride Ketogenic Diet, An Effective Treatment for resistant Epilepsy and A Comparison with Other Ketogenic Diets. Biomedical Journal, 36(1), 9.
Martikainen, M. H., Päivärinta, M., Jääskeläinen, S., &Majamaa, K. (2012). Successful treatment of related mitochondrial epilepsy with antiepileptic drugs and low glycaemic index diet. Epileptic
Miranda, M. J., Turner, Z., & Magrath, G. (2012). Alternative diets to the classical ketogenic dietwe be more liberal? Epilepsy Research, 100(3), 278–85. Neal E. Monitoring and side effects in Dietary Treatment of Epilepsy.In Neal E. PrImplementation of Ketogenic Therapy. Wiley & Sons, 2012
28
term monitoring of the ketogenic diet: Do’s and Don’ts. Epilepsy
lness In Neal E.Dietary Treatment of Epilepsy: Dhamija, R., Eckert, S., &Wirrell, E. (2013). Ketogenic diet. The Canadian Journal of Neurological
r, E., Benninger, F., Freilinger, M., Hauser, E., … Feucht, M. (2010). resistant childhood epilepsy-the
The Ketogenic and Modified Atkins Diet.
Ghazavi A, Tonekaboni SH, Karimzadeh P, Nikibakhsh AA, Khajeh A, Fayyazi A. The Ketogenic and Iran J Child Neurol. 2014 Summer;8(3): 12-
Karimzadeh P, Tabarestani S, Mahvelati F., Tonekaboni SH, Ghofrani M, Intractable seizure disorders
., Azargashb E., Ghofrani M., Abdollahe-Gorgi F. Low Glycemic Index Treatment in pediatric refractory epilepsy: The first Middle East report, Seizure 23
Gil, K. R., Christina Bergqvist, A. G., Blackford, R., … Wirrell, E. C. (2018). Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group.
line therapy? Expert Review of
Kania, B. A., & Rho, J. M. (2009). Ketogenic diets: an update for child
Kossoff, E. H. W., & Shields, W. D. (2014). Nonpharmacologic care for patients with Lennox-Gastaut 33.
, Christina Bergqvist, A. G., Blackford, R., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study
Larson, A. M., Pfeifer, H. H., & Thiele, E. A. (2012). Low glycemic index treatment for epilepsy in
Neurobiochemical mechanisms of 705.
chain Triglyceride Ketogenic Diet, An Effective Treatment for ic Diets. Biomedical Journal, 36(1), 9.
Martikainen, M. H., Päivärinta, M., Jääskeläinen, S., &Majamaa, K. (2012). Successful treatment of related mitochondrial epilepsy with antiepileptic drugs and low glycaemic index diet. Epileptic
Alternative diets to the classical ketogenic diet--can
Neal E. Monitoring and side effects in Dietary Treatment of Epilepsy.In Neal E. Practical
Chapter One
· Nordli, D. (2002). The ketogenic diet: uses and abuses. Neurology, 58(12 Suppl 7), S21· Practical Implementation of Ketogenic Therapy. Wiley· Rho, J. M. (2017). How does the ketogenic diet induce anti
4–10. · Swaiman's Paediatric Neurology E· ShahabeddinRezaei , Mona Kavoosi , Reza Shervin Badv , Mahmoud Mo
Zamani , Mahmoud Reza Ashrafi , Mir Saeed Yekaninejad and Maryam Mahmoudi,· The Influence of Ketogenic Diet on Liver Function in Children and Adolescents with Intractable
Epilepsy, Rezaei S, Kavoosi M, Shervin Badv R, Mohammadi M, ZKetogenic Diet on Liver Function in Children and Adolescents with Intractable Epilepsy, J Compr Ped. 2017 ;8(3)
· Thibert, R. L., Pfeifer, H. H., Larson, A. M., Raby, A. R., Reynolds, A. A., Morgan, A. K., & Thiele, E. A. (2012). Low glycemic index treatment for seizures in Angelman syndrome. Epilepsia, 53(9), 14981502.
· Tonekaboni S H, Mostaghimi M, Mirmiran P, Abbaskhanian A, AbdollahGorji F, Mohammad Ghofrani M., Azizi F. Efficacy of the Atkins Diet as Therapy for IntraIranian Medicine, 13 (6), November 2010
· Wheless JW, Kossoff EH. Ketogenic diets In: KF Swaiman, Ashwal S, et al eds. Swaiman’s Pediatric Neurology principles and practice. Elsevier, 2017
· Zupec-Kania BA, Brown J, MatsSymposium, March 2015, Manhattan Beach, CA, USA
Nordli, D. (2002). The ketogenic diet: uses and abuses. Neurology, 58(12 Suppl 7), S21Practical Implementation of Ketogenic Therapy. Wiley-Blackwell, UK, 2012 Rho, J. M. (2017). How does the ketogenic diet induce anti-seizure effects? Neuroscience Letters, 637,
Swaiman's Paediatric Neurology E-Book: Principles and Practice, 2017 ShahabeddinRezaei , Mona Kavoosi , Reza Shervin Badv , Mahmoud Mohammadi , Gholam Reza Zamani , Mahmoud Reza Ashrafi , Mir Saeed Yekaninejad and Maryam Mahmoudi,The Influence of Ketogenic Diet on Liver Function in Children and Adolescents with Intractable Epilepsy, Rezaei S, Kavoosi M, Shervin Badv R, Mohammadi M, Zamani G R, et al. The Influence of Ketogenic Diet on Liver Function in Children and Adolescents with Intractable Epilepsy, J Compr Ped.
Thibert, R. L., Pfeifer, H. H., Larson, A. M., Raby, A. R., Reynolds, A. A., Morgan, A. K., & Thiele, E. 2012). Low glycemic index treatment for seizures in Angelman syndrome. Epilepsia, 53(9), 1498
Tonekaboni S H, Mostaghimi M, Mirmiran P, Abbaskhanian A, AbdollahGorji F, Mohammad Ghofrani M., Azizi F. Efficacy of the Atkins Diet as Therapy for Intractable Epilepsy in Children Archives of Iranian Medicine, 13 (6), November 2010 Wheless JW, Kossoff EH. Ketogenic diets In: KF Swaiman, Ashwal S, et al eds. Swaiman’s Pediatric Neurology principles and practice. Elsevier, 2017
Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet Therapies Symposium, March 2015, Manhattan Beach, CA, USA
29
Nordli, D. (2002). The ketogenic diet: uses and abuses. Neurology, 58(12 Suppl 7), S21-4.
seizure effects? Neuroscience Letters, 637,
hammadi , Gholam Reza Zamani , Mahmoud Reza Ashrafi , Mir Saeed Yekaninejad and Maryam Mahmoudi, The Influence of Ketogenic Diet on Liver Function in Children and Adolescents with Intractable
amani G R, et al. The Influence of Ketogenic Diet on Liver Function in Children and Adolescents with Intractable Epilepsy, J Compr Ped.
Thibert, R. L., Pfeifer, H. H., Larson, A. M., Raby, A. R., Reynolds, A. A., Morgan, A. K., & Thiele, E. 2012). Low glycemic index treatment for seizures in Angelman syndrome. Epilepsia, 53(9), 1498–
Tonekaboni S H, Mostaghimi M, Mirmiran P, Abbaskhanian A, AbdollahGorji F, Mohammad Ghofrani ctable Epilepsy in Children Archives of
Wheless JW, Kossoff EH. Ketogenic diets In: KF Swaiman, Ashwal S, et al eds. Swaiman’s Pediatric
umoto JH. White Paper: Proceedings of Ketogenic Diet Therapies
Chapter Two
Traditional KD Methods
What is Classic KD? Classic KD, also known as Long Chainrefractory seizure consisting of a plus carbohydrate 3-4:1. This most common ratio; 4 g of fat to 1 g of protein plus carbohydrate (4:1) means that 90% of the energy comes from fat and 10% from protein and carbohydrate combined. Thedifferences between them are summarized in should be accurately calculated. In diet design, all meals should have the same ratio. Important considerations for calorie calculation consist of:
· RDA based on age (e.g. see · Weight, height and recent growth rate· Physical activity, the · Last 3-5 days of food intake record
Calories initially restricted to 80% to 90% of the daily recommendations. Although calorie restriction alone can reduce seizure in animal models, there is no clear evidence in humans. Avoidance of excessive calories, however, may improve the Calorie intake then may be adjusted to reach ideal growth. In patients with decreased growth rate, maximizing the calorie intake is one of the considerations. If a child is underweight, caloriegradually over time. In overweight children, growing rate should be regulated based on the appropriate weight for length. Weight loss can occur during the seizure control in children; however, in adults on MAD, the best efficacy was observed in patients with weight loss.
There is no scientific evidence for fluid restriction, and this is not recommended. Sufficient fluid consumption will help to prevent constipation and renal stones. As a low carbohydrate diet is a diuretic in itself, adequate hydration is crucial for all patents on KD.(e.g. see 7-6) Fat Ninety percent of calorie comes from plus protein ratio of 4:1. Important considerations for classic KD ratio choice:
· Diet ratio, in which seizure control happens, may vary amongst patients. Overall, a 4:1 ratio has more seizure control efficacy than lower ratios; however, wittolerability. This ratio should maintain urinary ketosis at 80
·
Traditional KD Methods
Classic KD, also known as Long Chain Triglyceride (LCT) diet, is a traditional treatment for refractory seizure consisting of a high-fat diet with a ratio of grams of fat to grams of protein
4:1. This most common ratio; 4 g of fat to 1 g of protein plus means that 90% of the energy comes from fat and 10% from protein and
combined. The diet can be initiated as inpatient or outpatient settings. The differences between them are summarized in Table 2-3. Calorie and protein requirements
accurately calculated. In diet design, all meals should have the same ratio.
Important considerations for calorie calculation consist of:
RDA based on age (e.g. see Table7-7) Weight, height and recent growth rate Physical activity, the degree of mobility, seizure level
5 days of food intake record
Calories initially restricted to 80% to 90% of the daily recommendations. Although calorie restriction alone can reduce seizure in animal models, there is no clear evidence in humans.
ive calories, however, may improve the efficacy of the diet.
Calorie intake then may be adjusted to reach ideal growth. In patients with decreased growth rate, maximizing the calorie intake is one of the considerations.
If a child is underweight, calories should be calculated based on current weight then increased gradually over time. In overweight children, growing rate should be regulated based on the
weight for length.
Weight loss can occur during the diet. There is no report of a relation between BMI and seizure control in children; however, in adults on MAD, the best efficacy was observed in
There is no scientific evidence for fluid restriction, and this is not recommended. Sufficient elp to prevent constipation and renal stones. As a low carbohydrate
in itself, adequate hydration is crucial for all patents on KD.(e.g. see
Ninety percent of calorie comes from long-chain triglycerides with ideal fat t
Important considerations for classic KD ratio choice: Diet ratio, in which seizure control happens, may vary amongst patients. Overall, a 4:1 ratio has more seizure control efficacy than lower ratios; however, wittolerability. This ratio should maintain urinary ketosis at 80-160 mg/dl. Although
30
Triglyceride (LCT) diet, is a traditional treatment for diet with a ratio of grams of fat to grams of protein
4:1. This most common ratio; 4 g of fat to 1 g of protein plus means that 90% of the energy comes from fat and 10% from protein and
diet can be initiated as inpatient or outpatient settings. The . Calorie and protein requirements
accurately calculated. In diet design, all meals should have the same ratio.
Calories initially restricted to 80% to 90% of the daily recommendations. Although calorie restriction alone can reduce seizure in animal models, there is no clear evidence in humans.
of the diet.
Calorie intake then may be adjusted to reach ideal growth. In patients with decreased growth
s should be calculated based on current weight then increased gradually over time. In overweight children, growing rate should be regulated based on the
between BMI and seizure control in children; however, in adults on MAD, the best efficacy was observed in
There is no scientific evidence for fluid restriction, and this is not recommended. Sufficient elp to prevent constipation and renal stones. As a low carbohydrate
in itself, adequate hydration is crucial for all patents on KD.(e.g. see Table
triglycerides with ideal fat to carbohydrate
Diet ratio, in which seizure control happens, may vary amongst patients. Overall, a 4:1 ratio has more seizure control efficacy than lower ratios; however, with less
160 mg/dl. Although
Chapter Two
Continuing ketosis is not always needed for seizure control. Sometimes seizure control may be achieved in lower ratios 4:1 is noexample, a 3:1 ratio (86% fat) or a 2:1 (83% fat) may be used for sufficient protein in infants, or other selected patients with special recurs the ratio can be adjusted upwards. used for a few months considering the risk of side effects and poor tolerability.
· If a patient is not able to tolerate 4:1 (usually in youngsters), the diet can start from 2.5:1 to 3:1 ratio and gradually reach thin any child who has difficulty tolerating the particularly helpful in order to provide more liberal diet in adolescents.
· The ratio also may be lowered in instances of severeor excessive ketosis causing symptoms such as lethargy. (May intervention though)
· MCT oil can be added to a classic diet as an adjuvant. This approach may be used to manage hyperlipidemiaprotein content.
· MCT also has laxative effects and can improve constipation. This oil is tasteless and can be easily mixed with foods.
· Diet restriction in the first 1better improve seizure control and after the 3introduced for selectedshould be suggested very carefully in selected patients.
Protein Protein is based on minimum requirements for growth. Serum albumin and total protein can reveal if the diet provides enough protein. If this compromises linear growth, then protein should be increased to the maximum level. The type of protein sources is also important to meet the requirements, and sometimes to decrease the amount of fat in every meal and manage hyperlipidemia. (e.g. see Carbohydrate The carbohydrate amount is very limited in classic KD and consumption of it can quickly reverse ketosis and result in seizure recurrence. All supplementations and medications should be carefully reviewed and substituted with carbohydrate free formula. Diet prescription Diet prescription in classic KD is ratio based. provided by fat, protein, andapproach, the calculation is summarized in carbohydrate, and protein should be divided into 3habits.
ketosis is not always needed for seizure control. Sometimes seizure control may be achieved in lower ratios 4:1 is not a set point for every patient. For example, a 3:1 ratio (86% fat) or a 2:1 (83% fat) may be used for sufficient protein in infants, or other selected patients with special needs. If ketosis decreases and seizure recurs the ratio can be adjusted upwards. However, ratios higher than 4.5used for a few months considering the risk of side effects and poor tolerability. If a patient is not able to tolerate 4:1 (usually in youngsters), the diet can start from 2.5:1 to 3:1 ratio and gradually reach the 4:1 optimal goal. This approach may be tried in any child who has difficulty tolerating the high-fat amount. Lower ratios are particularly helpful in order to provide more liberal diet in adolescents.The ratio also may be lowered in instances of severe dyslipidemiaor excessive ketosis causing symptoms such as lethargy. (May is notintervention though) MCT oil can be added to a classic diet as an adjuvant. This approach may be used to
hyperlipidemia or increase ketosis. It also allows for more carbohydrate or
MCT also has laxative effects and can improve constipation. This oil is tasteless and can be easily mixed with foods. Diet restriction in the first 1-3 months with lower carbohydrate or higher fbetter improve seizure control and after the 3rdmonth, more liberal diet can be
selected patients without negative impact on seizure control. This should be suggested very carefully in selected patients.
minimum requirements for growth. Serum albumin and total protein can reveal if the diet provides enough protein. If this compromises linear growth, then protein should be increased to the maximum level. The type of protein sources is also important to
t the requirements, and sometimes to decrease the amount of fat in every meal and . (e.g. see Table7-8)
The carbohydrate amount is very limited in classic KD and consumption of it can quickly in seizure recurrence. All supplementations and medications should
be carefully reviewed and substituted with carbohydrate free formula.
Diet prescription in classic KD is ratio based. Table 2-1 shows the definition and calories and carbohydrates in each ratio of classic KD. For an easy and fast is summarized in Table 7-5. After the calculation
protein should be divided into 3-4 meals and/or snacks adjusted with child
31
ketosis is not always needed for seizure control. Sometimes seizure t a set point for every patient. For
example, a 3:1 ratio (86% fat) or a 2:1 (83% fat) may be used for sufficient protein in ketosis decreases and seizure
However, ratios higher than 4.5≤:1 can be used for a few months considering the risk of side effects and poor tolerability. If a patient is not able to tolerate 4:1 (usually in youngsters), the diet can start from
e 4:1 optimal goal. This approach may be tried amount. Lower ratios are
particularly helpful in order to provide more liberal diet in adolescents. dyslipidemia, poor linear growth
is not the first
MCT oil can be added to a classic diet as an adjuvant. This approach may be used to is. It also allows for more carbohydrate or
MCT also has laxative effects and can improve constipation. This oil is tasteless and
3 months with lower carbohydrate or higher fat may more liberal diet can be
patients without negative impact on seizure control. This
minimum requirements for growth. Serum albumin and total protein can reveal if the diet provides enough protein. If this compromises linear growth, then protein should be increased to the maximum level. The type of protein sources is also important to
t the requirements, and sometimes to decrease the amount of fat in every meal and
The carbohydrate amount is very limited in classic KD and consumption of it can quickly in seizure recurrence. All supplementations and medications should
shows the definition and calories carbohydrates in each ratio of classic KD. For an easy and fast
calculation is done, total fat, cks adjusted with child
Chapter Two
The divided meals and snakes are essential to avoid rapid changes and to maintain glucose and ketosis levels as steady as possible for better seizure control. Each meal or snacks shoulhave the same ratio; this rule applies to all KD types’ e.g. same carbohydrates content in LGI or same MCT amount in MCT
Table 2-1. Explanation of the ratio system in classic KD
Ratio Fat (g) Protein plus CHO (g)
1:1 1 1 1:5:1 1.5 1 2:1 2 1 2.5:1 2.5 1 3:1 3 1 3.5:1 3.5 1 4:1 4 1 4.5:1 4.5 1 5:1 5 1
*CHO: Carbohydrate
Table 2-2. Step by step diet calculation based on ratiosDiet calculation steps Each diet unit 4 g fat + 1 g protein &
Each unit (4x9 kcal/g) + (1x4 kcal/g)
Total Calorie/day Units / day Total calorie Total fat g/day Units / day x 4
Total protein g/day
Total protein + CHO g/day Total CHO g/day
CHO: Carbohydrate *, see tables for calorie and protein requirements for infants and children based on RDA. **For 5:1 ratio, each calorie unit will be 49; (5× 9kcal/g) + (1×4kcal/g) =49
The divided meals and snakes are essential to avoid rapid changes and to maintain glucose and ketosis levels as steady as possible for better seizure control. Each meal or snacks shoulhave the same ratio; this rule applies to all KD types’ e.g. same carbohydrates content in LGI or same MCT amount in MCT-KD.
1. Explanation of the ratio system in classic KD Protein plus CHO (g) A calorie from fat
(%) 69 77 82 85 87 89 90 91 92
iet calculation based on ratios Ratio 4:1 Ratio 3:1
4 g fat + 1 g protein & CHO
3 g fat + 1 g protein & CHO
(4x9 kcal/g) + (1x4 kcal/g) = 40
3x9 kcal/g) + (1x4 kcal/g) = 31
Patients weight × kcal / daily requirement*Total calorie per day / 40 Total calorie per day / 31
Units / day x 4 Units / day x 3
Patients weight × protein requirement* g/kg/day e.g. 1
Units per day × 1
(Unit per day×1) - protein (g) = CHO g/day
see tables for calorie and protein requirements for infants and children based on RDA. **For 5:1 ratio, each calorie unit will be 49; (5× 9kcal/g) + (1×4kcal/g) =49
32
The divided meals and snakes are essential to avoid rapid changes and to maintain glucose and ketosis levels as steady as possible for better seizure control. Each meal or snacks should have the same ratio; this rule applies to all KD types’ e.g. same carbohydrates content in LGI
from fat A calorie from protein plus CHO (%)
31 23 18 15 13 11 10 9 8
Ratio 2:1 2 g fat + 1 g protein & CHO
2x9 kcal/g) + (1x4 kcal/g) = 22
Patients weight × kcal / daily requirement* Total calorie per day / 22
Units / day x 2
Patients weight × protein requirement* g/kg/day e.g. 1-2 g/kg
= CHO g/day
see tables for calorie and protein requirements for infants and children based on RDA.
Chapter Two
CASE: A 3- year old boy, on classic KD, ratio 4:1 as an example for diet calculation and meal plan.
CASE: a 3-year-old boy, 14 kg Classic KD, ratio 4:1 Calorie requirement: 102 / kg =1428 Calorie intake: (food intake history) Approximately: 1350 Average calorie: 1389 / day 90% calorie; 1250 / day 125 ÷ (14.4+16.8) =4:1 Per day Protein 16.8 Fat 125 CHO 14.45 Calorie 1250
Meal Plan Breakfast 1
- Orange juice 20 g (2.1 g CHO) - Butter 25 g (15 g fat) - Oil or olive oil 10 ml (14 g fat) - Egg 31 g For carbohydrate: - 17 g of group B vegetables: e.g. Spinach or tomato
(can be mixed with egg or cream like a puree)Or - twice from group A 34 g e.g. Lettuce (to make a
chicken salad)/ or tomato/broccoli
(food’s name) نام غذا املت گوجھ فرنگی املت گوجھ قارچ خاگینھ با نعنا خاگینھ با جعفری خاگینھ کدوسبز نرگسی اسفناج کوکو ھویج
*30% cream available in Iran
ar old boy, on classic KD, ratio 4:1 as an example for diet
boy, 14 kg Classic KD, ratio 4:1 Calorie requirement: 102 / kg =1428
ke: (food intake history)
90% calorie; 1250 / day
Protein requirement: 1.2Diet units per day 1250:40= Fat g/day= 4×31.25= 125 g / day (Carbohydrate + protein) g/day= 1×31.2531.25 - 16.80= 14.45 g carbohydrate
Per Meal
4.2 g 31.3 g
3.6 312.5 kcal
Breakfast 2
17 g of group B vegetables: e.g. Spinach or tomato eam like a puree)
twice from group A 34 g e.g. Lettuce (to make a
- Cream 30%* 50 g - Milk 3% (regular) 15 g - Mozzarella 12 g - Oil/or olive oil 18 g For the remaining carbohydrate:- 10g from fruits group B such as Banana, appleOr - 7 from group A such as melon, orange This combination should be diluted with water and/or ice and get mixed. Carbohydrate free sweeteners can be added.
33
ar old boy, on classic KD, ratio 4:1 as an example for diet
Protein requirement: 1.2×14= 16.8 g / day Diet units per day 1250:40= →31.25 Fat g/day= 4×31.25= 125 g / day (Carbohydrate + protein) g/day= 1×31.25
16.80= 14.45 g carbohydrate
For the remaining carbohydrate: B such as Banana, apple
7 from group A such as melon, orange
This combination should be diluted with water and/or ice and get mixed. Carbohydrate free sweeteners can be
Chapter Two
*Remember to have some source of Omegaoils. Omega-3 is a polyunsaturated fatty acid that may reducand development and brain function. Please see also -Remember that protein provided by carbohydrates has low biologic value, although it should be considered in protein calculation. It is better to consider high biologic sources as much
*Available in Iran.
Dinner - Egg 30 g or raw chicken 27 g - Mayonnaise* 30 g - Boiled potato** 15 g - Oil/olive oil 5 g
*Mayonnaise contains 0.3 g carbs but may vary in different brands.**Skinned before cooking
Meal Plan Lunch
- Red meat (raw) 15 g/raw fish 15 g*/ground beef 15 g/Tuna in oil 16 g/15 g raw chicken/15 g raw turkey
- Butter 20 g - Olive oil/oil 15 ml (15 g fat) For remaining CHO: 3.6 g: there can be 4 options1) Using both vegetables and fruits in the - 21 g vegetable group B e.g. Mushroom or 42 g
vegetable group A e.g. Broccoli - Fruit 14 g fruit from group B like apple/banana or
21 g fruits from group A-like strawberry. (They are good when mixed with cream)
(food’s name) نام غذا خورشت کرفس دلمھ کلم دلمھ برگ کاھو خوراک تاس کباب خورشت آلو سیاه خورشت اسفناج استیک با جعفری خورشت ھویج خورشت بامیھ سوپ قارچ خوراک لوبیا سبز
Snack Snack 1 Cream cheese* 42 g Peanut butter 5 Banana 9 g or 12 g Strawberry Oil/ olive oil 16 cc
*Remember to have some source of Omega-3 in the diet. It can be found in fish, such as tuna, salmon; and nut 3 is a polyunsaturated fatty acid that may reduce the risk of cardiac disease, necessary for growth
and development and brain function. Please see also Table 5-2 Remember that protein provided by carbohydrates has low biologic value, although it should be considered in
protein calculation. It is better to consider high biologic sources as much as possible.
Egg 30 g or raw chicken 27 g غذا (food’s name) کوکو سیب زمینی کوکو مرغ پوره سیب زمینی سالاد الویھ
*Mayonnaise contains 0.3 g carbs but may vary in different brands.
Red meat (raw) 15 g/raw fish 15 g*/ground beef 15 g/Tuna in oil 16 g/15 g raw chicken/15 g raw turkey
For remaining CHO: 3.6 g: there can be 4 options sing both vegetables and fruits in the recipe:
group B e.g. Mushroom or 42 g group A e.g. Broccoli
Fruit 14 g fruit from group B like apple/banana or strawberry. (They are
2) For Vegetable Lovers If the child more interested in the 3.6 g CHO will be: - 52 g of group B vegetables or 104 g of group A 3) Fruit lovers If the child prefers fruits, then 3.6 g CHO will be: - 24 g fruits from group B and 36 g from group A. 4)Other 21.5 g boiled potato/15 g cooked rice/ 15 g cooked macaroni You can choose either of 1lunch.
Snack 2 Cream 30% 48 g Fruit: 4 g group B or 5 g group ANut: Walnut 12 g (3.5 halves)Oil or Olive oil 10 cc
34
3 in the diet. It can be found in fish, such as tuna, salmon; and nut e the risk of cardiac disease, necessary for growth
Remember that protein provided by carbohydrates has low biologic value, although it should be considered in
If the child more interested in the vegetable group, total
52 g of group B vegetables or 104 g of group A
If the child prefers fruits, then 3.6 g CHO will be: 24 g fruits from group B and 36 g from group A.
21.5 g boiled potato/15 g cooked rice/ 15 g cooked
n choose either of 1st, 2nd, 3rd or 4th option for
B or 5 g group A Nut: Walnut 12 g (3.5 halves)
Chapter Two
Diet initiation (Fasting protocol, inpatient) Step one: (Day 1-2) · Hospitalization is required · Parent education · Change medications to carbohydrate free form
The period of fasting is optional and especially can be used in status epilepticus, ICU patients or any time when a rapid response is necessary. There is no difference in diet efficacy with or without fasting period, although fasting may induce a quick response. Fluid restriction is no longer necessary, and during the carbohydrate free fluids without restriction.
Blood glucose every (4-6 hours) and urine ketones (each void) will be checked.
Duration of fasting may vary from 12ketosis becomes consistently medium to large. Children and fasting is not recommended in infants. Strong ketosis is defined as a positive urine dipstick by hydroxybutyrate (BHB) of 3.6mmol/L although; using ≥4 mmol/L may be more effective. The mean serum Bpatients with greater than 90% seizure has been over 6mm/L. Table 2-3. Comparison of Ketogenic Diet initiations
Inpatient
Most Common Method2-5-day admission A rapid transition to ketosis Use an arbitrary goal ratio Diet adjustments every 18-24 hoursLimited food available in the hospitalAcute metabolic fluctuations may occur Step two: (Day 2-3) This step consists of the gradualratio constant (which is already planned e.g. 4:1,in one third calorie intervals; one third in the first day e.g. just breakfast, 2/3 in the second (breakfast and lunch), until the full calorie meals are introduced and tolerated. Following this, the patients should attend an outpatient clinic to monitor seizure control, ketosis (mostly by a dipstick
Diet initiation (Fasting protocol, inpatient)
Hospitalization is required for a period of fasting.
Change medications to carbohydrate free form
The period of fasting is optional and especially can be used in status epilepticus, ICU patients esponse is necessary. There is no difference in diet efficacy with or
without fasting period, although fasting may induce a quick response.
Fluid restriction is no longer necessary, and during the fasting period, the child can drink ids without restriction.
6 hours) and urine ketones (each void) will be checked.
Duration of fasting may vary from 12-38 hours to when the urinary ketosis is large, or urine ketosis becomes consistently medium to large. Children should not fast and fasting is not recommended in infants.
Strong ketosis is defined as a positive urine dipstick by ≥3+ (50–150 mg/dl), or serum βhydroxybutyrate (BHB) of 3.6–5.6 mM. The effective level in children is reported to start at 4
≥4 mmol/L may be more effective. The mean serum Bpatients with greater than 90% seizure has been over 6mm/L.
3. Comparison of Ketogenic Diet initiations Inpatient Outpatient
Most Common Method Evolving New Method3-4-week initiation A slower transition to ketosis Seizure control can be achieved with the
24 hours Diet adjustments every 7-14 daysLimited food available in the hospital Variety of foods available at ho
fluctuations may occur Less occurrence and severity of metabolic
gradual introduction of food over the 3 days. By keeping the KD ratio constant (which is already planned e.g. 4:1, or 3:1 in infants, etc.) diet is advanced daily in one third calorie intervals; one third in the first day e.g. just breakfast, 2/3 in the second (breakfast and lunch), until the full calorie meals are introduced and tolerated.
ts should attend an outpatient clinic to monitor seizure control, dipstick urine test), growth rate, and nutritional/medical follow
35
The period of fasting is optional and especially can be used in status epilepticus, ICU patients esponse is necessary. There is no difference in diet efficacy with or
period, the child can drink
6 hours) and urine ketones (each void) will be checked.
to when the urinary ketosis is large, or urine longer than 72 hours
150 mg/dl), or serum β-level in children is reported to start at 4
≥4 mmol/L may be more effective. The mean serum BHB level in
Outpatient
Evolving New Method
Seizure control can be achieved with the lowest ratio 14 days
Variety of foods available at home Less occurrence and severity of metabolic fluctuations
introduction of food over the 3 days. By keeping the KD or 3:1 in infants, etc.) diet is advanced daily
in one third calorie intervals; one third in the first day e.g. just breakfast, 2/3 in the second (breakfast and lunch), until the full calorie meals are introduced and tolerated.
ts should attend an outpatient clinic to monitor seizure control, urine test), growth rate, and nutritional/medical follow-up.
Chapter Two Monitoring during diet initiation Side effects such as nausea and vomiting, lethargy that may happen during this phase should be checked as they need close attention and monitoring. By starting the diet, the childvomiting. There are some symptoms that should be closely watched: (or ketone levels in infants may be requested more frequently than in older children. Blood glucose and bicarbonate, urine or blood ketone monitoringinterval may vary depending on the age and patient’s condition in different Hypoglycemia Hypoglycemia (asymptomatic: glucose < 40 mg/dl, and symptomatic patients) requires prompt treatment by 30-60 ml of juice, orange juIf the patient is not able to tolerate orally, IV dextrose should be used, although this rarely occurs.
· Hypoglycemia is usually transient, more common in infants and young children, and will improve by advancing diet.
· Hypoglycemia may present with tachypnoea, tachycardia, diaphoresis, excessive fatigue, altered mental status. (Table 2
Table 2-4. Signs and symptoms of Hypoglycemia
JitterinessPoor body tone
Lethargy Pallor
Poor feedingLow body temperature
Cold and clammy Gastrointestinal (GI) symptoms There is a possibility of different GI complaints due or ketosis, metabolic acidosis, or other non-diet related reasons · Nausea or abdominal pain may occur due to ketosis, especially when ketones rapidly
emerge, or when the patienttreated by a carbohydrate
· These symptoms are temporary, proper hydration and diphenhydramine (symptomatic treatment) are also helpful.
· Infants or nonverbal patients may develop f· In chronic cases, lowering the diet ratio is recomm
Monitoring during diet initiation
usea and vomiting, hypoglycemia, excessive ketosis, acidosis and lethargy that may happen during this phase should be checked as they need close attention and
child will become less active and may develop food refusa
There are some symptoms that should be closely watched: (Table 2-4) Regular blood glucose or ketone levels in infants may be requested more frequently than in older children.
Blood glucose and bicarbonate, urine or blood ketone monitoring is recommended, but the interval may vary depending on the age and patient’s condition in different
(asymptomatic: glucose < 40 mg/dl, and symptomatic patients) requires 60 ml of juice, orange juice or other carbohydrate
is not able to tolerate orally, IV dextrose should be used, although this rarely
is usually transient, more common in infants and young children, and will ng diet.
may present with tachypnoea, tachycardia, diaphoresis, excessive fatigue, Table 2-4) and alleviation of symptoms is recommended.
4. Signs and symptoms of hypoglycemia and hyper ketosis Hypoglycemia Hyper ketosis
Jitteriness Rapid breathingPoor body tone Increasing heart rate
Facial flushingIrritability
Poor feeding VomitingLow body temperature Lethargy
Cold and clammy Poor feeding
Gastrointestinal (GI) symptoms
possibility of different GI complaints due or ketosis, metabolic acidosis, or other
Nausea or abdominal pain may occur due to ketosis, especially when ketones rapidly patient is not able to tolerate high blood ketones
carbohydrate-containing drink, similar to hypoglycemia These symptoms are temporary, proper hydration and diphenhydramine (symptomatic
also helpful. Infants or nonverbal patients may develop food refusal or irritability due to GI discomfort.In chronic cases, lowering the diet ratio is recommended to decrease ketone levels
36
, excessive ketosis, acidosis and lethargy that may happen during this phase should be checked as they need close attention and
and may develop food refusal or
) Regular blood glucose or ketone levels in infants may be requested more frequently than in older children.
is recommended, but the interval may vary depending on the age and patient’s condition in different centers.
(asymptomatic: glucose < 40 mg/dl, and symptomatic patients) requires ice or other carbohydrate-containing liquids.
is not able to tolerate orally, IV dextrose should be used, although this rarely
is usually transient, more common in infants and young children, and will
may present with tachypnoea, tachycardia, diaphoresis, excessive fatigue, ) and alleviation of symptoms is recommended.
yper ketosis Rapid breathing
Increasing heart rate Facial flushing
Irritability Vomiting Lethargy
Poor feeding
possibility of different GI complaints due or ketosis, metabolic acidosis, or other
Nausea or abdominal pain may occur due to ketosis, especially when ketones rapidly ketones and should be
conditions. These symptoms are temporary, proper hydration and diphenhydramine (symptomatic
ood refusal or irritability due to GI discomfort. ended to decrease ketone levels
Chapter Two
Metabolic acidosis KD induces metabolic acidosis which is highest at diet initiaconcurrent carbonic anhydrase · Bicarbonate levels should be monitored. Acidosis can be managed by proper hydration;
however, in significant symptomatic patients who develop altered mental status, emesis or bicarbonate level<20 mEq/L, oral bicarbonate treatment.
· Vomiting is the most common reported side effect and can be due to KD or GI issues such as gastro esophageal reflux disease (GERD) or concurrent infection. This shoumanaged by anti-emetics like fluids without dextrose, if indicated.
Seizure frequency Increased seizure frequency may occur like any other AED initiation course. Carbohydrate intake; however, should be checked to rule out any overlooked source. Diet initiation - Classic KD This method can start in the clinic without Complete screening of the child before ststay in proximity of medical care administration and possible side effects. The nutritionist should make sure that parents are able to implement diet at home. Overall, a very cautious approach is required for patient selection. Diet process Diet begins with full calories, but the KD ratio increases daily from 1:1, 2:1, 3:1, to 4:1 to allow the patient to adapt to the This period may take 1-2 weeks depending on the patient tolerance. Gradual initiation of diet offers the same seizure control efficacy at 3rd month of diet with significant lower early side effects, such as hypoglycemiaof vomiting. Using exchange list/computer There is a method for meal planning by using exchange lists of carbohydrate, This method relies on averages, is not accurate as hand calculation, and maycenters. Nonetheless, it requires less time and practically easy to use, parents still need training. See some examples of exchange list in the complementary tables below. There are also various computer programs that can be used insteaKeto Diet Calculator, Electronic Ketogenic Manager (EKM) or Keto planner. The complementary tables are from
KD induces metabolic acidosis which is highest at diet initiation and may be worsened by concurrent carbonic anhydrase-inhibitor AEDs.
Bicarbonate levels should be monitored. Acidosis can be managed by proper hydration; however, in significant symptomatic patients who develop altered mental status, emesis or
bonate level<20 mEq/L, oral bicarbonate is recommended for short
Vomiting is the most common reported side effect and can be due to KD or GI issues such reflux disease (GERD) or concurrent infection. This shou
emetics like promethazine, metoclopramide and proper hydration, or IV fluids without dextrose, if indicated.
Increased seizure frequency may occur like any other AED initiation course. Carbohydrate should be checked to rule out any overlooked source.
Classic KD - (Outpatient, non-fasting protocol)
This method can start in the clinic without hospital admission and fasting Complete screening of the child before starting the diet should take place and the child should stay in proximity of medical care centers. The family should be well educated about the diet administration and possible side effects. The nutritionist should make sure that parents are
nt diet at home. Overall, a very cautious approach is required for patient
Diet begins with full calories, but the KD ratio increases daily from 1:1, 2:1, 3:1, to 4:1 to allow the patient to adapt to the increased fat component.
2 weeks depending on the patient tolerance. Gradual initiation of diet offers the same seizure control efficacy at 3rd month of diet with significant lower early side
hypoglycemia, weight loss, and acidosis, although, it does not reduce the rate
list/computer programs
There is a method for meal planning by using exchange lists of carbohydrate, This method relies on averages, is not accurate as hand calculation, and may
. Nonetheless, it requires less time and practically easy to use, parents still need training. See some examples of exchange list in the complementary tables below.
There are also various computer programs that can be used instead of hand calculation such as Keto Diet Calculator, Electronic Ketogenic Manager (EKM) or Keto planner.
The complementary tables are from Dr. Neal’s book, 2012.
37
tion and may be worsened by
Bicarbonate levels should be monitored. Acidosis can be managed by proper hydration; however, in significant symptomatic patients who develop altered mental status, emesis or
for short- or long-term
Vomiting is the most common reported side effect and can be due to KD or GI issues such reflux disease (GERD) or concurrent infection. This should be
and proper hydration, or IV
Increased seizure frequency may occur like any other AED initiation course. Carbohydrate
fasting protocol)
hospital admission and fasting (See Table 2-1). arting the diet should take place and the child should
should be well educated about the diet administration and possible side effects. The nutritionist should make sure that parents are
nt diet at home. Overall, a very cautious approach is required for patient
Diet begins with full calories, but the KD ratio increases daily from 1:1, 2:1, 3:1, to 4:1 to
2 weeks depending on the patient tolerance. Gradual initiation of diet offers the same seizure control efficacy at 3rd month of diet with significant lower early side
gh, it does not reduce the rate
There is a method for meal planning by using exchange lists of carbohydrate, protein, and fat. This method relies on averages, is not accurate as hand calculation, and may vary in different
. Nonetheless, it requires less time and practically easy to use, parents still need training. See some examples of exchange list in the complementary tables below.
d of hand calculation such as Keto Diet Calculator, Electronic Ketogenic Manager (EKM) or Keto planner.
Chapter Two
* Important: This doesn’t consider offal; liver should be avoided due to high Vi** The average fat per protein exchange is less than 1.0 g. But cheese and egg contain 3which is not usually necessary to count. Carbohydrate is negligible. · For protein exchange, the average fat per protein is less than
fat, but this is not usually counted. · For exchange, remember to consider carbohydrate and protein content, some are negligible e.g. butter/oil,
some like Mayonnaise contains carbohydrates that may differ depen· LCT supplement, See chapter 8
Table C-1. Exchange table for fat and protein* for claProtein exchange
Average 3 g protein per exchangeCooked red meat® 10 g Raw red meat 15 g Cooked white fish 15 g Raw white fish 15 g Cooked turkey 10 g Raw turkey 15 g Cooked chicken 10 g
Raw chicken 15g Raw egg ½ egg
Cheddar type cheese** 12 g Tuna fish or salmon in oil 12 g
* Important: This doesn’t consider offal; liver should be avoided due to high Vitamin A.** The average fat per protein exchange is less than 1.0 g. But cheese and egg contain 3which is not usually necessary to count. Carbohydrate is negligible.
For protein exchange, the average fat per protein is less than one g except for cheddar and egg with3fat, but this is not usually counted. For exchange, remember to consider carbohydrate and protein content, some are negligible e.g. butter/oil, some like Mayonnaise contains carbohydrates that may differ depending on the brand. LCT supplement, See chapter 8
1. Exchange table for fat and protein* for classic KD. Protein exchange Fat exchange
Average 3 g protein per exchange Average 15 g fat per exchange Butter 20g ) پختھ(گوشت قرمز
Raw red meat 15 g گوشت خام قرمز Margarine (75 % fat) ite fish 15 g پختھ(ماھی( Oil 15g
Raw white fish 15 g ماھی خام Mayonnaise 20g Double cream 30g )پختھ(گوشت بوقلمون
Raw turkey 15 g گوشت بوقلمون خام Calogen Cooked chicken 10 g پختھ(مرغ(
Raw chicken 15g مرغ خام Raw egg ½ egg خام( تخم مرغ(
type cheese** 12 g پنیر چدار سالمون در
ماھی تن یا روغن
38
tamin A. ** The average fat per protein exchange is less than 1.0 g. But cheese and egg contain 3–4 g of fat per exchange
one g except for cheddar and egg with3-4 g of
For exchange, remember to consider carbohydrate and protein content, some are negligible e.g. butter/oil, ding on the brand.
Fat exchange Average 15 g fat per exchange
Butter 20g کره Margarine (75 % fat) 20g مارگرین Oil 15g روغن Mayonnaise 20g مایونز Double cream 30g خامھ
30ml
Chapter Two
* The average protein content of each fruit exchange is 0.2 g, fat content is negligible. Each vegetable exchange contains an average of 0.9 g protein
Table C-2. Exchange table for carbohydrate in classical KD (average 2 g carbohydrate per exchange)
Vegetables (g)Beetroot cooked (pickled)
)ترشی(چغندر پختھ Cabbage cooked
برگ کلم پختھ Carrots cooked
ھویج پختھCarrots raw
ھویج خامCauliflower cooked
گل کلم پختھLeeks boiled
تره فرنگی پختھ Peas, frozen, boiled
نخود فرنگی پختھ و یخ زدهTomato raw
گوجھ ی خامTurnip cooked
شلغم پختھBeetroot cooked (pickled)
)ترشی(چغندر پختھ Cabbage cooked
برگ کلم پختھ Carrots cooked
ھویج پختھ
tent of each fruit exchange is 0.2 g, fat content is negligible. Each vegetable exchange
2. Exchange table for carbohydrate in classical KD (average 2 g carbohydrate per exchange)
Vegetables (g) Beetroot cooked (pickled) 36 Apple
سیبCabbage cooked 90 Banana
موزCarrots cooked 46 Blackberries
تمشکCarrots raw 34 Blackcurrants
انگورفرنگی سیاهCauliflower cooked 96 Grapes
انگور 76 Grapefruit
گریپ فروتPeas, frozen, boiled 20 Gooseberries (stewed
without sugar) نوعی انگورفرنگیTomato raw 64 Kiwifruit
کیوی Turnip cooked 100 Mango
انبھBeetroot cooked (pickled) 36 Melon
ھندوانھCabbage cooked 90 Orange
پرتقالCarrots cooked 46 Orange juice
آب پرتقال Peach
ھلو Pear
گلابی Raspberries
تمشک Strawberries
توت فرنگی
39
tent of each fruit exchange is 0.2 g, fat content is negligible. Each vegetable exchange
Fruits (g) Apple 20
Banana 8
Blackberries 40
Blackcurrants 30
Grapes 13
Grapefruit 30
Gooseberries (stewed نوعی انگورفرنگی
80
18
Mango 14
Melon 36
Orange 24
Orange juice 18
Peach 26
Pear 20
44
34
Chapter Two
Table C-4. Group A and B Group A 100 g
Vegetables CHO Protein
7 2 CHO: Carbohydrate. Fat in both groups is negligib
Common problems in the Hunger Because of the smaller portions in classic KD, children may feel hungry in the first 1However, by achieving higher ketosis as the diet proceeds, appetite will be Sometimes parents may feel guilty about the diet. This will add some emotional issues to the child adaptation process.
Table C-3. Vegetables and Fruits group A and B (our centre list)Group A
Vegetables FruitsAsparagus cooked مارچوبھ پختھ
Strawberryتوت فرنگی
Cabbage cooked پختھ کلم برگ
Tangerineنارنگی
Celery raw or cooked کرفس پختھ یا خام
Orangeپرتقال
Cucumber خیار
Grapefruitفروت
Eggplant cooked بادنجان پختھ
Dew melon/گرمک
Green pepper فلفل دلمھ ای خام یا پختھ
Greengageترش
Rhubarb raw ریواس خام
Melonخربزه
Tomato raw گوجھ فرنگی خام
Watermelonھندوانھ
Turnip cooked شلغم پختھ
Sweet lemonشیرین
Leek cooked پختھ تره فرنگی
Cantaloupeطالبی
Raw vegetables سبزی خوردن
کاھوی خامLettuce
اندیو خامEndive raw Radish raw ترب خامZucchini raw کدو سبز خامTomato juice اب گوجھ فرنگی
fruits and vegetables components Group A 100 g Group B 100 g
Fruits Vegetables CHO Protein CHO Protein 6.7 0.67 14 4
CHO: Carbohydrate. Fat in both groups is negligible. All numbers are in a gram.
Common problems in the first month:
Because of the smaller portions in classic KD, children may feel hungry in the first 1However, by achieving higher ketosis as the diet proceeds, appetite will be suppres
Sometimes parents may feel guilty about the diet. This will add some emotional issues to the
3. Vegetables and Fruits group A and B (our centre list) Group A Gr
Fruits Vegetables Strawberry توت فرنگی
Beet cooked چغندر پختھ
Tangerine نارنگی
Cabbage raw خام برگ کلم
Orange پرتقال
Brussels sprouts cooked کلم دلمھ ای پختھ
Grapefruit فروت گریپ
Carrot raw or cooked ھویج پختھ یا خام
Dew melon/honeydew گرمک
Green bean cooked لوبیا سبز پختھ
Greengage ترش سبز وجھگ
Kohlrabi cooked کلم قمری پختھ
Melon خربزه
Onion raw or cooked پیاز خام یا پختھ
Watermelon ھندوانھ
Pumpkin cooked پختھ یا حلوایی کدو
Sweet lemon شیرین لیمو
Tomato cooked یپختھ گوجھ
Cantaloupe طالبی
Okra (cooked) پختھ بامیھ
Cauliflower cooked گل کلم پختھ
Cabbage پختھ پیچ کلم
Mushroom raw خام قارچ
Spinach raw خام اسفناج
40
Group B 100 g Fruits
CHO Protein 10 1
Because of the smaller portions in classic KD, children may feel hungry in the first 1-2 weeks. suppressed.
