hypokalemic periodic paralysis and steven johnson's syndrome case management
DESCRIPTION
My 3rd Case Management on Hypokalemic Periodic Paralysis and Steven Johnson's Syndrome.TRANSCRIPT
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CASE MANAGEMENT ON HYPOKALEMIA ANDSTEVEN – JOHNSON’S SYNDROME
Christian Gallardo, MD1st Year Resident
Department of Internal MedicineUniversity of Perpetual Help
DALTA Medical Center
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Objectives
To discuss the diagnosis, management and treatment of Hypokalemia
To discuss the pathophysiology and clinical features of Hypokalemic Periodic Paralysis and Renal Tubular Acidosis
To discuss Allopurinol as one of the causes of Steven Johnson’s Syndrome
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General Data
S.A 31 year old Female Cavite City Roman Catholic Admitted for the first time
on July 30, 2009
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Chief Complaint:
RASHES on FACE
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History of Present Illness
Known Dyslipidemic and with Hyperuricemia recently diagnosed a month ago maintained on Simvastatin 20mg OD and Allopurinol 100mg OD.
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1 Day PTA
•(+) Rashes on malar and frontal area, cheeks and neck area with periorbital edema•(+) Cough, non productive•(+) Generalized weakness of both upper and lower extremities•Patient took 1 tablet of Lagundi tablet which offered relief.
History of Present Illness
(-) Nausea, vomiting, LBM
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History of Present Illness
Few Hours PTA
• Persistence of rashes on malar and frontal area, cheeks and neck area
• (+) ↑ periorbital edema and associated with swelling of the face
• (+) Cough• (+) ↑
generalizedweakness of upper and lower extremities
• (+) DOB• Admitted
(-) Nausea, vomiting, LBM
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Past Medical History
Hypokalemia – Cavite City Medical Center (2002) Unrecalled potassium levels Consultation at SJDH – Thyroid
Function Test - normal levels No Maintenance given
Bronchial Asthma – Last attack - 7 years old, no exacerbations afterwards
No Allergies No Operations No Hypertension, No Diabetes
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Family History
Hypokalemia – Father – with weakness episodes Had also cousins with hypokalemia on father
side Sister has also history of hypokalemia
maintained on potassium supplements
Hypertension – Maternal side Diabetes Mellitus – Maternal side Asthma – Maternal side Cervical Cancer and Breast Cancer –
Mother Leukemia – Maternal side Polycethemia Vera – Paternal side
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Personal and Social History
Non smoker Non Alcoholic Beverage
drinker Works as a nurse
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Review of Systems
General: (-) Weight Loss HEENT: (-) blurring of vision, (-)
sorethroat Cardiology: (-) chest pains, (-) PNDs, (-)
Orthopnea Gastroenterology: (-) abdominal pain, (-)
diarrhea, (-) constipation, (-) melena, (-) hematochazia
GUT: (-) dysuria, (-) oliguria (-)anuria Endocrinology: (-) polyuria, polydipsia,
polyphagia, Musculoskeletal: (-) myalgia Hematology: (-)easy brusability Neurology: (-) neuropathy, (-)seizures, (-)
headache
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General: Conscious, coherent, Not in cardiorespiratory distress
VS: BP: 110/80 CR: 92 bpmRR: 20 cpm T: 36.7°C
Skin: (+)maculopapular rashes, non-scaling, non – blanching on malar region, cheeks, frontal area, neck, abdomen, trunks, back, upper and lower extremities
HEENT: Anicteric Sclerae, pale PC, (+) conjunctival erythema, (+) oral mucocutaneous lesion, (-) TPC, (-) NAD, (-) CLAD
Physical Examination
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Physical Examination
Chest and Lungs: Symmetrical Chest Expansion, No retractions, (+) expiratory wheezes at both lung fields
Heart: Adynamic Precordium, PMI 5th ICS MCL, Normal Rate, Regular Rhythm, Normal S1 and S2, No S3 and S4, No Murmur.
