general pediatrics lmcc review

GENERAL PEDIATRICSLMCC Review

April 14, 2011

Feeding & Growth

Breastfeeding CPS recommends exclusive breastfeeding

for babies up to 6 months (when possible) Breast milk has a caloric content of ~20 Kcal/oz (0.67 Kcal/ml) Babies need 100-130 Kcal/Kg/day in 1st 3

months Feeding/growth spurts occur at 8-12 days,

3-4 wks, 3 mo, variably after that

When comparing human breastmilk and cow’s milk the following is correct

A. The ratio of whey:casein is higher in human milk

B. Cow’s milk has a lower solute load

C. Human milk has higher iron content

D. Cow’s milk has lactose, human milk does not

E. Human milk has sufficient vit D

Nutritionally, how does breast milk compare to cow’s milk?

Lower protein content - decreased solute load Greater whey:casein (70/30 vs 18/82, formula

60/40) CHO - both are lactose based (6.5% vs 4.5%) Fat - 30-50% vs 3.5-4% Vitamins - richer in A,C,E, lower in D & K Minerals - lower Fe but better absorbed

Breast Feeding Benefits for Baby

Immunological benefit secretory IgA, lactoferrin, lysozyme as GI

defence Lower rate OM, LRTI, gastro, possibly UTIs,

Hib Less allergenic Less constipating Better jaw/mandible development Attachment and ?Improved cognitive

functioning

Breast Feeding Benefits for Mother Postpartum weight loss & uterine involution Delayed return of fertility Bonding Pre-menopausal breast & ovarian Ca

reduced Economical benefit

Breast Feeding Issues Takes 3-7 days for milk supply to come in

risk of dehydration/ “breastfeeding jaundice” Jaundice is more common

Breastmilk jaundice “Mom/baby team”

ineffective latch/sore nipples/engorgement Infections

thrush , mastitis/abscess Reduced levels vitamin D & K

Vitamin D Supplementation Rickets is still a problem in Canada! 400 IU per day

All Breastfed infants Pregnant and nursing mothers in northern Canada Formula Fed infants living in northern Canada

800 IU per day - < 2 yrs breastfed babies with a risk factor

Home above 55 degree latitude, darker skin, sun avoidance Community with high prevalence Vit D deficiency

Vitamin D is found in milk, margarine, salmon, tuna, liver, kidney & from the sun

Contraindications to Breast Feeding Maternal infections

HIV, AIDS, active TB, malaria, herpes on breast, hepatitis

Maternal sepsis Psychotropic meds or others crossing Chemo/radiation Alcohol/drug abuse Infant Galactosemia (lactose) *May breastfeed even with VZV

Cow’s Milk Formula Always Fe fortified

Many choices!

Specialty formulas soy, lactose free, increased calories Protein hydrolysate formulas (eg.Alimentum, Nutramigen, Progestemil) Amino-acid based formulas (Neocate)

Introduction of Solids cereals 4-6 months vegetables fruits meats

cow’s milk should be postponed until at least 10-12 months of age (renal load)

2% or 1% milk should be postponed until after second birthday (inadequate fat content)

Rest gradually in this order

Infant Growth: Rules of Thumb Weight gain:

Regain birth weight by 10-14 days (max 10% loss)

Double birth weight by 5 months Triple birth weight by 1 year Quadruple birth weight by 2 years Increase by 5 lbs/year for rest of childhood

Growth - Height Average length at birth 50 cm (20 inches) Increases by 50% by 1 yr Doubles by 4 yrs Triples by 13 yrs

Ave growth 5-6 cm / yr (4 yrs-puberty)

Growth- Body Mass Index (BMI) BMI helps to quantify the weight to height

relationship BMI = weight(kg)/height (m)2 BMI of 20-25 is acceptable BMI > 30 indicates obesity BMI < 18 suggest severe anorexia or

Failure to thrive

Growth- Head Circumference

2 cm/month 1-3 months 1cm/month 3-6 months 0.5cm/month 6-12 months

Most of growth is in first yr! 12 cm in first yr, 2 cm in 2nd yr, 6-8 cm rest of life

Growth Monitoring Routinely recommended

Height, weight, head circumference Plot on appropriate chart considering ethnicity, genetic

syndromes (eg. Tri 21, Turner’s), and prematurity Prematurity, correct hc (18 mo), wt (24 mo),

ht (40 mo)

Only way to detect FTT Also detects chronic illness, feeding difficulties

What is Failure to Thrive? US National Center for Health Statistics defines FTT

for children < 2 yrs of age as:

Weight <3rd-5th centile for age on more than one occasion

Weight <80% of ideal body weight for age Weight crosses 2 major centile curves downwards

on a standardized growth curve Exceptions: genetic/familial short stature,

constitutional growth delay, SGA infants & preterm infants

Caloric needs

0-10 kg: 100 kcal/kg/day

10-20 kg: 1000 + 50 kcal/kg/day >20 kg: 1500 + 20 kcal/kg/day

Which growth curve demonstrates a growth pattern of FTT?

A. A

B. B

C. C

Which growth curve demonstrates a pattern of genetic short stature?