Sometimes parents may feel guilty about the diet. This will add some emotional issues to the
Group B Fruits Apple سیبGreen grape
سبز انگور Pineapple اناناسKiwifruit کیویNectarine شلیلPlum/prune
سیاه الو Pear گلابیApricots لوآزرد
Fig (fresh) انجیرتازهBanana موزCherries گیلاسGreengage گوجھ سبزشیرین
Chapter Two
There might be different possibilities when a child complains of hunger: adaptation to thsmaller portion, losing the pleasure of eating and finally real hunger. There are some suggestions to manage hunger: · Drinking decaffeinated zero drinks. · Freezing drinks (e.g. adding ice to the drinks)· Allowing a leaf of lettuce for each meal· Dividing meals into 4 equal meals or 3 meals and one snack to ensure stable calorie and
ketosis · Slightly decreasing calories to increase ketosis and suppress hunger.
Thirst This is not a common issue as ketosis usually suppresses the thirst. However, this is impoto ensure adequate fluid intake. Fluid should also be distributed regularly throughout the day. Giving too much fluid all at once may precipitate seizure activity and also may induce more thirst. Lost seizure control This may occur as with other There are suggestions to try in these situations. Considerations when seizure control is lost: (Kossoff, 2016) Reducing the calorie (or sometimes increasing) calories by 100 calories/day.Adding carnitine (usually 330 mg three times a day).Checking serum anticonvulsant levels and adjusting dose accordinglyReducing anticonvulsants! It may work. This is especially true in children on valproate and clonazepam. Fasting for 12 hours (As with the beginning of Ketogenic diet)Adding MCT oil (can increase ketosis, this is also another KD type)Increasing the ratio temporarily or permanently Reducing the ratio! Some children will improve on lower ketosis level.Intermittent fast; for example, 2 days a week (only one meal per day)Eliminating prepared meals and artificial sweeteners.
Diet discontinuation Important rules in all KD types For discontinuation in all diet types, concentrated sources of carbohydrates are the last components that should be returned to the diet when the child regains a fully normal diet without any issue. Ketone levels should be monitored until become negative. In case of seizure exacerbation, the diet should be set back at the last point/ratio at which the seizuunder control.
There might be different possibilities when a child complains of hunger: adaptation to thsmaller portion, losing the pleasure of eating and finally real hunger.
There are some suggestions to manage hunger:
Drinking decaffeinated zero drinks. Freezing drinks (e.g. adding ice to the drinks) Allowing a leaf of lettuce for each meal
meals into 4 equal meals or 3 meals and one snack to ensure stable calorie and
Slightly decreasing calories to increase ketosis and suppress hunger.
This is not a common issue as ketosis usually suppresses the thirst. However, this is impoto ensure adequate fluid intake. Fluid should also be distributed regularly throughout the day. Giving too much fluid all at once may precipitate seizure activity and also may induce more
This may occur as with other AEDs. In some children with good seizure control, it may recur. There are suggestions to try in these situations.
Considerations when seizure control is lost:
Reducing the calorie (or sometimes increasing) calories by 100 calories/day. Adding carnitine (usually 330 mg three times a day). Checking serum anticonvulsant levels and adjusting dose accordingly Reducing anticonvulsants! It may work. This is especially true in children on valproate and
h the beginning of Ketogenic diet) Adding MCT oil (can increase ketosis, this is also another KD type) Increasing the ratio temporarily or permanently Reducing the ratio! Some children will improve on lower ketosis level.
e, 2 days a week (only one meal per day) Eliminating prepared meals and artificial sweeteners.
Important rules in all KD types
For discontinuation in all diet types, concentrated sources of carbohydrates are the last at should be returned to the diet when the child regains a fully normal diet
without any issue. Ketone levels should be monitored until become negative. In case of seizure exacerbation, the diet should be set back at the last point/ratio at which the seizu
41
There might be different possibilities when a child complains of hunger: adaptation to the
meals into 4 equal meals or 3 meals and one snack to ensure stable calorie and
Slightly decreasing calories to increase ketosis and suppress hunger.
This is not a common issue as ketosis usually suppresses the thirst. However, this is important to ensure adequate fluid intake. Fluid should also be distributed regularly throughout the day. Giving too much fluid all at once may precipitate seizure activity and also may induce more
AEDs. In some children with good seizure control, it may recur.
Reducing anticonvulsants! It may work. This is especially true in children on valproate and
For discontinuation in all diet types, concentrated sources of carbohydrates are the last at should be returned to the diet when the child regains a fully normal diet
without any issue. Ketone levels should be monitored until become negative. In case of seizure exacerbation, the diet should be set back at the last point/ratio at which the seizure was
Chapter Two
Classic KD discontinuation Before the diet is discontinued, it should be used for at least an average of 3 months without success which means less than 50% seizure reduction. If the diet is successful, meaning more than 50 percent reduction in seizure frequency, it is usually continued for at least 2 years unless serious side effects develop. In children with seizure control more than 90%, the diet can be extended for a on KD has been reported. In GLUT-1 and PDHD, also in intractable epilepsy cases where there is a high risk of relapse, a longer duration is acceptable. In some cases, the diet is continued by switching to MAD or classic KD with a lower ratio. The recurrent risk is 20% and should be explained to the family. Risk factors for relapse include epileptic form EEG, structural abnormalities on neuroimaging, and tuberous sclerosis complex. Weaning from the diet is performed by gradually lowering the ratalthough, it can be discontinued immediately in emergency situations. One way of meaning is to reduce ratio by 0.25 to 0.5 every 1depending on ketones level and/or seizure control. The longer the duration of tslower the discontinuation process should be. An Alternative way is to gradually reduce eachmeal/snack ratio, for example: if the child is on a 3:1 ratio, do the breakfast on 2:1 to start, next week do the lunch 2:1, etc. When the ketosis becomes negative, weaning can proceed faster. Another approach is to change foods, one at a time. This is easier because no new calculation of ratio is required. For example: exchanging a portion of fat, like a heavy cream, to full cream milk (that reduceto half) over days or weeks, until it reaches just a simple milk. Reduce the oil/ butter of the meal slowly and substitute protein or carbohydrate. Replace fat with protein or carbohydrates in one meal at a time; continue for one week, and then nexweek continues with a new replacement. Switching between different types of KD to achieve seizure control is discussed in MCT Diet This diet was first developed in 1971 and further used by lower MCT amounts due to high GI side effects such as vomiting, abdominal In current MCT KD 30% to 60% of daily energy comes from MCT. This will be tailored based on the patient’s needs, tolerance and level of ketosis. Medium Chain Triglyceride (MCT) fat provides more ketones per grTriglyceride (LCT) fat because of its metabolic pathway. Therefore, more amounts of protein and carbohydrate are allowed. Fewer renal calculi, low bone density and growth retardation and fewer rereported compared to classic KD. Total cholesterol /HDL ratios are also lower, with no acidosis. Efficacy was reported as the same as classic KD.
Classic KD discontinuation
Before the diet is discontinued, it should be used for at least an average of 3 months without success which means less than 50% seizure reduction.
uccessful, meaning more than 50 percent reduction in seizure frequency, it is usually continued for at least 2 years unless serious side effects develop. In children with seizure control more than 90%, the diet can be extended for a longer duration; up to
and PDHD, also in intractable epilepsy cases where there is a high risk of relapse, a longer duration is acceptable. In some cases, the diet is continued by switching to MAD or
ratio.
ecurrent risk is 20% and should be explained to the family. Risk factors for relapse include epileptic form EEG, structural abnormalities on neuroimaging, and tuberous sclerosis
Weaning from the diet is performed by gradually lowering the ratio over 2although, it can be discontinued immediately in emergency situations.
One way of meaning is to reduce ratio by 0.25 to 0.5 every 1-2 weeks or every month, depending on ketones level and/or seizure control. The longer the duration of tslower the discontinuation process should be. An Alternative way is to gradually reduce eachmeal/snack ratio, for example: if the child is on a 3:1 ratio, do the breakfast on 2:1 to start, next week do the lunch 2:1, etc.
becomes negative, weaning can proceed faster. Another approach is to change foods, one at a time. This is easier because no new calculation of ratio is required. For example: exchanging a portion of fat, like a heavy cream, to full cream milk (that reduceto half) over days or weeks, until it reaches just a simple milk.
Reduce the oil/ butter of the meal slowly and substitute protein or carbohydrate. Replace fat with protein or carbohydrates in one meal at a time; continue for one week, and then nexweek continues with a new replacement.
Switching between different types of KD to achieve seizure control is discussed in
This diet was first developed in 1971 and further used by lower MCT amounts due to high GI as vomiting, abdominal pain, and diarrhea.
30% to 60% of daily energy comes from MCT. This will be tailored based on the patient’s needs, tolerance and level of ketosis.
Medium Chain Triglyceride (MCT) fat provides more ketones per grams than Long Chain Triglyceride (LCT) fat because of its metabolic pathway. Therefore, more amounts of protein and carbohydrate are allowed. Fewer renal calculi, hypoglycemia, ketoacidosis, constipation low bone density and growth retardation and fewer requirements of micronutrients are reported compared to classic KD. Total cholesterol /HDL ratios are also lower, with no acidosis. Efficacy was reported as the same as classic KD.
42
Before the diet is discontinued, it should be used for at least an average of 3 months without
uccessful, meaning more than 50 percent reduction in seizure frequency, it is usually continued for at least 2 years unless serious side effects develop. In children with
duration; up to 12 years
and PDHD, also in intractable epilepsy cases where there is a high risk of relapse, a longer duration is acceptable. In some cases, the diet is continued by switching to MAD or
ecurrent risk is 20% and should be explained to the family. Risk factors for relapse include epileptic form EEG, structural abnormalities on neuroimaging, and tuberous sclerosis
io over 2-3 months,
2 weeks or every month, depending on ketones level and/or seizure control. The longer the duration of the diet is, the slower the discontinuation process should be. An Alternative way is to gradually reduce each meal/snack ratio, for example: if the child is on a 3:1 ratio, do the breakfast on 2:1 to start,
becomes negative, weaning can proceed faster. Another approach is to change foods, one at a time. This is easier because no new calculation of ratio is required. For example: exchanging a portion of fat, like a heavy cream, to full cream milk (that reduces fat
Reduce the oil/ butter of the meal slowly and substitute protein or carbohydrate. Replace fat with protein or carbohydrates in one meal at a time; continue for one week, and then next
Switching between different types of KD to achieve seizure control is discussed in chapter 6.
This diet was first developed in 1971 and further used by lower MCT amounts due to high GI
30% to 60% of daily energy comes from MCT. This will be tailored
ams than Long Chain Triglyceride (LCT) fat because of its metabolic pathway. Therefore, more amounts of protein
, ketoacidosis, constipation quirements of micronutrients are
reported compared to classic KD. Total cholesterol /HDL ratios are also lower, with no
Chapter Two
Important tips about MCT· Ketosis in MCT-KD depends on the percentage of MCT, not ratio· It takes longer to begin seizure control due to the · MCT oil is more expensive. Coconut oil can be used as a substitute. Caprylic acid (C8),
capric acid (C10) in coconut oil have also more ketogenic potential.· Different studies show the same efficacy as classic KD with more GI side effects such as
nausea, vomiting or abdominal discomfort. These side statistical significance
· This diet provides steady glucose level similar to low glycaemic therapy.· It should not be used in patients who are on valproate because of the risk of liver failure. · MCT itself can be added to milk or any permitted foods and drinks as part of meal/snacks
or even as a salad dressing.· MCT oil is not proper for frying at a
container. During cooking, the · MCT may induce throat irritation. It is better to be mix
than just pour it over the food.
Diet initiation It can start either by fasting or nonRDA and protein should meet the classic KD. MCT-KD prescription Usually, 40%–55% energy from MCT can provide adequate ketosis as long as the child can tolerate that. Protein and carbohydrate percentage will be 10% and 15calorie, respectively. Long-chain triglyceride (LCT) provides the remaining daily calorie. Table 7-6 shows the calculation. In MCT diet, ketosis is not ratio related yet depends on MCT amount.
Table 2-5. Calculation for MCT diet
Total calorie requirements/day Based on RDA and diet history 45%* of calorie from MCT MCT g/day CHO g/day Protein g/day LCT g/day
* This is an example, not a fixed percentage **MCTs contain 8.3 calories per g Total daily MCT should be divided into all meals and snacks. Thidrink such as milk. Milk/MCT drink will make diet calculation easier.
Important tips about MCT-KD KD depends on the percentage of MCT, not ratio-related as classic KD
It takes longer to begin seizure control due to the gradual increase of MCT.MCT oil is more expensive. Coconut oil can be used as a substitute. Caprylic acid (C8),
C10) in coconut oil have also more ketogenic potential. Different studies show the same efficacy as classic KD with more GI side effects such as nausea, vomiting or abdominal discomfort. These side effects, however
is diet provides steady glucose level similar to low glycaemic therapy.It should not be used in patients who are on valproate because of the risk of liver failure. MCT itself can be added to milk or any permitted foods and drinks as part of meal/snacks
dressing. MCT oil is not proper for frying at a high temperature and should be kept in a dark glass container. During cooking, the temperature should be kept low for the whole time.MCT may induce throat irritation. It is better to be mixed well with milk or yogurt rather than just pour it over the food.
It can start either by fasting or non-fasting protocol. A calorie is calculated at 80RDA and protein should meet the daily requirement. All meals should have
55% energy from MCT can provide adequate ketosis as long as the child can tolerate that. Protein and carbohydrate percentage will be 10% and 15-18% of the
chain triglyceride (LCT) provides the remaining daily calorie. shows the calculation. In MCT diet, ketosis is not ratio related yet depends on MCT
5. Calculation for MCT diet Diet Calculation Steps
45%* of calorie from MCT 45% × total calorie/day/8kcal/g** 15% × total calorie/day/4kcal/g 10% × total calorie/day/4kcal/g Remaining calorie/day: 100- (45%+15%+10%)=30% 30%×Total calorie/day/9kcal/g
percentage
Total daily MCT should be divided into all meals and snacks. This can be added into the saladdrink such as milk. Milk/MCT drink will make diet calculation easier.
43
related as classic KD increase of MCT.
MCT oil is more expensive. Coconut oil can be used as a substitute. Caprylic acid (C8),
Different studies show the same efficacy as classic KD with more GI side effects such as however, didn’t reach
is diet provides steady glucose level similar to low glycaemic therapy. It should not be used in patients who are on valproate because of the risk of liver failure. MCT itself can be added to milk or any permitted foods and drinks as part of meal/snacks
temperature and should be kept in a dark glass should be kept low for the whole time.
ed well with milk or yogurt rather
is calculated at 80–100% of the requirement. All meals should have the same ratio as
55% energy from MCT can provide adequate ketosis as long as the child can 18% of the total daily
chain triglyceride (LCT) provides the remaining daily calorie. shows the calculation. In MCT diet, ketosis is not ratio related yet depends on MCT
salad as a dressing, or a
Chapter Two
CASE: A 3-year old boy on MCTKD as an example for diet calculation and meal plan.
CASE: 3-year-old boy, 14 kg MCTKDTotal calorie= 1250 kcal/day. Protein 14 g/day Milk allowance per day* 300 ml semi-skimmed milk = 14.1 g carbohydrate, 10.2 g protein, 5.1 g fat
Final daily plan 70 g MCT oil or 140 g Liquigen (if available) 300 mL of semi-skimmed milk Per day Per MealProtein 21 g 7 g LCT 37 g 12 g CHO 33 g 11 g
* This is based on child preferences and makes calculation easier asmeal/snack. The amount of CHO, total amount to have a final daily plan. Yogurt or dough could be also used instead of milk based on the child preference. MCT amount will be increased gradually (10 days to 2 weeks depending on the tolerance). Extra meals should be provided to meet calorie requirementsbiological value.
Diet process
· Regardless of initial fastingconsistent, meals are advanced daily in one third calorie intervals until full calorie intake is tolerated. MCT oil is divided into each meal; for example, 3 meal and 3 snaprevents too much MCT in one meal and subsequent instances of diarrhea or vomiting, MCT decreases by 10% and LCT increases by 10% for the following meal until the patient tolerates the diet.
· Alternatively, the diet is calculated by 30% of total calorie from LCT, 15% from carbohydrate, and 10% from protein. MCT period to reach 40-45% of total daily calorie intake.
year old boy on MCTKD as an example for diet calculation
old boy, 14 kg MCTKD 45% MCT goal
skimmed milk = 14.1 g carbohydrate,
45% energy from MCT= 562.5 MCT: 70 g/day 15% energy from carbohydraCHO: 47 g/day 10% energy from protein= 125 Protein: 31g/day Remaining 30% energy from fat= 375 LCT: 42 g/day Meal plan
70 g MCT oil or 140 g Liquigen (if available)
Meal
Breakfast 100 cc semi-skimmed milk and 18 g MCT, 7 g Protein, 12 g fat, 11 g CHO Lunch 100 cc milk and 18 g MCT, 11 g CProtein, 12g fat Snacks (two) 8 g MCT, 5.5 g CHO, 3.5 g meal, could be mid-morning or the afternoon)Before sleep 100 cc semi-skimmed milk and 18 g MCT
* This is based on child preferences and makes calculation easier as MCT will be added into milk in each meal/snack. The amount of CHO, protein, and fat provided by 300 ml of milk is deducted from the calculated total amount to have a final daily plan. Yogurt or dough could be also used instead of milk based on the child
MCT amount will be increased gradually (10 days to 2 weeks depending on the tolerance). Extra meals should requirements until MCT reaches the goal. Remember to use protein with high
egardless of initial fasting or non-fasting protocol, by keeping the MCT percentage consistent, meals are advanced daily in one third calorie intervals until full calorie intake is tolerated. MCT oil is divided into each meal; for example, 3 meal and 3 snaprevents too much MCT in one meal and subsequent diarrhea or other GI side effects. In
or vomiting, MCT decreases by 10% and LCT increases by 10% for the following meal until the patient tolerates the diet.
e diet is calculated by 30% of total calorie from LCT, 15% from carbohydrate, and 10% from protein. MCT then is gradually increased over a 7
45% of total daily calorie intake.
44
year old boy on MCTKD as an example for diet calculation
45% energy from MCT= 562.5
15% energy from carbohydrate= 187.5
10% energy from protein= 125
Remaining 30% energy from fat= 375
skimmed milk and 18 g MCT, 7 g rotein, 12 g fat, 11 g CHO
lk and 18 g MCT, 11 g CHO, 7 g
8 g MCT, 5.5 g CHO, 3.5 g Protein, 6 g fat (Half a morning or the afternoon)
skimmed milk and 18 g MCT
MCT will be added into milk in each fat provided by 300 ml of milk is deducted from the calculated
total amount to have a final daily plan. Yogurt or dough could be also used instead of milk based on the child
MCT amount will be increased gradually (10 days to 2 weeks depending on the tolerance). Extra meals should until MCT reaches the goal. Remember to use protein with high
, by keeping the MCT percentage consistent, meals are advanced daily in one third calorie intervals until full calorie intake is tolerated. MCT oil is divided into each meal; for example, 3 meal and 3 snacks. This
or other GI side effects. In or vomiting, MCT decreases by 10% and LCT increases by 10% for
e diet is calculated by 30% of total calorie from LCT, 15% from is gradually increased over a 7-10 days
Chapter Two
The daily milk allowance (based on the child’s preference/food record, can be calculated and subtracted from total daily protein and carbohydrate. It can then be used as the MCT source any time, even before sleep, to maintain ketosis overnight. LCT can be given as a fexchange. Exchanged tables are provided at the end of this c Monitoring Monitoring is similar to KD. · If seizure control requires adjusting diet, it is performed by increasing MCT up to 60% if
tolerated. MCT will gradually increase by 0.1every 1–3 days according to tolerance.
· Carbohydrate reduction for adjustment to improve ketosis should be reserved for patients who are not able to tolerate a higher MCT amount. (With lcalorie)
Gradual progression of the MCT oil is an important key to prevent GI side effects and increase patient tolerance. Caregivers should be trained order to prevent ongoing issues Discontinuation Weaning from MCT KD is performed by a slow decrease of MCT over 1done by decreasing 5-10 g of MCT daily, or per meal and/or snack, along with increasing protein (one exchange daily), until normal protein pothen be added in one exchange daily. To prevent seizure recurrence, low concentrate carbohydrate may be introduced when MCT reaches 30% in the diet. LCT may be added to meet the requirements while reducing MCT.
lk allowance (based on the child’s preference/food record, can be calculated and subtracted from total daily protein and carbohydrate. It can then be used as the MCT source any time, even before sleep, to maintain ketosis overnight. LCT can be given as a fexchange. Exchanged tables are provided at the end of this chapter
Monitoring is similar to KD.
If seizure control requires adjusting diet, it is performed by increasing MCT up to 60% if tolerated. MCT will gradually increase by 0.1–1.0 g per feed per day or 6 feeds per day
3 days according to tolerance. Carbohydrate reduction for adjustment to improve ketosis should be reserved for patients
ate a higher MCT amount. (With lowest level at 12% of daily
Gradual progression of the MCT oil is an important key to prevent GI side effects and increase patient tolerance. Caregivers should be trained in reducing MCT in case of any GI discomfort in order to prevent ongoing issues from resulting in severe complications.
Weaning from MCT KD is performed by a slow decrease of MCT over 1-10 g of MCT daily, or per meal and/or snack, along with increasing
protein (one exchange daily), until normal protein portions are achieved. The carbohydrate can then be added in one exchange daily.
To prevent seizure recurrence, low concentrate carbohydrate may be introduced when MCT reaches 30% in the diet. LCT may be added to meet the requirements while reducing MCT.
45
lk allowance (based on the child’s preference/food record, can be calculated and subtracted from total daily protein and carbohydrate. It can then be used as the MCT source any time, even before sleep, to maintain ketosis overnight. LCT can be given as a fat
If seizure control requires adjusting diet, it is performed by increasing MCT up to 60% if or 6 feeds per day
Carbohydrate reduction for adjustment to improve ketosis should be reserved for patients owest level at 12% of daily
Gradual progression of the MCT oil is an important key to prevent GI side effects and increase reducing MCT in case of any GI discomfort in
-3 months. It can be 10 g of MCT daily, or per meal and/or snack, along with increasing
rtions are achieved. The carbohydrate can
To prevent seizure recurrence, low concentrate carbohydrate may be introduced when MCT reaches 30% in the diet. LCT may be added to meet the requirements while reducing MCT.
Chapter Two
Table C-5. Exchange List for carbohydrates in MCT diet; this amount is for 5g CHO exchangeVegetable exchange (g) سبزیجاتBroccoli, raw 278 کلم بروکلی
Broccoli, boiled 455 کلم بروکلی پختھCabbage, boiled 227
برگ کلم پختھ Carrots, raw 83 ھویج خامCarrots, boiled 114 ھویج پختھCauliflower, 238boiled گل کلم پختھCelery, raw 556 کرفس خام
Zucchini, raw 287خام کدو خورشتی
Cucumber 285 خیار
Lettuce, raw 294 کاھو خامMushroom, 250 raw (1g) قارچ خامOnions, raw 63 پیاز خامOnion, spring 16 پیازچھ
runner beans 217 boiled, سبز پختھ لوبیا
Tomato, raw 161 گوجھ خام
* Whole grain wheat breakfast cereal
5. Exchange List for carbohydrates in MCT diet; this amount is for 5g CHO exchangeVegetable exchange (g) Rice, Pasta, cereals, Bread,
biscuits (g)نان، برنج Fruitsمیوه ھا
278 Rice, white, easy cook, 16 boiled
پختھ ) پخت سریع(برنج سفید
Apples, cooking, Stewed, no sugarسیب پختھ
455 Rice brown, boiled 16 برنج قھوه ای پختھ
Apples, eating 42 سیب
Cabbage, boiled 227 Spaghetti, white, 23 boiled
Banana موز
Spaghetti, whole 22 meat, boiled اسپاگتی، سبوس دار،پختھ
Cherries 53گیلاس
Carrots, boiled 114 Cornflakes 6 کورن فلکس
Kiwifruitکیوی
238 Rice Krispies 6 کریسپی برنج
Oranges پرتقال
556 Bread, white, 11 sliced نان سفید یک برش
Peaches ھلو
Zucchini, raw 287 Bread, wholemeal, 12 Average نان سبوس دار
Pears گلابی
Cucumber 285 Digestive biscuits, 7 Plain
کویت دایجستیو سادهبیس
Plums, raw, Averageآلوی خام
Lettuce, raw 294 Weetabix* 7
Strawberries توت فرنگی
250 raw (1g) Blackberrieتمشک
63 Vegetables Vegetables
Onion, spring 16 Peas, frozen, boiled 52 نخود فرنگی پختھ و یخ زده
Peppers, green, rawسبز خام
217 Sweet corn kernels, 26 Boiled ذرت پختھ
Tomatoes, puree گوجھ پختھ
161 Potatoes, old boiled 29 سیب زمینی پختھ
grain wheat breakfast cereal سبوسدار ولی کورنفلکس شبیھ صبحانھ نوعی
46
5. Exchange List for carbohydrates in MCT diet; this amount is for 5g CHO exchange Fruits میوه ھاApples, cooking, 62 Stewed, no sugar سیب پختھApples, eating 42 سیبBanana 22 موزCherries 53 گیلاس
Kiwifruit 47 کیویOranges 59 پرتقال
Peaches 66 ھلو
Pears 50 گلابی
Plums, raw, 57 Average آلوی خام Strawberries 83 توت فرنگیBlackberries° 98 تمشکVegetables
Peppers, green, 192 raw
سبز خامفلفل
Tomatoes, puree 35 گوجھ پختھ
Chapter Two
References · Bergqvist, A. G. C. (2012). Long
Epilepsy Research, 100(3), 261· Gilbert, D. L., Pyzik, P. L., & Freeman, J. M. (2000). The ketogenic diet: seizure control
correlates better with serum betaNeurology, 15(12), 787–90.
· Hoon, C. K., Yong, J. K., Dong, W. K., & Heung, D. K. (ketogenic diet for intractable childhood epilepsy: Korean multicentric experience. Epilepsia, 46(2), 272–279.
· Kossoff, E. H., Zupec-Kania, B. A., Amark, P. E., Blackford, R., … International Ketogenic Diet Study Group. (2009). of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2), 304
· Kossoff, E. H., Zupec-Kania, B. A., & Rneurologists. Journal of Child Neurology, 24(8), 979
· Lambert B.; Switching or discontinuing dietary treatment. In Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy. Fir
· Lee, P. R., & Kossoff, E. H. (2011). Dietary treatments for epilepsy: management guidelines for the general practitioner. Epilepsy &Behavior: E&B, 21(2), 115
· Liu, Y., & Wang, H.-S. (2013). MediumTreatment for Drug-resistant Epilepsy and A Comparison with Other Ketogenic Diets. Biomedical Journal, 36(1), 9.
· Magrath G and Elizabeth Neal E. The classical ketogenic diet, in Dietary Treatment of Epilepsy: Practical Implementation of Ketogeni
· Neal, E. G., Chaffe, H., Schwartz, R. H., Lawson, M. S., Edwards, N., Fitzsimmons, GH. (2009). A randomized trial of classical and mediumtreatment of childhood epilepsy.
· Nordli, D. R., Kuroda, M. M., Carroll, J., Koenigsberger, D. Y., Hirsch, L. J., Bruner, H. J., … De Vivo, D. C. (2001). Experience with the ketogenic diet in infants. Pediatrics, 108(1), 129
· Wirrell, E. C. (2008). Ketogeni17–9.
· Zupec-Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet Therapies Symposium, March 2015, Manhattan Beach, CA, USA
Bergqvist, A. G. C. (2012). Long-term monitoring of the ketogenic diet: Do’s and Don’ts. Epilepsy Research, 100(3), 261–6.
. L., Pyzik, P. L., & Freeman, J. M. (2000). The ketogenic diet: seizure control correlates better with serum beta-hydroxybutyrate than with urine ketones. Journal of Child
90. Hoon, C. K., Yong, J. K., Dong, W. K., & Heung, D. K. (2005). Efficacy and safety of the ketogenic diet for intractable childhood epilepsy: Korean multicentric experience. Epilepsia,
Kania, B. A., Amark, P. E., Ballaban-Gil, K. R., Christina Bergqvist, A. G., ., … International Ketogenic Diet Study Group. (2009). optimal
of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2), 304–17.
Kania, B. A., & Rho, J. M. (2009). Ketogenic diets: an update for child neurologists. Journal of Child Neurology, 24(8), 979–988. Lambert B.; Switching or discontinuing dietary treatment. In Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy. First Edition. 2012 Lee, P. R., & Kossoff, E. H. (2011). Dietary treatments for epilepsy: management guidelines for the general practitioner. Epilepsy &Behavior: E&B, 21(2), 115–21.
S. (2013). Medium-chain Triglyceride Ketogenic Diet, An resistant Epilepsy and A Comparison with Other Ketogenic Diets.
Biomedical Journal, 36(1), 9. Magrath G and Elizabeth Neal E. The classical ketogenic diet, in Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy First Edition. 2012. Neal, E. G., Chaffe, H., Schwartz, R. H., Lawson, M. S., Edwards, N., Fitzsimmons, GH. (2009). A randomized trial of classical and medium-chain triglyceride ketogenic diets in the treatment of childhood epilepsy. Epilepsia, 50(5), 1109–17. Nordli, D. R., Kuroda, M. M., Carroll, J., Koenigsberger, D. Y., Hirsch, L. J., Bruner, H. J., … De Vivo, D. C. (2001). Experience with the ketogenic diet in infants. Pediatrics, 108(1), 129Wirrell, E. C. (2008). Ketogenic ratio, calories, and fluids: do they matter? Epilepsia, 49 Suppl 8,
Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet Therapies Symposium, March 2015, Manhattan Beach, CA, USA
47
term monitoring of the ketogenic diet: Do’s and Don’ts.
. L., Pyzik, P. L., & Freeman, J. M. (2000). The ketogenic diet: seizure control hydroxybutyrate than with urine ketones. Journal of Child
2005). Efficacy and safety of the ketogenic diet for intractable childhood epilepsy: Korean multicentric experience. Epilepsia,
Gil, K. R., Christina Bergqvist, A. G., optimal clinical management
of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet
ho, J. M. (2009). Ketogenic diets: an update for child
Lambert B.; Switching or discontinuing dietary treatment. In Dietary Treatment of Epilepsy:
Lee, P. R., & Kossoff, E. H. (2011). Dietary treatments for epilepsy: management guidelines for
chain Triglyceride Ketogenic Diet, An Effective resistant Epilepsy and A Comparison with Other Ketogenic Diets.
Magrath G and Elizabeth Neal E. The classical ketogenic diet, in Dietary Treatment of Epilepsy:
Neal, E. G., Chaffe, H., Schwartz, R. H., Lawson, M. S., Edwards, N., Fitzsimmons, G., Cross, J. chain triglyceride ketogenic diets in the
Nordli, D. R., Kuroda, M. M., Carroll, J., Koenigsberger, D. Y., Hirsch, L. J., Bruner, H. J., … De Vivo, D. C. (2001). Experience with the ketogenic diet in infants. Pediatrics, 108(1), 129–33.
c ratio, calories, and fluids: do they matter? Epilepsia, 49 Suppl 8,
Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet
Chapter Three
KD for infants KD is recommended early in the treatment course for infant refractory epilepsy. KD in infants, however; is very potential side effects at this age also, di Important tips · Classic KD is recommended for infants less than 2 years of age in order to have an
accurate calculation of all diet components. Other KD types are not recommended. · The ketogenic ratio in infants during the first yea
ratio explanation, see · Hyper ketosis in infants is defined by a ketone level at · Patients should have a feeding assessment. If there is any feeding difficulty, which is
common in neurologic disorders, enteral feeding should be suggested. (see also chapter one)
· There are different sources to use alone, or in combination, to archive including Breast milk, keto formulas, pureed infant’s foods, carbohydrate free formula, fat/MCT supplementations. See KD products in chapter 8.
· In the case of formulasupplementation of A, D, E is required. Please see below, comparison of keto formula and regular formulas, also human/cow’s milk and formulas. Considering these differences can binfant’s diet e.g. if the you calculate the total daily calorie while infants are a combination of
KD is recommended early in the treatment course for infant refractory epilepsy. KD in infants, however; is very demanding and requires close medical attention because of the potential side effects at this age also, diet restrictiveness.
Classic KD is recommended for infants less than 2 years of age in order to have an accurate calculation of all diet components. Other KD types are not recommended. The ketogenic ratio in infants during the first year of life is usually 3:1 or 2.5:1. (For ratio explanation, see Table 7-1) Hyper ketosis in infants is defined by a ketone level at ≥5 mmol/l. Patients should have a feeding assessment. If there is any feeding difficulty, which is common in neurologic disorders, enteral feeding should be suggested. (see also chapter
re different sources to use alone, or in combination, to archive Breast milk, keto formulas, pureed infant’s foods, carbohydrate free formula,
fat/MCT supplementations. See KD products in chapter 8. formula, consumption is less than 500 ml per day, extra vitamin
supplementation of A, D, E is required. Please see below, Table 3comparison of keto formula and regular formulas, also human/cow’s milk and formulas. Considering these differences can be of great help when you deal with infant’s diet e.g. if the combination of natural milk with Ketocal®
you calculate the total daily calorie while infants are a combination of
KD for infants
48
KD is recommended early in the treatment course for infant refractory epilepsy. KD in and requires close medical attention because of the
Classic KD is recommended for infants less than 2 years of age in order to have an accurate calculation of all diet components. Other KD types are not recommended.
r of life is usually 3:1 or 2.5:1. (For
≥5 mmol/l. Patients should have a feeding assessment. If there is any feeding difficulty, which is common in neurologic disorders, enteral feeding should be suggested. (see also chapter
re different sources to use alone, or in combination, to archive the desired ratio Breast milk, keto formulas, pureed infant’s foods, carbohydrate free formula,
is less than 500 ml per day, extra vitamin Table 3-1 and Table 3-2 for
comparison of keto formula and regular formulas, also human/cow’s milk and e of great help when you deal with
® are used or when you calculate the total daily calorie while infants are a combination of milk.
Chapter Three
Table 3-1. Comparison of Ketocalmacro/micronutrients Nutrient per 100 kcal AptamilEnergy (kcal) 100Protein (g) 2 Carbohydrate (g) 11.1 (lactose)Fat (g) 5.3 (vegetable oil)Vitamin A (μg) 84Vitamin D (μg) 1.8Vitamin E (mg) 1.6Vitamin C (mg) 14Thiamin (mg) 0.076Riboflavin (mg) 0.189Folic acid (mg) 18Vitamin B6 (mg) 0.06Vitamin B12 (μg) 0.27Sodium (mg) 26Potassium (mg) 96Chloride (mg) 63Calcium (mg) 76Phosphorus (mg) 42Calcium / phosphorus 1.8:1Magnesium (mg) 7.6Iron (mg) 0.8Copper (μg) 61Zinc (mg) 0.76Manganese (mg) 0.011Iodine (μg) 18Selenium (μg) 2.3
Table 3-2. Differences between formula and hu
Human Milk
Carb 40% 11g/100kcal
Protein 6% 1.5 g/100
Fat 51% 5.7g/100
Calorie 0.67 kcal/cc
1. Comparison of Ketocal® with regular formula in providing
Aptamil® 1st Ketocal® 3:1 100 100
2.2 11.1 (lactose) 1(lactose) 5.3 (vegetable oil) 9.7 (palm, soya) 84 75.1 1.8 1.7 1.6 1.1 14 12 0.076 0.1 0.189 0.1 18 20 0.06 0.1 0.27 0.2 26 45.1 96 142 63 68.8 76 120 42 80 1.8:1 1.5:1 7.6 15 0.8 1.7 61 119 0.76 1.2 0.011 0.09 18 22.2 2.3 3
2. Differences between formula and human/cow milk Human Milk Infant formula Cow milk
11g/100kcal 41% 11g/100kcal
26% 6.9g/100 kcal
1.5 g/100 8.4% 2.1 g/100
20 % 4.9 g/100 kcal
5.7g/100 48% 5.3 g/100
50% 5.6 g/100 kcal
0.67 kcal/cc 0.67 kcal/cc 0.67 kcal/cc
49
with regular formula in providing
Ketocal® 4:1 100 2.1 0.4 (glucose syrup) 10 (soybean oil) 52.1 0.71 1 6.2 0.1 0.1 15.1 0.1 0.11 68.5 110 103 58.9 58.9 1:1 15.1 1 82.2 0.82 0.09 12.3 3
Child formula
53% 13.2 g/100 12% 3 g/kg/100 35% 4 g/kg/100 1 kcal/cc
Chapter Three
Patient selection KD is used in the treatment of refractory epilepsies/encephalopathy in infancy after the failure of two AEDs (likewise in children). Important indications include:
· Infantile spasm (particularity if resistant to · Epilepsy with migrating foci · Ohtahara syndrome · Specific metabolic disorders; GLUT1 and PDHD· Surgery candidates waiting for epilep· Some mitochondrial disorders
(See also chapter one for indications and contraindications) Baseline assessment Before diet initiation, baseline laboratory tests should be performed to make sure there is no preexisting disease or deficiency. This part is explained in chapter one. baseline monitoring in infants. (European guideline for infants)
Table 3-3. Advised baseline monitoringEssential Blood: - Full blood count - Renal profile (includes sodium,
urea, creatinine, bicarbonate,- Liver profile - Calcium, phosphate, magnesium- Glucose - Vitamin D - Lipid profile (repeat with fasting if elevated)- Free and total carnitine Urine: - Urinalysis, calcium: creatinine ratio- organic acids
KD is used in the treatment of refractory epilepsies/encephalopathy in infancy after the failure likewise in children). Important indications include:
Infantile spasm (particularity if resistant to first-line medications) Epilepsy with migrating foci
Specific metabolic disorders; GLUT1 and PDHD Surgery candidates waiting for epileptic surgery Some mitochondrial disorders
(See also chapter one for indications and contraindications)
Before diet initiation, baseline laboratory tests should be performed to make sure there is no prency. This part is explained in chapter one. Table 3
baseline monitoring in infants. (European guideline for infants)
3. Advised baseline monitoring Recommended
Renal profile (includes sodium, potassium, urea, creatinine, bicarbonate,and albumin)
Calcium, phosphate, magnesium
Lipid profile (repeat with fasting if elevated)
Urinalysis, calcium: creatinine ratio
Blood: - Vitamins A, E, B12 - Zinc, selenium, copper - Folate, ferritin
50
KD is used in the treatment of refractory epilepsies/encephalopathy in infancy after the failure
Before diet initiation, baseline laboratory tests should be performed to make sure there is no pre-Table 3-3 summarized the
Chapter Three
Calorie The recommendation for daily energy is 75% to 100% of Recommended Dietary Allowance (RDA). The final calorie count, however, is an averas well as RDA for age and gender considering recent growth rate. This may be adjusted later according to the individual’s needs and growth rate. fluid please see Table 7-5. Physical activity is also important for calorie calculation: For example, the presence of severe motor impartment, the degree Fat Classic KD with a 3:1 ratio is applied in most infants. Ador higher ratio (3.5 or 4:1) will be based on the should be performed carefully to meet the daily requirement and ensure an acceptable growth rate, particularly if the ratio is high (4:1). Infants have a low tolerance to MCT, but it can be also used by 10% to 25% of daily calorie to liberalize diet. MCT can be mixed with Protein Providing 10% of energy from combined protein and caprotein which is a minimum requirement. To prevent growth failure a protein to calorie ratio of 1.5 g protein / 100 kcal is recommended. DRA for protein should be met and adjusted in case of growth failure. Carbohydrate Carbohydrate should be greatly restricted and divided throughout the day to avoid hypoglycemia or excessive ketosis. Artificial sweeteners are not recommended for infants. Like other infants, as patients grow, solid foods should be introduced (based on agdevelopment). This will increase fiber in the diet which helps to prevent constipation. All supplements and medications should be carbohydrate free. Fluid There is no fluid restriction. Sufficient fluid consumption is essential and should be moniby physical examination, serial weighting, and some tests such as calcium: creatinine ratio and urine specific gravity.
The recommendation for daily energy is 75% to 100% of Recommended Dietary Allowance (RDA). The final calorie count, however, is an average of the patient’s food history and intake, as well as RDA for age and gender considering recent growth rate. This may be adjusted later according to the individual’s needs and growth rate. For RDA in infants, calorie, protein and
Physical activity is also important for calorie calculation: For example, the presence of severe degree of spasticity or frequent seizure of muscle contractions.
Classic KD with a 3:1 ratio is applied in most infants. Adjustment to a lower ratio (2.5 or 2:1) or higher ratio (3.5 or 4:1) will be based on the level of ketosis and tolerance. should be performed carefully to meet the daily requirement and ensure an acceptable growth
atio is high (4:1).
tolerance to MCT, but it can be also used by 10% to 25% of daily calorie to liberalize diet. MCT can be mixed with low-fat milk product to make a drink.
Providing 10% of energy from combined protein and carbohydrate usually means 1g/kg protein which is a minimum requirement. To prevent growth failure a protein to calorie ratio of 1.5 g protein / 100 kcal is recommended. DRA for protein should be met and adjusted in
arbohydrate should be greatly restricted and divided throughout the day to avoid or excessive ketosis. Artificial sweeteners are not recommended for infants.
Like other infants, as patients grow, solid foods should be introduced (based on agdevelopment). This will increase fiber in the diet which helps to prevent constipation. All supplements and medications should be carbohydrate free.
There is no fluid restriction. Sufficient fluid consumption is essential and should be moniby physical examination, serial weighting, and some tests such as calcium: creatinine ratio
51
The recommendation for daily energy is 75% to 100% of Recommended Dietary Allowance age of the patient’s food history and intake,
as well as RDA for age and gender considering recent growth rate. This may be adjusted later For RDA in infants, calorie, protein and
Physical activity is also important for calorie calculation: For example, the presence of severe muscle contractions.
justment to a lower ratio (2.5 or 2:1) of ketosis and tolerance. The calculation
should be performed carefully to meet the daily requirement and ensure an acceptable growth
tolerance to MCT, but it can be also used by 10% to 25% of daily calorie milk product to make a drink.
rbohydrate usually means 1g/kg protein which is a minimum requirement. To prevent growth failure a protein to calorie ratio of 1.5 g protein / 100 kcal is recommended. DRA for protein should be met and adjusted in
arbohydrate should be greatly restricted and divided throughout the day to avoid or excessive ketosis. Artificial sweeteners are not recommended for infants.
Like other infants, as patients grow, solid foods should be introduced (based on age and development). This will increase fiber in the diet which helps to prevent constipation. All
There is no fluid restriction. Sufficient fluid consumption is essential and should be monitored by physical examination, serial weighting, and some tests such as calcium: creatinine ratio
Chapter Three
Vitamins and minerals Supplementation should be considered according to RDA for age and Diet initiation · Parents should fill out the intake history form. · All infants (<12 months) should be admitted. · Fasting is not recommended for infants. · Diet should start stepwise by a 1:1 ratio, with a
3:1 ratio. For example, the diet can start with a hours with gradually changesvegetables with whipped heavy cream can be commenced once a
· A 3:1 formula is recommended for infants. A 4:1 formula is reserved for specific patients and should be used with caution.
· Infants can continue bottle or be mixed with a 3:1 ketogenic formuinfant’s need and diet ratio and given by bottle or tube.
· Sometimes, breastfeedingketogenic formula.
· Advancement is dependent upon seizure control
Vitamins and minerals
Supplementation should be considered according to RDA for age and weight. (See chapter 7)
Parents should fill out the intake history form. All infants (<12 months) should be admitted. Fasting is not recommended for infants. Diet should start stepwise by a 1:1 ratio, with a gradual increase to a clas:1 ratio. For example, the diet can start with a half-strength keto formula in the first 24
hours with gradually changes to full keto formula. Keto meals, such as pureed meats and vegetables with whipped heavy cream can be commenced once a day. A 3:1 formula is recommended for infants. A 4:1 formula is reserved for specific patients and should be used with caution. Infants can continue bottle or breastfeeding while on the diet. Expressed breast milk can be mixed with a 3:1 ketogenic formula. This amount should be calculated according to the infant’s need and diet ratio and given by bottle or tube.
breastfeeding may be allowed along with a calculated amount of 3:1
Advancement is dependent upon seizure control, tolerance and level of ketosis.
52
weight. (See chapter 7)
increase to a classical KD with a formula in the first 24
to full keto formula. Keto meals, such as pureed meats and day.
A 3:1 formula is recommended for infants. A 4:1 formula is reserved for specific patients
while on the diet. Expressed breast milk can la. This amount should be calculated according to the
may be allowed along with a calculated amount of 3:1
, tolerance and level of ketosis.
Chapter Three
CASE: A 10-month old boy/girl formula fed infant on the CK.
Three meals, one snack, formula feeds at least 3 W= 9 kg 3:1 ratio Calorie requirements: 800* kcal Protein requirements: 11 200 kcal/31= 6.45 dietary units. Fat: 19.35 g/day Protein: 3 g/day CHO: 3.45 g/day Continue breastfeeding/ or formula and introduce half strength 3:1 Ketocal® once/dayGradually substitute half strength for all daily formulas over a time.
After full substitution, change half strength to full strength one at a time Change a meal to keto meal one at a time over a time period Continue to achieve full diet
*Calorie is considered 600 kcal from formula and 200 kcal from meals for easy calculation.
Meal plan examples boiled carrot sticks, soft pieces of fruit as banana, slightly cooked apple/pear
Cheese cubs Hard-boiled egg/ omelette
Mixed Ketocal® with 30% cream and or butter to keep the ratio (Ketogenic yogurt) Meal 1 Cream 30% mix with Ketocal® *powder 3:1, water, olive oil,and banana.