Abdomen: Flat, Soft, NABS, Non-tender, No organomegaly
Extremities: No cyanosis, Full Pulses, No Edema
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Neurologic Examination
Oriented to time, person and place Cranial Nerves:
I: can smell II: 2-3mm PERTL, Fundoscopy: (+) ROR,
Clear Media, Distinct Cup borders, CDR: 1:3, AVR: 2:3, No exudates, No Hemorrhage
III, IV, VI: Full EOM V: (+) bilateral corneal reflex, good
masseter tone VII: (-) facial asymmetry VIII: can hear IX, X: (+) gag Reflex XI: can equally shrug shoulder XII: no tongue deviation
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Neurological Examination
(-) dysdiadochokinesia, (-) dysmetria, (-) Babinski (-) Brudzinski (-) Nuchal Rigidity
++1/5
++1/5 100%
3/53/5
++
++100%
100%
100%
++ ++
++++
3/5 3/5
1/5
1/5100%
100%
100%
100%
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Salient Features
35 year Old Female 1 month History of Allopurinol use Cough DOB History of hypokalemia and asthma Stong family history of hypokalemia on
father and siblings Conjunctival erythema Maculopapular Rashes on the face,
abdomen, trunks and extremities Wheezes at both lung field Generalized weakness of Upper and Lower
Extremities
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Working Impression:
Hypokalemia Probably Secondary to Renal Losses Hypokalemic Periodic
Paralysis vs Renal Tubular Acidosis
Hypersensitivity Vasculitis vs Steven-Johnson’s Syndrome Secondary to Allopurinol
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Problem #1: Weakness of extremities S> Body Weakness:
Weakness of upper and lower extremities
(-) vomiting, headache and fever O> conscious, coherent, not in
respiratory distress
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Laboratories1st HD 2nd HD 3rd HD
CBC
WBC 13.5 16.6 14.1
Hematocrit 0.46 0.43 0.43
Hemoglobin 146 128 139
Segmenters 0.77 0.74 0.58
Lymphocytes
0.17 0.15 0.28
Monocytes 0.04 0.09 0.09
Eosinophils 0.02 0.02 .05
Platelets Adequate
256 281
mmol/L1st HD 1st
HD9pm
2nd HD
Na 142 145
Cl 107
Mg 0.8
ABG
pH 7.32
pCO2 27.4
pO2 96.5
HCO3 14.0
S02 97.0
Simple Metabolic AcidosisWith adequate oxygenation
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Admsn, 9 pm
1st HD, 8 am
1st HD, 2 pm
1st HD,
10pm
2nd HD
2nd HD
3rd HD
3rd HD, 9am
4th HD
00.5
11.5
22.5
33.5
44.5
5
22.3
2.8 2.7
3.9 3.94.3
3.4
4.2
Potassium
3/5 upper extremity2/5 lower extremity
Potassium Trend
KCL drip: PNSS 8O cc + 40 meqs KCL x
6 hrs
KCL drip: PNSS 8O cc + 40 meqs KCL x
6 hrsOral K durule 2
durule TIDNaHCO3 1 Tab TID
Mg Oxide
1/5 upper extremity4/5 lower extremity
Spirinolactone 50mg/tab
then 25mg/tab BID
4 kalium Durule Q6hrsHypokalemia
2nd to1)
Hypokalemic Periodic Paralysis
2) RTA Type 1
2 kalium Durule TID
5/5 upper extremity5/5 lower extremity
Spirinolactone 25mg/tab BID
Repeat Electrolytes OPD basis
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Other LaboratoriesChemistry
Creatinine 94 mg/dL
Urine Potassium 34.4 mmol/L
Urine Osmolality 211mOsm/kg H2O
Urine Cl 68 mmol/L
Plasma Osmolality 212 mOsm/kg H20
Computation
Transtubular K+ Concentration Gradient (TTKG)
17.23
Anion Gap 21
Urine K (Uk) x Plasma Osmolality (Posm) TTKG = -------------------------------------------------------------- Plasma Potassium (Pk) x Urine Osmolality (Uosm)
Anion gap = Na- (Cl + HCO3)
Na 142 mmol/mL
Cl 107mmol/mL
HCO3 14 mmol/mL
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Weiner D and Dingo C. Hypokalemia – Consequences, Causes and Correction. American Journal of Nephrology 1999. Vol 2:1179-1188
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Primary (Familial) Autosomal dominant disorder Characterized by episodic attacks of
muscle weakness with concomitant hypokalemia (<3.5 mEq/L), which usually involves the four limbs.