A. A

B. B

C. C

FTT- Infant Growth Caveats to normal growth velocity:

>50% of babies shift their growth parameters upwards between birth and 3 months

Nearly 30% of well babies shift their parameters downwards between 3 and 18 months

Exclusively breastfed babies plot higher for wt at 0-6 months and lower at 6-12 months5

Growth Velocity Average age to “settle” on a growth curve is 13

months6

Downward shift seen with constitutional growth delay downward shift between 6 & 24 months may have decreased weight for height re-establish normal growth velocities by 3 yrs

*Genetically Programmed curve is established by 18-24 months

FTT- Assessment Grading of malnutrition:

Grade Wt for Age (%)

Wt for Ht (%)

0-Normal >90 >90

1-Mild 75-90 81-90

2-Moderate 60-74 70-80

3-Severe <60 <70

FTT- Assessment “Organic” vs. “non-organic”

Historical way of viewing FTT Refers to presence/absence of diagnosis of

major disease process or organ dysfunction “Non-organic” accounts for >80% Now felt to be more of a continuum Multifactorial process

FTT- Assessment Under-nutrition results from:

1) Decreased caloric intake

2) Inadequate caloric absorption

3) Increased caloric losses

4) Increased caloric requirements

FTT- Differential Diagnosis Decreased caloric intake:

Inadvertent (decreased breast milk, improper formula preparation)

Neglect or abuse Behavioural (ex. Food refusal) Pain (GERD, injury to mouth or esophagus) Fatigue /anorexia (anemia, cardiac dz, resp dz, RTA) Impaired swallowing (neurologic dz) Craniofacial abnormalities (cleft lip/palate, choanal

atresia) Toxin (lead)

FTT- Differential Diagnosis Inadequate Caloric Absorption & Increased Caloric Losses

GI Pancreatic insufficiency (CF) Liver disease (biliary atresia, chronic cholestasis) Generalized malabsorption (CF, Celiac, short gut) Diarrheal state (infectious, post-infectious) Persistent vomiting (pyloric stenosis, GERD) Inflammatory disorders (IBD) Allergic gastroenteropathy Protein-losing enteropathy

FTT- Differential Diagnosis Increased Caloric Losses cont’d

Renal Protein loss Carbohydrate loss

Inability to use nutrients Diabetes Mellitus Metabolic dz

FTT- Investigations Careful and complete history taking and physical

examination are ESSENTIAL Unless an illness other than primary under-

nutrition is suspected on Hx or P/E the yield of lab investigations is almost nil!

Lab testing helps with diagnosis in 1.4%

FTT- Investigations Non-specific “screening”

CBCD, ESR, lytes, BUN, Cr, venous gas Urine R&M, C&S

Markers of nutritional status Total protein, albumin, glu, Ca, PO4, Iron studies Zinc, vitamin levels

Dependent on specific aspects of Hx & P/E Liver function tests Thyroid studies Stool OB, reducing substances, culture, O&P, trypsin Sweat test, immunoglobulins & celiac screen, viral

serology (incl HIV), TB testing, immune w/up, metabolic w/up, CXR, ECG, milk scan

FTT- Management Admit if :

Suspect enviro. deprivation/neglect/abuse Suspect chronic dz which needs stabilizing Severe under-nutrition (consider if moderate)

ie. <60% of median weight for age Failed out-patient management

With hospitalization: greater likelihood of catch-up growth no change in developmental outcomes

FTT- Management Inter-disciplinary approach!!

Treat any underlying illness and provide nutritional support: Increase caloric intake

1.5-2 X RDA (120 kcal/kg/day x ideal wt)/current wt Estimate 150-200 kcal/kg/day

FTT- Management Mild under-nutrition

Ensure frequent feedings (q 3 hrs) Increase formula concentration (eg. 24 kcal/oz) Add calorie-rich foods to diet (butter, PB, oils) Provide dietary counseling Prescribe multivitamin with zinc and iron Follow-up frequently (?public health nurse) Expect catch-up growth at 2-3x regular rate in first

month

FTT- Management Moderate under-nutrition

Determine caloric intake Consider whether investigations are necessary Increase caloric intake (150-200 kcal/kg/day) Ensure adequate protein (3-4 g/kg/day) Consider meal supplements (eg. Pediasure) Add Multivitamin Consider therapeutic doses of iron Monitor weight gain

FTT- Management Severe under-nutrition

Admit Initiate re-feeding slowly Consider using elemental formula Consider diluting formula May need ng continuous feeds or tpn Follow fluid and lytes status closely

FTT- Long-term implications

Persistent growth deficits

Cognitive impairment

Behaviour problems

IMMUNIZATIONS

Immunization Schedule(Recommended by the Canadian Immunization Guide)

Age 2 mos 4 mos 6 mos 12 mos 18 mos 4-6 year q10 years

Vaccine

DaPTP-HIB, Pneum, Men DaPTP-HIB, Pneum,Men DaPTP-HIB, Pneum,Men MMR, Varicella, Pneum DaPTP-HIB, MMR DaPTP dTaP/dT

Contraindications to Vaccines Strict

- anaphylaxic or anaphylactic shock- encephalopathy

Precautions- febrile reaction > 40.5- shock collapse or hypotonia - hyporesponsive collapse- seizures

Contraindications to vaccines Anaphylaxis to eggs:

Influenza and yellow fever Severe immune deficiency:

All live vaccines (MMR, VZV, OPV, BCG, Yellow

fever, Oral cholera, Oral typhoid) Pregnancy:

MMR and Varicella Anaphylaxis to neomycin:

MMR and IPV

Immunization Reactions DaPTP/Hib

reactions occur within 72 hrs

MMR reactions occur 5-12 days

Delayed ImmunizationAge 1-6 years

0 mos:DPTP-Hib, MMR,HepB, P,V,MC

2 mos: Pentacel,MMR,HepB, Prevnar

4 mos: DPTP 12 mos:DPTP 4-6 yrs: DPTP 14-16 yrs: dTaP

> 7 Years Old 0 mos: TdaP,Polio,

MMR,V,Menj,HepB 2 mos: TdaP, Polio,

HepB,MMR, V 6-12 mos:

TdaP+Polio,HepB TdaP (no polio) q10

yrs thereafter

Other Immunizations

Hepatitis A & B Influenza HPV Rotavirus

INFECTIOUS DISEASE

Fever Temp > 38.3 Celsius rectal

0-1 mos: FSWU 1-3 mos:

toxic = FSWU non-toxic and low risk (WBC 5-15,

bands <5%, urine neg, well-looking, reliable family): investigate and follow

Otitis Media Incidence:

15% to 20% Peak:

6 to 36 months 4 to 6 years decreases > 6 years old

Etiology: S. pneumoniae, M. catarrhalis, non-type H. Flu, GAS & viral

Otitis Media Risk Factors

mid-face hypoplasia (Down Syndrome) daycare attendance Inuit/Aboriginal low SES 2nd hand smoke

Otitis Media Treatment:

1st line: Amoxil 2nd line: High dose Amoxil, Clavulin,

Macrolides, Cephalosporin T-Tubes if recurrent or persistent effusion

Complications: hearing loss chronic effusion mastoiditis meningitis

Sinusitis Mainly ethmoid sinus Predisposed by viral URTI, allergy,

asthma, periodontal disease Same bugs as OM +/- anaerobes Presents with fever, purulent nasal

discharge (> 10 days), periorbital tenderness, halitosis

Treat with same Abx as OM, may need longer course (3 weeks)

Meningitis Newborn period (LEG)

Listeria E. Coli Group B strep

2 months - 12 years (SHIN) Strep pneumonia, N. meningitides Less common Hib with immunization

Meningitis in abnormal hosts or anatomic defects:

increased Pseudomonas, Staph, Salmonella, Serratia

Complications SIADH seizures subdural effusions transient joint effusions (Ag-Ab reaction) hearing loss

Urinary Tract Infection Newborn M › F (2) Children F › M (10) Schoolgirls - 5% Recurrence - 80% Vesicoureteral reflux - 35% Renal scarring - 50% All children ‹ 3 yrs

U/S VCUG

Urinary Tract Infections UTI

KEEPPSS Klebsiella, E. Coli, Enterococcus, Proteus, Pseudomonas,

Staph, Strep

Pyelonephritis ampicillin + gentamycin IV initially then Amoxil or Septra po

VUR prophylactic Septra or nitrofurantoin (or Amoxil) Higher grades may require surgery

Osteomyelitis Organism

neonate: Staph, GBS, E.Coli > 3mos.: Staph, Hib, Strep,Pseudomonas SS disease: Salmonella

Diagnosis bone scan x-ray 10-12 days

Septic Arthritis Monoarticular (trauma) Organism

neonate group B strep, staph, E.Coli

>3 mos Staph aureus Strep, Hib

most common site is knee (40%)

Fever and Rash

A 14 year old male comes to you with a fever and rash for 2 days. He states that before the fever and rash he had started with red eyes, coryza, and cough. He has now developed a maculopapular rash starting at the head and moving to his toes. You look in his mouth and see little white ‘spots’. You are most concerned he has:

A. Kawasaki disease

B. Measles

C. Rubella

D. Scarlet Fever

E. Erythema Infectiosum

A 1 year old presents to your office with a new rash. Mum reports the baby had a high fever for two days with no associated symptoms. The fever has now disappeared as the rash appeared. The most likely organism causing the illness is:A. HHV-1B. HHV-2C. HHV-6D. Group A StrepE. Mycoplasma

Measles (First Disease) Paramyxovirus Stages

incubation: 8-13 days prodrome: 4 C’s then fever with rash skin rash (erythematous, maculopapular, starts at

hairline and spreads to face, trunk and extremities)

Complications pneumonia encephalitis SSPE

Scarlet Fever (Second Disease) Fever, pharyngitis, exanthem (starts in

axilla, groin, neck, blanches,circumoral pallor, Pastias lines)

Age › 3 yrs; recurrence Group A strep: erythrogenic toxin (2-5

days postStrep throat or GN) Treatment with Penicillin Complications

cellulitis Rheumatic fever

Rubella (Third Disease) Togavirus Congenital Postnatal

mild disease suboccipital nodes maculopapular pink rash, starts on face, neck

to extremities, spreads quicker than measles

Roseola Infantum (Fourth Disease)

HHV6 High fever rash maculopapular with

cessation of fever High WBC low WBC Febrile convulsions

Erythema Infectiosum (Fifth Disease) Parvovirus B19 No prodrome: red flushed face/slapped

cheek, maculopapular rash with lacelike appearance

Complications miscarriage/SA aplastic crises

The number one cause of death in children between 1 month and 1 year of age is:A. Child abuse

B. Sudden infant death syndrome

C. Apparent life threatening events

D. Drowning

E. Sepsis

ALTE/SIDS

Apparent Life Threatening Event: Definition Clinical symptoms frightening to caregiver Combo of apnea, colour change, tone

change, choking, gagging

Apnea: 20 seconds of breathing cessation or shorter if associated with tone change, colour change, CV change

ALTE Detailed Hx and Px Talk to person witnessing event Significant event?