Other appropriate products include carbohydrate or protein powder supplements that make calculation accurate. Other prepared baby foods can be used in very small amounts. low carbohydrate foods for babies. Remember that meals should be spread out during a day with the same ratio.*See Table 7- mixing guide for Ketocal
month old boy/girl formula fed infant on the CK.
, formula feeds at Important tips
A calorie from formula/breast milk and solid may vary depending on a number of breast/formulaefeeding per day and can be planned compatible with baby’s history intake At this age 3 to 4 feedings per day or24 to 30 ounces per day formula.A calorie from meals (solids) around 180kcal/day
/ or formula and introduce half strength 3:1 Ketocal® once/day
The formula can be also diluted by expressed breast milk
Gradually substitute half strength for all daily The gradual process is based on the level of ketosis and infant’s tolerance (see diet initiation monitoring, chapter 2)
substitution, change half strength to full
Change a meal to keto meal one at a time over a time Total meal calorie should be divided into e.g. 3 meals and one snack along with formula feeds
*Calorie is considered 600 kcal from formula and 200 kcal from meals for easy calculation.
Tips boiled carrot sticks, soft pieces of fruit as banana, Introducing finger foods orbits first. Then, offer
solids such as Ketogenic purées of fruit or vegetables made by adding oil, cream and protein powder. If protein powder is not available, yolk and/or white egg is a good substitute. As with other infants, the introductionshould include increasing textures and variety.
with 30% cream and or butter to
Meal 2 *powder 3:1, water, Ground almond (cooked) mixed with cream or fruit
puree.
Other appropriate products include carbohydrate or protein powder supplements that make calculation accurate. Other prepared baby foods can be used in very small amounts. Groundnuts like almond is a very good source of low carbohydrate foods for babies. Remember that meals should be spread out during a day with the same ratio.
mixing guide for Ketocal®
53
month old boy/girl formula fed infant on the CK.
from formula/breast milk and solid may breast/formulae
feeding per day and can be planned compatible with
At this age 3 to 4 feedings per day or 24 to 30 ounces per day formula.
from meals (solids) around 180-200
can be also diluted by expressed breast
The gradual process is based on the level of ketosis and infant’s tolerance (see diet initiation monitoring,
Total meal calorie should be divided into e.g. 3 meals and one snack along with formula feeds
*Calorie is considered 600 kcal from formula and 200 kcal from meals for easy calculation.
first. Then, offer solids such as Ketogenic purées of fruit or vegetables made by adding oil, cream and protein powder. If protein powder is not available, yolk and/or white
introduction of solids should include increasing textures and variety.
Ground almond (cooked) mixed with cream or fruit
Other appropriate products include carbohydrate or protein powder supplements that make calculation accurate. a very good source of
low carbohydrate foods for babies. Remember that meals should be spread out during a day with the same ratio.
Chapter Three
Some suggested combinations to d · Expressed breast milk along with carbohydrate
supplemental fat modular (emulsified LCT or MCT oils)· Ross Carbohydrate - Free Formula with Iron can be mixed with a carbohydrate polymer
such as Polycose powder, and a lipid source like micro lipid, to achieve the specific KD ratio. Please see some keto precipices that are suitable for infants in chapter 11.
For monitoring during diet initiation, see classic KD (chapter 2)The instructions how to use Keto
Diet Process If adequate ketosis (2-5 mmol/l in the calculation, re-evaluation is required for any possible carbohydrate source such as medications. Subsequently, the diet ratio should be changed. Different combinations can be used for infants in order to introduce solid foods. By keeping the diet ration constant, a combination of keto formula and solid foods, such as vegetables or fruits mixed with whipping cream, margarine, or oil, can be calculated and added. By introducing more carbohydrate, such as bread or potato at the age of 9amount of MCT can also be used for more liberation. MCT should be used with caution as it is poorly tolerated in infancy.
Monitoring and follow up Follow up is also fully explained in chapter one. Some important considerations in infants will be reviewed here.
Growth Because the growth rate during the first year is considerable, frequent visits to measugrowth parameters are necessary. diet discontinuation; particularly height measures. (See also chapter 5 for stepwise management of growth failure.)
Side effects reported in infants Most common side effects consist of GI issues such as constipation and reflux, altered lipid levels, renal stones, and acidosis. Other reported side effects include infection risk, bruising, fractures, pancreatitis, and lipoid
Discontinuation If the diet is not effective in infants, a 2the patient should quickly go to the next therapeutic option. Before considering the diet is ineffective, it is important to review diet calculatioand level of ketosis. Please see the
Some suggested combinations to design Keto formula
Expressed breast milk along with carbohydrate-free formula (such as RCF) plus supplemental fat modular (emulsified LCT or MCT oils)
Free Formula with Iron can be mixed with a carbohydrate polymer wder, and a lipid source like micro lipid, to achieve the specific KD
see some keto precipices that are suitable for infants in chapter 11.
For monitoring during diet initiation, see classic KD (chapter 2). The instructions how to use Ketocal formula is shown in Table 7-11 and Table
5 mmol/l in the blood) isn’t achieved after 2 weeks with proper evaluation is required for any possible carbohydrate source such as
uently, the diet ratio should be changed.
Different combinations can be used for infants in order to introduce solid foods. By keeping the diet ration constant, a combination of keto formula and solid foods, such as vegetables or
g cream, margarine, or oil, can be calculated and added. By introducing more carbohydrate, such as bread or potato at the age of 9-12 months, a small amount of MCT can also be used for more liberation. MCT should be used with caution as it
ted in infancy.
Monitoring and follow up
Follow up is also fully explained in chapter one. Some important considerations in infants will
Because the growth rate during the first year is considerable, frequent visits to measugrowth parameters are necessary. The growth rate is more of an issue in infants, even after diet discontinuation; particularly height measures. (See also chapter 5 for stepwise management of growth failure.)
Side effects reported in infants
on side effects consist of GI issues such as constipation and reflux, altered lipid acidosis. Other reported side effects include infection risk, bruising,
fractures, pancreatitis, and lipoid- pneumonia. (See also chapter 5)
If the diet is not effective in infants, a 2-week period is enough for discontinuation because the patient should quickly go to the next therapeutic option. Before considering the diet is ineffective, it is important to review diet calculation/ratio, the proper administration at home, and level of ketosis. Please see the discontinuation of classic KD in chapter 2.
54
free formula (such as RCF) plus
Free Formula with Iron can be mixed with a carbohydrate polymer wder, and a lipid source like micro lipid, to achieve the specific KD
see some keto precipices that are suitable for infants in chapter 11.
and Table 7-12.
) isn’t achieved after 2 weeks with proper evaluation is required for any possible carbohydrate source such as
Different combinations can be used for infants in order to introduce solid foods. By keeping the diet ration constant, a combination of keto formula and solid foods, such as vegetables or
g cream, margarine, or oil, can be calculated and added. By 12 months, a small
amount of MCT can also be used for more liberation. MCT should be used with caution as it
Follow up is also fully explained in chapter one. Some important considerations in infants will
Because the growth rate during the first year is considerable, frequent visits to measure rate is more of an issue in infants, even after
diet discontinuation; particularly height measures. (See also chapter 5 for stepwise
on side effects consist of GI issues such as constipation and reflux, altered lipid acidosis. Other reported side effects include infection risk, bruising,
week period is enough for discontinuation because the patient should quickly go to the next therapeutic option. Before considering the diet is
administration at home, of classic KD in chapter 2.
Chapter Three References · Kossoff, E. H., Zupec-Kania, B. A., Amark, P. E.,
Blackford, R., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2), 304
· van der Louw, E., van den Hurk, D., Neal, E., Leiendecker, B., Cross, J. H. (2016). Ketogenic diet guidelines for infants with refractory epilepsy. European Journal of Paediatric Neurology: EJPN: Official Journal of the European PaedSociety, 20(6), 798–809.
· Zupec-Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet Therapies Symposium, March 2015, Manhattan Beach, CA, USA
Kania, B. A., Amark, P. E., Ballaban-Gil, K. R., ChristinBlackford, R., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2), 304–17.
Louw, E., van den Hurk, D., Neal, E., Leiendecker, B., FitzsimmonCross, J. H. (2016). Ketogenic diet guidelines for infants with refractory epilepsy. European Journal of Paediatric Neurology: EJPN: Official Journal of the European Paed
809. Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet
Therapies Symposium, March 2015, Manhattan Beach, CA, USA
55
Gil, K. R., Christina Bergqvist, A. G., Blackford, R., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of children receiving the ketogenic diet: recommendations of the International
Fitzsimmon, G., Dority, L., … Cross, J. H. (2016). Ketogenic diet guidelines for infants with refractory epilepsy. European Journal of Paediatric Neurology: EJPN: Official Journal of the European Paediatric Neurology
Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet
Chapter Four
Modern Ketogenic diet
· Modified Atkins Diet (MAD) · Low Glycemic Index Diet (LGI)· Modified Ketogenic Diet (MKD)
MAD The Atkins diet was originally developed by Dr. Robert Atkins for management of obesity in the 1970s. In 2003, the first epilepsy patients in 2006 it was formally named the The Modified Atkins Diet (MAD) is approximately 1because the diet is not limited to a specific amount of calorie, Although 2:1 is a safe ratio, especially in the first month.Efficacy is reported close to classic KD, not the same, with more diet flexibility. Important tips about MAD 1) MAD is initiated as an outpatient setting
carbohydrate restriction. There is no particular preference in carbohydrate types; however, parents usually prefer low glycemic index types to get large quanti
2) Training for MAD can be undertaken in a clinic involving one hour with parents. Less weighing and measuring of foods are necessary, so preparing meals is easier with more dietary choices.
3) Patients and families are given high carbohydrate foods’ list learn about them. (Complementary Tables 1 and 2)
Diet initiation
· A 3-day food intake record should be completed by parents. This helps to set calorie goals based on individual needs and satiety.
· Parental training includes identifying reading, and weighting carbohydrates.
· High-fat foods with liberal protein, calorie, and fluids are encouraged.· Fiber supplements, fibrous carbohydrates such as fruits and vege
prevent constipation. · Fluid consumption is also encouraged. To estimate diet structure, calories can be calculated as 100% of the requirement for age, gender, with 60-65% of calories from fat, 20
Remember, as mentioned before, calorie and protein are not limited, and considering the calorie requirement or food record will help to set the calorie goal and maintain a sufficient growth rate or prevent too much weight gain. The iand BMI.
The ratio should be calculated as 2:1, especially in the limited to 10 g for children 2-12 years, 15 gr/day for adolescents and 20 g/day for increase (by 5-10 g/day), depending on seizure control. The higher carbohydrate content may be tried after 1-3 months and maintained between 10
Modern Ketogenic diet
Low Glycemic Index Diet (LGI) Modified Ketogenic Diet (MKD)
The Atkins diet was originally developed by Dr. Robert Atkins for management of obesity in the 1970s. In 2003, the first epilepsy patients received this diet under the supervision of Dr. Kossoff, and in 2006 it was formally named the Modified Atkins Diet to use in refractory epilepsies.The Modified Atkins Diet (MAD) is approximately 1-2:1 KD. This ratio may vary day by day
s not limited to a specific amount of calorie, fat, and protein as like classic KD. Although 2:1 is a safe ratio, especially in the first month. Efficacy is reported close to classic KD, not the same, with more diet flexibility.
Important tips about MAD
outpatient setting, and there is no guideline regarding foods, except for carbohydrate restriction. There is no particular preference in carbohydrate types; however, parents usually prefer low glycemic index types to get large quantities.
can be undertaken in a clinic involving one hour with parents. Less weighing and measuring of foods are necessary, so preparing meals is easier with more dietary choices.Patients and families are given high carbohydrate foods’ list and carbohydrate gram counter list to
about them. (Complementary Tables 1 and 2)
day food intake record should be completed by parents. This helps to set calorie goals based on individual needs and satiety.
ludes identifying high-fat foods, a listing of carbohydrateweighting carbohydrates.
foods with liberal protein, calorie, and fluids are encouraged. Fiber supplements, fibrous carbohydrates such as fruits and vegetables are recommended to
Fluid consumption is also encouraged.
To estimate diet structure, calories can be calculated as 100% of the requirement for age, 65% of calories from fat, 20-30% from protein, and 4-6% by carbohydrates.
s mentioned before, calorie and protein are not limited, and considering the calorie requirement or food record will help to set the calorie goal and maintain a sufficient growth rate or prevent too much weight gain. The initial plan can be then changed based on the patient’s needs
should be calculated as 2:1, especially in the first few months. Carbohydrate should be 12 years, 15 gr/day for adolescents and 20 g/day for
10 g/day), depending on seizure control. The higher carbohydrate content may be maintained between 10-30 g/day.
56
The Atkins diet was originally developed by Dr. Robert Atkins for management of obesity in the received this diet under the supervision of Dr. Kossoff, and
Modified Atkins Diet to use in refractory epilepsies. 2:1 KD. This ratio may vary day by day
protein as like classic KD.
Efficacy is reported close to classic KD, not the same, with more diet flexibility.
, and there is no guideline regarding foods, except for carbohydrate restriction. There is no particular preference in carbohydrate types; however, parents
can be undertaken in a clinic involving one hour with parents. Less weighing and measuring of foods are necessary, so preparing meals is easier with more dietary choices.
and carbohydrate gram counter list to
day food intake record should be completed by parents. This helps to set calorie goals based
carbohydrate-free products, label
tables are recommended to
To estimate diet structure, calories can be calculated as 100% of the requirement for age, size, and 6% by carbohydrates.
s mentioned before, calorie and protein are not limited, and considering the calorie requirement or food record will help to set the calorie goal and maintain a sufficient growth rate or
nitial plan can be then changed based on the patient’s needs
few months. Carbohydrate should be 12 years, 15 gr/day for adolescents and 20 g/day for adults. This may
10 g/day), depending on seizure control. The higher carbohydrate content may be
Chapter Four
If the calculated carbohydrate by calorie percentage is more than 10as new research shows strict carbohydrate regimen in the
The type of carbohydrate is not important, but as mentioned before, parents will choose the low
glycemic in order to have a bigger portion. In the first month, even 5 g per day of carbohydrate may be tried in children and it is beneficial. Please see complementary tables at the end of this chapter for the carbohydrate content of foods.
MAD initiation and maintenance
Table 4-1. Modified Atkins Diet (MAD) Protocol (2013)Diet initiation Baseline laboratory studies ● Complete blood count, ●Liver, kidney function tests, electrolytes ● Fasting lipid profile ●2-day food intake record obtained before initiation of the diet
Monitoring and maintenance (1st month) ●Weight checked weekly ● Ketones checked twice a week for at least the first month Maintenance (after 1st month) ●May increase net carbohydrates (by 5-10 g/day), change anticonvulsants, use Low-carb products (as deemed necessary) Discontinuation ●Increase carbohydrate limit by 10 g/day/week until 60 g/day
For MAD patient selection see chapter 6.
If the calculated carbohydrate by calorie percentage is more than 10 g/day, it should be decreased to 10 g/day as new research shows strict carbohydrate regimen in the first month will improve seizure control.
The type of carbohydrate is not important, but as mentioned before, parents will choose the low r to have a bigger portion. In the first month, even 5 g per day of carbohydrate may
be tried in children and it is beneficial. Please see complementary tables at the end of this chapter for the carbohydrate content of foods. MAD initiation and maintenance are summarized in Table 4-1.
1. Modified Atkins Diet (MAD) Protocol (2013) Fat/CHO Supplement/Fluid/extra●High-fat foods added liberally ●Carbohydrates: 10g/day for children, 15g/day for adolescents, 20g/day for adults (net carbohydrates counted = total carbohydrates minus fiber; no subtraction of sugar alcohols)
●Multivitamin and calcium supplement with vitamin D daily ● Clear carbohydratefluids encouraged●If possible, 400-calorie shthe first month
● Carbohydrate counting guides reviewed (from dietitians, books, Internet, apps)
● Avoid anticonvchanges ● Avoid low carbohydrate store-bought products
● Follow-up laboratory studies (including lipid profile) after 3 months
● Urine ketones checked weekly Food intake record months
●Then substitute regular meals, one per week
●A quicker titration off may be acceptable in some circumstances.
For MAD patient selection see chapter 6.
57
g/day, it should be decreased to 10 g/day month will improve seizure control.
The type of carbohydrate is not important, but as mentioned before, parents will choose the low r to have a bigger portion. In the first month, even 5 g per day of carbohydrate may
be tried in children and it is beneficial. Please see complementary tables at the end of this chapter for
Supplement/Fluid/extra ●Multivitamin and calcium supplement with vitamin D
● Clear carbohydrate-free fluids encouraged ●If possible, KetoCal®4:1
calorie shake daily) for the first month
● Avoid anticonvulsant
● Avoid low carbohydrate bought products
● Urine ketones checked
Food intake record at for 3
●A quicker titration off may eptable in some
circumstances.
Chapter Four
MAD prescription and writing the meal plan
One easy and practical way is food choice.(Neal, 2012) In this method, after calculating the diet, “One fat choice” is defined as 10 grams of fat and “one carbohydrate choice” is defined as 5 grams of carbohydrate. Free protein sources
· All meat and poultry including beef, pork, lamb, bacon, veal, ham, goose, Poussin, quail, pheasant.
· All fish including tuna (fresh or tinned in oil), salmon, sole, trout, sardines, herrings· Eggs in any forms of scrambled, fried, poached, soft and hard boiled.
Processed meats should be avoided as they contain carbohydrates such as flour or otherdiet ratio may vary from day to day based on the food from 1.5:1 to 2:1. Free fat sources Each of the following amounts contains 10 g of fat which is considered as one fat choice: · Butter: 3 teaspoons or 15 g · Olive oil or any vegetable oil: 10 ml (2 teaspoons or one tablespoon)· Margarine: 3 teaspoons · Cream 30%: 30 g or 6 teaspoons· Mayonnaise: 10 ml or one tablespoon
These two items have carbohydrate component that should be considered inl Carbohydrate sources One carbohydrate choice is defined as 5 g carbohydrate. Examples of 5 g carbohydrates choices are shown in Table4-2below. The foodscucumber, lettuce, raw spinach, mushroom
MAD prescription and writing the meal plan
One easy and practical way is food choice.(Neal, 2012) In this method, after calculating the diet, fat choice” is defined as 10 grams of fat and “one carbohydrate choice” is defined as 5 grams
All meat and poultry including beef, pork, lamb, bacon, veal, ham, venison, chickenpheasant.
All fish including tuna (fresh or tinned in oil), salmon, sole, trout, sardines, herringsEggs in any forms of scrambled, fried, poached, soft and hard boiled.
Processed meats should be avoided as they contain carbohydrates such as flour or otherday to day based on the food intake; however, a desirable diet ratio ranges
amounts contains 10 g of fat which is considered as one fat choice:
Olive oil or any vegetable oil: 10 ml (2 teaspoons or one tablespoon)
Cream 30%: 30 g or 6 teaspoons Mayonnaise: 10 ml or one tablespoon These two items have carbohydrate component that should be considered inl
carbohydrate choice is defined as 5 g carbohydrate. Examples of 5 g carbohydrates choices are below. The foods containing very low carbohydrate can be eaten freely
spinach, mushroom, mustard and cress, radish and rhubarb.
58
One easy and practical way is food choice.(Neal, 2012) In this method, after calculating the diet, fat choice” is defined as 10 grams of fat and “one carbohydrate choice” is defined as 5 grams
venison, chicken, turkey, duck,
All fish including tuna (fresh or tinned in oil), salmon, sole, trout, sardines, herrings
Processed meats should be avoided as they contain carbohydrates such as flour or other additives. The , a desirable diet ratio ranges
amounts contains 10 g of fat which is considered as one fat choice:
These two items have carbohydrate component that should be considered inlarger quantities.
carbohydrate choice is defined as 5 g carbohydrate. Examples of 5 g carbohydrates choices are can be eaten freely such as celery,
, mustard and cress, radish and rhubarb.
Chapter Four
Table 4-2. 5 g carbohydrate choicesApples (eating):½ medium-sized appleApricots (raw): 1 medium fruit Aubergine (cooked in oil): ½ medium aubergineAvocado (flesh): ½ medium avocadoBanana: ¼ average size banana Bean sprouts (raw): 4 tablespoonsBeetroot (boiled): 1 small (whole)Blackberries (raw): ×20 fruits Blueberries (raw): ×15 fruits Broccoli (cooked):4 tablespoons Brussel sprouts (boiled): 4 sproutsButternut squash (boiled): 2 tablespoonsCabbage/spring greens: 4 tablespoons (boiled or raw)Carrots (raw): 2 mediums Carrots (boiled): 2½ tablespoonsCauliflower (raw or boiled): 4 tablespoonsClementine/Satsuma: 1 fruit Zucchini (raw or cooked): 2 mediums French beans (cooked): 4 tablespoonsGrapefruit: ½ grapefruit Gherkins (pickled): 2 large, or 4 smallsLeeks (raw or cooked): 2 medium leeks (white part only)
Onions (spring): 5 medium spring onionsChopped onion: 2 tablespoons Oranges: ½ medium orange Pepper (red or yellow, raw or cooked): 5 ringsPlums: 1 fruit Pumpkin (boiled): 4 tablespoons Raspberries: ×20 Runner beans (boiled): 4 tablespoonsStrawberries: ×10 Tomato (tinned or raw): 3 tablespoons (tinned) or 1 mediumTurnip (boiled): 4 tablespoons
2. 5 g carbohydrate choices Name in Farsi (Persian)sized apple
oked in oil): ½ medium aubergine Avocado (flesh): ½ medium avocado
سیبلوآزرد
)درروغن شده سرخ آووکادو
Bean sprouts (raw): 4 tablespoons Beetroot (boiled): 1 small (whole)
led): 4 sprouts
Butternut squash (boiled): 2 tablespoons
موز)خام(جوانھ
چغندر پختھ)خام(توت سیاه )خام(بلوبری
بروکلی پختھپختھ لیکلم بروک
اسکواش پختھCabbage/spring greens: 4 tablespoons (boiled or raw)
poons Cauliflower (raw or boiled): 4 tablespoons
Zucchini (raw or cooked): 2 mediums
)خام یا پختھ(کلم )خام(ھویج پختھ ھویج
)خام یا پختھ(گل کلم کلمانتین
یا پختھ خام) خورشتی
French beans (cooked): 4 tablespoons
Gherkins (pickled): 2 large, or 4 smalls Leeks (raw or cooked): 2 medium leeks (white part only)
)پختھ(نخود فرنگی گریپ فروت خیارشور
)خام یا پختھ(تره فرنگی فقط قسمت سفید
Onions (spring): 5 medium spring onions
Pepper (red or yellow, raw or cooked): 5 rings
Runner beans (boiled): 4 tablespoons
پیازچھشده دپیازخور
پرتقال)زرد خام یا پختھلوآ
کدو پختھ تمشک)پختھ (زلوبیاه سب
Tomato (tinned or raw): 3 tablespoons (tinned) or 1 medium توت فرنگی
)کنسرو یا خام( گوجھ شلغم پختھ
59
Farsi (Persian)
سرخ(بادنجان
کلم بروک
گل کلم
خورشتی(کدوسبز
نخود فرنگی
تره فرنگی
زرد خام یا پختھ قرمز یا(فلفل
لوبیاه سب
گوجھ
Chapter Four
Here is an example of MAD diet prescription for
MAD for a child on 15 g carbohydrate per day9 years old boy 30 kg Calorie requirement: 70/kg/day = 2100 calorie
Calorie (food history): 1900 calorie Average Calorie requirement:2000 calorie/day
4 meals/day include approximately 18 fat choic/day (182 g fat=18 × 10 g)
Breakfast example
3 eggs 4 fat choice: -Two choices: Cream 30% -Two choices: Butter or olive oil -Half carbohydrate choice:2 tablespoons
املت یا پوره ی تخم مرغ
Lunch Red meat/ fish /ground beef/ chicken/t(60-70g) 5 fat choices: -3 choices: Butter or olive oil -2 choices: Mayonnaise (2 tablespoons)One carbohydrate choice: Tomato or eggplant
قلیھ ،کدو ،بادمجان ،آلو اسفناج ،آلو سیاه ،
Breakfast is the easy meal to skip carbohydrate based on the preference. But better to have a carbohydrate food in the morning sank. For lowered, and the fat component should be increased instead. That can be easily done by MCT oil. Change in Carbohydrate component
Here is an example of MAD diet prescription for a 9-year old boy.
MAD for a child on 15 g carbohydrate per day
Calorie requirement: 70/kg/day = 2100 calorie
Calorie (food history): 1900 calorie Average Calorie requirement:2000 calorie/day
Minimum Protein requirement 33 g/day + 42 g/day extra freely/day 15 g carbohydrate (fixed) distributed throughout a day Remain calorie: Fat 182 g (1640 calorie/day)
4 meals/day include approximately 18 fat choices /day
3 carbohydrate choices per day (15=5g ×3 or 2) carbohydrate choices and two half carbohydrate choices that equal
Dinner
Half carbohydrate choice:2 tablespoons broccoli
5 fat choices: -3fat choices butter or olive oilTwo fat choices: cream 30% -Minced meat/ chicken:60-70 g-One carbohydrate choices: Carrot, orCauliflower etc.
کوکو، کتلت سوپ، Snack
chicken/turkey
2 choices: Mayonnaise (2 tablespoons) Tomato or eggplant etc.
:انواع خورشت ھا
،کرفس ،قرمھ سبزیسبز لوبیا، ماھی
4 Fat choices: -Two choices: Cream 30% -Two choices: Olive oil Half carbohydrate choice: Strawberry (×5) apple (1/4 medium size) (After blending if the smoothie is too thick, water can be added, better to serve cold e.g. with ice) اسموتی
eal to skip carbohydrate based on the preference. But better to have a carbohydrate food in the morning sank. For increasing the ratio (and efficacy), protein should be
component should be increased instead. That can be easily done by in Carbohydrate component is usually the last alternative.
60
Minimum Protein requirement 33 g/day +
15 g carbohydrate (fixed) distributed throughout a
Remain calorie: Fat 182 g (1640 calorie/day)
3 carbohydrate choices per day ×3 or 2) carbohydrate choices and two half
equal 2.5 g
3fat choices butter or olive oil
70 g One carbohydrate choices: Carrot, or
Half carbohydrate choice: Strawberry (×5) apple
After blending if the smoothie is too thick, water can be added, better to serve cold e.g. with ice)
eal to skip carbohydrate based on the preference. But better to have a ratio (and efficacy), protein should be
component should be increased instead. That can be easily done by adding
Chapter Four
Diet process There are some things to be considered during the diet process:· For each patient, finding an adequate amount of fat to produce
if marginal efficacy or no/mild ketosis occurs, a higher fat intake will be required. One of the reasonsfor low ketone level is not enough fat and high protein intakes be rechecked. There are different ways other than food, to modify diet:
· Fat supplements such as ketogenic formulas, MCT oil, add Carnitine or Omega· Larger ketosis is especially important in the first month, during which urinary ketones should be
checked twice per week. After the first · Urinary ketosis usually starts at the end of the
achieved by the end of the first month. The Diet, ifA period of 2-month is required in order to evaluate diet efficacy. Although for all KDs, a 3period has been suggested.
· There is a new recommendation for higher fat intake in the first month by involving a 400KetoCal® 4:1 shake if available, or other highinstead. (Study by Kossoff et al, 2010). This higher ratio, close to 2:1, in the first month, will improve efficacy. Presumably, any other ketogenic supplements like MCT
· Another modification, not a recommendation, is calorie demonstrated a better response in some patients. (Kim et al. 2016)
Monitoring · Side effects such as acidosis, constipation, renal stones and
classic KD. As die is liberal, the child shouldn’t complain of hunger but if this occurs, one small snack consisting of fat and protein (with higher fat component) can be given.
· Monitoring includes checking ketosis (to BMI, weight changes, and growth parameters.
· For calorie reduction, the protein component should be cut off, but not fat. Increasing carbohydrates will depend on seizure control and patient tolerancconsidered in the first month.
For diet monitoring please also see Discontinuation Discontinuation includes slow tapering of the diet by increasing carbohydrate 10 g per day every 2weeks until ketosis is lost. When carbohydrate reaches 60 g per day, usually urinary ketones will be periodically negative. If seizure recurs, the diet should be returned to the last seizure this, switch from fat to big portions of profinal step is to commence switching to regular meals, one meal per week. The child will be off diet when all three meals become regular. High carbohydrate foods should be avoided until this step. The alternative way, if using the food choice method, is to slowly reduce fat choices by one or two choices daily, or every other day. At the same that will be 3-4 g per day. Protein intake can be adju
There are some things to be considered during the diet process: For each patient, finding an adequate amount of fat to produce ketosis is experimental: meaning that if marginal efficacy or no/mild ketosis occurs, a higher fat intake will be required. One of the reasonsfor low ketone level is not enough fat and high protein intakes instead. Therefore
ed. There are different ways other than food, to modify diet: Fat supplements such as ketogenic formulas, MCT oil, add Carnitine or OmegaLarger ketosis is especially important in the first month, during which urinary ketones should be
ked twice per week. After the first month, this could be spaced out. Urinary ketosis usually starts at the end of the first week, and medium to large levels will usually be achieved by the end of the first month. The Diet, if successful, will start workin
is required in order to evaluate diet efficacy. Although for all KDs, a 3
There is a new recommendation for higher fat intake in the first month by involving a 4004:1 shake if available, or other high-fat supplements such as whipping cream or oils
instead. (Study by Kossoff et al, 2010). This higher ratio, close to 2:1, in the first month, will improve efficacy. Presumably, any other ketogenic supplements like MCT may have similar effects. Another modification, not a recommendation, is calorie - restricted MAD (to 75%), which
response in some patients. (Kim et al. 2016)
Side effects such as acidosis, constipation, renal stones and weight loss are less common than in classic KD. As die is liberal, the child shouldn’t complain of hunger but if this occurs, one small snack consisting of fat and protein (with higher fat component) can be given. Monitoring includes checking ketosis (to ensure enough fat intake) and adjusting calories based on
growth parameters. For calorie reduction, the protein component should be cut off, but not fat. Increasing carbohydrates will depend on seizure control and patient tolerance. There is no rush to do this and may not be
For diet monitoring please also see Table 4-1 and chapter one.
Discontinuation includes slow tapering of the diet by increasing carbohydrate 10 g per day every 2weeks until ketosis is lost. When carbohydrate reaches 60 g per day, usually urinary ketones will be periodically negative. If seizure recurs, the diet should be returned to the last seizure this, switch from fat to big portions of proteins is recommended to continue for another 2 weeks. The final step is to commence switching to regular meals, one meal per week. The child will be off diet when all three meals become regular. High carbohydrate foods should be avoided until this step.
he alternative way, if using the food choice method, is to slowly reduce fat choices by one or two choices daily, or every other day. At the same time, carbohydrate decreases, for example,
4 g per day. Protein intake can be adjusted until achieving a normal diet.
61
ketosis is experimental: meaning that if marginal efficacy or no/mild ketosis occurs, a higher fat intake will be required. One of the reasons
instead. Therefore, food record should
Fat supplements such as ketogenic formulas, MCT oil, add Carnitine or Omega-3 fatty acids. Larger ketosis is especially important in the first month, during which urinary ketones should be
week, and medium to large levels will usually be successful, will start working within 2 to 4 weeks.
is required in order to evaluate diet efficacy. Although for all KDs, a 3-month
There is a new recommendation for higher fat intake in the first month by involving a 400-calorie fat supplements such as whipping cream or oils
instead. (Study by Kossoff et al, 2010). This higher ratio, close to 2:1, in the first month, will improve may have similar effects.
restricted MAD (to 75%), which
weight loss are less common than in classic KD. As die is liberal, the child shouldn’t complain of hunger but if this occurs, one small
and adjusting calories based on
For calorie reduction, the protein component should be cut off, but not fat. Increasing carbohydrates e. There is no rush to do this and may not be
Discontinuation includes slow tapering of the diet by increasing carbohydrate 10 g per day every 2 weeks until ketosis is lost. When carbohydrate reaches 60 g per day, usually urinary ketones will be periodically negative. If seizure recurs, the diet should be returned to the last seizure - free point. After
teins is recommended to continue for another 2 weeks. The final step is to commence switching to regular meals, one meal per week. The child will be off diet when all three meals become regular. High carbohydrate foods should be avoided until this step.
he alternative way, if using the food choice method, is to slowly reduce fat choices by one or two example, 1 g per meal
sted until achieving a normal diet.
Chapter Four
Low Glycemic Index diet (LGI diet)This diet was initially presented in Boston by Dr. E. Thiele and dietitian H. Pfeifer in 2002. Its aim was to make KD more tolerable with a liberal amount of caa glycemic index of less than 50.The glycemic index (GI) is a measure of how much a particular food will elevate the blood glucose compared to the same amount of glucose. This diet has the same efficacy as classic KD. The ratio of fat to carbohydrate and protein grams in this diet is 1:1 and with less fat compared to MAD and classic KD. Important tips about LGI · Treatment efficacy is not related to the level of ketones yet correlates with lower and steadier
blood glucose levels. · The weighting of foods isn’t required, and meals are based on the portion size.· Micronutrients, vitamins, and
vitamins such as B2, Vit C, andsee chapter 7
· For every food, the higher the acidity and fiber, the lower the GI. Moreover, adding some fat or protein to carbohydrates can lower its glycemic index because it slows down the GI motility and digestion process. Adding aci
· The method of cooking affects the GI, for example, the longer a food is cooked, the fasters its sugar digestion and absorption will be. (Higher GI)
· About the fruits, the ripper the fruit is, the highercompared to an unripe fruit containing more complex carbohydrates.
· Remember that total the total daily carbohydrate allowance includes dietary meaning that fiber is not subtracted out of a
Diet initiation The LGI diet can commence at the outpatient clinic along with the education sessions for families.A 3-5 day food intake record completed by parents will help to determine the calorie goal based oncurrent individual intake and habits. Disallowance nutritional requirements based on age and gender should be also considered in total calorie estimation. If the may occur. In case of inadequate calorie, metabolicmonitoring weight changes is important to adjust calorie, maintain adequate growth and avoid excess weight gain. · 60-70% of calories will be provided by fat and 20· Parents should be trained regarding the
and a list of low GI foods should be provided. (Complementary table C· For meal planning, each meal should have all three sources of fat,
with a gradual increase in the amount of protein (e.g. chicken, eggs, cheese, tofu, etc.) and fat (e.g. oil, butter, and mayonnaise).
· Fluid consumption should be encouraged· Carbohydrate amount is limited to 40
ones with a GI <50. Total dailypatient baseline carbohydrate intake can improve tolerability
Low Glycemic Index diet (LGI diet) This diet was initially presented in Boston by Dr. E. Thiele and dietitian H. Pfeifer in 2002. Its aim was to make KD more tolerable with a liberal amount of carbohydrate, by using carbohydrates with a glycemic index of less than 50.The glycemic index (GI) is a measure of how much a particular food will elevate the blood glucose compared to the same amount of glucose. This diet has the same
. The ratio of fat to carbohydrate and protein grams in this diet is 1:1 and with less fat compared to MAD and classic KD.
Treatment efficacy is not related to the level of ketones yet correlates with lower and steadier
of foods isn’t required, and meals are based on the portion size. minerals in LGI are higher than Classic or MAD and for some
and folate supplementation may not be necessar
For every food, the higher the acidity and fiber, the lower the GI. Moreover, adding some fat or protein to carbohydrates can lower its glycemic index because it slows down the GI motility
process. Adding acid, for example, a little of lemon juice lowers the GI.The method of cooking affects the GI, for example, the longer a food is cooked, the fasters its sugar digestion and absorption will be. (Higher GI) About the fruits, the ripper the fruit is, the higher the GI will be because it contains more sugar
unripe fruit containing more complex carbohydrates. the total daily carbohydrate allowance includes dietary
is not subtracted out of a patient's goal daily carbohydrate intake.
The LGI diet can commence at the outpatient clinic along with the education sessions for families.5 day food intake record completed by parents will help to determine the calorie goal based on
current individual intake and habits. Disallowance nutritional requirements based on age and gender should be also considered in total calorie estimation. If the daily caloric intake is high, weight gain may occur. In case of inadequate calorie, metabolic stress may increase seizure. Therefore, monitoring weight changes is important to adjust calorie, maintain adequate growth and avoid
70% of calories will be provided by fat and 20–30% from protein. (See chapter 7)trained regarding the elimination of high GI carbohydrates, exchange foods,
and a list of low GI foods should be provided. (Complementary table C-2). For meal planning, each meal should have all three sources of fat, protein, and
ual increase in the amount of protein (e.g. chicken, eggs, cheese, tofu, etc.) and fat (e.g. oil, butter, and mayonnaise). Fluid consumption should be encouraged Carbohydrate amount is limited to 40–60 g per day (10% of daily calories) and restricted to thones with a GI <50. Total daily carbohydrates also comprise fibers. Gradual tapering from
intake can improve tolerability
62
This diet was initially presented in Boston by Dr. E. Thiele and dietitian H. Pfeifer in 2002. Its aim rbohydrate, by using carbohydrates with
a glycemic index of less than 50.The glycemic index (GI) is a measure of how much a particular food will elevate the blood glucose compared to the same amount of glucose. This diet has the same
. The ratio of fat to carbohydrate and protein grams in this diet is 1:1 and with
Treatment efficacy is not related to the level of ketones yet correlates with lower and steadier
of foods isn’t required, and meals are based on the portion size. minerals in LGI are higher than Classic or MAD and for some
folate supplementation may not be necessary for all patients.
For every food, the higher the acidity and fiber, the lower the GI. Moreover, adding some fat or protein to carbohydrates can lower its glycemic index because it slows down the GI motility
d, for example, a little of lemon juice lowers the GI. The method of cooking affects the GI, for example, the longer a food is cooked, the fasters its
the GI will be because it contains more sugar
the total daily carbohydrate allowance includes dietary fiber in this diet patient's goal daily carbohydrate intake.
The LGI diet can commence at the outpatient clinic along with the education sessions for families. 5 day food intake record completed by parents will help to determine the calorie goal based on
current individual intake and habits. Disallowance nutritional requirements based on age and gender caloric intake is high, weight gain
stress may increase seizure. Therefore, monitoring weight changes is important to adjust calorie, maintain adequate growth and avoid
30% from protein. (See chapter 7) of high GI carbohydrates, exchange foods,
2). and carbohydrate,
ual increase in the amount of protein (e.g. chicken, eggs, cheese, tofu, etc.) and fat
60 g per day (10% of daily calories) and restricted to the carbohydrates also comprise fibers. Gradual tapering from
Chapter Four
LGI Prescription Table 4-3 shows stepwise calculation in the carbohydrate should be even in all meals and snakes combined with protein and fat to maintain a low glycemic index of all meals.
Diet calculation steps Total calorie requirements/day Based on RDA and diet history 1)A total calorie from fat/day
2)fat g/day
CHO is 40-60 g/day
3)Total calorie form CHO/day
4) A total calorie from protein/day
5)A total protein g/day
For meal planning, food exchange lists published by the American Dietetic Association is easy to useIn these lists, foods are divided into 7groups, and each group comprises of the foods that are alike in terms of carbohydrate fat, protein and calorie yet with the portion. For example, a very lean protein list has 35 calories and 1 gram of fat per serving; and one serving (portion) equals to two white eggs, or 28 g (1 oz) of turkey breast or chicken breast, skin removed, or 28 g (1 oz) of fat free cheese, etc. This also hfoods substitutions. For more information please see complementary of this chapter.
shows stepwise calculation in the LGI diet. Remember like KD, the distribution of carbohydrate should be even in all meals and snakes combined with protein and fat to maintain a low glycemic index of all meals.
Table 4-3. Calculation for LGI diet
Total calorie/day×60%
Total calorie from fat/9kcal/g
(40-60)×4=160-240 kcal/day
(Total calorie from the fat/day)-(total calorie from CHO/day)
Total calorie from protein/day/4kcal/g
exchange lists published by the American Diabetes Association and the is easy to use. (Table C-6 to 10)
are divided into 7groups, and each group comprises of the foods that are alike in terms of carbohydrate fat, protein and calorie yet with the different amount in one serving or
lean protein list has 35 calories and 1 gram of fat per serving; and one serving (portion) equals to two white eggs, or 28 g (1 oz) of turkey breast or chicken breast, skin removed, or 28 g (1 oz) of fat free cheese, etc. This also helps the families to have a foods substitutions. For more information please see complementary Tables (C
63
er like KD, the distribution of carbohydrate should be even in all meals and snakes combined with protein and fat to maintain a
(total calorie from CHO/day)
Diabetes Association and the
are divided into 7groups, and each group comprises of the foods that are alike amount in one serving or
lean protein list has 35 calories and 1 gram of fat per serving; and one serving (portion) equals to two white eggs, or 28 g (1 oz) of turkey breast or chicken breast, skin
elps the families to have a variety of s (C-6 to 10) at the end
Chapter Four
CASE: Diet calculation and meal plan for 1400 kcal/day Total Calorie requirements 1400 This is calculated based on the average food intake record and daily energy requirements. (see table7)
Final plan 1200-1400 calorie per day 83-93 g fat/day 80-100 g protein/day 40-60 g CHO/day
Breakfast 1Lunch -1 slice bread 28 g (10×10cm, size of a palm)-One teaspoon butter or olive oil - Two eggs - 28 g (1 oz) mozzarella cheese Egg and mozzarella can be mixed with olive oil or butter to make an omelet
Dinner -Cooked lentil (½cup) -90 g Fish or chicken (grilled or cooked)-1 tablespoon olive oil -Lettuce or mushroom -1 tablespoon mayonnaise
سالاد انواع ،Snack 1 - Cheese chips 40 g (cheddar cheese) - Fruit ½ apple or orange (small size), ½ fresh peach (medium size)
Cheese chips: Preheat the oven to 200°C (400°F). Place the cheese slices on a parchment paper that covers the baking plate. A small number of spices can be added on top of the10 minutes until they get crunchy.
CASE: Diet calculation and meal plan for 1400 kcal/day
food intake 60% calorie from fat: 1400 × 60%= 840840/9=93 g fat per day
Total CHO per day: 40-60 g per day160-240 calorie/day
Calorie from protein: 1400-(840+160400 kcal/day Protein:320-400/4: 80-100 g per dayMeal plan: 3 meals and 2 snacks Meal Snack: Fat 20-23g10-11g Protein 20-24g 10-CHO 10-16g 5-8 g Calorie 300-350 150
1 slice bread 28 g (10×10cm, size of a palm)
Egg and mozzarella can be mixed with olive oil or
-4 tablespoons rice or 1 slice breador ½ cup pasta -1/4 cup raw vegetables (cook after specifying the amount) -60 g beef (ground or stew), lamb, tuna i-14g (½oz)cheddar cheese -1.5 teaspoon olive oil
90 g Fish or chicken (grilled or cooked)
،خوراک عدس
اسفناج ،کرفس ،قرمھ سبزی کوکو مرغ ،کوکو ھویج کلم ،
مرغ سرخ کرده با برنج یا نان انواع ماکارونی یا پاستا
Snack 2
(small size), ½ fresh - Apple slices (1/3 apple) with - 1.5 tablespoon peanut butter
: Preheat the oven to 200°C (400°F). Place the cheese slices on a parchment paper that covers the can be added on top of the cheese slices. Bake them in the oven for about 8
64
60% calorie from fat: 1400 × 60%= 840
60 g per day
(840+160-240)= 320-
100 g per day
11g -١2 g 8 g
150-175
rice or 1 slice bread
1/4 cup raw vegetables (cook after specifying the
60 g beef (ground or stew), lamb, tuna in oil
قرمھ سبزی: خورشت ھا
،کوکو سبزی: کوکو،دلمھ مثل برگ کاھو
مرغ سرخ کرده با برنج یا نانانواع ماکارونی یا پاستا
: Preheat the oven to 200°C (400°F). Place the cheese slices on a parchment paper that covers the cheese slices. Bake them in the oven for about 8–
Chapter Four
Diet process Foods with high GI such as watermelon, bagels, and potatoes will be substituted by apples, cucumbers, oatmeal, peanuts, peas, corn carrots, kidney beans and none, or only the least processed carbohydrates, such as stone-ground whole wheat, pumpernickel A slow switch from milk to cream will also reduce the carbohydrate amThe carbohydrate amount should be determined individually, as some patients may use more than 60 g per day of carbohydrates and still show seizure improvement.One important point is to distribute carbohydrate throughout the day with eacwith a protein and fat sources. This helps to decrease the carbohydrate intake should be less than 5 Monitoring LGI is reported to have fewer side effects than classic KD. Minor sideconstipation, and vomiting. · Lethargy may be a symptom of acidosis, relative dehydration or elevated BUN. High protein
intake may cause elevated BUN and decline in renal function in patients with the renal disease, but not in patients with normal kidney function. Thus, renal disease is one contraindication to an LGI diet.
· Dehydration may also raise BUN level and should be avoided.· Metabolic acidosis may occur because fat is metabolized instead of carbohydrates, as th
source. This may worsen with concurrent use of topiramate or zonisamide. The child may develop lethargy, nausea, vomiting, and headache. Metabolic acidosis can be easily detected by a simple blood test and treated by an alkylation agent. (oral p
· Supplementation is also necessary as with other KD methods.· Ketones levels may exist in the blood and urine of the patients, but at a lower level or more likely
to be undetectable. Regular checking of ketones is not necessary.
If seizure continues, diet adjusting is based on carbohydrate types and amount. Two considerations are recommended: first ensuring that the patient takes proper low GI carbohydrates, second reducing total carbohydrate intake.Constipation should be treated promptly. Please also see chapter 5 for side effects.As with other KD types, compliance is important for seizure control, carbohydrate free medications should be used, supplementation is required, and enough fluid consumption is essential. During Illness This should be managed like other KDs. (see NPO situations in chapter 5). If the child is acidotic, potassium citrate with higher doses may be required. Diet discontinuation Slow tapering over a month is suggested by a As with other KDs, simple carbohydrates should be avoided until the final stage.
Foods with high GI such as watermelon, bagels, and potatoes will be substituted by apples, oatmeal, peanuts, peas, corn carrots, kidney beans and none, or only the least processed
ground whole wheat, pumpernickel bread, and nonA slow switch from milk to cream will also reduce the carbohydrate amount of the diet. The carbohydrate amount should be determined individually, as some patients may use more than 60 g per day of carbohydrates and still show seizure improvement. One important point is to distribute carbohydrate throughout the day with each meal/snack together with a protein and fat sources. This helps to decrease the overall GI of each meal. Daily variation of carbohydrate intake should be less than 5-10 g.