Age at onset of paralytic crises - within the first or second decade.
The frequency of attacks is maximal between 15 and 35 yr of age and then decreases with age .
Weakness may be focal or generalized, usually sparing
facial and respiratory muscles, and lasting for hours
(occasionally days) withgradual resolution.
Hypokalemic Periodic Paralysis
The precipitating factors: - carbohydrate- or sodium-rich meals, - emotional stress, - and rest after exercise - exposure to cold - events associated with increase epinephrine or insulinSerum potassium are normal during asymptomatic periods
Nand B, Vohra S. Hypokalemic Periodic Paralysis: An Unusual Cause. Hospital Physician January 2003
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Hypokalemic Periodic Paralysis Mutations in a calcium channel gene
(CACNA1S) or a sodium channel gene (SCN4A)
Majority of mutations in the CACNA1S gene located on chromosome 1q31-32.
Nand B, Vohra S. Hypokalemic Periodic Paralysis: An Unusual Cause. Hospital Physician January 2003
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Hypokalemic Periodic Paralysis The mechanism for depolarization-
induced attacks of weakness in HPP is not understood.
Missense mutations in the a1 subunit of the L-type calcium channel or less commonly in the voltage-gated sodium channel cause HPP.
Curiously, for both channel types the mutations occur in highly conserved arginine residues in the voltage-sensing segments. The calcium channel mutations cause a loss-of-function manifest as reduced current density and slower activation.
A study on fibres biopsied from a patient with the R528H calcium channel mutation detected a reduction in ATP-sensitive K current, which is more easily tied to depolarization with hypokalaemia and suggests a secondary channelopathy stemming from altered calcium homeostasis.
This observation does not readily explain the episodes of depolarization, weakness and hypokalaemia. In vitro, muscle fibres from patients with HypoPP depolarize in low K solution.
Venance S et al. The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain (2006), 129, 8–17
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Renal Tubular Acidosis
Characterized by Metabolic acidosis, secondary to defects
in renal tubular reabsorption of bicarbonate (HCO3)
Urinary excretion of hydrogen (H+) Glomerular function is little or not
affected All forms of RTA present
hyperchloremic metabolic acidosis, with normal anion gapPereira P, Miranda D, Oliveira E. Molecular Pathophysiology of
Renal Tubular Acidosis. Current Genomics, 2009, 10, 51-59
Distal nephron net acid secretion is impaired. High urine pH, even in the presence of systemic acidosis. Incomplete Distal RTA
Often no metabolic acidosis and the blood bicarbonate concentration is normalDefect in renal acid excretion must be demonstrated by a failure to lower urine pH below 5.5 following an NH4Cl load or a modified furosemide test.
Acquired distal RTA Often secondary to autoimmune diseases, such as Sjogren’s syndrome.Hypokalaemia is less troublesome than in the acquired autoimmune form of distal RT.
But it can become symptomatic, especially if a thiazide diuretic is prescribed to reduce hypercalciuria.
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Nand B, Vohra S. Hypokalemic Periodic Paralysis: An Unusual Cause. Hospital Physician January 2003
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Mechanism of Hypokalemia in Mg Deficiency Magnesium deficiency exacerbates
potassium wasting by increasing distal potassium secretion.
A decrease in intracellular magnesium, caused by magnesium deficiency, releases the magnesium-mediated inhibition of ROMK channels and increases potassium secretion.
•Magnesium deficiency alone, however, does not necessarily cause hypokalemia.•An increase in distal sodium delivery or elevated aldosterone levels may be required for exacerbating potassium wasting in magnesium deficiency.