ALTE Differential

Neuro- seizure GI- reflux Pulmonary- aspiration, apnea CV- arrhythmia Infection- sepsis, pertussis, RSV Metabolic Abuse

ALTE Investigations Labwork +/- infectious workup EEG ECG/Echo UGI/pH probe CT head Monitor

SIDS Unexpected death by Hx and Postmortem most common cause of death 1mo-1yr peak 2-4 months, 95% <6 months old

SIDS Infant Risk Factors:

prematurity age, sex prone sleeping bottle fed smoke environ. Thermal stress ethnicity prior illness winter months low birthweight

Maternal Risk Factors low age low SES low education smoker drug use poor nutrition

Gastroenterology/Pediatric Surgery

Colic Unexplained irritability/crying 2-3 wks to 3 mos At least 3 hrs/day > 3 days/week > 3 weeks duration ? Secondary to immature gut development

Colic ++Gas/legs drawn up; otherwise well No proven treatment - rhythmic

motion/sounds Ovol/Tylenol- no proven benefit Parental support and reassurance

Abdominal Pain Acute: Ddx includes gastro, hernia, UTI,

appendicitis, intussusception, malrotation, volvulus, HSP, SC Crisis, pneumonia, mesenteric adenitis,

Chronic: > 3 episodes affecting activities > 3 months Organic (<10%): constipation, IBD, mass, PUD,

GU, lactose intolerance Functional (90%): 8-10 yo peak, girls

predominate, vague crampy periumbilical pain with no awakening, ppting or alleviating factors. Normal growth.

Gastroesophageal RefluxGastroesophageal Reflux Incompetence of lower esophageal

sphincter improves by one year of age in most infants

Complications apnea in infants aspiration pneumonia chronic cough/wheeze esophagitis- dysphagia,hemetemesis, Fe

deficiency anemia

Gastroesophageal RefluxGastroesophageal Reflux Diagnosis

pH probe Ba swallow nuclear scan endoscopy

Gastroesophageal RefluxGastroesophageal Reflux

Treatment chalasia routine - attention to burping, small

frequent feeds, 300 prone

thickening feeds Medications:

H2 blockers or PPI if esophagitis or gastritis present

Domperidone for decreased gastric emptying

A 6 week old male infant presents with a one day history of increasing severity of vomiting. Mum notes he is very hungry but appears to forcefully vomit all that he has eaten in a ‘projectile’ fashion. She now worries he is becoming more lethargic and dehydrated. The typical abnormality on laboratory investigations would be:A. AnemiaB. EosinophiliaC. Hyperchloremic metabolic acidosisD. Hypochloremic metabolic alkalosisE. Respiratory alkalosis

Pyloric StenosisPyloric Stenosis

boy > girl 3 weeks - 3 months projectile vomiting (nonbilious) hungry infant feeds vigorously

Pyloric StenosisPyloric Stenosis O/E:- dehydration, lethargy, weight loss,

peristaltic waves L R, palpable “olive”

Lab- hypochloremic alkalosis

Investigation- U/S, UGI Rx: rehydrate and restore electrolyte

balance

- myotomy of pyloric muscle

IntussusceptionIntussusception

3 months - 3 years (up to 6 years) 5% anatomic abnormality found

Meckel’s polyp duplication

more frequent ileocolic or ileoileocolic


Clinical severe paroxysmal pain lethargy shock sausage shaped mass RUQ vomiting currant jelly stool


Lab x-ray

density or gasless right side

barium enema coil-spring sign

Treatment reduction by barium enema surgical reduction

Meckel Diverticulum 2% population year peak incidence 2 feet from ileocecal junction 35% ectopic gastric or pancreatic tissue painless rectal bleeding (typically bright red) 99Tc scan confirms diagnosis treatment: excision

A 4 year old girl presents with longstanding constipation. The mother reports her daughter has always been constipated and has been challenging to treat with many different therapeutic interventions being tried. They describe her stools as long and thin. She is noted to not be growing well when plotted on a growth curve. He neurological exam is normal. She appears to have good anal tone with the absence of stool in the rectum. The best test to pursue for confirmation of the diagnosis is:A. MRI of the spineB. Contrast enemaC. Colonoscopy with proximal large bowel biopsyD. Rectal biopsyE. No investigation required

Constipation Def’n: passage of bulky or hard stool at

infrequent intervals Retention of stool in rectum leads to

encopresis in up to 60% Most causes are not organic but due to

voluntary or involuntary retentive behavior

Constipation DDx Dietary Behavioral Obstruction Dehydration Structural defects (fissures) Metabolic (hypothyroid,hypoparaT,

hyperCa) Neuromuscular ( MMC, MD, spinal cord) Hirshsprungs

Constipation- Treatment Dietary: increased fluid/fibre Stool softeners or laxatives

Lactulose Lansoyl PEG powder

Bowel evacuation (enemas) Bowel/toileting regimen

Hirshsprung’s Disease Absence of ganglion cells in the bowel wall Most common cause of neonatal GI

obstruction Aganglionic segment NB: failure to pass meconium within 48

hrs

Hirshsprung’s Disease Constipation

onset after 2 yr encopresis large caliber stool normal growth normal nutrition normal anal tone stool in ampulla