LGI is reported to have fewer side effects than classic KD. Minor side effects include lethargy,
Lethargy may be a symptom of acidosis, relative dehydration or elevated BUN. High protein intake may cause elevated BUN and decline in renal function in patients with the
ut not in patients with normal kidney function. Thus, renal disease is one
Dehydration may also raise BUN level and should be avoided. Metabolic acidosis may occur because fat is metabolized instead of carbohydrates, as thsource. This may worsen with concurrent use of topiramate or zonisamide. The child may develop lethargy, nausea, vomiting, and headache. Metabolic acidosis can be easily detected by a simple blood test and treated by an alkylation agent. (oral potassium citrate) Supplementation is also necessary as with other KD methods. Ketones levels may exist in the blood and urine of the patients, but at a lower level or more likely to be undetectable. Regular checking of ketones is not necessary.
continues, diet adjusting is based on carbohydrate types and amount. Two considerations are recommended: first ensuring that the patient takes proper low GI carbohydrates, second reducing total carbohydrate intake.
romptly. Please also see chapter 5 for side effects.other KD types, compliance is important for seizure control, carbohydrate free medications
should be used, supplementation is required, and enough fluid consumption is essential.
This should be managed like other KDs. (see NPO situations in chapter 5). If the child is acidotic, potassium citrate with higher doses may be required.
Slow tapering over a month is suggested by a gradualincrease of the carbohydrateother KDs, simple carbohydrates should be avoided until the final stage.
65
Foods with high GI such as watermelon, bagels, and potatoes will be substituted by apples, oatmeal, peanuts, peas, corn carrots, kidney beans and none, or only the least processed
non-starchy vegetables. ount of the diet.
The carbohydrate amount should be determined individually, as some patients may use more than 60
h meal/snack together GI of each meal. Daily variation of
effects include lethargy,
Lethargy may be a symptom of acidosis, relative dehydration or elevated BUN. High protein intake may cause elevated BUN and decline in renal function in patients with the pre-existing
ut not in patients with normal kidney function. Thus, renal disease is one
Metabolic acidosis may occur because fat is metabolized instead of carbohydrates, as the primary source. This may worsen with concurrent use of topiramate or zonisamide. The child may develop lethargy, nausea, vomiting, and headache. Metabolic acidosis can be easily detected by a
Ketones levels may exist in the blood and urine of the patients, but at a lower level or more likely
continues, diet adjusting is based on carbohydrate types and amount. Two stepwise considerations are recommended: first ensuring that the patient takes proper low GI carbohydrates,
romptly. Please also see chapter 5 for side effects. other KD types, compliance is important for seizure control, carbohydrate free medications
should be used, supplementation is required, and enough fluid consumption is essential.
This should be managed like other KDs. (see NPO situations in chapter 5). If the child is acidotic,
of the carbohydrate content by 5-10 g. other KDs, simple carbohydrates should be avoided until the final stage.
Chapter Four
MKD This is a combination of 4 KDs comprising MCT, low glycemic index carbohydrates etc., proposed by Fitzsimmons and Sewell in 2015. The diet is flexible without calorie calculation, with 30carbohydrate per day and structured food selection and portion sizes. It appears to be the most adjusted KD based on personal needs and preference although its efficacy will be determfuture.
Complementary tables
C- 1. Examples of high carbohydrate foodsApple Pie (1 slice) پای سیبChocolate (dark, 28 g) 17.9 شکلات تلخDoughnut (glazed) 26.6 دوناتHard candy, jelly bean (28 g) 25
Macaroni and Cheese (1 cup) 40 ماکارونی و پنیرTortilla Chips 11.3 چیپس تورتیلا
C- 2. Carbohydrate gram counterExamples of foods with carbohydrate content per graFood
MILK, CREAM, AND BUTTER Butter (1 tsp) Heavy cream30% (2 tbsp) Milk (2%, 1 cup) (whole, 1 cup) Yogurt(plain, 1 cup) (from whole milk) NUTS AND SEEDS Almonds (whole, 2 tbsp) Hazelnuts (whole, 2 tbsp) Peanuts (2 tbsp) Pistachio Nuts (2 tbsp)
Pumpkin Seeds (2 tbsp) Walnuts (2 tbsp) Sesame seeds (1tbsp) CONDIMENTS, SAUCES, AND HERBSFresh Herbs (all types, 1 tbsp
This is a combination of 4 KDs comprising MCT, low glycemic index carbohydrates etc., proposed 2015. The diet is flexible without calorie calculation, with 30
per day and structured food selection and portion sizes. It appears to be the most adjusted KD based on personal needs and preference although its efficacy will be determ
1. Examples of high carbohydrate foods
61 Cheeseburger with Bun (115 g) ھمبرگر با پنیر
17.9 Croissant شیرینی کروسانت
Doughnut (glazed) 26.6 Hot Dog with Bun ھات داگ با نان ساندویج
25 Onion Rings پیازحلقھ ای سوخاری
40 Pizza (1 slice) پیتزا
11.3 White sugar(28 g) شکر سفید
2. Carbohydrate gram counter Examples of foods with carbohydrate content per gram (Dr. Atkins’ new diet revolution, 2002)
CHO (g)
Food
CHEESE 0 Cheddar (shredded, 2 tbsp)
0,8 Cream Cheese (2 tbsp)
11,7 11,4
Feta (crumbled, 2 tbsp) Persian feta (25 g)
11,4 Mozzarella (shredded, 2 tbsp) (whole milk)
Parmesan (shredded, 2 tbsp) GRAINS, BREADS,AND PASTA
3,6 Bagel (22 oz) 2,8 Biscuit (2 oz) 3,4 Corn Flakes (1 cup) 4,7 Pancake
(homemade, 15cm / 6" diameter)3,1 Pasta (cooked, ½cup) 1,7 Pasta (whole wheat, cooked,½1,4 Rice (white, cooked, 2 cups)
HERBS Tortilla (corn) 0,1 White Bread (1 slice) 14.9
66
This is a combination of 4 KDs comprising MCT, low glycemic index carbohydrates etc., proposed 2015. The diet is flexible without calorie calculation, with 30-40 g of
per day and structured food selection and portion sizes. It appears to be the most adjusted KD based on personal needs and preference although its efficacy will be determined in the
Cheeseburger with Bun (115 g) 33
Croissant 27
og with Bun 24
33
24
) 28
m (Dr. Atkins’ new diet revolution, 2002) Food CHO
(g)
0,2 0,8 0,8 0,5
a (shredded, 2 tbsp) 0,3
0,3 PASTA
38 27,6 24,2
Pancake (homemade, 15cm / 6" diameter)
21,8
19,8 ½ cup) 18,6
) 22,3 12,1 14,9
Chapter Four
Garlic (1 clove) Ginger Root (sliced, 1 tbsp) Honey (1 tsp) Jam (1 tsp) Ketchup (1 tbsp) Olives (green, 5) Pickle Relish (1 tbsp) Basil(1tbs) Fresh Herbs (all types, 1tbsp) BEANS Chickpea (½cup) Lentils (½cup) Navy (½cup) Red Kidney (½cup) Soybeans (½cup) VEGETABLES Artichoke (1) Asparagus (6 spears) Beans (green, ½cup) Broccoli (½cup) Cabbage (green, ½cup) Carrot (medium) Cauliflower (6 florets) Celery (1 stalk) Corn (½cup) Cucumber (½small) Eggplant (½cup) Endive (½cup) Leek (1) Lettuce (1 cup) Mushrooms (½cup) Okra (4 oz) Onion (1) Onions (green, 1/4 cup) Peas (green, ½cup) Peppers (green, ½cup) Potato (sweet, 1) Potato (white, ½cup) Pumpkin (½cup) Spinach (raw, 1 cup) Squash (butternut, ½cup) Tomato (1 small) Zucchini (1 small) Basil(1tbs) ریحان▪without sugar
1 Whole Grain Bread (1 slice) 0,9 MEAT, POULTRY, AND FISH 5,8 Meat, Poultry, Fish (6 oz) 4,6 Eggs (1) 4,2 Beef Salami (3 oz) 2.4 2,5 OILS AND DRESSINGS 5,4 Mayonnaise (1 tsp) 0,9 Olive Oil, Canola, Safflower, etc(1tsp) 0,1 Salad Dressing (Caesar, 2 tbsp)
Italian (2 tbsp) 22,5 Ranch (2 tbsp) 19,9 Salsa (red, 1 tbsp) 23,9 Soy Sauce (1 tbsp) 19,8 9,9
FRUITS 13,4 Apple (1 medium) 3,8 Applesauce (1/4cup) 4,9 Apricots (fresh, 1) 3,9 Avocado (1) 1,9 Banana (1 small) 7,3 Blueberries (1/4 cup) 4,4 Cantaloupe (1/4 cup) 1,5 Cherries (1/4 cup) 16 Fig (fresh, 1) 2,5 Grapes (1/4 cup) 3,3 Honeydew (1/4 cup) 1,8 Juice, lemon (1 tbsp) 12,6 Juice, orange (½cup) 1,3 Juice, tomato (½cup) 1,4 Kiwifruit (1) 7,5 Orange (1) 9,5 Mango (1/4 cup) 1,8 Peach (1 medium) 9,9 Pear (1 medium) 4,8 Pineapple (1/4 cup) 22,4 Plum (1) 15,4 Raspberries (1/4, cup) 9,9 Strawberries (1/4 cup) 1,1 Tangerine (1) 10,8 Watermelon (1/4 cup) 4,2 5,7 0,9
67
11,8
0-trace Eggs (1) 0,6
2,4
0,1 (1tsp) 0
0,6 3
1,4 0,8 1
21 6,9
3,9 Avocado (1) 14,9
23,7 5,1 3,3 4,8
Fig (fresh, 1) 9,6 7,1 3,9 1,3
13,4 5,1
11,3 16,3
7 10,9 25,1 4,8
Plum (1) 8,6 3,6 2,7
Tangerine (1) 7,8 2,8
Chapter Four
C-3. Glycemic index of different foodsFood Breads and pastry Croissant شیرینی کروسانتMuffin apple▪ مافین سیبPita bread نان پیتاMuffin Corn مافین ذرتDairy product Whole milk
شیر کامل Chocolate milk
یرشکاکائو Grains Barley جو بارلیRice, Basmati برنج باسماتیFruits Apple سیبCherries گیلاسMango انبھOrange پرتقالPineapple اناناسPlum لوآ
Prunes لوی سیاهآ
Strawberries توت فرنگیBeans Black-eyed لوبیا چشم بلبلcooked bean in sauce
لوبیا خوراک Kidney beans لوبیا قرمزSplit Peas لپھNavy bean لوبیا سفید
3. Glycemic index of different foods GI Food 67
Hamburger bun نان ھمبرگر
38
Blueberry muffin مافین بلوبری
57 Pumpernickel نان جو سیاه سبوس دار
49 Digestive دایجستیو
33 Yogurt (plain, Low fatm
ماست42 Soy Milk
شیرسویا 25 Rice, brown
برنج قھوه ای58 Rye bread
نان چاودار آرد درشت 38 Banana
موز22 Coconut
رگیلنا 61 Kiwi
کیوی43 Peach
ھلو66 Grape
انگور24 Pears
گلابی29 Resins
کشمش40
50 Baked beans
ختھلوبیای پ 56 Chickpeas
نخود27 Lentils
عدس32 Soy beans
سویا31 Pinto Beans
لوبیا چیتی
68
Food GI 61
59
41
59
Yogurt (plain, Low fatm sugar) 33
44
55
62
52
45
47
28
46
33
64
48
33
29
16
45
Chapter Four
Vegetables Beet چغندر
64
Carrots ھویج
71
Cauliflower گل کلم
15
Lettuce کاھو
10
Mushroom قارچ
10
Potato, baked سیب زمینی پختھ
85
Sweet potato شیرین سیب زمینی
54
Tomatoes گوجھ
15
Snack foods/candy/Foods Hummus (حمص)
6
Coconut Milk 41 popcorn پف فیل
55
Ice cream یبستن
61
Spaghetti, boiled 5 minutes× بکش شدهآماکارونی
37
Pizza cheese اپنیر پیتز
60
64 Broccoli
کلم بروکلی71 Cabbage
برگ کلم15 Eggplant
جانمباد 10 Fig
انجیر10 Onion
پیاز85 Green peas
نخود سبز54 Sweet corn
ذرت شیرین15
Jam ( )مربا
41 Instant Noodles 55 Peanut
بادام زمینی61 Macaroni and cheese
ماکارونی و پنیر37 Spaghetti
اسپاگتی60 Walnut
گردو
69
15
10
15
61
10
Green peas 48
55
51
47 15
64
41
15
Chapter Four
C-4 Examples of Foods with High GI (70Baked potato سیب زمینی پختھ
85
Cookie (کلوچھ ) 9278 کراکرDonut دونات
76
French Fries سیب زمینی سرخ شده
75
Rice cookie کیک برنجی
87
Rice, instant برنج، فوری
91
Pretzel چوب شور
83
Wild Carrot ( زردک(وحشی ھویج
97
C-5. Example of Foods with the lowVegetable Broccoli 10 Cabbage 15 Cauliflower 15 Carrot (raw) 16 Carrot (boiled) 41 Eggplant 15 Frozen green peas 39 Frozen sweet corn 47 Green beans 15 Lettuce 10 Mushroom 10 Red paper 10 Tomato 15 Sweet potato 48 Yam 35
4 Examples of Foods with High GI (70-100) 85 Corn Flakes
کورن فلکس84
92 Waffles 76 78 Jellybeans 80 76 Mashed potato
پوره سیب زمینی70
75 Rice, white برنج سفید
87
87 Pumpkin کدو تنبل
75
91 Watermelon ھندوانھ
72
83 White bread نان سفید
70
97
low GI (≤55) Fruits Snacks/foods
10 Cherries 22 Hummus 15 Grapefruit 25 Nutella 15 Plums 24 Milk Chocolate16 Peach 28 Peanut 41 Apple 34 Walnut 15 Pears 41 Cashew 39 Dried Apricots 43 Oatmeal crackers 47 Grape 43 Plain yogurt 15 Kiwi 47 Spaghetti 10 Oranges 40 Milk 10 Strawberry 40 Wholegrain bread 10 Prune 29 Corn chips
70
Snacks/foods
Hummus 6 33
Milk Chocolate 42 Peanut 13 Walnut 15 Cashew 25 Oatmeal crackers 55 Plain yogurt 33
32 lk 31
n bread 46 Corn chips 42
Chapter Four
Food exchange list tables
C-6 Starches Each Serving = 15 g carbohydrate, 3 g protein, 0Food Serving Breads and flours Biscuit (2 1/2 inches across) Bread (white, whole1 slice (1 oz) Wheat, pumpernickel, rye) Iranian bread (Sangak, Lavash, barbari) BreadReduced-calorie 2 slices (1 ½ Flour, cornmeal, wheat germ Grains Barley, cooked Pasta, cooked Quinoa, cooked Rice, white or brown cooked Tabbouleh prepared Starchy Vegetables Corn
Peas, green Pumpkin, canned, no sugar added Yam, sweet potato Snacks Popcorn, popped Pretzels Beans, Peas,and Lentils (Cooked) Each Serving = 15 g carbohydrate, 7 g protein, 0Baked beans Beans (black, garbanzo, kidney , lima, navy, pinto, white) Refried beans, canned
Each Serving = 15 g carbohydrate, 3 g protein, 0-1 g fat, 80 calories Serving Food
1 Bun (hotdog or hamburger)
Pancake
1 slice (1oz)
2 slices (1 ½ oz) Pita bread
3Tbsp dry Naan Indian Bread Cereals
1/3 cup Bran
1/3 cup Oatmeal cooked
1/3 cup Shredded wheat, plain 1/3 cup Sugar-coated cereals
½cup Potato
½ cup French fried (oven baked)
½cup Boiled (all kinds) 1 cup Baked with skin
1/2 cup Mashed, with milk
3 cups Snack chips, regular 3/4 oz
Each Serving = 15 g carbohydrate, 7 g protein, 0-3 g fat, 125 calories
1/3 cup Lentils (brown, green, yellow)ack, garbanzo, kidney ½ cup Peas (black-eyed, split)
½ cup
71
Food Serving
½ bun (1 oz)
Pancake 1/4-inch-thick 1 (4 inches across)
½ pocket (6 inches across)
Naan Indian Bread 1/4 (8 by 2 inches)
½cup
½cup
Shredded wheat, plain ½cup coated cereals ½cup
1 cup(2 oz)
Boiled (all kinds) ½ cup Baked with skin 1/4 large (3 0z)
Mashed, with milk ½ cup
9-13 (3/4 oz)
Lentils (brown, green, yellow) ½ cup eyed, split) ½ cup
Chapter Four
C-7. Fruits (The weight includes peel, skin, rinds,Each Serving = 15 g carbohydrate, 0 g protein, 0 g fat, 60 caloriesFruit Serving Apple, unpeeled, small 1 (4 oz) Apples, dried 4 ringsApricots, fresh 4 wholeApricots, dried 8 halved Dates 3 Dried fruits 2 TbspFigs 2 mediumGrapefruit, large 1/2 (11 oz)Grapes, small 17 (3 oz)Honeydew 1 slice or 1 cup cubed (10 oz)Kiwi 1 Raisins 2 TbspRaspberries 1 cup Strawberries 1 1/4 cup whole berriesFruit Juice Apple juice 1/2 cup Grape juice 1/3 cup Orange juice 1/2 cupPrune juice 1/3 cup
C-8. Milk and yogurt Milk Carbohydrate(g)
Skim 12 Low-fat 12 Whole 12 Serving size: one cup for all kind of milk and 8
7. Fruits (The weight includes peel, skin, rinds,and seeds) Each Serving = 15 g carbohydrate, 0 g protein, 0 g fat, 60 calories
Serving Fruit 1 (4 oz) Banana
4 rings Blueberries 4 whole Cantaloupe
halved Cherries, fresh Mango
2 Tbsp Nectarine, small 2 medium Orange, small 1/2 (11 oz) Peach, fresh, medium17 (3 oz) Pear, fresh, large 1 slice or 1 cup cubed (10 oz) Pineapple, fresh Plums, fresh, small
2 Tbsp Prunes 1 cup Tangerines, small 1 1/4 cup whole berries Watermelon1
1/2 cup Fruit juice blends, 1/3 cup Grapefruit juice 1/2 cup Pineapple juice 1/3 cup
Carbohydrate(g) Protein(g) Fat (g)
12 8 traces 8 5 8 8
cup for all kind of milk and 8 oz for yogurt
72
Serving 1/2 large
3/4 cup 1 cup cubed (11 oz)
, fresh 12 (3 oz) 1/2 cup or 1/2 small
1 (5 oz) 1
, medium 1 Pear, fresh, large 1/2
le, fresh 3/4 cup Plums, fresh, small 2 (5 oz)
3 angerines, small 2(8 oz)
1/4 cup cubes
Fruit juice blends, 100% juice1/3 cup 1/2 cup 1/2 cup
Calorie
90 5 120 8 150
Chapter Four
C-9. Non-starchy Vegetables Serving size = 1/2 cup cooked Each Serving = 5 g CHO, 2 g protein, 0 g fat, 25 Cal 1 cup raw 1/2 cup vegetable juice Artichoke and Artichoke hearts Asparagus Baby corn Beans (green, wax, Italian) Bean sprouts Beets Borscht Broccoli Brussels sprouts Cabbage - all types Carrots Cauliflower Creeley Coleslaw, no dressing Turnips cucumber water chestnuts Egg plant Zucchini Greens (collard, kale, mustard, turnip)Green onions or scallions Leeks Mixed vegetables (without corn or peas) Mushrooms
starchy Vegetables h Serving = 5 g CHO, 2 g protein, 0 g fat, 25 Cal
Okra Onions
Oriental
Tomato
s (collard, kale, mustard, turnip)
Leeks
73
h Serving = 5 g CHO, 2 g protein, 0 g fat, 25 Cal
Okra Onions Oriental radish or daikon
Pea pods (snow peas) Peppers (all varieties)
Radishes Sauerkraut
Soybean sprouts Spinach
Summer squash Sugar snap peas Tomato (fresh or canned,sauce)
Tomato/vegetable juice
Chapter Four
C-10. Meats and Meat SubstitutesLean selection (کم چرب) Each Serving = 0 g carbohydrate, 7 g protein, 0Beef 1 oz Cheeses 1 oz tuna, canned in water or oil(low fat:0-3g) Egg whites 2 Fish 1 oz Beans, Peas, and Lentils (Cooked) Each Serving = 15 g carbohydrate, 7 g protein, 0 Medium Fat Selections (چربی متوسط) Most of the meat products fall into this categoryEach Serving = 0 g carbohydrate, 7 g protein, 4Cheese, reduced-fat cheeses (4-7g) Mozzarella Feta cheese Beef (most of the beefproducts, ground beef, short ribs, tongue Egg Sausage1 oz Veal, cutlet 4-7 grams of fat per oz High-Fat Selections Each Serving = 0 g carbohydrate, 7 g protein, 8 or more g fat, 100 caloriesCheese, all regular cheese Including cheddar and swiss Processed meats (≥8 g fat per oz) bologna, pastrami, hard salami Hot dog: beef, pork, turkey, chicken or combination (10 per pound sized package)Peanut butter Contains unsaturated fat
meat portions are: 2 ounces meat (2 meat exchanges) = 1 small chicken leg or thigh, 1/2 cup cottage cheese or tuna; 3 ounces meat (3 meat exchanges) = 1 medium pork chop, 1 small hamburger, 1/2 of a whole chicken breast, 1 unbreacooked meat, about the size of a deck of cards.
10. Meats and Meat Substitutes
Each Serving = 0 g carbohydrate, 7 g protein, 0-3 g fat, 45 calories 1 oz Poultry, skinless
, canned in water or oil
Shrimp Hot dog with 0-3 g fat per oz
rving = 15 g carbohydrate, 7 g protein, 0-3 g fat, 125 calories
this category Each Serving = 0 g carbohydrate, 7 g protein, 4-7 g fat, 75 calories
1 oz Fish, fried
1 oz Lamb: ground, rib roast 1 oz Chicken with skin 1 oz Fried chicken, ground turkey
1 Ricotta cheese 1 oz
Each Serving = 0 g carbohydrate, 7 g protein, 8 or more g fat, 100 calories 1 oz
1 oz
combination (10 per pound sized package) 1 tbsp
meat portions are: 2 ounces meat (2 meat exchanges) = 1 small chicken leg or thigh, 1/2 cup cottage cheese or tuna; 3 ounces meat (3 meat exchanges) = 1 medium pork chop, 1 small hamburger, 1/2 of a whole chicken breast, 1 unbreaded fish fillet, cooked meat, about the size of a deck of cards.
74
1 oz 1 oz
1 oz 3 g fat per oz 1
1oz
Lamb: ground, rib roast 1 oz 1 oz
n, ground turkey 1oz
1/4 cup, 2 oz
1 oz
meat portions are: 2 ounces meat (2 meat exchanges) = 1 small chicken leg or thigh, 1/2 cup cottage cheese or tuna; 3 ounces meat (3 meat exchanges) = 1 medium pork
ded fish fillet,
Chapter Four
C-11. Fats Monounsaturated Fats Each Serving = 0 g carbohydrate, 0 g protein, 5 g fat, 45 caloriesAvocado 2TNuts: Almonds 6 nutsBrazil 2 nutsCashews 6 nutsFilberts (hazelnuts) 5 nutsMacadamia 3 nutsMixed (50% peanuts) 6 nutsPeanuts 0 nuts Pecans 4 halves Pistachios 16 nuPolyunsaturated Fats Margarine 1tsp Mayonnaise: Nuts Reduced fat 1Tbsp Regular 1 tsp Oils: corn, cottonseed, flaxseed, grape seed,safflower, soybean, sunflower Seeds: flaxseed, pumpkin, sunflower, sesameSalad dressing: Reduced-fat (check the label for carbohydrate count) Regular Saturated Fats Butter 1 tsp Cream Heavy (30%) 1Tbsp Light 1 ½ Tbsp Oil: coconut, palm, palm kernel 1tsp
Each Serving = 0 g carbohydrate, 0 g protein, 5 g fat, 45 calories 2Tbsp (1 oz)
Oils: Olive oil, canola oil, peanut oil
6 nuts Olives 2 nuts Black (ripe) 6 nuts Green (stuffed) 5 nuts 3 nuts 6 nuts 0 nuts 4 halves 16 nuts
1tsp
1Tbsp Pignolia (pine nuts) 1 tsp Walnuts
corn, cottonseed, flaxseed, grape seed, 1 Tsp
flaxseed, pumpkin, sunflower, sesame 1 Tbsp fat 2Tbsp
for carbohydrate count) 1 Tbsp
1 tsp Coconut, shredded Cream cheese
1Tbsp Regular 1 ½ Tbsp Reduced fat(light)
: coconut, palm, palm kernel 1tsp
75
oil, canola oil, peanut oil 1tsp
8 large 10 large
1 Tbsp 4 halves
2Tbsp
1Tbsp(1/2 oz) 1 ½ Tbsp(3/4 oz)
References
· A decade of the modified Atkins diet (2003Epilepsy &Behavior. 29: 437–442, 2013
· Atkins Robert C.Dr. Atkins’ new diet revolution. Avon Books/HarperCollins, New York, 2002.· Choose Your Foods: Exchange Lists for Diabetes
Association, 2008 · How Do I Use the Modified Atkins Diet? In KETOGENIC DIETS Treatments for Epilepsy and Other
Disorders. Kossoff E.H., Freeman JM, Turner Z, Rubenstein JE, Demos medical publishing, FIFTNew York, 2016
· Kim JA, Yoon J-R, Lee EJ et al. Efficacy of the classic ketogenic and the modified Atkins diets in refractory childhood epilepsy. Epilepsia.57:51–
· Kossoff EH, Zupec-Kania BA, Amark PE et al. Optimal clinical management ofketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50:3042009
· Kossoff EH, Rowley H, Sinha SR, Vining EPG.A prospective study of the modified Atkins diet for intractable epilepsy in adults. Epilepsy. 2008 49:316
· Kossoff EH, Zupec-Kania BA, Rho J.M., Ketogenic Diets: An Update for Child Neurologists, Journal of Child Neurology, 24, 979-988, 2009
· Kossoff EH, Dorward LJ, Turner Z, RD1, and Pyzik PL. Prospective Study of the Modified AtkiCombination With a Ketogenic Liquid Supplement During the Initial Month.
· Kumada T, Miyajima T, Hiejima I. Modified Atkins Diet and Low Glycemic Index Treatment for Medication-Resistant Epilepsy: Current Trends in Ketogenic Diet. J Neurol Neuroph
· Lambert B. Switching or discontinuing dietary treatment, In Neal E. Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy. First Edition. 2012.
· Muzykewicz DA, Lyczkowski DA, Memon N. et al. index treatment in pediatric epilepsy. Epilepsia, 50:1118
· Pfeifer HH. The Low Glycaemic Index Treatment In Neal E. Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy, First Edition.Wiley& Sons,
· Vitaflo dietitians, Neal E. Guidelines; practical implementation of the ketogenic diet for dietary management of epilepsy and neurometabolic disease.2016. PART 1, 2016
Atkins diet (2003–2013): Results, insights, and future directions. Kossoff H. E. 442, 2013
Atkins Robert C.Dr. Atkins’ new diet revolution. Avon Books/HarperCollins, New York, 2002.Choose Your Foods: Exchange Lists for Diabetes. American Dietetic Association and American Diabetes
How Do I Use the Modified Atkins Diet? In KETOGENIC DIETS Treatments for Epilepsy and Other Disorders. Kossoff E.H., Freeman JM, Turner Z, Rubenstein JE, Demos medical publishing, FIFT
R, Lee EJ et al. Efficacy of the classic ketogenic and the modified Atkins diets in refractory –58, 2016
Kania BA, Amark PE et al. Optimal clinical management ofketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50:304
ossoff EH, Rowley H, Sinha SR, Vining EPG.A prospective study of the modified Atkins diet for ts. Epilepsy. 2008 49:316–319
Kania BA, Rho J.M., Ketogenic Diets: An Update for Child Neurologists, Journal of
Kossoff EH, Dorward LJ, Turner Z, RD1, and Pyzik PL. Prospective Study of the Modified AtkiCombination With a Ketogenic Liquid Supplement During the Initial Month. Kumada T, Miyajima T, Hiejima I. Modified Atkins Diet and Low Glycemic Index Treatment for
Resistant Epilepsy: Current Trends in Ketogenic Diet. J Neurol Neurophysiol. 2013Lambert B. Switching or discontinuing dietary treatment, In Neal E. Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy. First Edition. 2012. Muzykewicz DA, Lyczkowski DA, Memon N. et al. Efficacy, safety, and tolerability of the low glycemic index treatment in pediatric epilepsy. Epilepsia, 50:1118–1126, 2009 Pfeifer HH. The Low Glycaemic Index Treatment In Neal E. Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy, First Edition.Wiley& Sons, 2012
flo dietitians, Neal E. Guidelines; practical implementation of the ketogenic diet for dietary management of epilepsy and neurometabolic disease.2016. PART 1, 2016
76
2013): Results, insights, and future directions. Kossoff H. E.
Atkins Robert C.Dr. Atkins’ new diet revolution. Avon Books/HarperCollins, New York, 2002. . American Dietetic Association and American Diabetes
How Do I Use the Modified Atkins Diet? In KETOGENIC DIETS Treatments for Epilepsy and Other Disorders. Kossoff E.H., Freeman JM, Turner Z, Rubenstein JE, Demos medical publishing, FIFTH Edition.,
R, Lee EJ et al. Efficacy of the classic ketogenic and the modified Atkins diets in refractory
Kania BA, Amark PE et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50:304–317,
ossoff EH, Rowley H, Sinha SR, Vining EPG.A prospective study of the modified Atkins diet for
Kania BA, Rho J.M., Ketogenic Diets: An Update for Child Neurologists, Journal of
Kossoff EH, Dorward LJ, Turner Z, RD1, and Pyzik PL. Prospective Study of the Modified Atkins Diet in
Kumada T, Miyajima T, Hiejima I. Modified Atkins Diet and Low Glycemic Index Treatment for ysiol. 2013
Lambert B. Switching or discontinuing dietary treatment, In Neal E. Dietary Treatment of Epilepsy: Practical
lity of the low glycemic
Pfeifer HH. The Low Glycaemic Index Treatment In Neal E. Dietary Treatment of Epilepsy: Practical
flo dietitians, Neal E. Guidelines; practical implementation of the ketogenic diet for dietary management
Chapter Five
Managing side effects
“The KD is an effective treatment but it is not entirely benign. It must be prescribed thoughtfully, implemented carefully, and monitored closely.”Nordli, D. (2002).
Side effect classifications Side effects are divided into early (in the All Side effects are summarized in in chapter 3. Late complications are discussed here.
Table 5-1. Short-term, long-term Immediate, Short term Acidosis Constipation Exacerbation of GERD* Excessive ketosis Fatigue Food refusal Hypoglycemia Increased seizure frequency Vomiting
*GERD: gastroesophageal reflux disease
Managing side effects
fective treatment but it is not entirely benign. It must be prescribed thoughtfully, implemented carefully, and monitored closely.”
Side effects are divided into early (in the first month) and late complications (after the All Side effects are summarized in Table 5-1.For early side effects please see diet initiation section in chapter 3. Late complications are discussed here.
term, and rare side effects of KD Rare (case report) Long-term erm
Basal ganglia changesBruising EsophagitisFanconi's renal tubular acidosisGallstonesHepatitis HyperuricemiaHypocalcemiaHypomagnesemiaHyponatremiaHypoproteinemiaIncreased illnessesLate-onset egg allergyLipoid pneumoniaPancreatitisSelenium deficiency subsequent cardiomyopathy, prolonged QT interval, and sudden death)
Bone fractures Decreased bone mineral density Dyslipidemia Kidney stones Poor linear growth Secondary carnitine deficiency Vitamin D deficiency Weight loss (or insufficient weight gain)
reflux disease
77
fective treatment but it is not entirely benign. It must be prescribed
tions (after the first month). .For early side effects please see diet initiation section
e (case report)
Basal ganglia changes
Esophagitis Fanconi's renal tubular acidosis Gallstones
Hyperuricemia Hypocalcemia Hypomagnesemia Hyponatremia Hypoproteinemia Increased illnesses
onset egg allergy Lipoid pneumonia Pancreatitis Selenium deficiency (including subsequent cardiomyopathy, prolonged QT interval, and sudden
Chapter Five
Table 5-2Reported side effects among classic KD patients of our center are summarized Side Effects inNo of patients 302 Mean age 4.8 years old
Early side effects/no
Hypoglycemia 13Nausea/vomiting 18Fever 4Epistaxis GI Bleeding 2UTI 1
*Mofid Children'sHospital, Tehran, IranMeantime on the diet had been at least 12 months in the nonresponsive group except for urgent di*Phenobarbital and phenytoin toxic level Managing side effects 1) GI Gastrointestinal symptoms are common and may occur in 75% of the patients. The ketone bodies may cause anorexia/nausea, vomiting or feeding refusal especially when their levels rapidly rise at diet initiation. Constipation, which is one of the most common reported side effects, can be prevented by higher fiber vegetables like adding some prunes (or any nonTreatment includes using more oil (MCT) than saturated fat, carbohydrate free laxatives such as polyethylene glycol. Impaction may need serial enemas are other options. Constipatiocommon in classic KD than other diet types due to GI reflux is treated by H2 blockers or proton pump inhibitors. Fat can lower the esophageal tone and delay gastric emptying. It may present as abdominal discomfort or back arching in younger children. Children may also complain ofheartburn, frequent vomiting, or hiccupping. Liver enzyme and function tests are recommended every 3liver and gallstone disease are reported in KD. Pancreatitis may occur in KD. Infants are particularly at risk because of relative deficiency in pancreatic lipase activity. Amylase and lipase level are important in case of abdominal pain. Abdominal pain may have different etiologies in abdominal examination.
Reported side effects among classic KD patients of our center are summarized Effects in classic KD patients of our center *
Early side effects/no Diet discontinuation
Late side effects/no
Hypoglycemia 13 None High TG 73Nausea/vomiting 18 2 High Chol 59Fever 4 1 Low appetite 2Epistaxis 1 None Gastritis 1GI Bleeding 2 2 AEDs toxicity * UTI 1 None Cardiomyopathy 1
Renal stone 1 Pneumonia 1
, Iran had been at least 12 months in the improved group and 3 months in the
group except for urgent discontinuation in 5 patients. *Phenobarbital and phenytoin toxic level
Gastrointestinal symptoms are common and may occur in 75% of the patients. The ketone bodies may cause anorexia/nausea, vomiting or feeding refusal especially when their
, which is one of the most common reported side effects, can be prevented by higher
ing some prunes (or any non-absorbable fibers), and increased fluid intake. Treatment includes using more oil (MCT) than saturated fat, carbohydrate free laxatives such as polyethylene glycol. Impaction may need serial enemas are other options. Constipatiocommon in classic KD than other diet types due to high-fat content and low fiber in the
blockers or proton pump inhibitors. Fat can lower the esophageal tone and
discomfort or back arching in younger children. Children may also , frequent vomiting, or hiccupping.
Liver enzyme and function tests are recommended every 3-6 months to monitor liver integrity. Fatty eported in KD.
Pancreatitis may occur in KD. Infants are particularly at risk because of relative deficiency in pancreatic lipase activity. Amylase and lipase level are important in case of abdominal pain. Abdominal pain may have different etiologies in KD patients and requires a complete
78
Reported side effects among classic KD patients of our center are summarized
Late side effects/no Diet discontinuation
High TG 73 None High Chol 59 None Low appetite 2 None Gastritis 1 None
1 1 Cardiomyopathy 1 1 Renal stone 1 None Pneumonia 1 None
group and 3 months in the
Gastrointestinal symptoms are common and may occur in 75% of the patients. The ketone bodies may cause anorexia/nausea, vomiting or feeding refusal especially when their
, which is one of the most common reported side effects, can be prevented by higher absorbable fibers), and increased fluid intake.
Treatment includes using more oil (MCT) than saturated fat, carbohydrate free laxatives such as polyethylene glycol. Impaction may need serial enemas are other options. Constipation is more
content and low fiber in the diet. blockers or proton pump inhibitors. Fat can lower the esophageal tone and
discomfort or back arching in younger children. Children may also
6 months to monitor liver integrity. Fatty
Pancreatitis may occur in KD. Infants are particularly at risk because of relative deficiency in pancreatic lipase activity. Amylase and lipase level are important in case of abdominal pain.
complete history and
Chapter Five
2) Metabolic
Hyperuricemia and acidosis have been reported in KD.Bicarbonate level should be monitored and supplementation in symptomatic cases presenting wvomiting, or lethargy is recommended. Carnitine deficiency This is more common in younger agesvalproic acid, and poor nutritional status. It may be asymptomatic or present with excessive fatigue, generalized muscle weakness, or systemic issues such as hepatitis and cardiomyopathy. Hyperlipidemia Hyperlipidemia is common and can happen in up to 60% of the patients at any time after beginning the diet. A lipid profile is recommended as a the first year. Classic KD mainly includes saturated fats and can lead to high triglycerides and cholesterol, LDL, VLDL and decrease in anti-atherogenicarachidonic acids to docohexaeonateespecially in MAD and is less common in LGI, and MCT KD.There are different modifications for hyperlipidemia: 1) Decreasing the cholesterol and saturated fats of the diet; e.olive oil, nuts, and seeds. Don’t forget to consider carbohydrate component in nuts, of course. (Table 5.3) 2) Adding medium-chain triglyceride oil, and carnitine.3) Changing protein intake to lower saturated fat sources;whole eggs. Usually,eggs and other proteins with high saturated fats are a common component of the diet. 4) Supplementation with Omega 36 to omega3 ratios) or adding oils that are rich in omega 3 such as Flaxseed, canola, soybean oil or actual fish including sardines, herring, different types of fat in oils, meats, the diet or modify it based on the fat types to lower cholesterol and saturated fatomega 3 fatty acids. If these modifications don’t correct hyperlipidemia, lowering the diet ratio is recommended. There is no report on using Statins in KD children. Usually, a normal lipid profile can be achieved by these nutritional modifications and there is no need for diet discontinuation.
have been reported in KD. Bicarbonate level should be monitored and supplementation in symptomatic cases presenting wvomiting, or lethargy is recommended.
This is more common in younger ages and may be caused by prolonged use of AEDs especially valproic acid, and poor nutritional status. It may be asymptomatic or present with excessive
generalized muscle weakness, or systemic issues such as hepatitis and cardiomyopathy.
Hyperlipidemia is common and can happen in up to 60% of the patients at any time after beginning the diet. A lipid profile is recommended as a baseline and monitored every 3 months in
Classic KD mainly includes saturated fats and can lead to high triglycerides and cholesterol, LDL, atherogenic-HDL. High poly unsaturated fatty acids,
docohexaeonate are also reported. This may also occur in other KD types especially in MAD and is less common in LGI, and MCT KD. There are different modifications for hyperlipidemia: 1) Decreasing the cholesterol and saturated fats of the diet; e.g. reduce the
seeds. Don’t forget to consider carbohydrate component in nuts, of course.
chain triglyceride oil, and carnitine. 3) Changing protein intake to lower saturated fat sources; for example, white eggs instead of
eggs and other proteins with high saturated fats are a common component of
4) Supplementation with Omega 3-Fatty acids (fish oils) 1-2 g four times/day (that reduces omega ) or adding oils that are rich in omega 3 such as Flaxseed, canola, soybean oil
or actual fish including sardines, herring, tuna, and salmon. Please also see Table 5.3different types of fat in oils, meats, seeds, and nuts. This is very helpful when you want to design the diet or modify it based on the fat types to lower cholesterol and saturated fat
If these modifications don’t correct hyperlipidemia, lowering the diet ratio is recommended. There tatins in KD children. Usually, a normal lipid profile can be achieved by
these nutritional modifications and there is no need for diet discontinuation.
79
Bicarbonate level should be monitored and supplementation in symptomatic cases presenting with
and may be caused by prolonged use of AEDs especially valproic acid, and poor nutritional status. It may be asymptomatic or present with excessive
generalized muscle weakness, or systemic issues such as hepatitis and cardiomyopathy.
Hyperlipidemia is common and can happen in up to 60% of the patients at any time after and monitored every 3 months in
Classic KD mainly includes saturated fats and can lead to high triglycerides and cholesterol, LDL, acids, a higher ratio of
also reported. This may also occur in other KD types
g. reduce the butter and use more seeds. Don’t forget to consider carbohydrate component in nuts, of course.
for example, white eggs instead of eggs and other proteins with high saturated fats are a common component of
2 g four times/day (that reduces omega ) or adding oils that are rich in omega 3 such as Flaxseed, canola, soybean oil
Table 5.3 that shows when you want to design
the diet or modify it based on the fat types to lower cholesterol and saturated fat or provide more
If these modifications don’t correct hyperlipidemia, lowering the diet ratio is recommended. There tatins in KD children. Usually, a normal lipid profile can be achieved by
Chapter Five
® Dried, not sweetened. There is no cholesterol in these nuts.
Table 5-3. Cholesterol, and different types of fat Nut/seed no in 1 oz
Protein
Almond 22 Brazil nuts 7 Cashews 17 Coconut® Flax seeds Many Hazelnut 19 Hemp seeds (hulled) Many Macadamias 11 Peanuts 28 Pecans(halves) 19 Pine nuts 153 Pistachio 46 Pumpkin seeds 142 Sesame seeds Many Sunflower seeds 300 Walnuts (halves) 14
Dried, not sweetened. There is no cholesterol in these nuts.
3. Cholesterol, and different types of fat in foods Protein
(g) Fat (g)
Total Saturated Mono Poly
6 14 1 9 3.5 4 19 5 7 7 4 13 3 8 2 2 18 16 0.77 0.2
5 12 1 2 8 4 17 2 13 2 8.4 13.5 1.3 1.5 10.6 2 22 3 17 <1 7 14 2 7 4 3 20 2 12 6 7 14 2 5 6
6 13 2 7 4 7 13 2 4 6 5 14 2 5 6 6 14 2 3 9 4 18 2 3 13
80
Net CHO(g)
3 1.3 8.4
2 0.5 2.3
1 1.5 3.8 1.1 2.7 5.8 2.2 2.6 3.7
2
Chapter Five
Fat/oil Type of Fat MonounsaturatedAlmond Oil Avocado Oil Butter Canola Oil Coconut Oil 6% Corn Oil Cottonseed Oil Grape Seed Oil Hazelnut Oil Macadamia Nut Oil 83% Margarine (Hard) Margarine (Soft) Mustard Oil Olive Oil Palm Oil Palm Kernel Oil Peanut Oil Rice Bran Oil Safflower Oil Sesame Oil Soybean Oil Sunflower Oil Walnut Oil Wheat Germ Oil
Monounsaturated Polyunsaturated 73% 19% 70% 10% 30% 4% 62% 32%
6% - Lowest 2% - Lowest (tie) 25% 62% 26% 50% 17% 71% 76% 14%
83% - Highest 3% 14% 16% 47% 33% 76% 23% 77% 9% 38% 10% 12% 2% - Lowest (tie) 49% 33% 47% 33% 13% 77% - Highest 40% 46% 24% 61% 20% 69% 19% 67% 30% 50%
81
Saturated 8% 20% 6% 6%
92% - Highest 13% 24% 12% 10% 14% 80% 20%
1% - Lowest 14% 52% 86% 18% 20% 10% 14% 15% 11% 14% 20%
Chapter Five
Cholesterol/saturated fat in 100 g of different foodsFood Portion Cholesterol (mg)Saturated fat Total fat
Dairy products Milk not fat 1 cup Milk low fat 1 cup Milk Whole 1 cupYogurt not fat 1 cupYogurt whole 1 cupCheddar cheese Fat/oil Butter Margarine Vegetable oil Meat/protein Egg Pinto beans ½ cupBeef ground lean 3.5 ozChicken no skin Chicken liver Turkey no skin Fish Salmon 3.5 ozTuna in water Lamb 3.5 ozShrimp
3) Higher chances of infection Pneumonia, sepsis and nonspecific febrile illnesses are reported. 4) Renal calculi Renal stones can occur in 10% of individuals with KD. They are more uric acids stones than calcium oxalate. This risk increases with dehydration or cois less chance of kidney stones in MCT, LGI, and MCT.Oral citrates or Polycitra-K (carbohydratepreventing stone formation. Diet discontinuation is not required, but early diagnosis and monitoring are crucial, with a urine analysis and urine two approaches regarding oral citrates; some centers use it for all KD patients as prophylaxis, while others reserve it for children with elevated Ca/ 5) Growth Decreased linear growth has been reported in KD,common in infants and younger children. Older children usually grow almost normally. Management includes a stepwise approach: The first step is checking diet calculation/administr
100 g of different foods Saturated fat Total fat
1 cup 4 0 1 cup 10 2 1 cup 33 5 1 cup 10 0 1 cup 29 5
1oz 30 6
1tsp 11 3 1tsp 0 3 1tsp 0 5
1 212 2
½ cup 0 0 3.5 oz 78 7
3 oz 55 0.6 4 oz 390 2 4 oz 76 0.5
3.5 oz 63 2
3 oz 26 0 3.5 oz 106 6
3 oz 161 0
Pneumonia, sepsis and nonspecific febrile illnesses are reported.
es can occur in 10% of individuals with KD. They are more uric acids stones than calcium oxalate. This risk increases with dehydration or concurrent use of Topiramate or Zonisamide. There is less chance of kidney stones in MCT, LGI, and MCT.
carbohydrate-free products) and sufficient fluid intake are helpful in preventing stone formation. Diet discontinuation is not required, but early diagnosis and monitoring
crucial, with a urine analysis and urine calcium-creatinine ratio in each followtwo approaches regarding oral citrates; some centers use it for all KD patients as prophylaxis, while others reserve it for children with elevated Ca/Cr ratio.
Decreased linear growth has been reported in KD, and growth follow up is mandatory. This is more common in infants and younger children. Older children usually grow almost normally. Management
calculation/administration/supplementation pitfalls.