Huang C and Kuo E. Mechanism of Hypokalemia in Magnesium Deficiency. J Am Soc Nephrol 18: 2649–2652, 2007
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Hemorrhagic and crusted lesions affecting the lip, palate and buccal mucusa
Hemorrhagic and erythematous and vesicular lesion of generalised distribution
Problem #2: Rashes
Presence of erythema of the conjunctiva
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1st HD 2nd HD 3rd HD 4th HD
Urine Culture 12,000 col/mL Diptheroids
CXR Normal
Hbs AG Non- reactive
Hbs AB Non-reactive
SGOT 41
SGPT 88
Problem #2: Rashes
Increase in rashes, Increase
Conjunctival
Redness CRP Negative
ESR 1 mm/hr
ANA Negative (1:40 dilution)Hydrocortisone IV
HydroxyzineMometasone
Furoate
Rashes:Etiology
- Drug induced?
Allopurinol-Induced Dress Syndrome
Hypersensitivity Vasculitis
Steven Johnson’s Syndrome
1st HD
CBC
WBC 13.5
Hematocrit 0.46
Hemoglobin 146
Segmenters 0.77
Lymphocytes
0.17
Monocytes 0.04
Eosinophils 0.02
Platelets Adequate
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Describes the association of drug (allopurinol) together with eosinophilia and systemic symptoms (hepatitis and progressive renal failure)
Drug Rash with
Eosinophilia and Systemic
Syndrome
Allopurinol-Induced DRESS Syndrome
Markel, A. Allopurinol-Induced Dress Syndrome . Internal Medicine American Journal 2005. Vol 7: 656-660.
Skin rash, fever, lymph node enlargement and single or multiple organ involvementSkin: maculopapular eruption and facial edemaStarts 2-6 week after commencement of medication
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Allopurinol Dress Syndrome Prevelence: 2% of taking drug
Criteria
A documented intake of Allopurinol
Lack of exposure to a different drug causing a similar picture
Presence of at least 2 major criteria and 1 minor criteria• Major Criteria
- Worsening renal function - Acute hepatocellular injury - Rash, manifesting by toxic epidermal necrolysis, erythema multiforme, diffuse maculopapular rash or exfoliative dermatitis•Minor Criteria include fever, leukocytosis and eosinophila
Markel, A. Allopurinol-Induced Dress Syndrome . Internal Medicine American Journal 2005. Vol 7: 656-660.
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1st HD 2nd HD 3rd HD 4th HD
Problem #2: Rashes
Increase in rashes, Increase
Conjunctival
Redness
Skin Biopsy -
contemplated
2nd HD
CBC
WBC 16.6
Hematocrit 0.43
Hemoglobin 128
Segmenters 0.74
Lymphocytes
0.15
Monocytes 0.09
Eosinophils 0.02
Platelets 256
Mild clearing of rashes
DesloratadineButamirate
Citrate
Rashes:Etiology
- Drug induced?
Hypersensitivity Vasculitis
Steven Johnson’s Syndrome
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Laboratories
CRP Negative
ESR 1 mm/hr
ANA Negative (1:40 dilution)
Urine Culture 12,000 col/mL Diptheroids
CXR Normal
Hbs AG Non- reactive
Hbs AB Non-reactive
SGOT 41
SGPT 88
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Hypersensitivity Vasculitis American College of Rheumatology
1990 Criteria for the Classification of
Hypersensitivity Vasculitis 3+ present, sesitivity 71%,
specificity 84%
Ruddy et al. Kelly’s Textbook of Rheumatology 6th Edition 2001. Volume 2:1197
• Age > 16 y/o• Possible offending drug• Palpable Purpura• Maculopapular rash• Skin Lesion biopsy showing granulocytes (neutrophils, eosinophils or both) in a privascular or extravascular location around arteriole or venule
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Hypersensitivity Vasculitis
Clinical Manifestation Palpable purpura, fever, urticaria,
arthralgia, lymphadenopathy Begin 7-10 days after antigen exposure Includes glomerulonephritis, instertitial
nephritis, hepatocelluar injury and GI, CNS or pulmonary vasculitis
Ruddy et al. Kelly’s Textbook of Rheumatology 6th Edition 2001. Volume 2:1197
Diagnosis:
Biopsy
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1st HD 2nd HD 3rd HD 4th HD
Problem #2: Rashes
Increase in rashes, Increase
Conjunctival
Redness
Clearing of rashes
BetamethasonePrednisone 15mg
TIDPetroleum Jelly
TID
Rashes:Etiology
- Drug induced: Allopurinol
Steven Johnson’s Syndrome
3rd HD
CBC
WBC 14.1
Hematocrit 0.43
Hemoglobin 139
Segmenters 0.58
Lymphocytes
0.28
Monocytes 0.09
Eosinophils .05
Platelets 281
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Steven Johnson’s Syndrome(SJS) Life treatening, bullous,
mucocutaneous disease, generally considerd to be immune-medicated reactions to drugs. Epidermal necrosis, extensive
detachment to the epidermis, erosions of mucous membranes and severe constitutional syndrome
Halevy S, Ghislain PD, Mockenhaupt M et al. Allopurinol is the most common cause of SJS and TEN in Europe and Israel. Journal of American Academy of
Dermatology 2008:58:25-32.