Hirshsprung’s onset at birth no encopresis small stools assoc. FTT/poor

nutrition abdominal distension normal anal tone ampulla empty

Hirshsprung’s DiseaseHirshsprung’s Disease

Diagnosis: rectal biopsy

shows absence of ganglion cells rectal manometry barium enema ->transition zone

Treatment: resection aganglionic bowel colostomy, then pull through at

6 to 12 months of age

Umbilical hernia Defect of central fascia beneath umbilicus Most common condition of abdominal wall Almost never incarcerate Strong family history and racial propensity Medical risk factors:

Congenital hypothyroidism Prematurity

Umbilical hernia DDx:

Small omphalocele (looks like fixed hernia) Supra-umbilical hernia

Refer to surgery if: Ring defect > 1.5 - 2 cm in diameter “elephant’s trunk” appearance Incarceration Not closing by 2 years of age

UROLOGY / NEPHROLOGY

Cryptorchidism 3.4% of NB, 0.7% of children > 1 year of age Bilateral in 20% Consequences- tumour, infertility, torsion,hernia Differential diagnosis

Ectopic testes Retractile testes Absent testes

surgical correction <2 year of age

Hypospadius Sibling risk - 10% Undescended testes - 10-15% Not associated with UT anomalies Do not circumcise

Enuresis Nocturnal , Diurnal, both Primary vs. Secondary 10% 5yr old, 5% 10 yr old, 1% 18 yr old Male > Female Familial maturational defect in bladder control

Enuresis Treatment

Rule out underlying cause (UTI, constipation, IDDM)

Motivational maneuvers- star charts Alarm Medication- DDAVP, TCA

The typical features of nephrotic syndrome include:A. Hypoalbuminemia, proteinuria, edema,

hyperlipidemia

B. Proteinuria, hematuria, hypertension, edema

C. hyperglycemia, glucosuria, ketonuria, weight loss

D. Proteinuria, hematuria, arthritis, purpura

Proteinuria Def”n: >150mg/24 hr (>4mg/m2/hr) Nonpathologic:

Postural- incr. in upright position 10x Collection done in supine and upright

positions Febrile Exercise

Proteinuria Tubular:

Hereditary- cystinosis, Wilson dis., RTA Acquired- antibiotics, ATN, cystic diseases,

heavy metal Glomerular:

Nephrotic syndrome Glomerulonephritis, tumour, drug, congenital

Nephrotic Syndrome Minimal change disease the most common

(76%) Membranous (8%), Focal Segmental (7%) Diagnosis

proteinuria(>40 mg/m2/hr) hypoalbuminemia hyperlipidemia edema

Nephrotic Syndrome Treatment

prednisone Renal biopsy

Unusual age (<1yr, adol.) Steroid resistant/ frequent relapse HTN, decreased renal function

Complications hypercoagulability infections Drug side effect- steroids, immunosuppressants

Marked eyelid edema in a 2-year-old boy with minimal change disease and nephrotic syndrome. Eyelid edema in any child should prompt the performance of urinalysis, rather than the presumption of allergy.

Severe scrotal edema in a 6-year-old boy with nephrotic syndrome.

Hematuria Microscopic (> 5 rbc/hpf) vs. Macroscopic Approach based on anatomy:

Kidney Ureter Bladder Urethra

Hematuria Glomerular Tubulointerstitial

ATN Infections Hypercalciuria Drugs

Hematologic causes platelets, SS disease, renal vein thombosis

Anatomic abnormalities Tumour, trauma, cysts, vascular

Exercise

Hematuria: Work-up STEP 1:

CBC, urine culture, Cr, C3, U/S 24 hr urine- Cr, protein, calcium

STEP 2: ASOT/antiDNase-B, T/S, ANA, coags, urine RBC

morphology, SS screen, VCUG (if infection/lower tract suspect)

STEP 3: biopsy

Glomerulonephritis Proteinuria, hematuria, hypertension, edema Etiology: Acute post Strep GN, IgA Nephropathy,

HSP, SLE Nephritis, RPGN, MPGN Acute Post Strep GN:

school-aged, mean age 7, 2:1(M:F), 1-2 wks between infection and presentation, dark urine, edema

Dx: UA, ASOT, anti DNAase B, low C3 (and in 6-8 wks) Management: fluid/Na restriction, diuretics +/- antiHTN 98% recover completely, sx resolve in 3-4 wks

Hemolytic Uremic Syndrome Diarrheal and non-diarrheal causes E. Coli 0157:H7 verotoxin Present with diarrhea followed by bloody

diarrhea 5-7 days later- Triad

microangiopathic hemolytic anemia thrombocytopenia renal failure

Hemolytic Uremic Syndrome Treatment

supportive lasix infusion Dialysis

No role for antibiotics, steroids Prognosis:

10-30% morbidity 5-10% mortality

RHEUMATOLOGY

A 5 year old female is admitted with acute abdominal for the past week. It is intermittent and severe. You are called to her bedside the next day for an evolving rash that is mainly on the lower limbs and appears to be raised bruising. At the same time she passes what appears to be a bloody stool. You note on review of the investigations from last night that she has 10-20 RBC/hpf on urinalysis.