82
0 3 8 0 7 9 4 4
1-2 5 1
18 2.3 5.5 2
12 0.7 14 0.5
es can occur in 10% of individuals with KD. They are more uric acids stones than calcium opiramate or Zonisamide. There
products) and sufficient fluid intake are helpful in preventing stone formation. Diet discontinuation is not required, but early diagnosis and monitoring
follow-up visit. There are two approaches regarding oral citrates; some centers use it for all KD patients as prophylaxis, while
and growth follow up is mandatory. This is more common in infants and younger children. Older children usually grow almost normally. Management
ls.
Chapter Five
A second step would involve calorie utilization issues e.g. testing for acid to rule out any underlying disorder such as mild metabolic conditions that may be missed in the pre-assessment period, or fecal fat or stool elastase for absorption problems. After this, increase/maximize calorie and protein content and follow up the result. Albumin and total protein levels will also show if the dietarythe ratio is suggested. An endocrinology consultation and IGFHowever, there is no data on growth hormone treatment in KD patients.In case of any undesired weight changes, increase or decrease, calorie intake shouadjusted accordingly. 6) Specific vitamin and/or mineral deficiencies Hypocalcemia in 2% and hypomagnesemia in 5% of childrendeficiency or other deficiencies may happen especially if supplementationadministered by caregivers. Vit. D level is better to be monitored every 6 months. Extra doses of multivitamins and minerals may be required if levels are still low with usual supplementation. (See chapter 7) 7) Cardiac Problems This includes cardiomyopathy and prolonged QT. The Etiology is unknown, but in some cases may associate with selenium deficiency or regular cardiac evaluation such as echocardiography. A blood sewith additional supplementation if necessary. 8) Bone health AEDs can increase the risk of rickets and osteoporosis as so many children who are about to get KD already have poor bone health. This makes Vit. D supplemeimportant. Ambulance and exercise should be encouraged especially in children who are unable to walk by using mobility aids. Osteoporosis takes more time to develop, and different mechanisms other than Vit. D defisuch as chronic acidosis and low IGFbone mass in these children, regular CMP*, Mg, Phosphate level every 3 to 6 monthsevaluated , Vit. D level monitoring every 6 months, and a once a year is recommended. For children with decreased bone mineral content, maximized calcium and Vit. D supplementations are recommended and exercise strongly encouraged. Even helpful in non-ambulatory children. *Comprehensive Metabolic Panel: measures kidney and liver function, electrolyte levels includes Glucose (fasting or non-fasting), Sodium (Na), Potassium (K), Chloride (Cl), Carbon dioxide (bicarbonate) (CO2), BUN (blood urea nitrogen), Creatinine BUN/creatinine ratio, Calcium (Ca), Magnesium (Mg), Protein (total), Albumin, Bilirubin (direct or total) Alkaline phosphatase (ALP), Aspartateaminotransferase (AST), Alanine
A second step would involve calorie utilization issues e.g. testing for acylcarnitineacid to rule out any underlying disorder such as mild metabolic conditions that may be missed in the
or fecal fat or stool elastase for absorption problems. After this, increase/maximize calorie and protein content and follow up the result. Albumin and total
dietary protein is sufficient. If the growth issue continuesthe ratio is suggested. An endocrinology consultation and IGF-1, GH level can be considered. However, there is no data on growth hormone treatment in KD patients. In case of any undesired weight changes, increase or decrease, calorie intake shou
6) Specific vitamin and/or mineral deficiencies Hypocalcemia in 2% and hypomagnesemia in 5% of children were reported. Selenium or deficiency or other deficiencies may happen especially if supplementation is not properly suggested or administered by caregivers. Vit. D level is better to be monitored every 6 months. Extra doses of multivitamins and minerals may be required if levels are still low with usual supplementation. (See
This includes cardiomyopathy and prolonged QT. The Etiology is unknown, but in some cases may associate with selenium deficiency or hypocarnitinemia. There is no current recommendation for regular cardiac evaluation such as echocardiography. A blood selenium check is recommended, along with additional supplementation if necessary.
AEDs can increase the risk of rickets and osteoporosis as so many children who are about to get KD already have poor bone health. This makes Vit. D supplementation (400 ID daily)
Ambulance and exercise should be encouraged especially in children who are unable to walk by using
Osteoporosis takes more time to develop, and different mechanisms other than Vit. D defisuch as chronic acidosis and low IGF-1, may also be involved. To ensure bone health and normal bone mass in these children, regular CMP*, Mg, Phosphate level every 3 to 6 months
, Vit. D level monitoring every 6 months, and a dual-energy X-ray absorptiometry (DEXA)
For children with decreased bone mineral content, maximized calcium and Vit. D supplementations are recommended and exercise strongly encouraged. Even weight-bearing exercise using standers
*Comprehensive Metabolic Panel: measures kidney and liver function, electrolyte levels includes fasting), Sodium (Na), Potassium (K), Chloride (Cl), Carbon dioxide
ood urea nitrogen), Creatinine BUN/creatinine ratio, Calcium (Ca), Magnesium (Mg), Protein (total), Albumin, Bilirubin (direct or total) Alkaline phosphatase (ALP),
(AST), Alanine aminotransferase (ALT), Glomerular filtration r
83
acylcarnitine& urinary organic acid to rule out any underlying disorder such as mild metabolic conditions that may be missed in the
After this, increase/maximize calorie and protein content and follow up the result. Albumin and total issue continues, lowering
1, GH level can be considered.
In case of any undesired weight changes, increase or decrease, calorie intake should be reviewed and
reported. Selenium or Vit. D is not properly suggested or
administered by caregivers. Vit. D level is better to be monitored every 6 months. Extra doses of multivitamins and minerals may be required if levels are still low with usual supplementation. (See
This includes cardiomyopathy and prolonged QT. The Etiology is unknown, but in some cases may . There is no current recommendation for
lenium check is recommended, along
AEDs can increase the risk of rickets and osteoporosis as so many children who are about to get KD would be even more
Ambulance and exercise should be encouraged especially in children who are unable to walk by using
Osteoporosis takes more time to develop, and different mechanisms other than Vit. D deficiency, 1, may also be involved. To ensure bone health and normal
bone mass in these children, regular CMP*, Mg, Phosphate level every 3 to 6 months should be ray absorptiometry (DEXA)
For children with decreased bone mineral content, maximized calcium and Vit. D supplementations exercise using standers is
*Comprehensive Metabolic Panel: measures kidney and liver function, electrolyte levels includes fasting), Sodium (Na), Potassium (K), Chloride (Cl), Carbon dioxide
ood urea nitrogen), Creatinine BUN/creatinine ratio, Calcium (Ca), Magnesium (Mg), Protein (total), Albumin, Bilirubin (direct or total) Alkaline phosphatase (ALP),
(ALT), Glomerular filtration rate (GFR)
Chapter Five 9) Long-term complications (>2 years on KD) There is a higher risk of bone fractures, kidney stones, and decreased growth. The recommendation for long-term KD patients is periodic screening for bone health and cardiovaAs with any other therapeutic modalities, diet continuation; benefits and side effects should be periodically weighed for each child, to decide if diet should continue or not. Patients should be carefully monitored for developing complicatimanaged by diet modifications.
term complications (>2 years on KD) risk of bone fractures, kidney stones, and decreased growth. The recommendation
KD patients is periodic screening for bone health and cardiovascular system. As with any other therapeutic modalities, diet continuation; benefits and side effects should be periodically weighed for each child, to decide if diet should continue or not. Patients should be carefully monitored for developing complications as many complications can be prevented or
84
risk of bone fractures, kidney stones, and decreased growth. The recommendation scular system.
As with any other therapeutic modalities, diet continuation; benefits and side effects should be periodically weighed for each child, to decide if diet should continue or not. Patients should be
can be prevented or
References · Bergqvist, A. G. C. (2012). Long
Research, 100(3), 261–6. · Dhamija, R., Eckert, S., &Wirrell, E. (2013). Ketogenic diet. The Canadian Journal of Neurological
Sciences. Le Journal CanadienDes Sciences Neurologiques, 40(2), 158· Kossoff, E. H., Zupec-Kania, B. A., Amark, P. E.,
Blackford, R., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study Group. Epilepsia, 50(2), 304–17.
· Kossoff, E. H., Zupec-Kania, B. A., & Rneurologists. Journal of Child Neurology, 24(8), 979
· Lee, P. R., & Kossoff, E. H. (2011). Dietary treatments for epilepsy: management guidelines for the general practitioner. Epilepsy & Behavior:
· Nordli, D. (2002). The ketogenic diet: uses and abuses. Neurology, 58(12 Suppl 7), S21· USDA National Nutrient Database for Standard Reference· Zupec-Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet Therapie
Symposium, March 2015, Manhattan Beach, CA, USA
Bergqvist, A. G. C. (2012). Long-term monitoring of the ketogenic diet: Do’s and Don’ts. Epilepsy
Wirrell, E. (2013). Ketogenic diet. The Canadian Journal of Neurological Sciences. Le Journal CanadienDes Sciences Neurologiques, 40(2), 158–67.
Kania, B. A., Amark, P. E., Ballaban-Gil, K. R., Christina Bergqvist, A. G., ., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of
children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study 17.
Kania, B. A., & Rho, J. M. (2009). Ketogenic diets: an update for child neurologists. Journal of Child Neurology, 24(8), 979–988. Lee, P. R., & Kossoff, E. H. (2011). Dietary treatments for epilepsy: management guidelines for the general practitioner. Epilepsy & Behavior: E&B, 21(2), 115–21. Nordli, D. (2002). The ketogenic diet: uses and abuses. Neurology, 58(12 Suppl 7), S21USDA National Nutrient Database for Standard Reference
Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet TherapieSymposium, March 2015, Manhattan Beach, CA, USA
85
term monitoring of the ketogenic diet: Do’s and Don’ts. Epilepsy
Wirrell, E. (2013). Ketogenic diet. The Canadian Journal of Neurological
Gil, K. R., Christina Bergqvist, A. G., ., … International Ketogenic Diet Study Group. (2009). Optimal clinical management of
children receiving the ketogenic diet: recommendations of the International Ketogenic Diet Study
ho, J. M. (2009). Ketogenic diets: an update for child
Lee, P. R., & Kossoff, E. H. (2011). Dietary treatments for epilepsy: management guidelines for the
Nordli, D. (2002). The ketogenic diet: uses and abuses. Neurology, 58(12 Suppl 7), S21-4.
Kania BA, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet Therapies
Chapter Six
How to choose diet type for patients
Currently, there are no specific demographics or particular criteria to predict who is more likely to respond to KD, or which type of KD is more appropriate. The selection of diet type is currently performed on an individual basis, and factors such nutritional habits, severity, type of patient selection decreases the dropoutthe diet principle is the same in almost all KDs, the preparation can affect child tolerance and family compliance. (See
*First month in children, adolescents and adults (In adults higher carbohydrate 20J 8 g on a 4:1 / 16 g on a 3:1 / 30 g on a 2:1 / 40There are some researches showing particular mutations may respond well to KD while others wiFor instance, SCN1A or STXBP1 are reported to have a dramatic response rate compared to CDKL5 with a poor response. More researches may provide a chance for a morefuture. Classic KD candidates
Classic KD can be used in individuals from infancy through adulthood. Proper candidates:1) First treatment option in GLUT1 deficiency or pyruvate dehydrogenase deficiency (PDHD). 2) Early in the treatment course: Certain epilepsy syndromes which are refractory are shown to be
more responsive to classic KD; for example, Myoclonic Astatic epilepsy (MAE) and Dravet Syndrome.
3) Infantile spam if first-line4) Status epilepticus, FIR
improvement on classic KD5) Infants with MAE and partial epilepsy with migrating foci.6) Infancy (under the age of 2 years7) LGS with or without VNS8) Some conditions such as tube9) In families needing structure and planned meals
Table 6-1. KD methods with different preparation and management KD Medical supervision Yes Supplementation Yes Measuring foods Weighted
Meal plan Yes Allowed CHO (g) In 1000 Calorie
8-16 gJ
Calorie control Yes Diet initiation HospitalOverall design Entire food
eaten at Calculated by dietitian
How to choose diet type for patients
Currently, there are no specific demographics or particular criteria to predict who is more likely to KD, or which type of KD is more appropriate. The selection of diet type is currently
performed on an individual basis, and factors such as the age of the child, family circumstances, nutritional habits, severity, type of epilepsy and whether or not a rapid response is required. Careful
dropout rate and increased efficacy and durability of diet. Although the diet principle is the same in almost all KDs, the degree of liberation and method of meal
olerance and family compliance. (See Table 6-1 below)
th in children, adolescents and adults (In adults higher carbohydrate 20-30 g may be considered)8 g on a 4:1 / 16 g on a 3:1 / 30 g on a 2:1 / 40–60 g on a 1:1
There are some researches showing particular mutations may respond well to KD while others wiCN1A or STXBP1 are reported to have a dramatic response rate compared to CDKL5
with a poor response. More researches may provide a chance for a more
n individuals from infancy through adulthood. Proper candidates:First treatment option in GLUT1 deficiency or pyruvate dehydrogenase deficiency (PDHD). Early in the treatment course: Certain epilepsy syndromes which are refractory are shown to be
sponsive to classic KD; for example, Myoclonic Astatic epilepsy (MAE) and Dravet
line medications fail. tatus epilepticus, FIRES (Febrile Illness Related Epilepsy S
improvement on classic KD ith MAE and partial epilepsy with migrating foci.
Infancy (under the age of 2 years, KCD is recommended) LGS with or without VNS
tube-fed patients, formula-only fed infants In families needing structure and planned meals
1. KD methods with different preparation and management MCT MAD Yes Yes Yes Yes
Weighted Weighted Estimated
Yes Yes Optional J 48 g 5g*
10* 15*
Yes No Hospital Hospital Home Entire food should be
each meal. Calculated by dietitian
Easier to prepare calculated by dietician
Liberal in fat and protein. Less dietitian support
86
Currently, there are no specific demographics or particular criteria to predict who is more likely to KD, or which type of KD is more appropriate. The selection of diet type is currently
of the child, family circumstances, response is required. Careful
rate and increased efficacy and durability of diet. Although and method of meal
below)
30 g may be considered)
There are some researches showing particular mutations may respond well to KD while others will not. CN1A or STXBP1 are reported to have a dramatic response rate compared to CDKL5
personalized KD diet in the
n individuals from infancy through adulthood. Proper candidates: First treatment option in GLUT1 deficiency or pyruvate dehydrogenase deficiency (PDHD). Early in the treatment course: Certain epilepsy syndromes which are refractory are shown to be
sponsive to classic KD; for example, Myoclonic Astatic epilepsy (MAE) and Dravet
Syndrome) which showed
LGI Yes Yes
Estimated Measured or estimated
Yes 40-60 g
Yes Home
Liberal in fat and
Less dietitian
Low glycemic index carbohydrates and exchange lists for planning meals
Chapter Six
MCT diet candidates MCT KD is a good choice for patients who are;1) Picky 2) Not able to follow classic KD or 3) Patients with large appetites who like milk and starch, or are interested in
in their diet, and/or a variety of foods.4) Hyperlipidemia
Patients with chronic vomiting or diarrhea before diet initiation are not candidates for this type of KD. Also, concurrent use of valproic MAD Candidates 1) Children in busy, large families 2) Children with limited tolerance for extremely high3) Patients who need more protein than KD can provide.4) Where hospital admission or a fasting period is not possible due to cost or other 5) Children with idiopathic generalized epilepsies (e.g., absence epilepsy, juvenile myoclonic epilepsy) 6) Children who need urgent treatment (e.g., same day) in the Overall, for adolescents and adults, the MAD is probably the better optioptions. In emergency situations, such as infantile spasms or refractory status epilepticus, classical KD, not the MAD, is indicated because time does matter. Usually classic KD’s effect on seizure will take less time to evolve.
LGI Diet candidates 1) If the child could not tolerate KD or less calculation/weighing of foods and more food choices.3) Persistent hyperlipidemia 4) Persistent symptoms from overketosisOverall it is suggested for children over 2 Switching diet There are different reasons for switching diet. Seizure control Switching from the MAD/LGI to the classical KD can further improvif there has been an initial partial response.In other words, higher ketone levels can be achieved by changing MAD to classic KD, or by adding some Ketogenic supplements to MAD, or by increasing ratio in classic KD. Some studies suggest that a strict diet in the first 1 to 3 months is critical for seizure control. Moreover, switching from KD to MAD or LGIT is possible without losing efficacy after 3 months of strict diet. Reducing the KD ratio after the
MCT KD is a good choice for patients who are;
Not able to follow classic KD or MAD. Also, the LGI diet doesn’t control their seizure. Patients with large appetites who like milk and starch, or are interested inin their diet, and/or a variety of foods.
Patients with chronic vomiting or diarrhea before diet initiation are not candidates for this type of valproic acid is a contraindication to MCD KD.
1) Children in busy, large families 2) Children with limited tolerance for extremely high-fat foods
who need more protein than KD can provide. 4) Where hospital admission or a fasting period is not possible due to cost or other
Children with idiopathic generalized epilepsies (e.g., absence epilepsy, juvenile myoclonic
6) Children who need urgent treatment (e.g., same day) in the clinic. Overall, for adolescents and adults, the MAD is probably the better option due to more food
In emergency situations, such as infantile spasms or refractory status epilepticus, classical KD, not the MAD, is indicated because time does matter. Usually classic KD’s effect on seizure will take
1) If the child could not tolerate KD or MAD because of high-fat content, or if the family prefers less calculation/weighing of foods and more food choices.
overketosis (e.g., vomiting or fatigue). Overall it is suggested for children over 2 year's old/teenagers who need a more liberal diet
There are different reasons for switching diet.
Switching from the MAD/LGI to the classical KD can further improve seizure control, especially partial response.
In other words, higher ketone levels can be achieved by changing MAD to classic KD, or by adding some Ketogenic supplements to MAD, or by increasing ratio in classic KD.
dies suggest that a strict diet in the first 1 to 3 months is critical for seizure control. Moreover, switching from KD to MAD or LGIT is possible without losing efficacy after 3 months
ratio after the first 3 months is reported to have the same result
87
, the LGI diet doesn’t control their seizure. Patients with large appetites who like milk and starch, or are interested in more carbohydrate
Patients with chronic vomiting or diarrhea before diet initiation are not candidates for this type of
4) Where hospital admission or a fasting period is not possible due to cost or other factors Children with idiopathic generalized epilepsies (e.g., absence epilepsy, juvenile myoclonic
on due to more food
In emergency situations, such as infantile spasms or refractory status epilepticus, classical KD, not the MAD, is indicated because time does matter. Usually classic KD’s effect on seizure will take
content, or if the family prefers
old/teenagers who need a more liberal diet
e seizure control, especially
In other words, higher ketone levels can be achieved by changing MAD to classic KD, or by adding some Ketogenic supplements to MAD, or by increasing ratio in classic KD.
dies suggest that a strict diet in the first 1 to 3 months is critical for seizure control. Moreover, switching from KD to MAD or LGIT is possible without losing efficacy after 3 months
rted to have the same result.
Chapter Six
Tolerance/compliance issues 1) Switching from classic KD to MAD/LGI. The child may find classic KD difficult to tolerate, or the family may not be willing to continue even partially effective, switching to a more liberal diet may help to prevent
2) Switching from MCTKD to classic KD may be suggested due to the GI side effects of MCT oil particularly in younger children, or if manMCT products in each food or snack, etc. 3) Switching to classic KD or MADKD, if weight loss is an issue in MAD or LGI.Adolescents, or any child who is interested in a more liberal diet, may switch more choices. MCTKD suits children who are interested in higher carbohydrate content in foods. This can also be planned before starting the diet with a proper food record and an approach; However, switching to other die How to switch Chaining from one type to another KD
Table 6-2. How to switch between diet types
Educate parents
Calculate diet, meals,and snacks
Classic to MAD/LGI Classic to MCT
▪Gradually reduce classic KD ration slowly to a ratio (See discontinuation in chapter 2)
▪Exchange one meal at a time overa set time period; few days/every week
▪Once the 1:1 ratio is tolerated; with sufficient/stcommence the new diet
▪ For example; First week: MCT breakfast, classic lunch dinner and snacks. Next week: MCT breakfast and lunch, classic dinner and snacks, etc.
1) Switching from classic KD to MAD/LGI. The child may find classic KD difficult to tolerate, or the family may not be willing to continue weighing and preparing foods. If the diet has been effective, or even partially effective, switching to a more liberal diet may help to prevent nonadherence
2) Switching from MCTKD to classic KD may be suggested due to the GI side effects of MCT oil particularly in younger children, or if managing MCT diet is too hard for the family due to adding MCT products in each food or snack, etc.
3) Switching to classic KD or MADKD, if weight loss is an issue in MAD or LGI.Adolescents, or any child who is interested in a more liberal diet, may switch to MAD or LGI to have more choices. MCTKD suits children who are interested in higher carbohydrate content in foods. This can also be planned before starting the diet with a proper food record and an
, switching to other diet types is a feasible option if compliance is a concern.
Chaining from one type to another KD is also stepwise, are shown in Table 6-2, below
2. How to switch between diet types
MAD/LGI to MCT MAD/LGI to classic Classic to MAD/LGI
▪ introduce MCT and decrease LCT until target ratio
▪Gradually increase the ratio from 1:1 to 2:1 and higher until target ratio
Gradually reduce classic KD ration slowly to a 1:1
(See discontinuation in
▪protein and increase the carbohydrate over a week/or a set time period
▪ Target ratio depends on seizure control and tolerance
Once the 1:1 ratio is tolerated; with sufficient/stable ketosis, commence the new diet
88
1) Switching from classic KD to MAD/LGI. The child may find classic KD difficult to tolerate, or the et has been effective, or
nonadherence.
2) Switching from MCTKD to classic KD may be suggested due to the GI side effects of MCT oil aging MCT diet is too hard for the family due to adding
3) Switching to classic KD or MADKD, if weight loss is an issue in MAD or LGI. to MAD or LGI to have
more choices. MCTKD suits children who are interested in higher carbohydrate content in foods. This can also be planned before starting the diet with a proper food record and an individualized
t types is a feasible option if compliance is a concern.
, below.
MAD/LGI to MCT
▪ Graduallyintroduce MCT and decrease LCT until target ratio
▪ Slowly decrease the protein and increase the carbohydrate over a week/or a set time period
References
· Chamita R, Eckert S, Wirrell E. Ketogenic Di· Ko A, Jung DE, Kim SH, Kang H
Diet for Specific Genetic Mutation in Developmental and Epileptic Encephalopathy. Front. Neurol. 9:530, 2018
· Kossoff EH, A decade of the modified Atkins diet (2003Epilepsy & Behavior. 29: 437–442, 2013
· Kumada T, Miyajima T, Hiejima I. Modified Atkins Diet and Low Glycemic Index Treatment for Medication-Resistant Epilepsy: Cu
· Liu Y-M C., Wang H-S.; Mediumresistant Epilepsy and A Comparison with Other Ketogenic Diet, Biomed J, 36:9
· Louw E. van der, Dorine van den Hurk D. van der, Neal E. et al.; Ketogenic diet guidelines for infants with refractory epilepsy, Epilepsy. 20: 798
· Muzykewicz DA, Lyczkowski DA, Memon N et al. index treatment in pediatric epilepsy. Epilepsia, 50:1118
· Nordli D.; The ketogenic diet: Uses and abuses, 58, S21-S24, 2002· Zupec-Kania B, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet, Therapies
Symposium. Manhattan Beach, CA, 2015
Chamita R, Eckert S, Wirrell E. Ketogenic Diet. Can J Neurol Sci., 2013, 40: 158-167Ko A, Jung DE, Kim SH, Kang H-C, Lee JS, Lee ST, Choi JR and Kim HD. The Efficacy of Ketogenic Diet for Specific Genetic Mutation in Developmental and Epileptic Encephalopathy. Front. Neurol.
A decade of the modified Atkins diet (2003–2013): Results, insights, and future directions. 442, 2013
Kumada T, Miyajima T, Hiejima I. Modified Atkins Diet and Low Glycemic Index Treatment for Epilepsy: Current Trends in Ketogenic Diet. J Neurol Neurophysiol, 2013S.; Medium-chain Triglyceride Ketogenic Diet, An Effective Treatment for Drug
resistant Epilepsy and A Comparison with Other Ketogenic Diet, Biomed J, 36:9-15, 2013der, Dorine van den Hurk D. van der, Neal E. et al.; Ketogenic diet guidelines for infants
with refractory epilepsy, Epilepsy. 20: 798-809, 2016 Muzykewicz DA, Lyczkowski DA, Memon N et al. Efficacy, safety, and tolerability of the low glycemic
ment in pediatric epilepsy. Epilepsia, 50:1118–1126, 2009 Nordli D.; The ketogenic diet: Uses and abuses, 58, S21-S24, 2002
Kania B, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet, Therapies Symposium. Manhattan Beach, CA, 2015
89
167 The Efficacy of Ketogenic
Diet for Specific Genetic Mutation in Developmental and Epileptic Encephalopathy. Front. Neurol.
2013): Results, insights, and future directions.
Kumada T, Miyajima T, Hiejima I. Modified Atkins Diet and Low Glycemic Index Treatment for rrent Trends in Ketogenic Diet. J Neurol Neurophysiol, 2013 chain Triglyceride Ketogenic Diet, An Effective Treatment for Drug-
15, 2013 der, Dorine van den Hurk D. van der, Neal E. et al.; Ketogenic diet guidelines for infants
Efficacy, safety, and tolerability of the low glycemic
Kania B, Brown J, Matsumoto JH. White Paper: Proceedings of Ketogenic Diet, Therapies
Chapter Seven
Practical points in KD· Common medications · Monitoring ketosis and compliance· Managing Illness · Supplementation · Tables
Common mediation informationThis is a simple list to mention common medications used in 1) H2 blockers (Ranitidine) or Proton2) Potassium citrate to prevent nephrolithiasis (Poly
Oral citrate can reduce folic acid absorption due to the anemia. Dose: Starting from 2mEq/kg/day; for a young child e.g.15 mEq twice daily and for an older child 30 mEq twice daily
3) Constipation management Polyethylene glycol products are carbohydrate free so appropriate for constipation treatment. (Starting doseis 17 g per day). Other medications: Milk of Magnesia, mineral oil.
4) Bicarbonate to correct acidosisTypically want to keep HCO3 1mEq/kg when HCO3 - <20 mEq/L, IV or by oral citrate.
Surgery and anesthesia: PotentiallyAccording to new recommendation (Keto diet study group) propofol that is used for sedation (e.g. MRI procedure), or status epilepticus should be avoided in the acid oxidation and may lead to propofol infusion syndrome. This syndrome is a condition characterized by myocardial failure, acidosis. However, there are other reports about safe usage of propofol mentioning thatdose (for anesthesia induction) is not high enough to induce propofol infusion syndrome. Remember that patients with mitochondrial disorders are also sensitive to propofol effects and caution is recommended. Overall, anesthesia is relatively safe in a bicarbonate, and electrolytes level. Normal saline is a better choice instead of ringer lactate. Seizure exacerbation and acid-baseconsidered for every patient.
General consideration for reducing medication carbohydrate content, please remember:Carbohydrate content of medications widely differs, and a All medications (other than AEDs) maymedication that children may commonly need. As a rule, liquid forms should be avoided as they usually have more carbohydrate as sweetens. Remember this trend about carbohydrate content:Liquids > chewable/disintegrating tablets > tablets/capsulesGenerics may contain more carbohydrate than brand products. Sometimes, the by making sugar free medication or informing you if IV forms of medication can be used instead with some dose adjustment. An advice parentmedication their child is supposed Some medication such as anti-histamines may lower seizure threshold without having carbohydrates.As a last option if you have to give a medication containing carbohydrates, add some butter or oil to make it Ketogenic.
Practical points in KD
Monitoring ketosis and compliance
Common mediation information This is a simple list to mention common medications used in KD.
blockers (Ranitidine) or Proton-pump inhibitors for GERD 2) Potassium citrate to prevent nephrolithiasis (Poly-Citra)
Oral citrate can reduce folic acid absorption due to the alkaline environment and the risk of megaloblastic ng from 2mEq/kg/day; for a young child e.g.15 mEq twice daily and for an older child
Polyethylene glycol products are carbohydrate free so appropriate for constipation treatment. (Starting doseOther medications: Milk of Magnesia, mineral oil.
4) Bicarbonate to correct acidosis - levels >20 mEq/L, begin supplementation with sodium bicarbonate
<20 mEq/L, IV or by oral citrate.
sia: Potentially dangerous medication According to new recommendation (Keto diet study group) propofol that is used for sedation (e.g. MRI procedure), or status epilepticus should be avoided in the ketogenic diet because it impairs fatty
may lead to propofol infusion syndrome. This syndrome is a condition characterized by myocardial failure, rhabdomyolysis, hyperlipidemia and metabolic acidosis. However, there are other reports about safe usage of propofol mentioning thatdose (for anesthesia induction) is not high enough to induce propofol infusion syndrome. Remember that patients with mitochondrial disorders are also sensitive to propofol effects and caution is
y safe in a keto diet by close monitoring of serum glucose, PH,electrolytes level. Normal saline is a better choice instead of ringer lactate.
base disturbances may happen during and after surgery and should
General consideration for reducing medication carbohydrate content, please remember:Carbohydrate content of medications widely differs, and a small amount of it may disrupt ketosis. All medications (other than AEDs) may have this issue includes antibiotics and over the counter medication that children may commonly need. As a rule, liquid forms should be avoided as they usually have more carbohydrate as sweetens. Remember this trend about carbohydrate content:
hewable/disintegrating tablets > tablets/capsules Generics may contain more carbohydrate than brand products. Sometimes, the
making sugar free medication or informing you if IV forms of medication can be used instead An advice parent to read labels/ask the pharmacist
their child is supposed to take (Table7-1). histamines may lower seizure threshold without having carbohydrates.
to give a medication containing carbohydrates, add some butter or oil to
90
environment and the risk of megaloblastic ng from 2mEq/kg/day; for a young child e.g.15 mEq twice daily and for an older child
Polyethylene glycol products are carbohydrate free so appropriate for constipation treatment. (Starting dose
levels >20 mEq/L, begin supplementation with sodium bicarbonate
According to new recommendation (Keto diet study group) propofol that is used for sedation (e.g. diet because it impairs fatty
may lead to propofol infusion syndrome. This syndrome is a life-threatening , hyperlipidemia and metabolic
acidosis. However, there are other reports about safe usage of propofol mentioning that its pediatric dose (for anesthesia induction) is not high enough to induce propofol infusion syndrome. Remember that patients with mitochondrial disorders are also sensitive to propofol effects and caution is
diet by close monitoring of serum glucose, PH, electrolytes level. Normal saline is a better choice instead of ringer lactate.
disturbances may happen during and after surgery and should be
General consideration for reducing medication carbohydrate content, please remember: amount of it may disrupt ketosis.
have this issue includes antibiotics and over the counter medication that children may commonly need. As a rule, liquid forms should be avoided as they usually have more carbohydrate as sweetens. Remember this trend about carbohydrate content:
Generics may contain more carbohydrate than brand products. Sometimes, the pharmacist can help making sugar free medication or informing you if IV forms of medication can be used instead
pharmacist about every
histamines may lower seizure threshold without having carbohydrates. to give a medication containing carbohydrates, add some butter or oil to
Chapter Seven
Table 7-1: Carb / Non-Carb Ingredients of medicationshttps://www.charliefoundation.org/resourcesCarbohydrate Ingredients
● Glycerin ● Maltodextrin ● Magnasweet ● Organic acids: Ascorbic acid, citric acid, lactic acid ● Propylene glycol ● Sugars: dextrose, fructose, glucose, lactose, sucrose, sugar, palm sugar, agave nectar, cane syrup, cane juice, corn syrup, honey ● Sugar alcohols: erythritol, isomalt, glycerol, mannitol, maltitol, sorbitol, xylitol● Starches: cornstarch, hydrogenated starchhydrolysates (HSH), pregelatinized starch, sodium starch glycolate
Free foods There are some free foods include sugarsparkling drinks, and sweeteners such as saccharine and aspartame. We don’t have these products available here in Iran, but we can make a drink by mixing water with few slices of cucumber, lemon or fresh basil leaves and keep it in the fridge for one hour, then filter it down through a fine strainer and use the water as a beverage. Remember that liquidsSome plant-based ingredients such asabsorbable carbohydrate and can be used in KD.recommended regarding their calorie and carbohydrate content. Monitoring ketosis and compliance Ketosis Urinary ketosis is usually monitored 2(not in LGI) especially in the first month until stable ketosis is achieved.80–160 mg/dl which is equal to as 3+ to 4+ or large initiation. It is important to perform this test accurately e.g. not contaminated, no damp surrounding etc. Urine ketone test seems to have had some pitfalls and limitations (in interpretation. The home blood testing measures serum βcontrol. This test also requires proper collection and accurate laboratory procedure. However, thereno need for a regular checkup (see also follow up tips in chapter one). between 4 and 6 mmol/L although this has not been confirmed yet. (Infants:
Carb Ingredients of medications https://www.charliefoundation.org/resources-tools/resources-2/low
ngredients Non-Carbohydrate Ingredients
● Organic acids: Ascorbic acid, citric acid,
● Sugars: dextrose, fructose, glucose, lactose, sucrose, sugar, palm sugar,
rup, cane juice,
● Sugar alcohols: erythritol, isomalt, glycerol, mannitol, maltitol, sorbitol, xylitol ● Starches: cornstarch, hydrogenated starch hydrolysates (HSH), pregelatinized starch,
· Acesulfame potassium · Aspartame · Carboxymethylcellulose· Cellulose · Hydroxymethylcellulose· Magnesium stearate· Microcrystalline cellulose· Polyethylene glycol· Saccharine · Superose · Stevia (rebiana)
There are some free foods include sugar-free drinks and such as flavored zerosweeteners such as saccharine and aspartame. We don’t have these products
available here in Iran, but we can make a drink by mixing water with few slices of cucumber, lemon or it in the fridge for one hour, then filter it down through a fine strainer and
use the water as a beverage. Remember that liquids should be divided throughout the day.such as cellulose, carboxymethylcellulose (Table 7
absorbable carbohydrate and can be used in KD. However, Sugar alcohols like isomaltrecommended regarding their calorie and carbohydrate content.
Monitoring ketosis and compliance
Urinary ketosis is usually monitored 2-3 times per week by dipstick especially in classic KD/ MAD month until stable ketosis is achieved. Urine test detects acetoacetate
160 mg/dl which is equal to as 3+ to 4+ or large ketosis; usually occur over 2 weeks afterinitiation. It is important to perform this test accurately e.g. not contaminated, no damp surrounding etc. Urine ketone test seems to have had some pitfalls and limitations (Table 7-2) that should be considered
ing measures serum β-hydroxybutyrate (BHB) level, which correlates with seizure control. This test also requires proper collection and accurate laboratory procedure. However, there
checkup (see also follow up tips in chapter one). Levels should be maintainedbetween 4 and 6 mmol/L although this has not been confirmed yet. (Infants: ≤5mmol/L)
91
2/low-carb Carbohydrate Ingredients
Acesulfame potassium (AceK)
Carboxymethylcellulose
Hydroxymethylcellulose Magnesium stearate Microcrystalline cellulose Polyethylene glycol
Stevia (rebiana)
zero-calorie water, diet sweeteners such as saccharine and aspartame. We don’t have these products
available here in Iran, but we can make a drink by mixing water with few slices of cucumber, lemon or it in the fridge for one hour, then filter it down through a fine strainer and
throughout the day. Table 7-1) contain non-
r, Sugar alcohols like isomalt not routinely
es per week by dipstick especially in classic KD/ MAD Urine test detects acetoacetate
usually occur over 2 weeks after diet initiation. It is important to perform this test accurately e.g. not contaminated, no damp surrounding etc.
) that should be considered
hydroxybutyrate (BHB) level, which correlates with seizure control. This test also requires proper collection and accurate laboratory procedure. However, there is
Levels should be maintained ≤5mmol/L)
Chapter Seven
Table 7-2. Urinary ketone test limitationsReasons for low urine ketones:
Not enough urine on the dipstickIllness Taking carbohydrate in error Exercise
Compliance: Urinary ketosis pints out compliance, however: Efficacy (seizure control) shows more correlation with blood BHB. Early morning and checking urinary ketones. However, urine as urine ketones can be low in the morning and higher in the evening. Managing illness Different issues may happen during illness and the · Ketone level reduces just before starting illness, although some
low calorie and fluid intake or higher metabolism due to fever or illness. · Medications may also affect ketones e.g. steroids reduce ketones. · Seizures may exacerbate during illness.
In course of fever, diarrhea, and vomiting, proper hydration is critical with clear, sugaras tolerated, oral hydration fluids (ORS) or ringer-lactate) if indicated. ORS carbohydrate should be calculated. Blood glucose and kshould be monitored and treated if necessary. When vomiting stops, feeding should be gradually restarted by fluid consumption and semisolid meals which are better tolerated. In case of prolonged vomiting or diarrhea, fat should reduce by using halfoil or in MCT-KD, half of MCT in each meal for a day and then slowly return to the amount over the next couple of days. In NPO situations Like other children, children on KD may get sick. For the are crucial. 1) Dextrose free IV fluid should be initiated to maintain adequate hydration. 2) Glucose monitoring every 4-6 hours is recommended (depending on the age and clinical co
child). It should be spaced out while diet gradually restarted and stopped3) For acidosis monitoring, a daily metabolic panel is recommended until the 4) Hypoglycemia (Blood glucose less than 40 mg/dl) should be treated by bolu
dextrose D10W. Achieving 50-80 mg/dl is the goal. Blood glucose should be rechecked, and persistenthypoglycemia should be treated with glucose infusion. Resuming feeds usually prevents hypoglycemia.
5) Metabolic acidosis (bicarbonate <20 mEq/l) may occur due to either excessive ketosis or hypoglycemia. Patients on topiramate orzonisamide are, particularly at risk.started at 1-2 mEq/kg/day divided 2
6) In persistent acidosis and/or refractory symptomatic patients, supplementation should increase and can be changed to IV infusion of sodium acetate.
2. Urinary ketone test limitations
Reasons for low urine ketones: Limitation of urine ketone test:
nough urine on the dipstick Dilution may show low ketone level.Indicating past few hours condition instead of current. Not suitable in hyper ketosis(reads up to16mmol/L)
ut compliance, however: Efficacy (seizure control) shows more correlation Early morning and post-dinner urine samples are the most reliable time for
checking urinary ketones. However, urine should be checked at different times of the day ifas urine ketones can be low in the morning and higher in the evening.
Different issues may happen during illness and the child should be visited by a physician.Ketone level reduces just before starting illness, although sometimes hyper ketosis may occur due to very low calorie and fluid intake or higher metabolism due to fever or illness. Medications may also affect ketones e.g. steroids reduce ketones. Seizures may exacerbate during illness.
vomiting, proper hydration is critical with clear, sugaras tolerated, oral hydration fluids (ORS) or dextrose-free IV solution (0.45% or 0.9% saline or
lactate) if indicated. ORS carbohydrate should be calculated. Blood glucose and kshould be monitored and treated if necessary. When vomiting stops, feeding should be gradually restarted by fluid consumption and semisolid meals which are better tolerated. In case of prolonged vomiting or diarrhea, fat should reduce by using half amount of cream, butter,
KD, half of MCT in each meal for a day and then slowly return to the amount over the next couple of days. In formula-fed patients, half strength formula can be tried.
hildren on KD may get sick. For the management of acute
Dextrose free IV fluid should be initiated to maintain adequate hydration. 6 hours is recommended (depending on the age and clinical co
child). It should be spaced out while diet gradually restarted and stopped For acidosis monitoring, a daily metabolic panel is recommended until the diet is advanced. Hypoglycemia (Blood glucose less than 40 mg/dl) should be treated by bolus infusion of 0.25 g/kg
80 mg/dl is the goal. Blood glucose should be rechecked, and persistenthypoglycemia should be treated with glucose infusion. Resuming feeds usually prevents hypoglycemia.
<20 mEq/l) may occur due to either excessive ketosis or hypoglycemia. Patients on topiramate orzonisamide are, particularly at risk. Treatment by oral
2 mEq/kg/day divided 2-3 times daily. fractory symptomatic patients, supplementation should increase and can be
changed to IV infusion of sodium acetate.
92
Limitation of urine ketone test:
Dilution may show low ketone level. Indicating past few hours condition instead
Not suitable in hyper ketosis(reads up to
ut compliance, however: Efficacy (seizure control) shows more correlation most reliable time for
should be checked at different times of the day if possible
should be visited by a physician. times hyper ketosis may occur due to very
vomiting, proper hydration is critical with clear, sugar-free drinks IV solution (0.45% or 0.9% saline or
lactate) if indicated. ORS carbohydrate should be calculated. Blood glucose and ketosis should be monitored and treated if necessary. When vomiting stops, feeding should be gradually
amount of cream, butter,
KD, half of MCT in each meal for a day and then slowly return to the previous patients, half strength formula can be tried.
of acute illness, certain rules
6 hours is recommended (depending on the age and clinical condition of the
is advanced. s infusion of 0.25 g/kg
80 mg/dl is the goal. Blood glucose should be rechecked, and persistent hypoglycemia should be treated with glucose infusion. Resuming feeds usually prevents hypoglycemia.
<20 mEq/l) may occur due to either excessive ketosis or hypoglycemia. bicarbonate should be
fractory symptomatic patients, supplementation should increase and can be
Chapter Seven
Supplementation
Most of the micronutrients are lacking in KD and should be supplemented and monitored. Although a high level of Vitamin A and E is also reported, and fatRecommended supplementations are shown in enough micronutrients, but this is not a fixed supplementation
Table 7-3 Supplementations in KD Required Optional
Multivitamin with minerals (and trace minerals) Calcium with vitamin D
Oral citrates (PolycitraLaxatives: MiraLAXoil, a
Carbohydrate free products should be used
To have an idea about supplementation in different types of KD,amount in the diet, the lower the micronutrients will be. Important points include:1) How much of RDA is being provided by diet for each patient depending on the weightdiet type? 2) Monitoring results Based on this information, supplementation should be individually adjusted with extra or lower doses and ensure patients’ safety. Dietary Reference Intakes based on age are provided in this website:http://nationalacademies.org/hmd/Reports/2006/DietaryNutrient-Requirements.aspx Vitamin D and Calcium Supplementation with low-dose 400 IUof vitamin D deficiency is serum 25-months. Remember that we gain most of our peak bone mass duringin will increase if this process is left incomplete. Also, yearly DEXA for screening is now recommended because osteoporosis in KD also seems to occur due to Vitamin D independent mechanisms. DEXA (dualquick test with radiation equal to oneA daily dose of 600 - 1200 mg of oral calcium in a (See also RDA Table7-9 for Vitamin D and Calcium) Carnitine Carnitine deficiency may interrupt ketone formation so free carnitine level should be checked. Serum level<20 μmol/L is usually considered deficiency although different ageexist. Risk factor for deficiency is multiple AEDs, especially valproic acid.
are lacking in KD and should be supplemented and monitored. Although level of Vitamin A and E is also reported, and fat-soluble vitamins levels also require tracking.
Recommended supplementations are shown in Table 7-3. Usual multivitamin supplements provide enough micronutrients, but this is not a fixed supplementation.
3 Supplementations in KD Optional Extra supplementation
Oral citrates (Polycitra-K) Laxatives: MiraLAX®, mineral oil, a glycerin suppository
Additional selenium, magnesium, zinc, phosphorus, vitamin D, Carnitine MCT oil or coconut oil (source of MCT) Salt (sodium to add to modular formulas if used for greater than age 1 year)
Carbohydrate free products should be used
idea about supplementation in different types of KD, remember that the higher the fat amount in the diet, the lower the micronutrients will be. Important points include:1) How much of RDA is being provided by diet for each patient depending on the weight
supplementation should be individually adjusted with extra or lower
Dietary Reference Intakes based on age are provided in this website: http://nationalacademies.org/hmd/Reports/2006/Dietary-Reference-Intakes-Essential
dose 400 IU vitamin D in the multivitamin is recommended. Definition
-OH< 32 ng/dl and should be measured at baselinemonths. Remember that we gain most of our peak bone mass during childhood and risk of fractures in will increase if this process is left incomplete.
Also, yearly DEXA for screening is now recommended because osteoporosis in KD also seems to cur due to Vitamin D independent mechanisms. DEXA (dual-energy X-ray absorptiometry is a
quick test with radiation equal to one-twentieth of a standard chest X-ray. 1200 mg of oral calcium in a sugar-freeform is recommended while on
for Vitamin D and Calcium)
Carnitine deficiency may interrupt ketone formation so free carnitine level should be checked. rum level<20 μmol/L is usually considered deficiency although different age
Risk factor for deficiency is multiple AEDs, especially valproic acid.
93
are lacking in KD and should be supplemented and monitored. Although soluble vitamins levels also require tracking. . Usual multivitamin supplements provide
Extra supplementation
Additional selenium, magnesium, zinc, phosphorus, vitamin D,
MCT oil or coconut oil (source of
Salt (sodium to add to modular formulas if used for greater than age
remember that the higher the fat amount in the diet, the lower the micronutrients will be. Important points include: 1) How much of RDA is being provided by diet for each patient depending on the weight/age and
supplementation should be individually adjusted with extra or lower
Essential-Guide-
vitamin D in the multivitamin is recommended. Definition baseline and every 6
childhood and risk of fractures
Also, yearly DEXA for screening is now recommended because osteoporosis in KD also seems to ray absorptiometry is a
is recommended while on KD.
Carnitine deficiency may interrupt ketone formation so free carnitine level should be checked. rum level<20 μmol/L is usually considered deficiency although different age-based levels may
Chapter Seven
Carnitine supplement is indicated in case of clinical or bioparents may want to try it as an adjuvantcontrol,and energy level by using carnitine. mg/kg daily and increase as needed; however, it may differ based on individual requirements. supplement should be L-Carnitine isomer only, and required dose is usually less than 50 mg/kg/day. Selenium Selenium deficiency has been reported even after a few months of therapy.selenium deficiency in 20% of their patients and recommended a or diet provides less than 70% of RDA. Blood selenium monitoring and extra supplement more than daily prescribed multivitamin which iabout 20 µg, may be required. This is to prevent subsequent cardiac complications. Adding high selenium foods such as Brazil nuts is beneficial, if available. Alternatively, sunflower seeds with high selenium seem a proper substitute source.Other sources of selenium include Cooking tips Advice parents to start with simple recipes. When the diet is proved to be efficient, then more variety can be considered. Other helpful points: ü As a general rule, remember that herbs and spices all have carbohydrate and just a pinch is
allowed. ü Other ingredients such as catsup, lemon juice, vinegar, soy sauce, and baking chocolate can be
used in very small quantities (suchü Advise parent to ask a dietitianü Meal ingredients can be cooked together or separately. Remember, differences in the
process such as fluctuations in temperature maü Regular butter, cream, mayonnaises have less carbohydrate than diet ones that are available in
the market, so better to buy the regular one. ü A parent should get used to reading all labels to ensure what the food components really
They should be careful while reading labelsfiber which will be the total carbohydrate of a given product.Carbohydrates = Total Carbohydrates
ü Remember that fiber does count in daily total carb(LGI) diet. It differs from other ketogenic diets because it affects the
Carnitine supplement is indicated in case of clinical or biochemical deficiency. But sometimes adjuvant. There are some reports about increased well
energy level by using carnitine. The recommendation is to start at a as needed; however, it may differ based on individual requirements.