SJS (<10% BSA)Toxic epidermal necrolysis (TEN) (>30% BSA)
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Steven Johnsons Syndrome(SJS) European Case-Control Surveillance
of severe cutaneous adverse drug reactions (EuroSCAR) A daily dose of 200mg or more of
allopurinol was associated with increased risk for SJS and TEN compared with lower daily doses.
Risk: Recent users ≤ 8 weeks
Halevy S, Ghislain PD, Mockenhaupt M et al. Allopurinol is the most common cause of SJS and TEN in Europe and Israel. Journal of American Academy of
Dermatology 2008:58:25-32.
Singapore and Taiwan studies: 28% of SJS due to allopurinol
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Final Diagnosis:
Hypokalemia Secondary to Hypokalemic Periodic Paralysis
Steven Johnson’s Syndrome Drug Induced (Allopurinol)
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Hypokalemia
The average daily potassium intake: 70 mEq.
90% of potassium excreted in the urine and the vast majority of the remainder in the stool.
Most potassium is present in the intracellular space.
Weiner D and Dingo C. Hypokalemia – Caonsequences, Causes and Correction. American Journal of Nephology 1999. Vol 2:1179-1188
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Hypokalemia
Potassium is freely filtered at the glomerulus, followed by reabsorption of approximately 85% by the proximal tubule and the loop of Henle.
Weiner D and Dingo C. Hypokalemia – Caonsequences, Causes and Correction. American Journal of Nephology 1999. Vol 2:1179-1188
↓ Serum K from 3.5 to 3.0 mEq/LTotal body potassium deficit = 100 to 300 mEq,
↓ 2.0 mEq/L Total body deficit = 600 to 800 mEq.
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Reabsorbtion and Secretion
Weiner D and Dingo C. Hypokalemia – Consequences, Causes and Correction. American Journal of Nephrology 1999. Vol 2:1179-1188
Principal cell is the most numerous cell, comprising 60 to 70% of the CCD, and is believed to be responsible for potassium secretion.
CCD A- and B-type intercalated cells (A cell and B cell), which comprise the remainder of the CCD, are modeled to reabsorb luminal potassium.
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Other Causes of Hypokalemia
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Manifestation of Hypokalemia Symptoms generally do not become
manifest until the serum is below 3.0 meq/L
Severe muscle weakness or paralysis – occur usually < 2.5 meq/L Lower extremities progresses to the
trunk and upper extremities
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
Other clinical manifestations:Respiratory muscle weakness, ileus, distention, anorexia, nausea, vomiting, cramps and tetany
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Cardiac Arrythmias and ECG Abnormalities Premature atrial beat and ventricular
beat, sinus beat, paroxysmal atrial tachycardia, atrioventricular block and ventricular tachycardia.