What is the best treatment option you can offer to provide her with the best long term prognosis?

A. Supportive care

B. IVIG

C. Aspirin

D. Antibiotics to cover Group A strep

E. IV steroids

With this condition, what will you tell the family with regards to necessary followup?

A. She will need an echocardiogram as there is a risk of coronary aneurysms

B. She will need regular renal monitoring as there is a risk of long term renal impairment

C. There is no need for regular monitoring after the illness resolves as there are no long term sequelae

D. She will need long term antibiotic prophylaxis

A 2 year old male was seen three days ago for a three day history of fever and neck swelling. He was sent home with antibiotics to treat a cervical adenitis that measures 2x2cm. He now returns 3 days into the antibiotics with persistent fever and no improvement in his neck swelling. You note he has red conjunctiva and his lips are red and cracked. The mother is worried he is reacting to the medication as he has a new rash.

What would be the next best step in managing this child’s situation? Admit for IV antibiotics Change his oral antibiotics and follow up

in 2 days to ensure good response Send off serology for EBV infection and

discontinue the antibiotics Admit for IVIG Perform an echo and if abnormal initiate

IVIG

Henoch Schonlein Purpura Immune-mediated systemic vasculitis of small

vessels Often preceding URTI Ages 4-10yr; recurs in 1/3 Classically involves,

skin GI joints (75% have arthralgias) kidney (20% develop gross hematuria)

HSP - Skin Palpable nonthrombocytopenic

purpura May be presenting sign in only 50%

of patients. Purpura involves dependent areas of

the body. Edema of the hands, feet, and scalp

often an early finding (20-40%)

HSP - Gastrointestinal 45 to 85% of children with HSP

major hemorrhage (5%) intussusception (2%) occult bleeding (33%)

HSP Treatment

Steroids used if severe/life-threatening GI involvement

Doesn’t affect renal outcome Must monitor for renal involvement- may

occur late Watch for recurrence education

Kawasaki DiseaseCriteria F ever for 5 days Plus 4 of 5:

E xanthem E xtremities L ymph nodes (>1.5 cm) M ucosal changes C onjunctivits

“feel my conjunctivitis”

Kawasaki Disease CVS

coronary aneurysms- 20% of cases Risk < 5% if treated

Phases Acute- febrile 1-10 days Subacute (peeling, afebrile, high

platelets/ESR) 10-21 days Convalescent (normalize plt/ESR) >21 days

Kawasaki DiseaseAssociated Features Uveitis Hydropic gallbladder Carditis Pancreatitis arthritis

Sterile pyuria (urethritis)

Diarrhea Aseptic meningitis

Kawasaki Disease Treatment

IVIG Aspirin- high followed by low dose Echo at baseline and at 6-8 weeks for

coronary aneurysms

ONCOLOGY

Acute Lymphocytic Leukemia Most prevalent malignancy under 15 yo-

immature lymphoid cells accumulate in the BM (lymphoblasts)

80% of all cases of acute leukemias : peak 2-6yo

Poor prognosis if < 2yrs or > 10yrs, WBC > 50,000, CNS or testicular involvement at diagnosis, T-Cell or B-Cell, Pseudodiploidy (presence of translocations within leukemic cells), and hypodiploidy (< 46 chr. in leukemic cells), L3 morphology of blasts

ALL- Clinical Features Bone marrow failure: anemia, low plts,

neutropenia Lymphadenopathy Hepatosplenomegaly Bone pain

Acute Lymphocytic Leukemia Relapse 30 - 40%

bone marrow CNS testes

Long term problems secondary tumours infertility learning disability

Neuroblastoma Malignancy of neural crest cells Most common neoplasm in infants Most in first 4 yrs (mean=2yo) Most common site of primary tumor is abdomen (abd.

mass ,abd. pain ,HTN ) Thoracic tumors: resp. distress H & N-Horner syn. Constitutional s/sx Metastases common to bone, BM, liver lung (50%) High catecholamines in 90%

Wilms’ Tumor 5-6% of childhood Ca: 75% before 5yo Palpable abdominal mass, HTN, hematuria Bilateral in 5% Association with aniridia,

hemihypertrophy, and Beckwith Wiedmann Syndrome in 12-15%

Retinoblastoma Bilateral (40%)

hereditary - AD osteogeneic carcinoma

Unilateral (60%) sporadic

Brain Tumors Second most common tumor-most common

solid tumor cerebellum 40%, brainstem and 4th ventricle

15%, suprasellar 15% Presentation depends on location Common signs: vomiting, papilledema,

lethargy, headache, personality change, ataxia, loss of vision, seizures, focal neurologic signs, nystagmus

HEMATOLOGY

This child is at risk for:A. Overwhelming sepsis

B. Bone pain

C. Hypersplenism

D. Stroke

E. Growth failure

With this blood smear, you would expect the CBC to show:A. Low Hgb, low MCV, high retics, high

RDW

B. Low Hgb, low MCV, low retics, high RDW

C. Low Hgb, high MCV, low retics, normal RDW

D. Low Hgb, normal MCV, normal retics

With this blood smear, which statement is most accurateA. This condition is autosomal recessiveB. Hemolysis is not a feature of this diseaseC. Bony crises are a common sequelaeD. The osmotic fragility test is a diagnostic

testE. Hemoglobin electrophoresis is a

diagnostic test

Anemia Low Retics (underproduction)