Carnitine isomer only, and required dose is usually less than 50 mg/kg/day.
Selenium deficiency has been reported even after a few months of therapy. Some centers reported selenium deficiency in 20% of their patients and recommended a screening test if child symptomatic or diet provides less than 70% of RDA. Blood selenium monitoring and extra supplement more than daily prescribed multivitamin which iabout 20 µg, may be required. This is to prevent subsequent cardiac complications. Adding high selenium foods such as Brazil nuts is beneficial, if available. Alternatively, sunflower seeds with high selenium seem a proper substitute source. Please see Table 7-10 for RDA indifferent ages.
include tuna, beef, chicken, turkey, and enriched grains.
Advice parents to start with simple recipes. When the diet is proved to be efficient, then more
As a general rule, remember that herbs and spices all have carbohydrate and just a pinch is
Other ingredients such as catsup, lemon juice, vinegar, soy sauce, and baking chocolate can be used in very small quantities (such as 2 grams, about1/8 teaspoon).
dietitian about extracts or flavors they are willing to useMeal ingredients can be cooked together or separately. Remember, differences in the process such as fluctuations in temperature may change the keto ratio. Regular butter, cream, mayonnaises have less carbohydrate than diet ones that are available in the market, so better to buy the regular one.
should get used to reading all labels to ensure what the food components reallyThey should be careful while reading labels and look for the total carbohydrate amount minus fiber which will be the total carbohydrate of a given product. Carbohydrates = Total Carbohydrates – fiber Remember that fiber does count in daily total carbohydrate allowance of low glycemic index (LGI) diet. It differs from other ketogenic diets because it affects the glycemic
94
chemical deficiency. But sometimes . There are some reports about increased well-being, seizure
is to start at a lowdose of 10 as needed; however, it may differ based on individual requirements. The
Carnitine isomer only, and required dose is usually less than 50 mg/kg/day.
Some centers reported test if child symptomatic
Blood selenium monitoring and extra supplement more than daily prescribed multivitamin which is about 20 µg, may be required. This is to prevent subsequent cardiac complications. Adding high selenium foods such as Brazil nuts is beneficial, if available. Alternatively, sunflower seeds with
for RDA indifferent ages. turkey, and enriched grains.
Advice parents to start with simple recipes. When the diet is proved to be efficient, then more
As a general rule, remember that herbs and spices all have carbohydrate and just a pinch is
Other ingredients such as catsup, lemon juice, vinegar, soy sauce, and baking chocolate can be
about extracts or flavors they are willing to use Meal ingredients can be cooked together or separately. Remember, differences in the cooking
Regular butter, cream, mayonnaises have less carbohydrate than diet ones that are available in
should get used to reading all labels to ensure what the food components really are. and look for the total carbohydrate amount minus
ohydrate allowance of low glycemic index glycemic index of the food.
Chapter Seven
Preparing foods Advise parents to select foods which are familiar They can also play some tricks based on the child feeding preference for example:Making a salad with chopped lettuce and mayonnaise, this helps food’s portion seem larger or mixing cream (خامھ) /milk with sugar-freeUsing almond flour, coconut or hazelnut flour for making is very helpful to make foods and deserts for children. Remember that these flours may need a modified recipe, for example, more eggs or yeast. Alsgeneral. Foods that can be used as free: 25 grams of lettuce; one walnut, macadamia nut, or pecan; three filberts; or three ripe (black) olives.A parent should know about the importance of snacks to keep keideal weightdon’t have much-storedprotein will be used instead that will lead to decrease ketosis. To complete cooking tips one simple keto recipe is ex Keto cheese sticks (اسنک پنیری کتوژن
· 5 stick mozzarella cheese (you can cut the cheese into sticks or cubs if sticks are not available.یامی توانید پنیر رابھ شکل استوانھ یا مکعب ببرید
· One large egg ( یک عدد تخم مرغ ) · 1-2tbsp almond or coconut flour (· Very small amount of herp for seasoning, based on the preference e.g. dry parsley or garlic powder
· Olive oil for frying (یتون برای سرخ کردن· 1-2 tsp parmesan cheese (optional)
How to prepare: Beat the egg in small bowl. Place flour, and herb, salt and paper in a plastic bag and shake well. Dip the mozzarella sticks/cubs into the egg, then into a fridge for one hour. Fry the pieces in a pan with a hot oil; around 200 C° (400 F°), for 5 minutes. (This prevents cheese from melting). Cook them until browned in all sides.
خوب و بریزید یزرفر یکپلاست یک با یکداخل پلاست بعد درو یددرتخم مرغ بغلطان .یزکنیدفر یخچال ساعت در
یرب شدن پنآ از باشد تا یقهزمان پختن پنج دق
Advise parents to select foods which are familiar to the child, similar to their usual family foods. They can also play some tricks based on the child feeding preference for example:
with chopped lettuce and mayonnaise, this helps food’s portion seem larger or mixing free sweetener.
Using almond flour, coconut or hazelnut flour for making bread, cookies or cakes (instead of wheat) is very helpful to make foods and deserts for children. Remember that these flours may need a modified recipe, for example, more eggs or yeast. Also, heavy cream is a good substitute for milk in
Foods that can be used as free: 25 grams of lettuce; one walnut, macadamia nut, or pecan; three filberts; or three ripe (black) olives.
should know about the importance of snacks to keep ketosis stable. stored fat to break down, so if the interval of meals gets long, stored
protein will be used instead that will lead to decrease ketosis.
To complete cooking tips one simple keto recipe is explained here.
(اسنک پنیری کتوژن5 stick mozzarella cheese (you can cut the cheese into sticks or cubs if sticks are not available
یامی توانید پنیر رابھ شکل استوانھ یا مکعب ببرید ).استوانھ ای شکل
onut flour ( رد بادام یا نارگیلآدو قاشق غذاخوری یا یک ) Very small amount of herp for seasoning, based on the preference e.g. dry parsley or garlic powder
یا پودرسیر بستھ بھ سلیقھ برای طعم دارکردن یتون برای سرخ کردنروغن ز )
2 tsp parmesan cheese (optional)
Beat the egg in small bowl. Place flour, and herb, salt and paper in a plastic bag and shake well. Dip the mozzarella sticks/cubs into the egg, then into a plastic bag to be coated. Ffridge for one hour. Fry the pieces in a pan with a hot oil; around 200 C° (400 F°), for 5 minutes.
cheese from melting). Cook them until browned in all sides.
در فلفل را نمک و شک وخ يسبز ،ردآ .یدکاسه خوب بزندرتخم مرغ بغلطان ابتدا را یرپن يها قطعه سپس .مخلوط شوند
ساعت در یکحداقل سپس آنها را .شود پوشیده با آرد کاملازمان پختن پنج دق داغ و کاملا یدروغن با .سرخ کردن در روغن است
.مدند غذا حاضر استآدر يقطعات به رنگ قهوه ا
95
similar to their usual family foods. They can also play some tricks based on the child feeding preference for example:
with chopped lettuce and mayonnaise, this helps food’s portion seem larger or mixing
, cookies or cakes (instead of wheat) is very helpful to make foods and deserts for children. Remember that these flours may need a
o, heavy cream is a good substitute for milk in
Foods that can be used as free: 25 grams of lettuce; one walnut, macadamia nut, or pecan; three
tosis stable. Usually, children with fat to break down, so if the interval of meals gets long, stored
5 stick mozzarella cheese (you can cut the cheese into sticks or cubs if sticks are not available. استوانھ ای شکل(پنج تکھ پنیرموزارلا
Very small amount of herp for seasoning, based on the preference e.g. dry parsley or garlic powder ادویھ مقدار کمی سبزی خشک
Beat the egg in small bowl. Place flour, and herb, salt and paper in a plastic bag and shake well. Dip bag to be coated. Freeze the pieces in the
fridge for one hour. Fry the pieces in a pan with a hot oil; around 200 C° (400 F°), for 5 minutes.
یهدستورته
کاسه خوب بزن یک در تخم مرغ رامخلوط شوند تا کاملا یدتکان بده
کاملا تا یدرد خوب تکان دهآسرخ کردن در روغن است يمرحله بعدقطعات به رنگ قهوه ا که یوقت .شود جلوگیري
Chapter Seven
Useful Tables In this section, some useful tables (7for protein, calorie, fluid, and minerals in infants/children. This information is helpful for diet calculation. Table 7-4: Foods Value for diet calculation Weight Protein Cream (30%) خامھ
100
Milk (3%) regular شیر معمولی
1 cup(240 ml)
Cream cheese پنیر خامھ ای
30 g
Plain yogurt ماست ساده
100
Feta cheese پنیرفتا
28 g
Mozzarella پنیر موزارلا
28 g
Cheddar پنیرچدار
28 g
Egg whole raw تخم مرغ خام
44
Egg boiled whole تخم مرغ پختھ
50
Egg whole scrambled تخم مرغ نیمرو
46
Egg white (cooked) ی سفیده
)پختھ(تخم مرغ
One large
Egg yolk (cooked) زرده ی تخم مرغ
)پختھ(
One large
Poultry انواع مرغ
100 g
Meat گوشت
100 g
Tuna in water بآماھی تن در
85 g
Tuna in oil ماھی تن درروغن
85 g
Fish cooked (average) ماھی پختھ
85 g
In this section, some useful tables (7-4 TO 7-10) are shown including different foods' value, RDA for protein, calorie, fluid, and minerals in infants/children. This information is helpful for diet
Foods Value for diet calculation Protein (g) Fat (g)
100 1.7 30
1 cup(240 ml) 8 8
30 g 2.4 7.5
100 3.5 3
28 g 2 10
28 g 6 6
28 g 7 8
5 4
6 5
6 7
One large 3.6 N
One large 2.7 4.5
100 g 13.3 6.70
100 g 13.3 6.70
85 g 22 1
85 g 25 7
85 g 22 5
96
ding different foods' value, RDA for protein, calorie, fluid, and minerals in infants/children. This information is helpful for diet
Carbs (g)
3.5
11
1
5
1
1
Trace
N
N
N
0
N
0
0
0
0
0
97
Ground beef Extra lean گوشت گوسالھ کم چرب
100 g 22 5 0
Drumstick ران مرغ
100 g 25 15.50 0
Chicken breast سینھ مرغ
100 g 33.43 4.70 0.51
Cooked Shrimp میگو پختھ
100 g 16.43 0.85 0
Quail سینھ بلدرچین
100 g 22.68 2.98 0
Turkey ران بوقلمون
100 g 27.84 9.82 0
Turkey سینھ بوقلمون
100 g 21.13 0.70 3.52
Fat Butter کره
20 g N 15 N
Oil روغن
10 ml 0 10 g 0
Mayonnaise مایونز
20 g N 15 0.3
Carbohydrate Lavash Bread نان لواش
10 g 0.9 0.05 6.39
Sangak bread کنان سنگ
10 g 0.87 0.04 5.51
Taftoon bread نان تافتون
10 g 0.93 0.06 6.05
Barbari bread نان بربری
10 g 0.82 0.08 5.64
Rice (cooked) برنج پختھ
1 cup(158 g) 4 N 45
Macaroni ماکارونی
1 cup (140 g) 7 1 40
Potato سیب زمینی
135 g 2 N 27
Corn ذرت
77g 3 1 19
Lentil عدس
1 cup (198 g) 18 1 40
Pinto bean لوبیا چیتی
1 cup (171 g) 14 1 44
Orange juice ب پرتقالآ
From one orange
0.6 0 9
One orange (peeled, without seeds) یک پرتقال پوست کرده بدون دانھ
131 g 1 0 15
Apple juice ب سیبآ
Canned /bottled(240 ml)
N 0 29
Chapter Seven
One apple (peeled, sliced)
پوست کنده سیب
110 g
Vegetables group B
جات گروه بسبزی
100 g
Fruits group B میوه ھای گروه ب
100 g
Mint نعناع
20 g
Parsley جعفری
20 g
Onion پیاز
20 g
Cherry گیلاس
20 g
Palm لوآ
10 g
White beans لوبیا سفید
10 g
White rice flour ردبرنجآ
10 g
Other Peanut butter کره بادام زمینی
100
Walnut گردو
28 g
Almond بادام درختی
28 g
Cashew بادام ھندی
28 g
Pistachio پستھ
28 g
N: negligible. Reference/; mostly from Nutritive values of foods, USDA, 2
110 g N 0
100 g 2 0
100 g 0.67 0
20 g 0.69 0.10
20 g 0.65 0.08
20 g 0.23 0.01
20 g 0.18 0.01
10 g 0.16 0.05
10 g 1.98 0.14
10 g 0.80 0.10
100 26 50
28 g 4 18
28 g 6 14
28 g 4 13
28 g 6 15.26
ible. Reference/; mostly from Nutritive values of foods, USDA, 2
98
16
7
6.7
1.71
1.24
1.10
3.22
6.38
6.26
8
22
4
6
9
4.73
Chapter Seven
RDA Tables
1) WHO 2007safe level for growth; +1.96 SD. 2)FAO 2013
Table 7-7: Calorie requirement per body weigh based on age Age
0-6 6-12 1-3 4-6 7-10 11-14 15-18
Table 7-5. Recommended daily calorie, protein,Age/months Weight
(kg)* 1-3 3.8-5.9 4-6 6-7.9 7-12 8-10
*Ideal weight/age, ▪RDA from Austria, France, Germany, The Netherlands, UK, USA
Table 7-6: Fluid maintenance in childre1–10 kg
10–20 kg
>20 kg
WHO 2007safe level for growth; +1.96 SD. 2)FAO 2013
ent per body weigh based on age Age Calorie requirement
kcal/kg/body weight 108 2.298 102 1.290 1.170 1Male Female Male 55 47 1 1 45 40 0.9 0.8
. Recommended daily calorie, protein,and fluid based on RDA Kcal/day▪ Protein g/kg/day
WHO/FAO100-95 1.7795-85 1.2485-80 1.12
RDA from Austria, France, Germany, The Netherlands, UK, USA
Fluid maintenance in children 100 ml/kg
1000 ml + 50 ml/kg for each kg >10 kg
1500 ml + 20 ml/kg for each kg >20 kg
99
Protein/kg
108 2.2 1.6
102 1.2 90 1.1 70 1
Male Female 55 47 1 1 45 40 0.9 0.8
fluid based on RDA in infants Protein g/kg/day
WHO/FAO 1.77-1.361 1.24-1.121 1.12-0.862
Chapter Seven
Table 7-8: Safe level of protein iAge/years The safe
protein intake g/kg/day
0.5 1.31 0.5-1 1.14 1.5 1.03 2 0.97 3 0.90 4 0.86 5 0.85 6 0.89 7 0.91 8 0.92 9 0.92 10 0.91
From WHO, 2007
*Adequate intake (AI)
Table 7-10. Recommended daily allowance for Selenium
Age (yr) RDA (µg/day)
<0.5 15* 0.5-1 20* 1-3 20 4-8 30 *Adequate intake (AI) Dietary Reference Intakes for Institute of Medicine, National Academies Press (US); 2011
Table7-9: Recommended Dietary Allowances (RDA) For Calcium and Vit D Age Calcium mg 0-6 months 200* 7-12 months 260* 1-3 years 700 4-8 years 1000 9-13 years 1300 14-18 1300
Safe level of protein intake The safe level of protein intake g/kg/day
Age/years The safe level of protein intake g/kg/day Girls
11 0.90 0.9112 0.89 13 0.88 0.9014 0.87 0.8915 0.85 0.8816 0.84 0.8717 0.83 0.8618 0.82 0.85
. Recommended daily allowance for Selenium
Age (yr) RDA (µg/day) Age (yr) RDA(µg/day)
9-13 40 14-18 55
Adequate intake (AI) Calcium and Vitamin D.
Institute of Medicine, National Academies Press (US); 2011
Recommended Dietary Allowances ) For Calcium and Vit D
Tolerable Upper-
Vit D IU(µg) Calcium mg Vit D (µg) 400 (10) 1000 25 400 (10) 1500 38 600 (15) 2500 63 600 (15) 2500 75
13 years 1300 600 (15) 3000 18 1300 600 (15) 3000 100
100
level of protein intake
Girls Boys 0.90 0.91 0.89 0.90 0.88 0.90 0.87 0.89 0.85 0.88 0.84 0.87 0.83 0.86 0.82 0.85
RDA(µg/day)
Adequate intake (AI)
-level intake
Calcium mg Vit D (µg) 1000 25 1500 38 2500 63 2500 75
100 3000 100
Chapter Seven
Table 7-11: Ketocal 4:1 PowderDilution to be determined by medical presciptionDilution Powder 20 kcal/fl oz 9,5 g 24 kcal/fl oz 11,4 g 30 kcal/fl oz 14,3 g 45 kcal/fl oz 21,4 g
Table 7-12: Ketocal 3:1 PowderDilution to be determined by medical presciptionDilution Powder 20 kcal/fl oz 9,5 g 24 kcal/fl oz 11,4 g 30 kcal/fl oz 14,3 g 45 kcal/fl oz 21,4 g A gram scale is necessary for proper preparation of formulaKetocal® is the only available ketogenic diet formula in Iran
Ketocal 4:1 Powder Dilution to be determined by medical presciption
Water Final Volume 91 ml 100 ml 89 ml 100 ml 86 ml 100 ml 79 ml 100 ml
Ketocal 3:1 Powder Dilution to be determined by medical presciption
Water Final Volume 90 ml 100 ml 89 ml 100 ml 86 ml 100 ml 79 ml 100 ml
or proper preparation of formula is the only available ketogenic diet formula in Iran
101
Osmolality 110 mOsm/kg 130 mOsm/kg 170 mOsm/kg 260 mOsm/kg
Osmolality 100 mOsm/kg 120 mOsm/kg 150 mOsm/kg 240 mOsm/kg
Chapter Seven
Practical forms (checklists) in KD Form no.1 Food history This form is designed for the motherwith details. Moreover, specific questions will be asked about child nutritional habits or preferences. Based on the gathered information,appropriate idea about the daily calorie, child.
روزه غذایی
3 days of food history
Second dayروزدوم
Third day روزسوم
Practical forms (checklists) in KD therapy
is designed for the mother (family) to explain 2-3 days of child’s intake with details. Moreover, specific questions will be asked about child nutritional habits or preferences. Based on the gathered information, the dietitian will have an appropriate idea about the daily calorie, and type of KD that better suits a particular
روزه غذایی 3یادآمد 3 days of food history
Meals/ snacksه ی وعده ومیان وعد
First day روزاول
Second day روزدوم
Breakfastصبحانھ
Morning snackمیان وعده صبح
Lunch
Afternoon snackعصرانھ
Dinner
Night میان وعده شب
102
3 days of child’s intake with details. Moreover, specific questions will be asked about child nutritional habits
will have an and type of KD that better suits a particular
Meals/ snacks وعده ومیان وعد
غذا
Breakfast صبحانھ
Morning snack میان وعده صبح
Lunch ناھار
Afternoon
snack عصرانھDinner شام
Night Snack میان وعده شب
Chapter Seven
(Not picky or choosy about foods)
:کالری مصرفی کودک بر اساس شرح حال و ثبت تغذیھ ی دریافتی
)یھنظر مادرومتخصص تغذ
Which type of KD is suggested?
Explanations :والدین/توضیحات مادر
( Nutritional habits ) عادات و علایق تغذیھ ای کودک
؟بسیار بھ کربوھیدرات علاقھ دارد
Interested in carbohydrate foods?
.سیاربھ تنوع غذایی علاقھ دارد
Interested in variety and changes in the meals
(Picky eater) ھر غذایی را نمی خورد
میخورد فقط غذاھای محدودی را
Interested in limited/certain kinds of foods
(Not picky or choosy about foods) سان گیر است
Dietitian note :متخصص تغذیھ
کالری مصرفی کودک بر اساس شرح حال و ثبت تغذیھ ی دریافتی
(Daily calorie intake based on the food
نظر مادرومتخصص تغذ ( کدام نوع رژیم کتوژن را مناسب می دانید؟
Which type of KD is suggested? (Parents and dietitian’
103
توضیحات مادر
عادات و علایق تغذیھ ای کودک
بسیار بھ کربوھیدرات علاقھ دارد
Interested in carbohydrate foods?
سیاربھ تنوع غذایی علاقھ داردب
Interested in variety and changes in the meals
ھر غذایی را نمی خورد
فقط غذاھای محدودی را
limited/certain kinds of foods
سان گیر استآدر مورد غذا
متخصص تغذیھ توضیحات
کالری مصرفی کودک بر اساس شرح حال و ثبت تغذیھ ی دریافتی
(Daily calorie intake based on the food history)
کدام نوع رژیم کتوژن را مناسب می دانید؟
Parents and dietitian’s opinion)
Chapter Seven
Form no.2 Dietitian report form
Shahid Beheshti University of Medical SciencesMofid children's Hospital
Patient Code: ……..
Attending Physician: ….………….Dietitian: ………………..
Family name
Name
Mother's education
Family size
Current Address
Emergency phone number
Food or drug Allergy
What are the allergy symptoms?
Beheshti University of Medical Sciences Mofid children's Hospital
….………….
Date of Birth
Gender Family name
..... / .............. / ..
Father's education
status
Mother's education
status
Gastrointestinal disorders
Back archingFood Low appetite HeartburnThe severity
Vomiting/nausea Abdominal discomfort/pain Restlessness after eating Diarrhea Constipation
What are the allergy symptoms?
104
Father's Name Date of Birth
..... / .......
Relative relationship
between parents
Gastrointestinal disorders
Back arching Food refusal Low appetite
eartburn The severity of each symptom
Chapter Seven
105
Chapter Seven
Form no.3
Screening questions for feeding difficulties
Pediatric screening questions to identify potential feeding difficulties 1) What does your child eat on a typical da
2) What is the child usual meal time duration?
3) Where do meals take place?
4) Does your child express hunger?
5) Was your child born prematurely or with other
6) How is your child’s development, especially speech?
7) Does your child walk on grass and s
8) Does your child choke, gag or vomit with feedings?
9) Fussy or irritable only during feedings?
10) Any pocketing of foods in cheeks/mouth
Red flags: Limited food repertoire (less than 10
and textures, food rigidities
Mealtime duration >1hour, grazing, lack of mealtime structure, developmentally inappropriate feeding
methods (bottle, syringe, spoon
Distracted eating, sleep eating, inability to self
Constipation, grazing, filing up on liquids at meals, turning head away from the
presented on utensil expressing hunger
Delayed feeding milestones, frequent respiratory infections/pneumonia
Delayed milestones, autism spectrum disorder, speech apraxia child’s development, especially speech
Sensory integration dysfunction, problems with smells, wet or messy food, not able to brush teeth
Vomiting frequency, decline in participation during mealt
congestion or wet vocal quality after eating
Any signs of reflux, food allergies, Eosinophilic Esophagitis (EoE) irritable only during feedings.
Lack of endurance to chew, extended mealtimes, overstuffs mouth, and requires liquid to get food down
Screening questions for feeding difficulties
Pediatric screening questions to identify potential feeding difficulties What does your child eat on a typical day?
What is the child usual meal time duration?
Does your child express hunger?
Was your child born prematurely or with other health problems?
How is your child’s development, especially speech?
Does your child walk on grass and sand?
Does your child choke, gag or vomit with feedings?
Fussy or irritable only during feedings?
cheeks/mouth?
Limited food repertoire (less than 10-15 foods), liquid diet, inability to progress to
Mealtime duration >1hour, grazing, lack of mealtime structure, developmentally inappropriate feeding
methods (bottle, syringe, spoon-feeding of liquids
Distracted eating, sleep eating, inability to self-feed, does not participate in family
Constipation, grazing, filing up on liquids at meals, turning head away from the
expressing hunger
Delayed feeding milestones, frequent respiratory infections/pneumonia
Delayed milestones, autism spectrum disorder, speech apraxia child’s development, especially speech
Sensory integration dysfunction, problems with smells, wet or messy food, not able to brush teeth
Vomiting frequency, decline in participation during mealtimes, multiple swallows to get food down,
congestion or wet vocal quality after eating
flux, food allergies, Eosinophilic Esophagitis (EoE) irritable only during feedings.
Lack of endurance to chew, extended mealtimes, overstuffs mouth, and requires liquid to get food down
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Pediatric screening questions to identify potential feeding difficulties
15 foods), liquid diet, inability to progress to age-appropriate foods
Mealtime duration >1hour, grazing, lack of mealtime structure, developmentally inappropriate feeding
s not participate in family-style meals
Constipation, grazing, filing up on liquids at meals, turning head away from the caregiver when food is
Delayed milestones, autism spectrum disorder, speech apraxia child’s development, especially speech
Sensory integration dysfunction, problems with smells, wet or messy food, not able to brush teeth
imes, multiple swallows to get food down,
flux, food allergies, Eosinophilic Esophagitis (EoE) irritable only during feedings.
Lack of endurance to chew, extended mealtimes, overstuffs mouth, and requires liquid to get food down
Chapter Seven
Form no.4
Seizure chart/history/ (for parents) حال وضعیت سلامتی ارزیابی بیماري توسط پزشک است که معمولا شامل شرح
انتخابکودك در هاي تعیین نوع وتعداد تشنج
تان را مشاهده کند، هاي کودك از آنجا که پزشک شما ممکن است نتواند تشنج
و توصیف تشنج کودك تان توسط شما
راجع ) ند معلم یا پرستارمان(از افرادي که در هنگام تشنج کودك تان در کنار او بوده اند
.معمولا فردي که تشنج داشته قادر نیست که آن را به یاد بیاورد
خود را بسازید وبه پزشک در تشخیص و
.خواهید کرددرمان کودك خود نقش فعال ایفا
art/history/ (for parents) ارزیابی بیماري توسط پزشک است که معمولا شامل شرح مهمی از حال پزشکی بخش بسیار
تعیین نوع وتعداد تشنج. ستا ها کودك و توصیف دقیقی از ویژگی ها، زمان شروع و تعداد تشنج
از آنجا که پزشک شما ممکن است نتواند تشنج .ددرمان مناسب اهمیت حیاتی دار
مشاهده ي دقیق .ها کمک بزرگی به او خواهد کرد
از افرادي که در هنگام تشنج کودك تان در کنار او بوده اند .والدین عزیزبسیار اهمیت دارد
معمولا فردي که تشنج داشته قادر نیست که آن را به یاد بیاورد. به جزئیات حادثه سؤال کنید
خود را بسازید وبه پزشک در تشخیص و تشنج با ثبت تشنج هاي کودك خود در این جدول، شما می توانید جدول
درمان کودك خود نقش فعال ایفا به این ترتیب شما هم در تشخیص و
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حال پزشکی بخش بسیار شرح
کودك و توصیف دقیقی از ویژگی ها، زمان شروع و تعداد تشنج
درمان مناسب اهمیت حیاتی دار
ها کمک بزرگی به او خواهد کرد توصیف دقیقی از تشنج
والدین عزیزبسیار اهمیت دارد
به جزئیات حادثه سؤال کنید
با ثبت تشنج هاي کودك خود در این جدول، شما می توانید جدول
به این ترتیب شما هم در تشخیص و. درمان کمک نمایید
Chapter Seven
(Seizure chart)
شماره ي پرونده
چهارشنبه پنجشنبه جمعه
(Seizure chart) تشنج جدول: تاریخ تولد نام و نام خانوادگی
سن در هنگام اولین تشنج
)اگر تشخیص داده شده(نام بیماري یا سندرم صرع
شنبه یکشنبه دوشنبه سه شنبه
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نام و نام خانوادگی شماره ي تماس
سن در هنگام اولین تشنج سن فعلی
نام بیماري یا سندرم صرع
ساعت/تاریخ
دقیقا (توصیف تشنج )توضیح دهید
مدت تشنج تعداد
)چند بار تکرار شده( دارویا داروها
چند بار در روز (مقدار یلیچند م هر بارو
)گرم گرید یمصرف يداروها
ل تحریک تشنج عام )اگر وجود داشته(
تعداد کل تشنج در روز بیهوشی یا بی حالی
اگر بوده و ( تشنجبعد)چقدر طول کشیده
تاریخ شروع داروي جدید و نام دارو
ي گریبا رنگ د()مشخص نمایید
اگر (توضیحات دیگر )لازم است
Chapter Seven
:تشنج
(How to fill this form, guide for the parents)
یم دهینام هیعلائم اول ایکه پرودروم (را تجربه کرده بود از چیز تشنج آیا درست قبل از شروع تشنج چگونه شروع شد؟
؟بیعجیا بوي دیدن یا شنیدن چیزي از کیکدام بیاز کدام بخش بدن آغاز شد و به ترت
کودك شما در طول تشنج قادر به پاسخ دادن بود؟چرخش سر، چنگ زدن به ع،یپلک زدن سر
د؟یچرخ یم شکدام شد؟ در یم دهیپرش در بدن کودك شما د
مدفوع خود را از دست داد؟ ایکودك شما کنترل ادرار يماریل آن، ابتلاء به بثچراغ گردان، و م ون،
یا سردرد شد؟ بخشی از بدن خود، خستگی، گیجی و
تشنج راهنمایی براي پر کردن جدول
(How to fill this form, guide for the parents) قبل از بروز تشنج ، مشغول چه کاري بود؟
را تجربه کرده بود يماریدال بر ب یاز تشنج علائم شیپ يروزها ایکودك شما ساعت ها تشنج چگونه شروع شد؟ ؟يقرار یب ایاضطراب جه،یسرگ ،یخلق
دیدن یا شنیدن چیزي توهم ایدر سر دل، احساس ترس بیعج یحسمثلا ازاز کدام بخش بدن آغاز شد و به ترت یحالت نیدر بدن او مشاهده شد؟ چن يرارادیغ ایکودك شما در طول تشنج قادر به پاسخ دادن بود؟ ایآ نمود؟ ریپلک زدن سر دن،یات جولب ها، حرک دنیسینمونه ل يبرا(داشت؟ سم ؟)هدفشیچشم ها ای دیلرز یپلک کودك شما م ایآ شد؟ یم رهیخ يکودك شما به نقطه ا
پرش در بدن کودك شما د ایآ زد؟ یم ادیفر ایکرد یم هیکودك شما گر ایآ بدن کودك شما سفت شد؟ بود؟
کرد؟ رییتنفس کودك شما تغ تیوضع ایکرد؟ آ رییرنگ پوست کودك شما تغکودك شما کنترل ادرار ایآ؟ زبانش را گاز گرفت ایکودك شما لب ون،یزینور صفحه تلو ،یخوابیب مانند(تشنج وجود داشت يبرا
؟ییغذا ي ه، قطع مصرف دارو، حذف وعدمثل سرماخوردگی یا تببخشی از بدن خود، خستگی، گیجی و کودك شما دچار ضعف موقت در
آسیبی به کودك رسید؟ آیا در اثر تشنج این علائم چقدر طول کشید؟ .لطفاتاریخ شروع داروي جدید یا رژیم کتوژن را با رنگ دیگري مشخص کنید
109
(How to fill this form, guide for the parents) قبل از بروز تشنج ، مشغول چه کاري بود؟ "کودك شما دقیقا
کودك شما ساعت ها ایآخلق راتییمانند تغ) شوند
مثلا از خاصی شکایت کرد؟ی یرمعمولیحرکت غ ایآ
یبدن را درگ يقسمت هاسمیکودك شما اتومات ایآ
هدف یلباس، راه رفتن بکودك شما به نقطه ا ایآبدن کودك شما سفت شد؟ ایآ
بود؟ شتریسمت بدن او برنگ پوست کودك شما تغ ایآایکودك شما افتاد؟ آ ایآبرا ندهانزیبرانگ اعواملیآ
مثل سرماخوردگی یا تب دیجدکودك شما دچار ضعف موقت در آیا پس از تشنج
این علائم چقدر طول کشید؟لطفاتاریخ شروع داروي جدید یا رژیم کتوژن را با رنگ دیگري مشخص کنید
Chapter Seven
Form no. 5 Refractory seizure/epilepsy assessment form (for physicians)
Name Age
Epileptic syndrome or any other neurologic/ non
If known
under investigation
Explanation
Current seizure type/s*
If a mixed type, what type is prominent?
Current seizure duration and frequency
What time during the day? At nights? On awakening? etc.
Is aura present? Explain the aura
Is there a cluster of seizures?
Any triggers (light, fasting, etc.)
Current developmental abilities (abilities in each area)
Any visual/hearing issue? Any skin, GI, gonadal or other systemic findings?
General physical examination
Refractory seizure/epilepsy assessment form (for physicians)
Age Date of birth
Epileptic syndrome or any other neurologic/ non-neurologic underlying etiology
Explanation
110
Refractory seizure/epilepsy assessment form (for physicians)
Gender
neurologic underlying etiology
111
Neurologic examination Consciousness and awareness Cranial nerve DTR Tone Strength Cerebellar test Primitive reflex/s
Any focal neurologic finding
Any movement disorder LTM Report Results of other consults (if any)
Previous Treatments/medication, VNS, KD, Surgery, IVIg, plasmapheresis
*Please specify the type: Absence, atypical absence, myoclonic, focal with or without changing in awareness, (where the focality is?), GTC, mixed type, drop attacks, head drops, atonic seizures, tonic, focal tonic, focal clonic, Focal with secondary generalization, autonomic symptoms, infantile spasm (flexion/extension/mixed or start with focality)
Chapter Seven
Form no 6.
Past medical history (for physicians)
Past medical history assessment ExplanationNeonatal History Birth weight / Type of delivery Head circumference Any issue during pregnancy Apgar score/resuscitation Neonatal seizure Any admission/etiology (Check discharge summary and investigations)Any traumatic events in the neonatal Developmental History Age at walking Age at talking Slow progression / regression Delay:(which area) When did regression or slowing start?Vision/hearing issues? Seizure history Age at first seizure Type of first seizure Any neonatal seizure Please specify all type of previous seizures:Any history of status epilepticus? Any febrile status? How many times of admission?When was the last time the seizure was under control? For how long?medication/s?
Family history Any seizure disorder in the familyWhich family member/s has been affected?Did the family member have easily contorted seizure?Consanguineous Parents? What type?Any neurologic disorder/mental retardation in the family?Any undiagnosed disease in the family?Any non-neurologic issue in the family?Any abortion, stillbirth, neonatal death in the family? Any surgery in the past Any hospital admission? Any other systemic disease? Any trauma, especially brain and spine?
Past medical history (for physicians)
Past medical history assessment Explanation
Birth weight / Type of delivery Head circumference
d investigations) neonatal period?
Age at walking
did regression or slowing start?
Any neonatal seizure Please specify all type of previous seizures: Any history of status epilepticus?
How many times of admission? he last time the seizure was under control? For how long? With which
Any seizure disorder in the family Which family member/s has been affected? Did the family member have easily contorted seizure?
? What type? Any neurologic disorder/mental retardation in the family? Any undiagnosed disease in the family?
neurologic issue in the family? , neonatal death in the family?
Any trauma, especially brain and spine?
112
With which
Chapter Seven
Form no.7 Pre KD checklist for physicians
Pre-KD checklist
Date:
Name Date of birth Gender
Suggested date/age for KD initiation
Referred for KD because of:
metabolic disorder multiple AEDs
Other (please specify):
Epileptic syndrome or underlying etiology if known
Based on: Investigation/
assessment Details
□ Growth assessment/
Any Failure to thrive
Weight/height, BMI, Z score, head circumference
□ 3 days food history record and national preferences (dietitian report)
Check if the form is filled
□ Feeding assessment/Dysphasia /Required G-tube? Dietitian or GI report
Feeding difficulty screening form results (please explain),GI assessment if swallowing difficulties or recurrent aspiration
□ Suggested KD type Classic, MCT, Atkins, LGI
□ Suggested ratio/MCT percentage/CHO g per day to start
This depKD type, treatment goal and response/patient tolerance
□ Systemic disease Any heart/Unresolved GI symptoms/Kidney infection, disease, stone/ Osteopenia
for physicians
Name Date of birth Gender
Suggested date/age for KD initiation
Referred for KD because of: Refractory seizure
metabolic disorder multiple AEDs non-tolerance/allergy
Epileptic syndrome or underlying etiology if known:
Details Results & date that is done
Weight/height, BMI, Z score, head circumference
Check if the form is filled
Feeding difficulty screening form results (please explain), GI assessment if swallowing difficulties or recurrent aspiration
Classic, MCT, Atkins, LGI
This depends on the KD type, treatment goal and response/patient tolerance
Any heart/Unresolved GI symptoms/Kidney infection, disease, stone/ Osteopenia
113
e that is Explanation
114
□ Hematology full panel WBC, Hg, HCT, MCV,
MCH, MCHC, Plt count
□ Electrolytes/ anion gap Na,K, Ca, P,Mg,VBG,
BS, (serum bicarbonate),chloride, Anion gap. Uric acid, Bun/Cr. Zinc &selenium
□ Liver enzyme /function
SGPT, SGOT, Alp, LDH, PT, INR, Bilirubin, Albumin, total protein
□ Fasting Lipid profile TG, Cholesterol:
LDL/HDL
□ Urine exam U/A, Urine calcium/cr ratio
□ R/O Neuro-metabolic Serum Carnitine and
acyl Carnitine profile Free fatty acids: SCFAs, VSCFAs, LCFAs, VLCFAs
□ R/O Neuro-metabolic Urine organic acid/serum amino acids
□ Please add any other metabolic study if any
Ammonia, Lactate, pyruvate HPLC: Blood amino acids, Urine amino acids,
□ EEG( recent) Any abnormality, focal findings (where), generalized, Please specify
□ Neuroimaging (recent) MRI, MRS, CT Scan, etc.
Please specify if focal (wherein the brain) /what the lesion is any contrast study? Any generalized finding
□ Any neurophysiology study (if done)
VEP, ABR, EMG, etc.
□ CSF study (if done) Glucose, lactase, pyruvate, protein, neurotransmitter
□ Genetic study (if done) Please specify the method and findings and where it is done
115
□ Current AEDs* (also, level if available)
Dose and numbers per day/ how long patient is on these medications/ Level if done
□ Current seizure status/type and frequency per day
Seizure table is helpful. Please specify all types of the seizure □ that is happening
□ When was the last seizure
Type/duration/frequency
□ Current developmental abilities in the motor, language, and mental areas
Explain the abilities and specify if appropriate with age/delay (in which area)/regression
□ Refractory seizure form Check if the form is filled
□ Neurology examination findings
Check if complete already (refractory seizure form) or now
□ The result of all consultations
Ophthalmology, GI, Cardiology×, Endocrine, etc.
□ Renal ultrasound if any (optional baseline)
especially if the history of renal stone in the family
*Please consider tapering Phenobarbital and Primidone before KD initiation, if possible.
Patients on Valproic acid should not be started on the MCT diet.
Patients on Valproic acid are at risk for Carnitine deficiency, and this may worsen during KD.
Patients on Topiramate and Zonisamide are at risk of metabolic acidosis and renal stones, may
worsen during KD.
Please make a note with a different color if a patient is on these medications to remember the
risk s and consider closer follow up, discontinuation, extra caution such as blood work,
ultrasound or early treatment with L-Carnitine or Polycitra-K.
Contraindicated if there is a history of cardiac disease in the family such as arrhythmia,
cardiomyopathy. ECG and Echocardiography may be considered.
□ Absence, atypical absence, myoclonic, focal with or without decreased awareness, (where the
facility is), GTC, Mixed type, drop attacks, Atonic seizures, Tonic, Focal tonic, Focal with secondary
generalization. Is there any aura or postictal, which type of aura and how long aura or postictal last?
▪ Zinc and selenium check if possible.
Chapter Seven
Form no.8 Physician note on KD follow up
Post KD assessment Date Name Months after diet initiation: Diagnosis: Seizure status (seizure chart) Height/weight/BMI, head circumference in infants (Growth chart) Consciousness and awareness New Blood work results With date Any GI symptoms/complaints Any infection/Fever Any complaints Please specify side effects Physical exam General exam Neuro exam New intervention Change in diet Change in AEDs Add Policytra (reason?) Add L-Carnitine (reason?) Any new consult (GI, Nephro..etc) Dietitian report
ician note on KD follow up
Date Name Date of birth Current AgeMonths after diet initiation:
Explanation
116
urrent Age Gender
Chapter Seven
Form no. 9 AEDs history
Medication information
Medication Name
Highest Dose, if ever reached full dose?
Proper for the seizure type?
Form no.10
Investigation results
Investigation results Date:
General Explanation
BS, Bun, Cr, Ammonia, Lactate, pyruvate, Na, K, P, Ca, Mg, VBG, CK, Liver enzymes and function Please add others
Abdominal ultrasound Chest x-ray/bone survey if done
Any biopsy Genetic study if done
Echocardiography if done
Form no. 9 AEDs history
Proper for the seizure
Duration with full dose
Allergy/rash/reaction/ Intolerance; side effects, non-compliant
Nonmedications
General Explanation Neurologic Explanation
Neurometabolic Study: HPLC Amino acids (blood, urine)Urine organic acids Please add others
CSF study Neuroimaging (MRI, MRS, CTScan), etc. EEG report Electrophysiology: EMG, NCV, ABR, VEP, etc. Free and acyl Carnitine level
117
Non-seizure medications
Why discontinued?
Neurologic Explanation
HPLC Amino acids (blood, urine)
Neuroimaging (MRI, MRS, CT
Electrophysiology: EMG, NCV,
Chapter Seven
Recommended Investigations and
1) Investigations for Patients on KD
This is for non-complicated cases. In every patient, decisions should be made based on his/her specific patient condition. Urinary ketones every week in the first month · After stable ketosis once per week (th
visit/6 month's visit, or if seizure recurs
Blood ketones · Blood ketones will be checked if no urinary ketosis or urinary ketones is positive with no seizure control· In infants (<2 years of age) blood ketones should be checked at least twice during
the diet, (first month) and should not exceed 5 mmol/l.
Mandatory blood work at first month: · Full blood count including platelets· Renal profile (includes sodium, potassiu· Liver profile (enzymes and function)· Calcium, Magnesium · Uric acid · Glucose · Fasting Lipid profile · Carnitine (Free and Total) · U/A, Ca/Cr ratio
Mandatory blood work at 3rd month, then every 3 months in the f· Full blood count including platelets· Renal profile (includes sodium, potassium, urea, creatinine, bicarbonate (VBG), and albumin)· Liver profile (enzymes and function)· Calcium, Magnesium · Fasting Lipid profile · Carnitine (Free and total) · U/A, Ca/Cr ratio · Vitamin D level
Recommended Investigations and intervals
Investigations for Patients on KD
complicated cases. In every patient, decisions should be made based on his/her
Urinary ketones every week in the first month
After stable ketosis once per week (the second month) and then just during illness, before the visit, or if seizure recurs
Blood ketones will be checked if no urinary ketosis or urinary ketones is positive with no seizure controllood ketones should be checked at least twice during
irst month) and should not exceed 5 mmol/l.
Mandatory blood work at first month: Full blood count including platelets Renal profile (includes sodium, potassium, urea, creatinine, bicarbonate (VBG), and albumin)Liver profile (enzymes and function)
month, then every 3 months in the first year:Full blood count including platelets Renal profile (includes sodium, potassium, urea, creatinine, bicarbonate (VBG), and albumin)Liver profile (enzymes and function)
118
complicated cases. In every patient, decisions should be made based on his/her
e second month) and then just during illness, before the third-month
Blood ketones will be checked if no urinary ketosis or urinary ketones is positive with no seizure control lood ketones should be checked at least twice during the induction phase of
m, urea, creatinine, bicarbonate (VBG), and albumin)
irst year:
Renal profile (includes sodium, potassium, urea, creatinine, bicarbonate (VBG), and albumin)
Chapter Seven
· Zinc, copper (optional) · Clotting screening (Infants) · Vitamin C, Vitamin B 12, Ferritin (Optional, Infants)· Vitamins A, D, E level (Optional, Infants)
The same investigations should be done every 6 months in theInvestigations that are suggested to be done after one year (and yearly · Renal Ultrasound · Bone mineral density (DEXA scan) (After the second year)· Zinc, selenium, copper · Vitamin D level · Vitamin C, Vitamin B 12, Ferritin (Infants)· Vitamins A, D, E level (Infants)· Free fatty acids (Infants)
Vitamin C, Vitamin B 12, Ferritin (Optional, Infants) Vitamins A, D, E level (Optional, Infants)
The same investigations should be done every 6 months in the second year. Investigations that are suggested to be done after one year (and yearly afterward
Bone mineral density (DEXA scan) (After the second year)
Vitamin C, Vitamin B 12, Ferritin (Infants) Vitamins A, D, E level (Infants)
119
afterward):
References
· Bergqvist C. Dietary treatments and bone health. In Neal E. Practical Implementation of Ketogenic
Therapy. First Edition. 2012. Wiley & Sons · Bergqvist C, Chee CM, Lutchka
complication of the Ketogenic diet. Epilepsia. 2003. 44: 610· Chaffe H.: Ketone monitoring and management of illness. In Neal E.