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
Depression of the ST segment, decrease in the amplitude of the T wave and increase in the amplitude of U waves which occur at the end of the T wave.U waves in the lateral precordial leads V4 to V6
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Manifestation of Hypokalemia Rhabdomyolysis
K<2.5meq/L can lead to rhabdomyolysis, muscle cramps and myoglobinuria
Renal Abnormalities (reversible) Urinary concentrating abilities, increased
renal ammonia production due to intracellular acidosis, increased renal bicarbonate reabsorbtion and hypokalemic nephropathy
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
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Correction of Hypokalemia
There is no strict correlation between the serum K concentration and total body K stores – can only be approximated in chronic hypokalemia
K deficit of 200-400 meq Lower the serum K by 1 meq/L
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
Transcellular Potassium Redistribution
•This estimation mentioned does not apply
• Diabetic ketoacidosis- may have normal plasma potassium levels despite losses due to insulin and fluids
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Correction of Hypokalemia (Oral) K preparations:
Intravenous or oral potassium is generally preferred over K citrate or K bicarbonate Metabolic alkalosis due to diuretic therapy,
vomiting, hyperaldosterism K citrate or K bicarbonate
Hypokalemia and metabolic acidosis
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
Oral therapy is usually preferred over parenteral replacement of
potassium
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Correction of Hypokalemia (IV) Intravenous administration
Who cannot eat Had severe symptomatic hypokalemia
20-40 meq/L – peripheral vein More concentrated solutions can be
infused into large veins in severe symptomatic hypokalemia
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
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Correction of Hypokalemia (IV) Maximum recommended rate (IV):
10-20meq/hr However, 40-100meq/hr – patients with
paralysis of life threatening arrhythmias Should be prepared as 10-20 meqs of K
in 100ml of fluid to avoid accidental administration of IV K.
Careful monitoring of physiologic effects of severe hypokalemia is essential
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
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Correction of Hypokalemia (IV) Saline rather than dextrose is
recommended Dextrose can lead to transient 0.2-1.4
meq/L reduction in the serum K, particularly if only 20 meq/L of KCl
Can induce arrhythmias in susceptible patients Mediated by dextrose-stimulated release
of insulin and enhancing the activity of cellular NA-K-ATPase pump
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
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Correction of Hypokalemia
Mild to moderate hypokalemia (3.0-3.5 meq/L) Directed toward replacing the lost
potassium and treating the underlying disorder (vomiting or diarrhea).
10-20 KCL 2-4x/day (20-80meqs/day) Sequential monitoring of the serum K
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
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Correction of Hypokalemia
Severe Hypokalemia (<2.5 to 3.0 meq/L or symptomatic) Potassium must be given more rapidly to
patients with severe hypokalemia Caution must be exercise
When treated, will tend to drive K into cells and worsen hypokalemia
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
• Serum K can rise acutely by as much as 1 – 1.5 meq/L after oral dose of 40-60meq and by 2.5 to 3.5 meq/L after 135-160 meq
• Larger doses - gastric irritation but is transient
• 2 meq/L = 400-800 meqs K deficit• Oral: 40-60 meqs 3-4X/day until serum K above 3.0 to 3.5 or symptoms resolve and thereafter reduce dose and frequency
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Correction of Hypokalemia
For chronic replacement – serum K should be measured 3-4x a month
Oral KCl preparation salt substitutes sprinkled on food (50-65meqs KCl per level teaspoon)
Rose B, Sterns R, Post T. Clinical Manifestation and Treatment of Hypokalemia. UpToDate 2009.
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Summary
HPP is a rare disorder of uncertain cause characterized by potentially fatal episodes of muscle weakness or paralysis.
RTA present hyperchloremic metabolic acidosis, with normal anion gap.
SJS is a life treatening, bullous, mucocutaneous disease, generally considerd to be immune-medicated reactions to drugs.
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Summary
Oral therapy is usually preferred over parenteral replacement of potassium
Mild to moderate hypokalemia (3.0-3.5 meq/L) 10-20 KCL 2-4x/day (20-80meqs/day)
Severe Hypokalemia (<2.5 to 3.0 meq/L or symptomatic) Potassium must be given more rapidly to
patients with severe hypokalemia
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