MCV- Low- iron, thal,lead, chronic - Normal- renal, thyroid, infection

- High- B12, folate, fanconi High Retics (increased loss)

Blood Loss Hemolysis- intrinsic

- extrinsic

Iron Deficiency Anemia Decrease in RBC Hb due to inadequate Fe stores Etiology: poor dietary intake, early transition to

cow’s milk, high requirements with growth spurts, cow’s milk intolerance, chronic GI blood loss

Diagnosis: microcytic anemia, low serum iron, ferritin,

S/sx: irritability, anorexia, lethargy, pale, tachycardia, systolic murmur

Spherocytosis Autosomal Dominant, northern European abnormal membrane protein- Spectrin leads to hemolysis- less flexible newborn- jaundice < 24 hr disease can be mild, moderate or severe mild- mod. Anemia, reticulocytosis and

splenomegaly Dx- osmotic fragility test Rx.- support, splenectomy

From CD Atlas, MCCQE Toronto Notes, 2003

G6PD Most common RBC enzyme defect X-linked, African, Mediterranean susceptible to oxidation stress--> Hgb precip.-->

membrane damage Precipitating agents-

infection, vit. C, fava bean, benzene, Medications (nitrofurantoin, antimalarial, sulfa, nalidixic acid, vit. K analog)

intermittent hemolysis, chronic hemolysis, incidental with anemia and retics

smear- bite cells

Sickle Cell Disease Newborn asymptomatic

first sign (6 mos) - hand-foot syndrome Crises

sequestration vaso-occlusive aplastic(Parvovirus) hyperhemolytic infections

Prevent infections (considered asplenic)

From CD Atlas, MCCQE Toronto Notes, 2003

Classic HemophiliaClassic Hemophilia Factor VIII deficiency:

X-linked ie asymptomatic female carriers transmit to sons

Symptoms: excessive bruising with ambulation intramuscular hematomas from minor trauma hemarthrosis: often spontaneous hematuria risk for intracranial hemorrhage & bleeding into the

neck

Classic HemophiliaClassic Hemophilia

Severity depends on level FVIII in plasma mild 6% to 30% moderate 1% to 5% severe < 1%

Pts with mild FVIII deficiency experience only prolonged bleeding following tooth extraction, surgery or bleeding

Lab: PT normal, PTT greatly prolonged

Classic HemophiliaClassic Hemophilia

Treatment:

- prevention of trauma

- FVIII concentrates

- strict avoidance of drugs that affect platelet function

Immune Thrombocytopenic Purpura (ITP) Thrombocytopenia mediated by auto antibodies

causing destruction Peak age 2-6yo: associated with antecedent viral

infection Most resolve in 4-6wks: 20% duration > 6mos Clinical Features: purpura, petechiae, bleeding

from mouth, gums, kidney, GIT, no hepatosplenomegaly

Treatment- IVIG, steroids

CHILD ABUSE

Child Abuse Physical - 80% Sexual - 15% Physical and emotional neglect Munchausen by proxy 2nd cause of death in 1-6mos 1/3:1/3:1/3- <1yo:1-6yo:>6yo Premature and developmentally impaired have

3x risk

Red Flags Recurrent injury/ingestion Injury poorly explained/ out of proportion High risk injuries: scald, cigarette,spiral fracture, retinal

hemorrhage Injury pattern/site High risk environment

preterm, neurologic impairment single parent family stress psychiatric illness

ADOLESCENCE

Adolescence- HEEADSS Home Education Eating Activities Drugs Sexuality Suicide

Adolescent SuicideWarning Signs Depression Psychosomatic complaints Acting out Previous attempt Family history

ORTHOPEDICS

Growing Pains 3 - 6 years Night time: poorly localized Pain in shins, calves, thighs Heat, massage, Tylenol Normal physical exam

Congenital Dislocation Hip(Developmental Dislocation Hip)

Age: 2 months to 2 years Signs:

limitation of abduction tight adductors shortening of leg asymmetric skin folds + Galeazzi sign (one knee lower than other)

Congenital Dislocation HipAssess stability: Ortolani(reduce) &

Barlow(dislocate) signsTreatment: triple diaper first for mild cases of

subluxation (x 6 to 8 weeks) < 6 to 8 months, Pavlik harness > 8 months, traction closed reduction spica cast

Metatarsus Adductus Forefoot faces in rather than lining up with

2nd metatarsal May be secondary to intrauterine

positioning Early treatment is stretching If rigid may require casting to correct

An 8 year old male comes to clinic with a 5 day history of limp together with pain in the right hip and upper thigh. He is otherwise healthy and has been on no medications. Exam demonstrates limited ROM in the hip and a normal knee exam. An xray has been taken . The most likely diagnosis is.