Epilepsy: Practical Implementation of Ketogenic Therapy. First Edition.2012. Wiley & Sons. · Edmonton Epilepsy Association, a · Gebhardt SE, Thomas RG. Nutritive Value of Foods. Washin
Agriculture, Agricultural Research Service. · Geil Patti Bazel, Choose Your Foods: Exchange Lists for Diabetes, American Dietetic Association,
and American Diabetes Association; 2008· Kossoff EH, Zupec-Kania BA, Amark PE et
the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group.2009. Epilepsia, 50:304–317
· Neal E. Monitoring and side effects in Dietary Treatment of Epilepsy: Practical ImplemenKetogenic Therapy, First Edition. 2012. Wiley & Sons
· Neal EG, B. Zupec-Kania B, Pfeifer HH. Carnitine, nutritional supplementation, discontinuation of ketogenic diet therapies. Epilepsy Research. 2012 100: 267
· Neal E, Carnitine in DietaryKetogenic Therapy, First Edition. 2012. Wiley & Sons
· Neal E, Gwyneth Magrath and Elizabeth Neal Classic Ketogenic diet In Neal E. Dietary Treatment of Epilepsy: Practical Implementation of Ketog
· Soysal E, Gries H., Wraya C.: Pediatric patients on the anesthesia—a medical record review. Journal of Clinical Anesthesia, 170
· The Charlie Foundation for Ketogenictools/resources-2/low-carb/item/1137
· van Delft R, Lambrechts D, Verschuure P. Blood betaseizure reduction due to ketogenic diet than do ketones in the urine.2010. Seizure 19: 36
· Van der Louw E, Van den Hurk D, Neal E. et al. Ketogenicrefractory epilepsy. Epilepsy. 2016. 20: 798
· Welcome to Ketomeds, A source of information for carbohydrate content in medications (http://www.ketomeds.com/
Bergqvist C. Dietary treatments and bone health. In Neal E. Practical Implementation of Ketogenic Edition. 2012. Wiley & Sons
, Lutchka L, et. al. Selenium deficiency associated with cardiomyopathy: A complication of the Ketogenic diet. Epilepsia. 2003. 44: 610-620 Chaffe H.: Ketone monitoring and management of illness. In Neal E. Epilepsy: Practical Implementation of Ketogenic Therapy. First Edition.2012. Wiley & Sons. Edmonton Epilepsy Association, a guide for parents, Epilepsy education series, 2011. Gebhardt SE, Thomas RG. Nutritive Value of Foods. Washington, DC. 2002. U.S. Department of Agriculture, Agricultural Research Service.
Your Foods: Exchange Lists for Diabetes, American Dietetic Association, American Diabetes Association; 2008
Kania BA, Amark PE et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group.2009.
Neal E. Monitoring and side effects in Dietary Treatment of Epilepsy: Practical ImplemenKetogenic Therapy, First Edition. 2012. Wiley & Sons
Kania B, Pfeifer HH. Carnitine, nutritional supplementation, discontinuation of ketogenic diet therapies. Epilepsy Research. 2012 100: 267Neal E, Carnitine in Dietary Treatment of Epilepsy In Neal E. Practical Implementation of Ketogenic Therapy, First Edition. 2012. Wiley & Sons Neal E, Gwyneth Magrath and Elizabeth Neal Classic Ketogenic diet In Neal E. Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy, First Edition. 2012 Wiley & Sons
C.: Pediatric patients on the ketogenic diet undergoing a medical record review. Journal of Clinical Anesthesia, 170–175, 2016.
The Charlie Foundation for Ketogenic therapies: https://www.charliefoundation.org/resourcescarb/item/1137-carbohydrate-non-carbohydrate-ingredients
van Delft R, Lambrechts D, Verschuure P. Blood beta-hydroxybutyrate correlates better with seizure reduction due to ketogenic diet than do ketones in the urine.2010. Seizure 19: 36Van der Louw E, Van den Hurk D, Neal E. et al. Ketogenic diet guidelines for infants with refractory epilepsy. Epilepsy. 2016. 20: 798-809 Welcome to Ketomeds, A source of information for carbohydrate content in medications http://www.ketomeds.com/)
120
Bergqvist C. Dietary treatments and bone health. In Neal E. Practical Implementation of Ketogenic
Selenium deficiency associated with cardiomyopathy: A
Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy. First Edition.2012. Wiley & Sons.
for parents, Epilepsy education series, 2011. gton, DC. 2002. U.S. Department of
Your Foods: Exchange Lists for Diabetes, American Dietetic Association,
al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group.2009.
Neal E. Monitoring and side effects in Dietary Treatment of Epilepsy: Practical Implementation of
Kania B, Pfeifer HH. Carnitine, nutritional supplementation, and discontinuation of ketogenic diet therapies. Epilepsy Research. 2012 100: 267—271
Treatment of Epilepsy In Neal E. Practical Implementation of
Neal E, Gwyneth Magrath and Elizabeth Neal Classic Ketogenic diet In Neal E. Dietary Treatment enic Therapy, First Edition. 2012 Wiley & Sons
diet undergoing general 175, 2016.
https://www.charliefoundation.org/resources-ingredients
hydroxybutyrate correlates better with seizure reduction due to ketogenic diet than do ketones in the urine.2010. Seizure 19: 36–39
diet guidelines for infants with
Welcome to Ketomeds, A source of information for carbohydrate content in medications
Chapter Eight
KD in
Mechanism of action KD is effective in neurometabolic disorders through different mechanisms.
1) It can bypass the aberrant metabolic pathway and prevent the production and accumulation of toxic substances.
2) Ketone bodies also provide an appropriate energy fuel for the developing brain. 3) KD can improve the clinical symptoms of these
cognitive issues.
Applying KD in neurometabolic disorders is similar of treatment is required. KD should be started as soon as the diagnosis is made and maintained until adolescent to provide enough energy for the developing brain and protect it from metabolic insult. Therefore, these patients are vulnerable to the longbone density issues. Patients should have regular monitoring and supplementations. (Please see term side effects, chapter 5) Important tips
In neurometabolic KD patients, specific ratio and diet type should be considered based on the age. This is particularly important to meet RDA in different ages and maintain the compliance. For example, 3:1 ratio will be sufficient in infancy to provide appropriate propreschool and school children 3:1 to 4:1 ratio is acceptable and in older children and adolescents MAD is recommended.
It is very critical to remember that in some metabolic disorders such as urea cycle defects high and/or non-selectiveness of protein content can lead to hyper not an option. In contrast, MAD is a proper alternative in patients with GLUT1In PDHD, KCD is also preferred.
Of note, KD mimicking the fasting without catabolicatabolic states can be easily evoked by avoiding food or water restriction. Thus, the goal is to achieve ketosis without having patients fall into a Patients will benefit from a sufficient fluid intake, and appropriate protein content in the diet.
Well-known metabolic disorders treated by KD include glucose transporter type 1 (GLUT1) deficiency and pyruvate dehydrogenase deficiency (PDHD) that will be briefly discussed in this chapter.
Neurometabolic disorders in which KD has been successfully tried are summarized in (Please also see Table 1-1 and Table 1
KD in Neurometabolic Disorders
KD is effective in neurometabolic disorders through different mechanisms. It can bypass the aberrant metabolic pathway and prevent the production and accumulation of
Ketone bodies also provide an appropriate energy fuel for the developing brain. KD can improve the clinical symptoms of these diseases, for instance, refractory seizure and
Applying KD in neurometabolic disorders is similar to the refractory seizures, however: a treatment is required. KD should be started as soon as the diagnosis is made and maintained until
adolescent to provide enough energy for the developing brain and protect it from metabolic insult. fore, these patients are vulnerable to the long-term side effects such as growth retardation and
Patients should have regular monitoring and supplementations. (Please see
bolic KD patients, specific ratio and diet type should be considered based on the age. This is particularly important to meet RDA in different ages and maintain the compliance. For example, 3:1 ratio will be sufficient in infancy to provide appropriate propreschool and school children 3:1 to 4:1 ratio is acceptable and in older children and adolescents
It is very critical to remember that in some metabolic disorders such as urea cycle defects high eness of protein content can lead to hyper ammonia, therefore
not an option. In contrast, MAD is a proper alternative in patients with GLUT1In PDHD, KCD is also preferred. Of note, KD mimicking the fasting without catabolism. In patients with metabolic disorders, catabolic states can be easily evoked by avoiding food or water restriction. Thus, the goal is to achieve ketosis without having patients fall into a catabolic state which is
t from a gradual increase in the ratio (over several weeks or even months), sufficient fluid intake, and appropriate protein content in the diet.
known metabolic disorders treated by KD include glucose transporter type 1 (GLUT1) te dehydrogenase deficiency (PDHD) that will be briefly discussed in this
disorders in which KD has been successfully tried are summarized in Table 1-4 in chapter one)
121
It can bypass the aberrant metabolic pathway and prevent the production and accumulation of
Ketone bodies also provide an appropriate energy fuel for the developing brain. instance, refractory seizure and
to the refractory seizures, however: a long period treatment is required. KD should be started as soon as the diagnosis is made and maintained until
adolescent to provide enough energy for the developing brain and protect it from metabolic insult. term side effects such as growth retardation and
Patients should have regular monitoring and supplementations. (Please see long-
bolic KD patients, specific ratio and diet type should be considered based on the age. This is particularly important to meet RDA in different ages and maintain the compliance. For example, 3:1 ratio will be sufficient in infancy to provide appropriate protein for growth. In preschool and school children 3:1 to 4:1 ratio is acceptable and in older children and adolescents
It is very critical to remember that in some metabolic disorders such as urea cycle defects high therefore, MAD or LGI is
not an option. In contrast, MAD is a proper alternative in patients with GLUT1-DS and GSD IIIa.
sm. In patients with metabolic disorders, catabolic states can be easily evoked by avoiding food or water restriction. Thus, the goal is to
state which is life-threatening. (over several weeks or even months),
known metabolic disorders treated by KD include glucose transporter type 1 (GLUT1) te dehydrogenase deficiency (PDHD) that will be briefly discussed in this
disorders in which KD has been successfully tried are summarized in Table 8-1
Chapter Eight
Table 8-1. Neurometabolic disorders and successful treatment with Ketogenic diet
GABA metabolism disorders
GSDs
Mitochondrial disorders
Non-ketotic hyperglycinemia
Phosphofructokinase (PFK) deficiency
Purine metabolism
Urea cycle disorders
KD has been tried for neurodegenerative disorders such as Alzheimer’s, Parkinson’s, ALS or evtraumatic brain injury mostly for its neuroprotective effect, which is beyond the scope of this book.
1. Neurometabolic disorders and successful treatment with Ketogenic
GABA metabolism disorders Succinic semialdehyde dehydrogenase (SSADH)deficiency (in Mice)
GSD III GSD V (McArdle's disease)
*Pyruvate dehydrogenase complex (PDHC) deficiency*Mitochondrial DNA depletion syndromes (MDS):Such POLG-related disorders*Disorders of mitochondrial transcription and translation such as MELAS*Disorders of the mitochondrial respiratory chain (MRC) such as Isolated Complex I deficiency (NADH ubiquinone oxidoreductase deficiency) Isolated complex II deficiencyComplex IV deficiency andComplex I/IV deficiency
Significant improvement in seizure decreased glycine level
sphofructokinase (PFK) deficiency
Adenylosuccinate lyase (ADSL) deficiency
Argininosuccinate lyase (ASL) deficiency
KD has been tried for neurodegenerative disorders such as Alzheimer’s, Parkinson’s, ALS or evtraumatic brain injury mostly for its neuroprotective effect, which is beyond the scope of this book.
122
1. Neurometabolic disorders and successful treatment with Ketogenic
Succinic semialdehyde dehydrogenase (SSADH)
GSD V (McArdle's disease)
dehydrogenase complex (PDHC) deficiency *Mitochondrial DNA depletion syndromes (MDS):
related disorders *Disorders of mitochondrial transcription and translation such as MELAS *Disorders of the mitochondrial respiratory chain
ted Complex I deficiency (NADH ubiquinone oxidoreductase deficiency) Isolated complex II deficiency Complex IV deficiency and
Significant improvement in seizure frequency
Adenylosuccinate lyase (ADSL) deficiency
Argininosuccinate lyase (ASL) deficiency
KD has been tried for neurodegenerative disorders such as Alzheimer’s, Parkinson’s, ALS or even traumatic brain injury mostly for its neuroprotective effect, which is beyond the scope of this book.
Chapter Eight KD and GLUT1deficiency GLUT1 deficiency patients have mutations in the SLC2A1 gene encoding Glut1 protein. This defect results in failure of glucose transport into the brain. Ketone bodies can enter the brain via a different mechanism and serve as an alternative fuel. Therefore, KD is the most effective treatment for these patients, although it may not control all symptoms CKD can provide significant seizure control and movement disorder improvement. Furthermore, early diagnosis and treatment were reported to improve the outcome; first 1 to 6 months after birth has been suggested as the best time that diethis period damage is not reversible. Usually, after diet initiation patients will be able to gain some cognitive skills. MAD is also reported has effects option for older children or teenagers. This means CKD can be switched to MAD later in the treatment course. As we mentioned earlier, these patients are vulnerable to because of long-standing therapy and should have regulchapter 5) LGI is not an option for these patients because of the insufficient level of ketosis. Triheptanoin is a triglyceride with three 7deficiency. It can enhance the TCAGLUT1 and fatty acid oxidation defects and KD is considered the first choice of therapy. It is worth to remember that a widedeficiency from refractory epilepsy in infancy to movement disorder/dystonia in childhood or paroxysmal exertional dyskinesia in late childhood/adolescent.
KD and PDHD PDHD is a severe mitochondrial pyruvate into acetyl-CoA through the tricarboxylic acid cycle. Pyruvate instead is metabolized to lactate and production of adenosine triphosphatase (ATP) via the mitochondrial respiratory chain isimpaired. This disorder has different phenotypes; commonly with a neurodegenerative nature contractor to GLUT1. Patients usually receive other medications such as cofactor replacement with thiamine, activation of PDC with dichloroacetate, carnitine or antsurvival rate has been improved in recent reports compare to the past; however, many factors other than ketogenic diet therapy are involved in the male gender will have a higher mortality rate. Like GLUT1 deficiency, there is a wide spectrum of PDC deficiency phenotypes includingencephalopathy and/or seizures, and isolated neuropathy with normal cognitive functioning. After KD initiation, Ketone bodies can provide an alternative fuel for the brain and low carbohydrate content of the diet will decrease the lactate and pyruvate production. Seizure improvement after KD is well established. There are some reports about the sleep disturbance, speech/language development, social functioning, and frequency of hospitalizations. Important factors related to diet efficacy was reported as: 1) Disease phenotype (less efficacy in prenatal cases because brain damage already started in utero). 2) Achieving and maintaining ketosis: clinically effective blood ketone level was reported at 3mmol/l.
Starting diet early in life with greater carbohydratneurologic outcome and mental development. To our knowledge, the only choice that should be used in this disease and none of the
GLUT1 deficiency patients have mutations in the SLC2A1 gene encoding Glut1 protein. This esults in failure of glucose transport into the brain. Ketone bodies can enter the brain via a
mechanism and serve as an alternative fuel. Therefore, KD is the most effective treatment these patients, although it may not control all symptoms in a given patient.
CKD can provide significant seizure control and movement disorder improvement. Furthermore, early diagnosis and treatment were reported to improve the outcome; first 1 to 6 months after birth has been suggested as the best time that diet initiation may improve neurologic outcome and after this period damage is not reversible. Usually, after diet initiation patients will be able to gain some
on both on seizure and movement disorders and option for older children or teenagers. This means CKD can be switched to MAD later in the treatment course. As we mentioned earlier, these patients are vulnerable to long
therapy and should have regular follow up. (See long
LGI is not an option for these patients because of the insufficient level of ketosis. Triheptanoin is a triglyceride with three 7-carbon fatty acids used in animal studies of GLUT1
TCA cycle to produce energy; its usage is still under investigation in GLUT1 and fatty acid oxidation defects and KD is considered the first choice of therapy.
wide spectrum of phenotypes has been recognized for a deficiency from refractory epilepsy in infancy to movement disorder/dystonia in childhood or paroxysmal exertional dyskinesia in late childhood/adolescent.
PDHD is a severe mitochondrial disorder that prevents glucose from being metabolized from through the tricarboxylic acid cycle. Pyruvate instead is metabolized to adenosine triphosphatase (ATP) via the mitochondrial respiratory chain is
impaired. This disorder has different phenotypes; commonly with a neurodegenerative nature contractor to GLUT1. Patients usually receive other medications such as cofactor replacement with thiamine, activation of PDC with dichloroacetate, carnitine or antiepileptic medications. survival rate has been improved in recent reports compare to the past; however, many factors other than ketogenic diet therapy are involved in the outcome, for instance, neonatal or infantile onset and
mortality rate. Like GLUT1 deficiency, there is a wide spectrum of including neonatal death, hypotonia, developmental delay,
encephalopathy and/or seizures, and childhood-onset intermittent ataxia/episodic dystonia orisolated neuropathy with normal cognitive functioning. After KD initiation, Ketone bodies can provide an alternative fuel for the brain and low carbohydrate content of the diet will decrease the lactate and pyruvate production. Seizure
KD is well established. There are some reports about the positivesleep disturbance, speech/language development, social functioning, and frequency of
Important factors related to diet efficacy was reported as:
ase phenotype (less efficacy in prenatal cases because brain damage already started in utero). 2) Achieving and maintaining ketosis: clinically effective blood ketone level was reported at 3
Starting diet early in life with greater carbohydrate restriction has been suggested to improve neurologic outcome and mental development. To our knowledge, the classic ketogenic diet is the only choice that should be used in this disease and none of the KD alternatives is indicated
123
GLUT1 deficiency patients have mutations in the SLC2A1 gene encoding Glut1 protein. This esults in failure of glucose transport into the brain. Ketone bodies can enter the brain via a
mechanism and serve as an alternative fuel. Therefore, KD is the most effective treatment in a given patient.
CKD can provide significant seizure control and movement disorder improvement. Furthermore, early diagnosis and treatment were reported to improve the outcome; first 1 to 6 months after birth
t initiation may improve neurologic outcome and after this period damage is not reversible. Usually, after diet initiation patients will be able to gain some
on both on seizure and movement disorders and would be an option for older children or teenagers. This means CKD can be switched to MAD later in the
long-term side effects long-term side effects on
LGI is not an option for these patients because of the insufficient level of ketosis. carbon fatty acids used in animal studies of GLUT1
cycle to produce energy; its usage is still under investigation in GLUT1 and fatty acid oxidation defects and KD is considered the first choice of therapy.
spectrum of phenotypes has been recognized for a GLUT1 deficiency from refractory epilepsy in infancy to movement disorder/dystonia in childhood or
being metabolized from through the tricarboxylic acid cycle. Pyruvate instead is metabolized to adenosine triphosphatase (ATP) via the mitochondrial respiratory chain is
impaired. This disorder has different phenotypes; commonly with a neurodegenerative nature contractor to GLUT1. Patients usually receive other medications such as cofactor replacement with
iepileptic medications. The overall survival rate has been improved in recent reports compare to the past; however, many factors other
, for instance, neonatal or infantile onset and mortality rate. Like GLUT1 deficiency, there is a wide spectrum of
neonatal death, hypotonia, developmental delay, intermittent ataxia/episodic dystonia or
After KD initiation, Ketone bodies can provide an alternative fuel for the brain and low carbohydrate content of the diet will decrease the lactate and pyruvate production. Seizure
positive effect on ataxia, sleep disturbance, speech/language development, social functioning, and frequency of
ase phenotype (less efficacy in prenatal cases because brain damage already started in utero). 2) Achieving and maintaining ketosis: clinically effective blood ketone level was reported at 3–3.5
e restriction has been suggested to improve ketogenic diet is the
alternatives is indicated.
References
· DeBrosse S. D., Okajima K., Zhang S., Nakouzi G., Schmotzer C.L., LuskM.B., Grahame G., Kerr D.S. Spectrum of neurological and survival outcomes in pyruvate dehydrogenase complex (PDC) deficiency: Lack of correlation with genotype. Moleculand Metabolism 107; 394–402, 2012
· Klepper J., Ketogenic dietary therapy in neurometabolic Practical Implementation of Ketogenic Therapy
· Leen W.G., Mewasingh L., Verbeek M.M., Kamsteeg E., van de Warrenburg B.P., Willemsen M.A. Movement Disorders in GLUT1 Deficiency Syndrome Respond to the Modified Atkins Diet. Movement Disorders, Vol. 28, No. 10, 2013
· Paoli A., Bianco A., Damiani E.,Bosco G., Ketogenic Diet Neurodegenerative Diseases, BioMed Research International, July 2014
· Scholl-Bürgi S., Höller A, Pichler k., Michel M., Haberlandt, E., Karall. D. Ketogenic diets in patients with inherited metabolic disorders
· Sofou K., Dahlin M., Hallböök T., Lindefeldt M., Viggedal G., Darin N. Ketogenic diet in pyruvate dehydrogenase complex deficiency: short245, 2017
osse S. D., Okajima K., Zhang S., Nakouzi G., Schmotzer C.L., Lusk-Kopp M., Frohnapfel M.B., Grahame G., Kerr D.S. Spectrum of neurological and survival outcomes in pyruvate dehydrogenase complex (PDC) deficiency: Lack of correlation with genotype. Molecul
402, 2012 Klepper J., Ketogenic dietary therapy in neurometabolic diseases Dietary Treatment of Epilepsy: Practical Implementation of Ketogenic Therapy, by Elizabeth Neal, John Wiley & Sons, Ltd.2012
h L., Verbeek M.M., Kamsteeg E., van de Warrenburg B.P., Willemsen M.A. Movement Disorders in GLUT1 Deficiency Syndrome Respond to the Modified Atkins Diet. Movement Disorders, Vol. 28, No. 10, 2013 Paoli A., Bianco A., Damiani E.,Bosco G., Ketogenic Diet in Neuromuscular and Neurodegenerative Diseases, BioMed Research International, July 2014
Bürgi S., Höller A, Pichler k., Michel M., Haberlandt, E., Karall. D. Ketogenic diets in patients with inherited metabolic disorders, J Inherit Metab Dis 38:765–773, 2015Sofou K., Dahlin M., Hallböök T., Lindefeldt M., Viggedal G., Darin N. Ketogenic diet in pyruvate dehydrogenase complex deficiency: short- and long-term outcomes. J Inherit Metab Dis. 40:237
124
Kopp M., Frohnapfel M.B., Grahame G., Kerr D.S. Spectrum of neurological and survival outcomes in pyruvate dehydrogenase complex (PDC) deficiency: Lack of correlation with genotype. Molecular Genetics
Dietary Treatment of Epilepsy: Elizabeth Neal, John Wiley & Sons, Ltd.2012
h L., Verbeek M.M., Kamsteeg E., van de Warrenburg B.P., Willemsen M.A. Movement Disorders in GLUT1 Deficiency Syndrome Respond to the Modified Atkins Diet.
in Neuromuscular and
Bürgi S., Höller A, Pichler k., Michel M., Haberlandt, E., Karall. D. Ketogenic diets in 773, 2015
Sofou K., Dahlin M., Hallböök T., Lindefeldt M., Viggedal G., Darin N. Ketogenic diet in pyruvate term outcomes. J Inherit Metab Dis. 40:237–
Chapter Nine
KD in the adult setting is briefly discussed in this chapter.Traditionally, KD has been used in children with the idea that adults wouldn’t comply with the diet restrictions. This opinion was gradually reconsidered in the adults comes from chronic epileptic patients such as Lennox Gastaut or metabolic cases like GLUT1 deficiency transferred into adult services. Other than refractory seizures, KD is also tried for status epilepticus and super refractory strefractory epilepsy of adult patients as an effective therapy and different types of KD has been successfully tried, MAD, LGI,adherence. Recently, a modifiedreported effective. This novel diet has least restriction meaning that just a high fat low carbohydrate diet is encouraged without calorie, protein or fluid restrictions. MAD, however, is the first and common choice of KD used in an similar to children in an outpatient clinic with the same method and monitoring. Likewise, MAD is more tolerable and CKD is more effective, CKD may start at the beginThe reported response rate is at least 50%, although it may vary in different centers offering different KD types and usually,compliant cases. Symptomatic generalizfrequency in some studies. Women may develop menstrual irregularity. KD was also tried in pregnancy but its impact on the with inherited metabolic disorders typically diagnosed in childhood, and is necessary for adults. High blood Cholesterols level is not a contraindication although more frequent checkup is recommended and particular diet considerations may hAcute pancreatitis is an absoluteOther than seizure control, other benefits of KD is also appealing to adults for example weight loss, cognitive improvement, avoiding multi AEDs therapy, and the overaanti-inflammatory effects may relate to it. Important tips
In adults, ketosis response happens more quickly compare to children, between two to five days (mean 2.4 days) in classic KD and mean of 5 days in MAD. Furthermore, 2 montof KD is enough duration to evaluate its efficacy (Which is at least 3 months in children).
In adult MAD, more carbohydrate component is allowed; 20 mg in the first month (subtracting fiber) and up to 25 and 30 after stable ketosis.
The short-term common side effects include gastrointestinal symptoms such as constipation or bloating, weight loss, kidney stones,was reported while on diet. Although the patient already had preseems that balancing saturated fat/cholesterol versus included in diet design to avoid cardiovascular complication. Please also see table 5managing blood lipids and using different types of fat in dibe determined and whether
LDL and cholesterol level usually rise in first months but usually return to a diet continuation.
Multivitamin and mineral Sup
In Iran, keto diet is offered in adultuniversities. There are many similarities between protocol for MAD in an adult that is
KD in Adults setting is briefly discussed in this chapter.
Traditionally, KD has been used in children with the idea that adults wouldn’t comply with the diet restrictions. This opinion was gradually reconsidered in the 1990s. Anotadults comes from chronic epileptic patients such as Lennox Gastaut or metabolic cases like GLUT1 deficiency transferred into adult services. Other than refractory seizures, KD is also tried for status epilepticus and super refractory status in adults. Taken together, KD is now offered in refractory epilepsy of adult patients as an effective therapy and different types of KD has been successfully tried, MAD, LGI, and MCTD can liberalize the diet and increase compliance and
modified ketogenic diet (MKD) has been introduced in adults that also reported effective. This novel diet has least restriction meaning that just a high fat low carbohydrate diet is encouraged without calorie, protein or fluid restrictions.
is the first and common choice of KD used in an adult setting. Diet initiation is similar to children in an outpatient clinic with the same method and monitoring. Likewise, MAD is more tolerable and CKD is more effective, CKD may start at the beginning then twitch to MAD.
response rate is at least 50%, although it may vary in different centers offering usually, the common reason for discontinuation is diet restriction and non
compliant cases. Symptomatic generalized epilepsies appeared to show more reduction in seizure frequency in some studies. Women may develop menstrual irregularity. KD was also tried in pregnancy but its impact on the fetus is still unknown. Contraindications are similar to children
ted metabolic disorders typically diagnosed in childhood, and usually,is necessary for adults. High blood Cholesterols level is not a contraindication although more frequent checkup is recommended and particular diet considerations may h
absolute contraindication of a keto diet. Other than seizure control, other benefits of KD is also appealing to adults for example weight loss, cognitive improvement, avoiding multi AEDs therapy, and the overa
effects may relate to it.
In adults, ketosis response happens more quickly compare to children, between two to five days (mean 2.4 days) in classic KD and mean of 5 days in MAD. Furthermore, 2 montof KD is enough duration to evaluate its efficacy (Which is at least 3 months in children). In adult MAD, more carbohydrate component is allowed; 20 mg in the first month (subtracting fiber) and up to 25 and 30 after stable ketosis.
common side effects include gastrointestinal symptoms such as constipation or bloating, weight loss, kidney stones, and high blood lipids. One case of Myocardial infarction was reported while on diet. Although the patient already had pre-existing high chseems that balancing saturated fat/cholesterol versus polyunsaturatedincluded in diet design to avoid cardiovascular complication. Please also see table 5managing blood lipids and using different types of fat in diet design. Longbe determined and whether lipid-lowering agents may be of some benefits.LDL and cholesterol level usually rise in first months but usually return to a
and mineral Supplementation and regular follow up are recommended.
adult setting of different centers such as Tehran and Isfahan medical
e are many similarities between Keto diet in adults and children. We mention Johan adult that is brief and practical. (Table 9-1)
125
Traditionally, KD has been used in children with the idea that adults wouldn’t comply with the . Another implication in
adults comes from chronic epileptic patients such as Lennox Gastaut or metabolic cases like GLUT1 deficiency transferred into adult services. Other than refractory seizures, KD is also tried
atus in adults. Taken together, KD is now offered in refractory epilepsy of adult patients as an effective therapy and different types of KD has been
MCTD can liberalize the diet and increase compliance and ketogenic diet (MKD) has been introduced in adults that also
reported effective. This novel diet has least restriction meaning that just a high fat low carbohydrate diet is encouraged without calorie, protein or fluid restrictions.
setting. Diet initiation is similar to children in an outpatient clinic with the same method and monitoring. Likewise, MAD
ning then twitch to MAD. response rate is at least 50%, although it may vary in different centers offering
the common reason for discontinuation is diet restriction and non-ed epilepsies appeared to show more reduction in seizure
frequency in some studies. Women may develop menstrual irregularity. KD was also tried in is still unknown. Contraindications are similar to children
usually, no screening test is necessary for adults. High blood Cholesterols level is not a contraindication although more frequent checkup is recommended and particular diet considerations may help to manage that.
Other than seizure control, other benefits of KD is also appealing to adults for example weight loss, cognitive improvement, avoiding multi AEDs therapy, and the overall neuroprotection and
In adults, ketosis response happens more quickly compare to children, between two to five days (mean 2.4 days) in classic KD and mean of 5 days in MAD. Furthermore, 2 months trial of KD is enough duration to evaluate its efficacy (Which is at least 3 months in children). In adult MAD, more carbohydrate component is allowed; 20 mg in the first month (subtracting
common side effects include gastrointestinal symptoms such as constipation or high blood lipids. One case of Myocardial infarction
existing high cholesterol, it polyunsaturated should be carefully
included in diet design to avoid cardiovascular complication. Please also see table 5-2 for Long-term safety is still to
agents may be of some benefits. LDL and cholesterol level usually rise in first months but usually return to a normal level by
plementation and regular follow up are recommended.
setting of different centers such as Tehran and Isfahan medical
Keto diet in adults and children. We mention John Hopkins
Chapter Nine
Table 9-1 - Johns Hopkins Adult Epilepsy Diet Center Modified Atkins Diet
Pre-Diet · Nutrition evaluation · Height and weight, calculation of body mass index· Three-day food record to calculate · Food preferences/practices (ex. religious)/prior diets, allergies, intolerances, aversions· Laboratory evaluations
The comprehensive metabolicAntiepileptic drug levels (if applicable)Urine calcium, creatinine, human chorionic gonadotropin (premenopausal woman)· Diagnostic studies
EEG/Epilepsy Monitoring Unit evaluation (if the MRI with epilepsy protocol (if applicable)
· Screening for cardio- and cerebrovascular risk factors, history of nephrolithiasis· Pre-Diet calendar of daily seizures, start,
Initiation Diet prescription · 20 g net carbohydrates per day (subtracting fiber) Modified Atkins Diet· Liberal fat intake to satiety· Daily multivitamin, calcium and vitamin D supplement (low carbohydrate version)· Maintain adequate hydration· Monitoring (documented on a calendar)
Seizures daily Urine ketones daily until reaching 40 mg/dL, then biweekly Weights weekly Start and end of menses (premenopausal women)Follow-up · Clinic visits at 3 and 6 months then annually· Monitoring
Seizure frequency Urine ketones Food records/compliance BMI changes Side effects
· Laboratory evaluations (annual unless otherwise specified)
The comprehensive metabolic panel, complete blood count, fasting lipid profile (at 3 months and more frequent if elevated)Antiepileptic drug levels Vitamin D, zinc, selenium levels, free and total carnitine levels· Diagnostic studies
Renal ultrasound (if nephrolithiasis suspected)ECG (if the history of heart disease)Carotid ultrasound (if prolonged fasting lipid elevation)Bone density scan (every 5 years, minimum)For recipes, please see chapter 11
Johns Hopkins Adult Epilepsy Diet Center Modified Atkins Diet
Height and weight, calculation of body mass index day food record to calculate predict caloric intake
Food preferences/practices (ex. religious)/prior diets, allergies, intolerances, aversions
metabolic panel, complete blood count, fasting lipid profile, vitamin D, Antiepileptic drug levels (if applicable) Urine calcium, creatinine, human chorionic gonadotropin (premenopausal woman)
EEG/Epilepsy Monitoring Unit evaluation (if the diagnosis is unclear) MRI with epilepsy protocol (if applicable)
and cerebrovascular risk factors, history of nephrolithiasiscalendar of daily seizures, start, and end of menses (premenopausal women)
20 g net carbohydrates per day (subtracting fiber) Modified Atkins DietLiberal fat intake to satiety Daily multivitamin, calcium and vitamin D supplement (low carbohydrate version)Maintain adequate hydration Monitoring (documented on a calendar)
daily Urine ketones daily until reaching 40 mg/dL, then biweekly
eekly Start and end of menses (premenopausal women)
Clinic visits at 3 and 6 months then annually
Urine ketones Food records/compliance BMI changes
Laboratory evaluations (annual unless otherwise specified)
metabolic panel, complete blood count, fasting lipid profile (at 3 months and more frequent if elevated)
Vitamin D, zinc, selenium levels, free and total carnitine levels
Renal ultrasound (if nephrolithiasis suspected) of heart disease)
Carotid ultrasound (if prolonged fasting lipid elevation) ne density scan (every 5 years, minimum)
126
Johns Hopkins Adult Epilepsy Diet Center Modified Atkins Diet Protocol
Food preferences/practices (ex. religious)/prior diets, allergies, intolerances, aversions
panel, complete blood count, fasting lipid profile, vitamin D,
Urine calcium, creatinine, human chorionic gonadotropin (premenopausal woman)
and cerebrovascular risk factors, history of nephrolithiasis end of menses (premenopausal women)
20 g net carbohydrates per day (subtracting fiber) Modified Atkins Diet
Daily multivitamin, calcium and vitamin D supplement (low carbohydrate version)
daily Urine ketones daily until reaching 40 mg/dL, then biweekly
eekly
Urine ketones Food records/compliance
metabolic panel, complete blood count, fasting lipid profile (at 3 months
References
· Adults and Ketogenic Diets In The Ketogenic and Modified Atkins Diets Treatments for Epilepsy and Other Disorders?, Kossoff EH, MD, FreemanJM, Turner Z, RubensteinFifth Edition, New York, 2016
· Cervenka M.C., Henry B.J., Felton E.A., Center: Experience, efficacy and challenges, Epilepsy & Behavior 58: 61
· Martin-McGill K.J., Jenkinsonaadults with refractory epilepsy: An evaluation of a
· Nei M., Ngo L., Sirven J.I, Sperling M.R.: Ketogenic diet in adolescents and adults Seizure, 439–442, 2014
· Williams T.J, Cervenka M.C: The role for ketogenic diets in epilepsy and status epilepticus in adults. Clinical Neurophysiology Practice, 154
· Zare M., Okhovat A.A., Esmaillzadeh A., in an adult with refractory epilepsy: A controlled randomized clinical trial. Iran J Neurol, 16(2): 722017.
Adults and Ketogenic Diets In The Ketogenic and Modified Atkins Diets Treatments for Epilepsy and Other Disorders?, Kossoff EH, MD, FreemanJM, Turner Z, Rubenstein JE, Demos medical publishing,
Edition, New York, 2016 Cervenka M.C., Henry B.J., Felton E.A., Pattona K., Kossoff E.H.: Establishing an Adult Epilepsy Diet Center: Experience, efficacy and challenges, Epilepsy & Behavior 58: 61–68, 2016
Jenkinsona M.D., Smith C.T., Marsona A.G.: The modified ketogenic diet for adults with refractory epilepsy: An evaluation of a setup service, Seizure 52: 1Nei M., Ngo L., Sirven J.I, Sperling M.R.: Ketogenic diet in adolescents and adults
Williams T.J, Cervenka M.C: The role for ketogenic diets in epilepsy and status epilepticus in adults. Clinical Neurophysiology Practice, 154–160, 2017 Zare M., Okhovat A.A., Esmaillzadeh A., Mehvari J., Najafi M.R., Saadatnia M.
with refractory epilepsy: A controlled randomized clinical trial. Iran J Neurol, 16(2): 72
127
Adults and Ketogenic Diets In The Ketogenic and Modified Atkins Diets Treatments for Epilepsy and JE, Demos medical publishing,
K., Kossoff E.H.: Establishing an Adult Epilepsy Diet 68, 2016
The modified ketogenic diet for service, Seizure 52: 1–6, 2017
Nei M., Ngo L., Sirven J.I, Sperling M.R.: Ketogenic diet in adolescents and adults with epilepsy.
Williams T.J, Cervenka M.C: The role for ketogenic diets in epilepsy and status epilepticus in adults.
aadatnia M., Modified Atkins diet with refractory epilepsy: A controlled randomized clinical trial. Iran J Neurol, 16(2): 72-7,
Chapter Ten
KD commercial products All products should be checked if they are fortified by vitamins and mineral (usually they are) and calculation is required making sure of enough Products characterized by star * symbol are currently available in Iran. They are not many right now! But we hope to have some of them very soon.
Ketocal® is the only available keto diet formula in Iran
KD formulas 1) Ketocal® Nutricia
A) Ketocal® powder 3:1 or 4:1* (fo
Europe: SHS) is a milk proteinB) Ketocal® liquid (ready to drink): 1:4
· Vanilla or unflavored · Supplemented with DHA and ARA and contains multi· Appropriate for enteral or oral feedings
C) KetoCal® multi-fiber LQ Complete vanilla
fiber (Nutricia)
2) KetoVie™ Formula
4:1 liquid formula with two different flavors; mild vanilla and chocolate Details: Indicated for 1 year of age + Contains DHA, insoluble and soluble fibersMildly sweet with sucralose and acesulfame potassium· MCT – 25% of the total calories · Whey protein – 100% · Net Carbohydrate – 0.4g (vanilla) and 1.1g (chocolate) · Carnitine – contains 50mg Carnitine per serving· Selenium – contains 22mcg per serving · Free water: 77%
KetoVie Peptide is also available for individuals with GI sensitivities (oral or tube feeding).3) KetoVOLVE ™ (Nutr-e-volution )
4:1 Ratio formula, MCT enriched
KD products
All products should be checked if they are fortified by vitamins and mineral (usually they are) and making sure of enough age-appropriate intake according to DRA.
racterized by star * symbol are currently available in Iran. They are not many right now! But we hope to have some of them very soon.
is the only available keto diet formula in Iran
powder 3:1 or 4:1* (for North America: Nutricia, Rockville, MD, U.S.A. and for Europe: SHS) is a milk protein-based, powdered formula. Water should be added.
liquid (ready to drink): 1:4
Supplemented with DHA and ARA and contains multi-fiber blend. Appropriate for enteral or oral feedings
LQ Complete vanilla flavoured4:1 ketogenicratio
4:1 liquid formula with two different flavors; mild vanilla and chocolate
Contains DHA, insoluble and soluble fibers Mildly sweet with sucralose and acesulfame potassium
25% of the total calories
0.4g (vanilla) and 1.1g (chocolate) ns 50mg Carnitine per serving
contains 22mcg per serving
is also available for individuals with GI sensitivities (oral or tube feeding).volution )
4:1 Ratio formula, MCT enriched powder
128
All products should be checked if they are fortified by vitamins and mineral (usually they are) and intake according to DRA.
racterized by star * symbol are currently available in Iran. They are not many right now!
r North America: Nutricia, Rockville, MD, U.S.A. and for based, powdered formula. Water should be added.
liquid feed with added
is also available for individuals with GI sensitivities (oral or tube feeding).
Chapter Ten Details · For individuals over the age of 1 years · Oral and tube fed individuals · Can be used in cooking and baking· 100% whey protein, high in large neutral and breac· Cholesterol free · Contains MCT mixed with LCT
acid (C10) and 57 g of LCT · Meets Vitamin D 400 IU (10 ug) per day· No artificial sweeteners
4) KetoVOLVE MKD (Nutr-e
· A 2:1 ratio a modified ketogenic
and protein combined. · With chocolate flavor in 20g sachets· For oral use, via feeding tube, as a shake or in baked products.
5) Keyo: · A chocolate flavored with a 3:1 ratio, ready to eat, semi· Contains fat (LCT) 30 g, protein 8 g, carbohydrate 2 g per 100g, vitamins, minerals, trace elements,
arachidonic acid (ARA) and docosahexaenoic acid (DHA), sugar and sweetener.
6) RCF (Abbott Nutrition, Columbus,
· Ross Carbohydrate Free Soy Formula Base with Iron is a · L-carnitine (3 mg/100 ml) and taurine (12 mg/100 ml).· It contains protein (4g), fat (7.2) in 100 ml, and vitamins and minerals (water should be added)
Modulars These products may be added to customize the diet for each child. Carbohydrate · Carbohydrate polymer such as Polycose powder (Abbott Nutrition)· Carbo Mass* (Karen) 3.8 kcal/g, No protein or fat, lactose free· Maxijul: Glucose polymer powder supplemen· Caloreen: Glucose polymer powder supplement (Nestlé)· Vita joule Carbohydrate powder supplement (Vita
For individuals over the age of 1 years Oral and tube fed individuals Can be used in cooking and baking 100% whey protein, high in large neutral and breached chain amino acids
Contains MCT mixed with LCT – Every 100g of contains 15g of MCT Oil of which 45% is Decanoic
Meets Vitamin D 400 IU (10 ug) per day
e-volution )
A 2:1 ratio a modified ketogenic dietproduct; 2grams of fat for every 1 gram of carbohydrates
With chocolate flavor in 20g sachets For oral use, via feeding tube, as a shake or in baked products.
:1 ratio, ready to eat, semi-solid food Contains fat (LCT) 30 g, protein 8 g, carbohydrate 2 g per 100g, vitamins, minerals, trace elements, arachidonic acid (ARA) and docosahexaenoic acid (DHA), sugar and sweetener.
RCF (Abbott Nutrition, Columbus, OH, U.S.A.)
Soy Formula Base with Iron is a lactose-free concentrated carnitine (3 mg/100 ml) and taurine (12 mg/100 ml).
It contains protein (4g), fat (7.2) in 100 ml, and vitamins and minerals (water should be added)
These products may be added to customize the diet for each child.
Carbohydrate polymer such as Polycose powder (Abbott Nutrition) (Karen) 3.8 kcal/g, No protein or fat, lactose free
Maxijul: Glucose polymer powder supplement (Nutricia) Caloreen: Glucose polymer powder supplement (Nestlé) Vita joule Carbohydrate powder supplement (Vitaflo)
129
Every 100g of contains 15g of MCT Oil of which 45% is Decanoic
; 2grams of fat for every 1 gram of carbohydrates
Contains fat (LCT) 30 g, protein 8 g, carbohydrate 2 g per 100g, vitamins, minerals, trace elements, arachidonic acid (ARA) and docosahexaenoic acid (DHA), sugar and sweetener.
concentrated liquid.
It contains protein (4g), fat (7.2) in 100 ml, and vitamins and minerals (water should be added)
Chapter Ten
Protein · Liquid Protein Fortifier, Abbott Nutrition· Protifar: Protein powder supplement (Nutricia)· Vitapro (Vitaflo): Protein powder supplement
Lipid
· Lipid emulsion such as Microlipid_ (Nestle, Vevey, Switzerlan· Calogen, Nutricia (Supplement of LCT)
Not for use in children <3 years of age. drinks, or for tube fed patients, in 3 flavors Neutral, Banana and S
· 100 ml: 450 kcal, 50 g Fat, 0 proteins, 0.1 g Carbohydrate· MCT oil
MCT Oil Nestle OmniCT oil: MCT oil made from fractioned coconut oil.Liquigen® (Nutricia): emulsified MCT oil This is a creamy and palatable alternative to traditional MCT oil fclassic KD and MAD.
Fiber Resource Optifibre: Soluble dietary fiber powder (Nestlé) Food thickener Thixo-D Cal Free Calorie free thickener (Sutherland Health) Sweeteners · Stevia* (Canderel):Every sachet: 0.4 kcal, 1.99· Sucralose* (Canderel):0 calorie, 0 CHO· Agave 1 teaspoon contains 5g
its sugars are fructose and glucose
Calorie free water Liquid Sweet, Low® Liquid Sweet 10products helpful as sweeteners. Carbohydrate free multivitamins
Ketovite liquid For infants ●Vitamin A as palmitate (1.7x 106 units/g) Vitamin D2 (ergocalciferol) Cyanocobalamin Choline chloride
●Fruiti Vits Vitamin, mineral and trace element mixture in powder form, Vita
Liquid Protein Fortifier, Abbott Nutrition Protein powder supplement (Nutricia)
Vitapro (Vitaflo): Protein powder supplement
Lipid emulsion such as Microlipid_ (Nestle, Vevey, Switzerland) to add fat and achieve the goal KD ratio.Calogen, Nutricia (Supplement of LCT) Not for use in children <3 years of age. Long-chain triglyceride fat emulsion can be added to foods and drinks, or for tube fed patients, in 3 flavors Neutral, Banana and Strawberry. 100 ml: 450 kcal, 50 g Fat, 0 proteins, 0.1 g Carbohydrate
OmniCT oil: MCT oil made from fractioned coconut oil. Liquigen® (Nutricia): emulsified MCT oil This is a creamy and palatable alternative to traditional MCT oil for use in MCTKD or modification of
Resource Optifibre: Soluble dietary fiber powder (Nestlé)
D Cal Free Calorie free thickener (Sutherland Health)
:Every sachet: 0.4 kcal, 1.99 g CHO Sucralose* (Canderel):0 calorie, 0 CHO Agave 1 teaspoon contains 5gr carbohydrate = 20 calories Glycemic Index is between 65its sugars are fructose and glucose
Liquid Sweet 10®, and Stevia® Pure Extract (liquid) are commercial helpful
Carbohydrate free multivitamins
2500 400 12.5 150.0
Vits Vitamin, mineral and trace element mixture in powder form, Vita
130
d) to add fat and achieve the goal KD ratio.
can be added to foods and
or use in MCTKD or modification of
carbohydrate = 20 calories Glycemic Index is between 65-85 &
iquid) are commercial helpful
units units microgram mg
Vits Vitamin, mineral and trace element mixture in powder form, Vita flo
Chapter Ten
NanoVM Solac nutrition Nano VM contains only 0.003g (0.006 g in 100% of the DRI’s) of carbohydrate per scoop. (Insignificant amount) contains 14 vitamins and 12 minerals. is a powdered, hypoallergenic, carbohydrateformulations are available for ages 1 Phlexy-vitssachets and tablets Vitamin, mineral, and trace element Pediatric Seravit The same vitamins and minerals in the form of powder (Nutricia) Useful links Cooking and baking There are different recipes to prepare keto mealssome links for families: https://www.charliefoundation.org/resourceshttps://www.charliefoundation.org/resourceswww.matthewsfriends.org We intend to design a website about cooprofessionals and parents based on Iranian cuisine and foods. We’ll get there soon.