A. Avascular necrosis of the head of the femurB. Slipped Capital Femoral EpiphysisC. Fractured femoral neckD. Septic arthritisE. Growing pains

Legg Calve Perthes Disease Avascular necrosis femoral head males > females 5 to 9 year old:sx: painful hip, limp,

referred pain X-ray: lucency, widening of the distance

and eventual distortion of the femoral head Treatment: casts to keep hip in abduction

& medial rotation surgery

This 12 year old child comes with a 2 week history of increasing pain in the right leg and limping. He has a hard time flexing his hip and prefers to keep it externally rotated when examined. He gives no history of trauma. There has been no illness. The best management of this child would be

A. Urgent consult with orthopedics

B. Semi-urgent referral to the ortho clinic

C. Routine referral to the ortho clinic

D. Expectant management, return if symptoms do not resolve in 2-4 weeks

E. Follow-up xrays in 2 weeks

Slipped Capital FemoralEpiphysis Most common in obese boys in adolescence femoral epiphysis slips posteromedially off the

metaphysis unilateral or bilateral gradual or sudden causes pain & limitation of motion abduction & internal rotation are limited Klein’s line on xray

Treatment: pinning to prevent further slipping

Osgood-Schlatter’s Disease Common cause of knee pain Mostly in athletic or overweight kids Pain arises in tibial tuberosity (at site of

attachment of patellar tendon)after exercise or kneeling

Treatment mainly supportive: includes reduced physical activity +/- bracing

DERMATOLOGY

Pityriasis rosea Prodrome rare: fever, malaise, arthralgia,

pharyngitis Herald patch: solitary 1-10cm annular papular

lesion anywhere on body 5-10 days later, widespread symmetrical

eruption involving trunk & proximal limbs < 1cm slightly raised pink/brown papules

eruption, fine scale, especially at periphery

Pityriasis Rosea Christmas tree pattern on back Duration: 2 to 12 weeks Mild to severe pruritus ? Viral trigger Treatment:

antipruritic/antihistamine lubricating lotion or topical cortosteroid if

moderate to severe pruritus

Impetigo + Prevalent during hot, humid months + Common in infants & children

Initiated by infection with Grp.A hem.strep Bullous impetigo 2o to Staph Aureus Grp.2

phase type Erythematous macule...vesicles & pustules

ImpetigoImpetigo

Bullae with erythematous halo...honey coloured crusts (non bullous form), superficial & rupture easily (bullous type

Spread by contact to other parts of body

Treatment: local measures...personal hygiene compresses to remove crusts systemic antibiotic

Atopic DermatitisAtopic Dermatitis

Inflammatory skin disorder

erythema...edema...intense pruritus...

exudation, crusting & scaling

> risk to develop allergies & asthma

Infancy: 1st 2 to 3 months weepy patches on

cheeks, neck, wrists, hands & extensor aspect extremities

Childhood: involvement of flexural

areas, especially popliteal & antecubital fossae

also, neck, wrist, behind ears, > with age; > drying & thickening of skin

Atopic Dermatitis


Other signs:

“Mask of atopic dermatitis” Hyperpigmentation of skin Lichenification


Itch - scratch - itch cycle Rx: antihistamines topical corticosteroids topical immune modulators (Protopic) hydrating lotions

Seborrheic DermatitisSeborrheic Dermatitis

Begins on scalp as “cradle cap” Involvement behind ears, sides of nose &

eyebrows Greasy, brownish scales Shorter course than atopic dermatitis Responds more rapidly to treatment than

atopic dermatitis

Treatment: General Guidelines

Keep bathing at minimum Use of non-soap cleansers Cotton garments/avoid wool Keep nails cut short Avoid environmental triggers Treat superinfections with systemic

antibiotics

Staphylococcal diaper dermatitis. There are numerous thin-walled pustules surrounded by erythematous halos, as well as multiple areas in which pustules have ruptured, leaving a collarette of scale around a denuded erythematous base.

Irritant or ammoniacal diaper dermatitis. Note the involvement of the convex surfaces and the sparing of the intertriginous creases.

Candidal diaper dermatitis. The eruption is bright red with numerous pinpoint satellite papules and pustules. Intertriginous areas are prominently involved.

Psoriatic diaper dermatitis. This child had a persistent diaper rash that did not respond to routine therapy. Note that scaling is not as intense as in psoriatic lesions seen elsewhere on the body.

Erythema Multiforme Erythema multiforme (EM

minor). A, The characteristic target lesions are symmetrically distributed. B, In these typical target lesions with central dusky areas, the peripheral rims are beginning to vesiculate. C, In this case, the peripheral rims have become frankly bullous. (C, Courtesy of Michael Sherlock, MD, Lutherville, Md.)

Stevens-Johnson Syndrome Stevens-Johnson syndrome (EM

major). A, Severe bullous and erosive lesions cover the face, neck, upper trunk, and proximal extremities. Note the ocular and oral involvement. B, Typical bullae, target lesions, and erosions of the lips are seen in this boy. C, This child has numerous vesicles and bullae of the oral mucosa along with formation of a shaggy white membrane consisting of sloughed debris. (C, Courtesy of Michael Sherlock, MD, Lutherville, Md.)

Milia White-yellow papules Epidermal inclusion cysts Face, gingivae, palate

Ebstein pearls Neonates:

Occur spontaneously Resolve spontaneously

(weeks-months) Older children:

Occur at site of trauma (blisters, abrasions)

May not resolve spontaneously

Erythema Toxicum Neonatorum White papule/pustule (1-2mm)

surrounded by red wheal Localized or generalized Palms and soles spared Usually starts ~ day 2 New lesions continue to

appear as rash waxes and wanes up to 10 days

Less common & delayed presentation in prems

Eosinophils on scraping BENIGN & SELF-LIMITING

CLASS OF 2011 FACULTY OF MEDICINE UNIVERSITY OF OTTAWA

GOOD LUCK!

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