Nano VM contains only 0.003g (0.006 g in 100% of the DRI’s) of carbohydrate per scoop. ant amount) contains 14 vitamins and 12 minerals.
is a powdered, hypoallergenic, carbohydrate-free vitamin and mineral supplement with 4 formulations are available for ages 1-3, 4-8, 9-18 years and for children 1-18 years who are tube fed.
trace element mixture in powder form (Nutricia)
The same vitamins and minerals in the form of powder (Nutricia)
to prepare keto meals, desserts, shakes, cookies, smoothies etc. Here are
https://www.charliefoundation.org/resources-tools/resources-2/find-recipes https://www.charliefoundation.org/resources-tools/resources-2/find-books
We intend to design a website about cooking tips in ketogenic diet and all information for both professionals and parents based on Iranian cuisine and foods. We’ll get there soon.
131
Nano VM contains only 0.003g (0.006 g in 100% of the DRI’s) of carbohydrate per scoop.
free vitamin and mineral supplement with 4 18 years who are tube fed.
, shakes, cookies, smoothies etc. Here are
king tips in ketogenic diet and all information for both professionals and parents based on Iranian cuisine and foods. We’ll get there soon.
Chapter Ten
132
Chapter Eleven
Iranian Keto foods and recipes In this chapter we introduce some Iranian foods that are prepared with calculated calories andratios. For some of these food specific keto products are used for example zero calorie sweetener(Canderel, or Erythritol), and flour/wellthat are available in Iran. These ground nuts can be used instead of wheat flour in cookies andbreads. There are varieties of oils in the market that can be used as a with the same amount. If butter or margarine is your preference, remember that 15 gram of oil isequal to 20 gram of butter/margarine. Heavy cream and mayonnaise also can be included insteadof oil considering their carbohydratebrand). 20 grams of mayonnaise and 30 grams of cream (30%) are equal to 15g grams of oil. Heavycream that is available in Iran is 30%.There are some commercial products available in Iran such as Kin different recipes like smoothies, soups and cookies for different age groups specifically, in infantfoods. They are also added to increase diet ratio similar to using MCT oil, for example.Commercial products have a known ratio on their labels therefore; cooking with them is easier.Different company websites usually present instructions for cooking with their products that canbe looked up on line. In chapter 9 different Keto products and their suppliers are introducedalthough many of them are not available in Iran. Available products in Iran are shown by * sign.An important reminder is also necessary here: for spices and dried vegetables which are commonlyused in Iranian cuisines, families can use their own preferenhave their own local food recipes; however, it should be explain to them that our suggested recipescan be easily modified according to their local herbs and spices. They can use a pinch of them anddiscuss it with the dietitians. For classic type of KDs we use the scale that weighs in 1increments. Recipes are written based on that. For example for egg instead of saying 1 regularegg (usually equal to 50 g) the weight in grams is mentioned.For adults, other than the suggested recipes in this chapter, a variety of salads are convenientchoices, considering high carbohydrate vegetables such as onion or tomato should be avoided.Wing kabab (without flour) is a very good source of fat and can be marinated in small aonion, olive oil saffron and high fat yogurt, and grilled in the oven.For infants, Fereni (فرنی) and 3:1 Soup with cream are among appropriate options.The recipes and photos in this chapter are prepared and written by Ms. Maryam Azizi, ourdietitian at Mofid Children’s Hospital I really appreciate her help.
In this chapter we introduce some Iranian foods that are prepared with calculated calories andratios. For some of these food specific keto products are used for example zero calorie sweetener
rythritol), and flour/well-grounded almond or hazel nut (فندق یا گردو کاملا آسیاب شدهthat are available in Iran. These ground nuts can be used instead of wheat flour in cookies andbreads. There are varieties of oils in the market that can be used as a fat source in these recipeswith the same amount. If butter or margarine is your preference, remember that 15 gram of oil isequal to 20 gram of butter/margarine. Heavy cream and mayonnaise also can be included insteadof oil considering their carbohydrate and protein content (usually very low but depends on thebrand). 20 grams of mayonnaise and 30 grams of cream (30%) are equal to 15g grams of oil. Heavycream that is available in Iran is 30%. There are some commercial products available in Iran such as Ketocal formulas. They can be usedin different recipes like smoothies, soups and cookies for different age groups specifically, in infantfoods. They are also added to increase diet ratio similar to using MCT oil, for example.
own ratio on their labels therefore; cooking with them is easier.Different company websites usually present instructions for cooking with their products that canbe looked up on line. In chapter 9 different Keto products and their suppliers are introducedalthough many of them are not available in Iran. Available products in Iran are shown by * sign.An important reminder is also necessary here: for spices and dried vegetables which are commonlyused in Iranian cuisines, families can use their own preference in cooking. Different cities in Iranhave their own local food recipes; however, it should be explain to them that our suggested recipescan be easily modified according to their local herbs and spices. They can use a pinch of them and
he dietitians. For classic type of KDs we use the scale that weighs in 1Recipes are written based on that. For example for egg instead of saying 1 regular
egg (usually equal to 50 g) the weight in grams is mentioned. n the suggested recipes in this chapter, a variety of salads are convenient
choices, considering high carbohydrate vegetables such as onion or tomato should be avoided.Wing kabab (without flour) is a very good source of fat and can be marinated in small aonion, olive oil saffron and high fat yogurt, and grilled in the oven.
and 3:1 Soup with cream are among appropriate options.The recipes and photos in this chapter are prepared and written by Ms. Maryam Azizi, our
tian at Mofid Children’s Hospital I really appreciate her help.
133
In this chapter we introduce some Iranian foods that are prepared with calculated calories and ratios. For some of these food specific keto products are used for example zero calorie sweetener
فندق یا گردو کاملا آسیاب شده( that are available in Iran. These ground nuts can be used instead of wheat flour in cookies and
fat source in these recipes with the same amount. If butter or margarine is your preference, remember that 15 gram of oil is equal to 20 gram of butter/margarine. Heavy cream and mayonnaise also can be included instead
and protein content (usually very low but depends on the brand). 20 grams of mayonnaise and 30 grams of cream (30%) are equal to 15g grams of oil. Heavy
etocal formulas. They can be used in different recipes like smoothies, soups and cookies for different age groups specifically, in infant foods. They are also added to increase diet ratio similar to using MCT oil, for example.
own ratio on their labels therefore; cooking with them is easier. Different company websites usually present instructions for cooking with their products that can be looked up on line. In chapter 9 different Keto products and their suppliers are introduced although many of them are not available in Iran. Available products in Iran are shown by * sign. An important reminder is also necessary here: for spices and dried vegetables which are commonly
ce in cooking. Different cities in Iran have their own local food recipes; however, it should be explain to them that our suggested recipes can be easily modified according to their local herbs and spices. They can use a pinch of them and
he dietitians. For classic type of KDs we use the scale that weighs in 1-gram Recipes are written based on that. For example for egg instead of saying 1 regular
n the suggested recipes in this chapter, a variety of salads are convenient choices, considering high carbohydrate vegetables such as onion or tomato should be avoided. Wing kabab (without flour) is a very good source of fat and can be marinated in small amount of
and 3:1 Soup with cream are among appropriate options. The recipes and photos in this chapter are prepared and written by Ms. Maryam Azizi, our
Chapter Eleven
Foods with 4:1 ratio1. Kuku
عدس یا مرغ،گوشت مثال عنوان به
موجود روغن از سوم یک و نمک ادویه،
.شود داغ کاملا تا میگذاریم شعله روي
بر را کوکو و برمیداریم را سرتابه سپس
Instructions: Kuku recipe is made by cooked and chopped stuff such as chicken, meat, lentils or raw chopped vegetables that mixed up with eggs, spice and oil. After mixing well, it will be added to a skillet to fry or cook in the oven.
Foods with 4:1 ratio
( شده وخرد پخته معمولا که غذایی ي ماده نوع براساس
ادویه، ، مرغ تخم کردن بااضافه ) سبزي کوکو مثل(است
روي و ریخته رادرتابه نروغ ي بقیه .کرد مخلوط مرغسپس .شود برشته تا گذاریم می را تابه در و کنیم
.شود برشته
: Kuku recipe is made by cooked and chopped stuff such as chicken, meat, lentils or raw chopped vegetables that mixed up with eggs, spice and oil. After mixing well, it will be added to a skillet to fry
134
براساس کوکو :تهیه دستوراست شده وخرد خام یا )پخته .شود می تهیه لازم درموادمرغ تخم با کاملا باید را موادکنیم می اضافه را مخلوط سپس هم دیگر طرف تا گردانیم می
: Kuku recipe is made by cooked and chopped stuff such
as chicken, meat, lentils or raw chopped vegetables that mixed up with eggs, spice and oil. After mixing well, it will be added to a skillet to fry
Chapter Eleven
يکوکو سبز
(Kuku sabzi) Calorie 292 292کالري
Ingredients: لازم مواد
Kuku vegetables’ 42 g خرد شود(Egg 41g گرم41 تخم مرغ
Oil 35 g گرم35 روغن
خرد شود زیر دیکه با دیشو ز،ی،گشنيجعفر ه،تر(سبزي کوکو
کوکوي عدس(Lentils Kuku)
Calorie 312 312 کالري
Ingredients: مواد لازم Cooked lentils 17 g گرم17عدس پختهEgg 21 g گرم 21تخم مرغ Butter or olive oil 39 g
گرم 39 تونیز روغن ایکره
اسفناجي کوکو
(Spinach Kuku) Calorie 390 390 کالري
Spinach 40 g گرم 40 اسفناج
Onion 5g گرم 5پیاز
Egg 47 g گرم47تخم مرغ
Oil 35 g گرم35تونیز ای عیروغن ما
135
سبزي کوکو
عدس پخته
روغن ما
Chapter Eleven
کوکوي لوبیا سبز (Green bean KukuCalorie 312 يکالر
Ingredients: مواد لازمGreen beans 40 g Egg 27 g گرم27تخم مرغOnion 6 g گرم6پیازOil or butter 39 g
مرغ يکوکو (Chicken Kuku) Calorie: 416 ۴١۶ی
Ingredients: مواد لازمChicken 15 g 15گرم Egg 14 g 14گرمCarrot 40 g 40گرمLeek 10 g 10گرمOnion 10 g 10گرمOlive oil 51 51گرم
136
.
(Green bean Kuku ) کالر 312
مواد لازم گرم40اسبزیلوب
تخم مرغ پیاز
گرم39روغن
یکالر
مواد لازم 15مرغ 14تخم مرغ 40هویج
10 یتره فرنگ 10ازیپ 51تونیروغن ز
Chapter Eleven
روغني حاوي را با این مواد مخلوط می کنیم و در تابه . شود روي شعله ي ملایم می گذاریممی گذاریم تا بپزد سپس سر تابه را برمی داریم و تا زمانی که هردوطرف برشته
به تابه روي گوشت گذاشت تا بخارپزشود و
Instructions: Mix the materials (including half of the oil), put them in the skillet until cooked.rings can be used raw or placed on top of the meat to steam and give taste to the meat.
را با این مواد مخلوط می کنیم و در تابه ) نیمی از روغن( وط کردهلمواد را هم مخمی گذاریم تا بپزد سپس سر تابه را برمی داریم و تا زمانی که هردوطرف برشته
تابه روي گوشت گذاشت تا بخارپزشود و یا در خام کنار کباب گذاشت وت به صور وه گوجه را می شود حلقه حلقه برید
Mix the materials (including half of the oil), put them in the skillet until cooked.rings can be used raw or placed on top of the meat to steam and give taste to the meat.
ي ا کباب تابه (Ground meat pan kabab)Calorie 312 يکالر
Ingredients: Ground meat 15 g Onion 15 g Tomato 20 g گرمCarrot 15 g گرمOlive oil 39 g گرم
137
مواد را هم مخ: هیدستور تهمی گذاریم تا بپزد سپس سر تابه را برمی داریم و تا زمانی که هردوطرف برشته
گوجه را می شود حلقه حلقه برید . گوشت طعم دهد
Mix the materials (including half of the oil), put them in the skillet until cooked. Tomato rings can be used raw or placed on top of the meat to steam and give taste to the meat.
(Ground meat pan kabab) کالر٣١٢
مواد لازم گرم15 گوشت چرخ کرده
گرم15پیاز گرم20گوجه
گرم15جیهو گرم39روغن
Chapter Eleven
2) Soup
سپس .میکن یمرا اضافه جیمرغ و هو پزشد و به هم میکن یخامه را اضافه م میدار ی
Instructions: Cook the barley with a glass of water, add chicken and carrot when half finished, add the butter or oil next. After cooking, add the heavy cream and mix them well.
3) Stews (خورشت ھا)
تفت داده) لازم هست جزء مواد اگر ازو اضافه کرد ولی اگر را هم می توان در ابتدا تفت داد
مثل زردچوبه پخته باشند یا به سادگی له شوند بهتر است زمانی اضافه شوند که گوشت یا مرغ پخته باشد ادویه جات میارت ملابا حر است خورشت بهتر. شوند
Instructions: In general, for the stew toss the meat/chicken and vegetables with oil (and onion if any) and fry for 10 minutes, then cook with one or two glass of water. If vegetables are to simply crash (like Okra) it is best to add when meat or chicken cooked. Add spies such as turmeric or cinnamon at first and seasoning such as pomegranate paste at the end.
پزشد میکه ن یبپزد و زمان میگذار یآب م وانیل کیجودوسر را با یشعله بر م يپس از پخته شدن سوپ را از رو .می
Cook the barley with a glass of water, add chicken and carrot when half finished, add r or oil next. After cooking, add the heavy cream and mix them well.
ازیپ و( مرغ با روغن ایخورشت ها ابتدا گوشت را هم می توان در ابتدا تفت دادجات سبزی. پخته می شود بآ وانی
پخته باشند یا به سادگی له شوند بهتر است زمانی اضافه شوند که گوشت یا مرغ پخته باشد ادویه جات شوند یاضافه م ب نارنج در آخرآ ایمثل رب انار ییها
In general, for the stew toss the meat/chicken and vegetables with oil (and onion if any) and fry for 10 minutes, then cook with one or two glass of water. If vegetables are to
is best to add when meat or chicken cooked. Add spies such as turmeric or cinnamon at first and seasoning such as pomegranate paste at the end.
خامهسوپ جو با (Creamy barley soup)Calorie 312 312يکالر
Ingredients: مواد لازمChicken 16 g گرم Barely 11 g 11 گرمHeavy cream 6 g Olive oil 36 g گرمCarrot 20g گرمParsley 5 g مگر
138
جودوسر را با :هیدستور تهیکن یکره را اضافه م ایروغن
.شود کنواختیتا میزن یم
Cook the barley with a glass of water, add chicken and carrot when half finished, add r or oil next. After cooking, add the heavy cream and mix them well.
خورشت ها ابتدا گوشت هیته يبرا: هیدستور تهیدو ل با یکی سپس .شود یم
پخته باشند یا به سادگی له شوند بهتر است زمانی اضافه شوند که گوشت یا مرغ پخته باشد ادویه جات ها یچاشن و ابتدا در نیدارچ و
.فتدیجا بکاملا تا پخته شود
In general, for the stew toss the meat/chicken and vegetables with oil (and onion if any) and fry for 10 minutes, then cook with one or two glass of water. If vegetables are to
is best to add when meat or chicken cooked. Add spies such as turmeric or cinnamon at first and seasoning such as pomegranate paste at the end.
Creamy barley soup) 312
مواد لازم 16مرغ 11جو دوسر
Heavy cream 6 g مگر 6 خامه گرم 36 روغن گرم 20 جیهو
5 يجعفر
Chapter Eleven
خورشت آلواسفناج(Spinach Plum stew) Calorie 390 390 کالري Ingredients: موادلازمMeat 28 g گرم 28گوشتSpinach 25 g 25 گرمOnion 6 g گرم 6ازیپ Plum 12 g 12گرمOlive oil 48 g 48 گرمLemon juice مویآبل
هیخورشت بام (Okra Stew) Calorie 416 416 يکالر Ingredients: موادلازمOkra 60 g 60 گرم بامیه Meat 18 g 18گرم گوشت Oil 52g گرم 52 روغنOnion 6 g مگر 6 ازیپ Tomato paste 5 g گرم
139
موادلازم گوشت
25اسفناج
12آلو تونیز ای عیروغن ما
کالر
موادلازم بامیه گوشت
روغن
گرم 5 رب گوجه
Chapter Eleven
)گوجه و کدوسبز ،وشت(TAS kabab) Calorie 416 641يکالر
Ingredients: موادلازمMeat: 20 g گرمZucchini: 30g گرمCarrot: 33 g 33 مگرOnion: 5 g گرم 5ازیپButter or olive oil 52g
گرم 52 تونیز اروغنیکره Tomato 20 g گوجه
خورشت فسنجان(Fesenjan or walnut stew)Calorie 416 641
Ingredients: مواد لازمMeat or chicken 20 g Carrot 40 g گرم 40جGround Walnut 5 g Olive oil 48 g گرمPomegranate paste 5 g Onion 5 g گرم 5ازی
140
وشتگ(تاس کباب
کالر
موادلازمگرم 20گوشت
30)یکدوخورشت(کدوسبز 33 جیهو
پButter or olive oil 52g
کره گوجه گرم ٢٠
(Fesenjan or walnut stew) 641يکالر
مواد لازمMeat or chicken 20 g گرم ٢٠ مرغ ایگوشت
جیهو Ground Walnut 5 g گرم 5شده ابیسآ گردوی
گرم 48روغن Pomegranate paste 5 g گرم 5رب انار
یپ
Chapter Eleven
يخورشت قرمه سبز (Gormeh-sabzi stew with meat) Calorie 390 390 Ingredients: مواد لازمMeat 28 g مگر28 گوشتOnion 10 g گرم10ازیOil 48 g گرم48روغنSpecific vegetables 65g
)روغن سرخ شده در و که خرد(Parsley, chives, Fenugreek, well chopped and fried)
اسبزیخورشت لوب (Green bean stew) Calorie 292 يکالر٢٩٢
Ingredients: لازمموادMeat 20 g گرم 20گوشتGreen beans 10 g Mushroom 8 g گرمCarrot 8 g مگرOlive oil 35 g گرم
141
sabzi stew with meat) 390يکالر
مواد لازمگوشت
یپ روغن
Specific vegetables 65g گرم65سبزيقرمه سبزی که خرد لهیشنبل یکم ،تره،يجعفر(
(Parsley, chives, Fenugreek, well chopped
٢٩٢
مواد گوشت
Green beans 10 g گرم 10سبز ایلوب گرم 8قارچ گر 8 جیهو
35 تونیز ای عیروغن ما
Chapter Eleven
خورشت کرفس(Celery stew with meat)Calorie 312 ٣١٢ Ingredients Meat 15 g Celery 50 g Parley and mint 24 g
گرم ٢۴ نعناع و جعفريOnion 6 g Oil 39 g مگر 39
جانمباد يخوراك مسما(khorak e bademjan, eggplant stew) Ingredients: Chicken 15 g Eggplant 30 g Carrot 10 g Tomato 15 g مOnion 6 گرمOil 39 g گرم٣٩
براي تهیه ي این خورشت بادمجان باید جداگانه سرخ
For this stew, Fry eggplant separately and add it when chicken or meat is cooked.
142
(Celery stew with meat) ٣١٢ يکالر
مواد لازم گرم 15گوشت
گرم 50کرفس Parley and mint 24 g
نعناع و جعفريگرم6 ازیپ
تونیز ای عیروغن ما
خوراك مسما(khorak e bademjan, eggplant stew)
Ingredients: مواد لازم Chicken 15 g گرم15مرغ Eggplant 30 g گرم30جان مباد Carrot 10 g مگر10جیهو
مگر15هرب گوج گرم6پیاز
٣٩ روغن براي تهیه ي این خورشت بادمجان باید جداگانه سرخ
.خراضافه گرددآدر و شود For this stew, Fry eggplant separately and add it when chicken or meat is cooked.
Chapter Eleven
لوآخوراك مرغ و (Khorak e morgh va aloo, Chicken and golden plum stew) Calorie 312 3يکالر
Ingredients: مواد لازمChicken 16g گرمGolden plum (Bokhara plum) 26
گرم 26 آلوبخارا Onion 10 g گرم10ازTomato paste 5 g Oil 39 گرم 39روغن Barberries (Iranian berries) 5 g
یرزاقاسمیم (Mirza Ghasemi) (Vegi)Calorie 312 يکالر٣١٢
Ingredients: مواد لازمEggplant 35 g گرمTomato 10 g گرمEgg 28 g 28 مگرOnion and garlic 6 g Butter or olive oil 39g
نکته مهم درتهیه ي این غذاکباب کردن بادنجان استImportant tip for this food is to use grilled eggplant.
143
(Khorak e morgh va aloo, Chicken and 312
مواد لازمگرم16مرغ
Golden plum (Bokhara plum) 26 g
ازیپ گرم 5فرنگی رب گوجه
Barberries (Iranian berries) 5 g گرم5زرشک
(Mirza Ghasemi) (Vegi) ٣١٢
مواد لازمگرم 35بادنجان گرم10یگوجه فرنگ
28 تخم مرغ Onion and garlic 6 g گرم6ریازوسیپ
r olive oil 39g گرم 39تونیز روغن ایکره نکته مهم درتهیه ي این غذاکباب کردن بادنجان است
Important tip for this food is to use grilled
Chapter Eleven
دهیم تا مواد را خوب مخلوط کرده و ورز میبا بخشی از . ن را به چهار قسمت تقسیم نموده و هر قسمت را گرد می کنیم
رام آن می گذاریم تا آ ب جوش سس تهیه کرده وگردي ها در
Instructions: Cook everything like a usual stew, mix them all and knead to make a sticky mixture. Then divide it into four parts and use your hands to shape them to around parts. Make a sauce with a portion of oil and half the tomato paste, salt, turmeric and boiled water. Add the rounds to the sauce and cook them slowly until done.
Calorie 416
Ingredients:
Ground meat 18 g
Carrot 20 g گرم 10معطر خشک يسبز
Mixed dried vegetables (parsley, trogon, Savo
Almond flour 10 g
Olive oil 48 g
Tomato paste 5 g
مواد را خوب مخلوط کرده و ورز می ،پس از پختن واضافه کردن مواد طبق دستور خورشت هان را به چهار قسمت تقسیم نموده و هر قسمت را گرد می کنیمآسپس . یک مخلوط چسبنده به دست بیایدب جوش سس تهیه کرده وگردي ها درآکمی زردچوبه و مقداري ،
g like a usual stew, mix them all and knead to make a sticky mixture. Then divide it into four parts and use your hands to shape them to around parts. Make a sauce with a portion of oil and half the tomato paste, salt, turmeric and boiled water. Add the
unds to the sauce and cook them slowly until done.
144
کوفته (Koofteh)
Calorie 416 416يکالر
Ingredients: مواد لازم
گرم18گوشت چرخ کرده
گرم 20 جیهو
) سبز د،ترخونیمرزه،شو ،يجعفر )
Mixed dried vegetables (parsley, trogon, Savory, Dill) 10 g Onion 5 g گرم5ازیپ
Almond flour 10 g گرم10آرد بادام
Olive oil 48 g گرم 48 تونیروغن ز
گرم 5فرنگی رب گوجه پس از پختن واضافه کردن مواد طبق دستور خورشت ها :هیدستور ته
یک مخلوط چسبنده به دست بیاید،نمک ،روغن و نصف رب گوجه
. بپزد و جا بیفتد
g like a usual stew, mix them all and knead to make a sticky mixture. Then divide it into four parts and use your hands to shape them to around parts. Make a sauce with a portion of oil and half the tomato paste, salt, turmeric and boiled water. Add the
Chapter Eleven
(Kashk
Eggplant 30 g
Ground walnut 13 g
Onion, garlic, dried mint 6 g گرم
.براي تهیه ي این خورشت بادنجان باید جداگانه سرخ شود
145
بادنجان کشک
(Kashk-e Bademjan) (Vegi)
Calorie 312 312يکالر
Ingredients: مواد لازم Eggplant 30 g گرم30بادنجان
Ground walnut 13 g گرم13شده ابیسآ يگردو
Kashk 10 g گرم10کشک
گرم 6 خشک نعناع و ریس و ازیپ
Oil 32 g گرم 32 روغن
براي تهیه ي این خورشت بادنجان باید جداگانه سرخ شود
Fry eggplant separately.
Chapter Eleven
Almond four (Or well-grounded almond
هم به گردو را. بتدا کمی تفت می دهیم و سپس با روغن سرخ می کنیم تا طلایی شود گلاب و زعفران را ،بآدر ظرفی جداگانه یک لیوان
.ی را اضافه می کنیم و کاملا مخلوط می نماییم .فرنی دارد مثلاین مخلوط شل است و غلظتی
.ردیرد شود تا حلوا کاملا شکل بگآجذب
Roast the almond flour until golden, add the walnut and roast it too. In a separate pan place one cup of water, rose water and saffron to boil, then add the sweetener and mix them all. At the end mix these two parts thoroughly. This is a loose mixtureFor Halwa, Roast almond flour completely until brown. The sugar/saffron content should be completely absorbed by the flour part.
Calorie 416
Ingredients: grounded almond) 20 g گرم20شده ابیس
Ground walnut 14 g Olive oil 37 g
Saffron and rose water (a pinch) Zero calorie sugar
بتدا کمی تفت می دهیم و سپس با روغن سرخ می کنیم تا طلایی شوددر ظرفی جداگانه یک لیوان . و کنار می گذاریم با هم تفت می دهیم
ی را اضافه می کنیم و کاملا مخلوط می نماییم میرژ شکر ،ش کردهتا به جوش بیاید سپس شعله را خامواین مخلوط شل است و غلظتی .خر این دو بخش را با هم مخلوط می کنیم
جذب شکر کاملا يحاو عیرنگ باشد و ما ییوخرما
Roast the almond flour until golden, add the walnut and roast it too. In a separate pan place one cup of water, rose water and saffron to boil, then add the sweetener and mix them all. At the end mix these two parts thoroughly. This is a loose mixture
r Halwa, Roast almond flour completely until brown. The sugar/saffron content should be completely absorbed by the flour part.
146
یکاچ (Kachi) Calorie 416 416يکالر
Ingredients: مواد لازم سآنرم اریبس بادام ایرد بادام آ
گرم 14شده ابیاس يگردو گرم 37تونیروغن ز
زعفران و گلاب به مقدار کم Zero calorie sugar یمیشکر رژ
بتدا کمی تفت می دهیم و سپس با روغن سرخ می کنیم تا طلایی شودا رد بادام راآ :هیدستور ته
با هم تفت می دهیم ن اضافه کرده وآتا به جوش بیاید سپس شعله را خامو می گذاریم
خر این دو بخش را با هم مخلوط می کنیمآدرمرحله ي سرخ دیحلوا آرد کاملا با يبرا
Roast the almond flour until golden, add the walnut and roast it too. In a separate pan place one cup of water, rose water and saffron to boil, then add the sweetener and mix them all. At the end mix these two parts thoroughly. This is a loose mixture.
r Halwa, Roast almond flour completely until brown. The sugar/saffron content should
Chapter Eleven
Halwa and Kachi share the same ingredients with different cooking instructions.
Calorie: 312
Ingredients:
Almond flour 15 g
Olive oil 27 g
Grounded walnut 10 g گرمRose water, zero sweetener
Calorie 312, 4: 1 Ratio
Ingredients:
Carrot 10 g
گرم 13شده ابیسآنرم Almond flour (or well-grounded almond) 13 g
Grounded walnut 9 g گرم 9شدهButter or olive oil 29 g
Halwa and Kachi share the same ingredients with different cooking instructions.
147
(Halwa) حلوا
Calorie: 312 312يکالر
Ingredients: مواد لازم
Almond flour 15 g گرم 15رد بادام آ
Olive oil 27 g گرم 27تونیروغن ز
گرم 10شده ابیسآ يگردو
Rose water, zero sweetener وگلاب یمیشکر رژ
Carrot Halwa 4: 1 Ratio 312يکالر
Ingredients: مواد لازم Carrot 10 g گرم10جیهو
نرم یلیبادام خ ایبادام ردآ
grounded almond) 13 g شده ابیسآفندق ایگردو
29 g گرم 29تونیز اروغنیکره
Halwa and Kachi share the same ingredients with different cooking instructions.
Chapter Eleven
ب روي شعله می گذاریم تا به جوش بیاید و مرتب به هم ب مخلوط نموده و به مخلوط روي شعله اضافه
.راي تزیین می توان ازکمی دارچین استفاده کرد
Roast the almond flour, add boilingwater and bring the mixture to boil. Stir it slowly until the flour cooks and most of the water cooks off. Blend the rest ingredients in boiled water and add to the mixture. Continue stirring until done. For decorating, you can use a small amount of cinnamon.
416 calorie
Ingredients:
Almond flour: 20
Butter or olive oil: 37 g
Ground walnut 14 g شدهZero calorie sweetener
Water and rose water and Saffron
ب روي شعله می گذاریم تا به جوش بیاید و مرتب به همآا مقداري ب و میده یتفت م یکمب مخلوط نموده و به مخلوط روي شعله اضافه آکمی ی مواد را باقبا. یکنواخت باشد و گلوله نشود
راي تزیین می توان ازکمی دارچین استفاده کردب .می کنیم و به هم می زنیم تا جا بیفتد
Roast the almond flour, add boilingwater and bring the mixture to boil. Stir it slowly until the flour cooks and most of the water cooks off. Blend the rest ingredients in boiled water
ontinue stirring until done. For decorating, you can use a small
148
شعله زرد (Shole zard)
416 calorie 416يکالر
Ingredients: موادلازم
20 g بادامرد آ 20گرم گرم 37تونیز اروغنیکره
شده ابیسآ يگردو گرم 14
Zero calorie sweetener لازم زانیبه م یمیشکر رژ
and Saffron گلاب وآب و زعفران کم رد بادام راآ :هیستور تهد
یکنواخت باشد و گلوله نشود می زنیم تامی کنیم و به هم می زنیم تا جا بیفتد
Roast the almond flour, add boilingwater and bring the mixture to boil. Stir it slowly until the flour cooks and most of the water cooks off. Blend the rest ingredients in boiled water
ontinue stirring until done. For decorating, you can use a small
Chapter Eleven
ن را به آید وآتمام مواد را در ظرفی ریخته و با هم زن برقی به هم می زنیم تا خمیر صاف و نرمی به دست بسیار کمی روغن چرب مقدار ده باگرم نمو
.ه ریخته و دو طرف نان را می پزیمPlace all the ingredients in a bowl and mix by an electric mixer to make the smoot and soft dough. Leave it in the room temperature for 20 mithe mixture in a non-stick pan greased with a thin oil layer. Cook both sides of the naan.
. میکن یم
رد آسپس مخلوط میکن یهم اضافه کرده وکاملا مخلوط مدرجه 160با حرارت در فر و میگذار ی
Add almond flour, backing powder, cinnamon and blended applecontainer beat the sugar and oil and add eggs and beat it well. Then mix two parts and make a paste. Divide the paste into small sizes and put it on baking dish. (160
Calorie 390
Ingredients:
Almond flour 28 g
Egg 19
Butter 41
Zero calorie sweetener
تمام مواد را در ظرفی ریخته و با هم زن برقی به هم می زنیم تا خمیر صاف و نرمی به دست گرم نمو سپس یک تابه ي نچسب را. دقیقه در حرارت اتاق می گذاریم
ه ریخته و دو طرف نان را می پزیمیک قاشق غذاخوري از مواد را در تابPlace all the ingredients in a bowl and mix by an electric mixer to make the smoot and soft dough. Leave it in the room temperature for 20 minutes. Then Place a tablespoon of
stick pan greased with a thin oil layer. Cook both sides of the naan.
مخلوط یپوره شده را در ظرف بیوس نیپودر ، دارچهم اضافه کرده وکاملا مخلوط م تخم مرغ را و میزن یم مکاملا به ه روغن را
یفر م ینیس يکوچک رو يدر اندازه ها رامده آبه دست
Add almond flour, backing powder, cinnamon and blended apple in a beat the sugar and oil and add eggs and beat it well. Then mix two parts and
make a paste. Divide the paste into small sizes and put it on baking dish. (160
کیک یزديYazdi cake Calorie 416 416کالري Ingredients: موادلازمAlmond flour 30 g گرمApple 12 g گرم12سیبEgg 23 g گرم 23تخم مرغHeavy cream 10g گرمOil 20g گرم20روغن Baking soda ½ tsp
يخورینصف قاشق چا Cinnamon ½ tsp یخورینصف قاشق چاZero sweetener یمیرژ شکر
149
(Naan) نانCalorie 390 4:1 390 يکالر
Ingredients: موادلازم Almond flour 28 g گرم 28 رد بادامآ
Egg 19 g گرم19تخم مرغ
Butter 41g گرم 41کره
Zero calorie sweetener یمیشکر رژ
تمام مواد را در ظرفی ریخته و با هم زن برقی به هم می زنیم تا خمیر صاف و نرمی به دست :هیدستور تهدقیقه در حرارت اتاق می گذاریم 20مدت
یک قاشق غذاخوري از مواد را در تابمی کنیم و Place all the ingredients in a bowl and mix by an electric mixer to make the smoot and
nutes. Then Place a tablespoon of stick pan greased with a thin oil layer. Cook both sides of the naan.
پودر ، دارچ نگیرد بادام، بکآ:هیدستور تهروغن را و جداگانه شکر یدر ظرفبه دست ریخمو میینما یم هرا اضاف .میکن یپخت م
in a bowl. In a separate beat the sugar and oil and add eggs and beat it well. Then mix two parts and
make a paste. Divide the paste into small sizes and put it on baking dish. (160 C or 320 F).
موادلازم
گرم 30آرد بادام سیب
تخم مرغگرم10خامه
Baking soda ½ tsp پودر گینبک
نصف قاشق چا نیدارچ شکر
Chapter Eleven
Calorie 416
Ingredients Milk 90 ml
White egg 16 g Heavy cream 20 g
Cacao ½ to 1 tsp نصف تا یک قاشق چایی خوريOil or MCT Oil 45 g 45 گرم
Zero calorie sweetener
150
کائوکا شیر ( Mocha) Calorie 416 416 کالري
Ingredients مواد لازم
Milk 90 ml 90 سی سی شیر White egg 16 g گرم 16تخم مرغ
Heavy cream 20 g گرم 20خامه کاکائو نصف تا یک قاشق چایی خوري
45ن ام سی تی یا روغ روغن Zero calorie sweetener شکر رژیمی
Chapter Eleven
Flixweed or Chia seed 1 tea spoon
Food and deserts with 3:1 ratio
Fesenjan stew (Walnut stew)
Calorie500 500کالري
Meat 22g گرم 22 گوشت
Ground walnut 15g گرم 15 شده
Onion 10 گرم10ازیپ
Pomegranate paste 5g گرم 5رب انار
Oil 49 گرم 49روغن
Zero
Zero calorie sugar
Rose water (or any zero calorie available flavour)d or Chia seed 1 tea spoon قاشق چایخوري 1
Food and deserts with 3:1 ratio
خورشت فسنجان
شده ابیسآ يگردو
رب انار
151
بستنی یخی Zero calorie(Popsicle)
Water بآZero calorie sugar یمیرژشکر
Rose water (or any zero calorie available flavour) 1 ریخاك شی یا تخم شربت
Chapter Eleven
خر یک لیوان آدر و در روغن سرخ می کنیم و سپس باقی مواد را کم کم اضافه می کنیم ورام بجوشد آدقیقه می گذاریم 15 را در این مرحله اضافه می کنیم و حدود
At first, caramelize onion, and add all material but kashk. After roasting the mixture, add one glass of water and bring it to boil. Then add Kashk and let them slowly cook fo20 minutes.
Chicken salad سالاد مرغ Calorie 500 500يکالر Ingredients: موادلازم Chicken 31g گرم 31مرغ Lettice 60 g گرم 60کاهو Tomato 36 g گرم 36گوجه Cucumber 30 g گرم 30 اریخ Mayonnaise 20 g گرم 20 ونزیCarrot 30 g گرم 30 جیهو Green paper 60 g گرم 60يفلفل دلمه ا
و در روغن سرخ می کنیم و سپس باقی مواد را کم کم اضافه می کنیم ورا در این مرحله اضافه می کنیم و حدود ب اضافه می کنیم تا به جوش بیاید کشک
At first, caramelize onion, and add all material but kashk. After roasting the mixture, add one glass of water and bring it to boil. Then add Kashk and let them slowly cook fo
یما
فلفل دلمه ا
Kalle josh (Vegi) کله جوشCalorie 500 500يکالر Ingredients: موادلازمKask 40 g گرم40کشکGround walnut 30 g Onion 10 g گرم10ازDried mint 5 g گرمOil 41g گرم 41 روغن Turmeric زردچوبه
152
و در روغن سرخ می کنیم و سپس باقی مواد را کم کم اضافه می کنیم و پیاز را خرد :هیدستور ته
ب اضافه می کنیم تا به جوش بیاید کشکآ.تا جا بیفتد
At first, caramelize onion, and add all material but kashk. After roasting the mixture, add one glass of water and bring it to boil. Then add Kashk and let them slowly cook for 15 to
کله جوش کالر
موادلازم کشک
Ground walnut 30 g گرم30شده ابیسآ يگردو ازیپ گرم5نعناع خشک
زردچوبه
Chapter Eleven
Snacks and deserts with 3:1 ratio
Apricot cake Calorie 500 500يکالر
Ingredients: موادلازم Almond flour 15 g گرم 15رد بادامEgg 42 گرم 42 مرغتخم Milk 60cc یس یس 60 ریش Apricot 12 g گرم12زردآلو Butter 51 گرم 51کره Baking soda ½ tsp يخوریپودرنصف قاشق چاVanilla 3 g گرم 3 لیوان
ب را اضافه می کنیم و مرتب مخلوط را
.مده و غلیظ شودآ
Roast the almond flour for few minutes and add one cup of watermixture continuously. Add the rest ingredients slowly while stirring and bring the mixture to boil. Continue stirring and let the water slowly evaporate and make a thick liquid.
Snacks and deserts with 3:1 ratio
رد بادام آ
پودرنصف قاشق چا نگیبک
ب را اضافه می کنیم و مرتب مخلوط را آشعله ي پایین گذاشته و کم کم روي ،در ظرفی ریختهآباقی مواد را کم کم اضافه می کنیم و به هم می زنیم تا به جوش
Roast the almond flour for few minutes and add one cup of water on low heat. Stir the inuously. Add the rest ingredients slowly while stirring and bring the mixture
to boil. Continue stirring and let the water slowly evaporate and make a thick liquid.
(Fereni) فرنی Calorie: 500 3:1 Ingredients: Almond flour 30 g Milk 48 g یسYolk egg 33g Oil 41g گرم
153
در ظرفی ریخته رد بادام راآ :هیدستور تهباقی مواد را کم کم اضافه می کنیم و به هم می زنیم تا به جوش به هم می زنیم و
on low heat. Stir the
inuously. Add the rest ingredients slowly while stirring and bring the mixture to boil. Continue stirring and let the water slowly evaporate and make a thick liquid.
Calorie: 500 3:1
Ingredients: موادلازم Almond flour 30 g گرم 30رد بادامآ
س یس 50 ریش Yolk egg 33g گرم 33تخم مرغ يزرده
41تونیروغن ز
Chapter Eleven
Smoothies Calorie 500 3:1 ٣:١ ۵کالري٠٠
Ingredients are the same just 13 grams of banana instead of strawberry. .به جاي توت فرنگی استفاده می شود
تخم مرغ از گرا .مخلوط کن ریختمی توان سفیده ي تخم مرغ را به صورت پخته یا خام با بقیه ي مواد در اضافه را مواد ي هیبق بعد شود سفت
Egg white can be used raw or cooked and place with other ingredients in a mixer. If using raw white egg beat it first until white and firm peaks, then add the rest ingredients. Adding ice gives smoothies a very good taste.
٣:١
(Banana smoothie) شیرموزIngredients are the same just 13 grams of banana instead of strawberry.
به جاي توت فرنگی استفاده می شود گرم موز 13 مشابه شیر توت فرنگی است فقط
شیر هویجMilk 90 cc
Carrot 25 gHeavy cream 10 g
Butter 58 gWhite egg 45g گرم
Zero calorie sweetener
می توان سفیده ي تخم مرغ را به صورت پخته یا خام با بقیه ي مواد در و دیسف تا دیومخلوط کن دیزین را در مخلوط کن بر
.ن می دهدآاضافه کردن یخ طعم خوبی به Egg white can be used raw or cooked and place with other ingredients in a mixer. If using
white egg beat it first until white and firm peaks, then add the rest ingredients. Adding ice gives smoothies a very good taste.
گی شیر توت فرن Strawberry smoothie Ingredients: موادلازمMilk 100cc 100 سی سیEgg white 43g سفیده ي تخم مرغStrawberry 20 g 20 گرمHeavy cream 15 g گرمButter 59 g گرم59کرهZero sweetener یمیشکررژ
154
(Banana smoothie) Ingredients are the same just 13 grams of banana instead of strawberry.
مشابه شیر توت فرنگی است فقط
(Carrot smoothie) Milk 90 cc یس یس100شیر
Carrot 25 g گرم25هویج Heavy cream 10 g گرم10خامه
Butter 58 g گرم58کره گرم 45سفیده ي تخم مرغ
Zero calorie sweetener یمیشکررژ
می توان سفیده ي تخم مرغ را به صورت پخته یا خام با بقیه ي مواد در : هیدستور تهن را در مخلوط کن برآابتدا دیکن یخام استفاده م
اضافه کردن یخ طعم خوبی به . دیکن Egg white can be used raw or cooked and place with other ingredients in a mixer. If using
white egg beat it first until white and firm peaks, then add the rest ingredients.
موادلازم100شیر
سفیده ي تخم مرغ گرم 43 20یتوت فرنگ گرم15خامه
کره شکررژ
155
Appendix
Almond بادام درختی
Apple سیب Apple Pie پاي سیب Applesauce سیب زمینیلوآزرد Apricots
Asparagus cooked مارچوبه پخته Avocado آووکادو Baked potato سیب زمینی پخته Banana موز Basil ریحان
)خام(جوانه لوبیا Bean sprouts (raw) Beef Salami کالباس گوشت گاو Beet cooked چغندر پخته
)ترشی(چغندر پخته Beetroot cooked (pickled) Biscuit بیسکوئیت Blackberry تمشک
رفرنگی سیاهانگو Blackcurrants Brussels sprouts cooked کلم دلمه اي پخته Butter کره Cabbage کلم پیچ پخته Cabbage cooked برگ کلم پخته
پخته کلم برگ Cabbage cooked لم برگ خامک Cabbage raw
Canola روغن مایع کانولا Cantaloupe طالبی Carrot raw or cooked هویج پخته یا خام Carrots cooked هویج پخته Carrots raw هویج خام Cashew بادام هندي Cauliflower cooked گل کلم پخته Celery کرفس Celery raw or cooked کرفس پخته یا خام Cheddar پنیرچدار Cherries گیلاس Chicken breast سینه مرغ Chickpea نخود Chocolate (dark) شکلات تلخ
156
لینارگ Coconut
Cooked Shrimp میگو پخته Cookie کلوچه Corn ذرت Corn Flakes کورن فلکس Cream خامه Cream cheese پنیر خامه اي Croissant شیرینی کروسانت Cucumber خیار Dew melon/honeydew گرمک Donut دونات Drumstick ران مرغ Egg boiled whole تخم مرغ پخته
)پخته(ي تخم مرغ سفیده Egg white (cooked) Egg whole raw تخم مرغ خام Egg whole scrambled تخم مرغ نیمرو Eggplant cooked بادنجان پخته Endive raw اندیو خام Feta cheese پنیرفتا Fig (fresh) انجیرتازه French Fries سیب زمینی سرخ شده
گورفرنگیوعی انن Gooseberries (stewed Grapefruit گریپ فروت Grapes انگور Green bean cooked لوبیا سبز پخته Green grape انگور سبز Green pepper فلفل دلمه اي خام یا پخته Greengage گوجه سبز ترشنیبست Ice cream
Kiwifruit کیوي Kohlrabi cooked کلم قمري پخته
پخته رنگیتره ف Leek cooked Lentils عدس Lettuce کاهوي خام Mango انبه Mashed potato پوره سیب زمینی Mayonnaise سس مایونز Melon خربزه
157
Milk Chocolate شیرکاکائو
Mint نعناع Mozzarella پنیر موزارلا
ذرتین ماف Muffin Corn Mushroom raw قارچ خام Nectarine شلیل Okra (cooked) بامیه پخته Olive Oil روغن زیتون Olives زیتون Orange پرتقال Orange juice آب پرتقال Pancake پن کیک Peach هلو
ینیبادام زم Peanut Peanut butter کره بادام زمینی Pear گلابی Peas, frozen, boiled نخود فرنگی پخته و یخ زده Pepper فلفل Pickle Relish ترشی سیر Pineapple اناناس
یتیچ یالوب Pinto Beans Pistachio پسته
ایتزپ یرپن Pizza cheese Plum/prune الو سیاهلیپف ف popcorn
Poultry انواع مرغ Pretzel چوب شور
سبوس دار اهینان جو س Pumpernickel pumpkin کدو تنبل Pumpkin cooked کدو حلوایی یا پخته Quail بلدرچین Radish raw ترب خام Raspberries تمشک raw vegetables سبزي خوردن Resins کشمشمریواس خا Rhubarb raw Rice cookie کیک برنجی Rice, white برنج سفید
158
Soy Milk شیرسویا
Soy Sauce سس سویا Soybeans سویا Spinach raw اسفناج خامپهل Split Peas
Strawberries توت فرنگی Sweet lemon لیمو شیرین Tangerine نارنگی
پخته گوجه Tomato cooked Tomato juice آب گوجه Tomato raw گوجه ي خام Turkey بوقلمون Turnip cooked شلغم پخته Vegetables سبزیجات Waffles وافل Walnut گردو Watermelon هندوانه White bread نان سفید White sugar شکر سفید
)زردك(وحشی هویج Wild Carrot Zucchini raw کدو سبز خام
159
160