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Metabolic alkalosis

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Metabolic alkalosis

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Metabolic alkalosis

  1. 1. Metabolic Alkalosis:Metabolic Alkalosis: the Missing Puzzle Piecethe Missing Puzzle Piece By Prof. Ashraf Abdel Basset Professor of Pediatrics
  2. 2. Case PresentationCase Presentation
  3. 3. History:History: O A three-year old previously healthy female presented with recurrent episodes of quadriparesis.
  4. 4. Examination:Examination:  Apparently healthy female.  Her blood pressure was 90/60.  Apart from generalized hypotonia, her neurological examination was unremarkable.
  5. 5. She was followed in the pediatric neurology outpatient clinic as a case of periodic paralysis. Few months later, she developed a similar attack of quadriparesis.
  6. 6. Laboratory Investigations:Laboratory Investigations: Serum creatinine 0.5 mg/dl. PH 7.56, HCO3 37. Serum Na 138 mEq/L. Serum K 2.2 mEq/L. Serum Cl 93 mEq/L. Urinary Chloride 30 mEq/L.
  7. 7. So….So…. The main abnormality is “hypokalemicThe main abnormality is “hypokalemic metabolic alkalosis withmetabolic alkalosis with ↑↑ urine chloride”.urine chloride”.
  8. 8. Lumen Ca2+ , Mg2+ , Na+ , NH4 + Na+ 2Cl- K+ K+ Lumen (+) voltage Ca++ furosemide TRPV4 ROMK KCNJ1 ATP Cl- 3Na+ 2K+ ClC-Kb Barttin Blood Paracellin-1 +8 mV Cl- ClC-Ka CaSR NKCC2 SLC12A1 Thick ascending limb, Loop of HenleThick ascending limb, Loop of Henle
  9. 9. Distal convoluted tubuleDistal convoluted tubule Na+ 2Cl- K+
  10. 10. Classification of Metabolic AlkalosisClassification of Metabolic Alkalosis Chloride responsive Chloride resistant (Urine chloride<15mEq/L) (Urine chloride>20mEq/L) Metabolic alkalosis • Vomiting • Low Chloride intake • Pyloric stenosis • Chloride losing diarrhea • GI fistula • Cystic Fibrosis With Hypertension •Hyperaldosteronism •Renal artery stenosis •Renin secreting tumor •Liddle syndrome •11β-HSD deficiency •11β-Hydroxylase deficiency •17α-OH/17,20-lyase deficiency • Licorice abuse With Normal Blood Pressure Bartter syndrome Gitelman syndrome Diuretics therapy Alkali loading
  11. 11. Back to Our PatientBack to Our Patient What is the most likely diagnosisWhat is the most likely diagnosis??
  12. 12. Chloride responsive Chloride resistant (Urine chloride<15mEq/L) (Urine chloride>20mEq/L) Metabolic alkalosis • Vomiting • Low Chloride intake • Pyloric stenosis • Chloride losing diarrhea • GI fistula • Cystic Fibrosis With Normal Blood Pressure •Bartter syndrome •Gitelman syndrome •Diuretics therapy •Alkali loading With Hypertension •Hyperaldosteronism •Renal artery stenosis •Renin secreting tumor •Liddle syndrome •11β-HSD deficiency •11β-Hydroxylase deficiency •17α-OH/17,20-lyase deficiency • Licorice abuse Urinary Chloride 30 mEq/LUrinary Chloride 30 mEq/L
  13. 13. Chloride responsive Chloride resistant (Urine chloride<15mEq/L) Urine chloride>20mEq/L Metabolic alkalosis • Post-diuretics therapy • Vomiting • Low Chloride intake • Pyloric stenosis • Chloride losing diarrhea • GI fistula • Cystic Fibrosis With Normal Blood Pressure •Bartter syndrome •Gitelman syndrome •Diuretics therapy •Alkali loading With Hypertension •Hyperaldosteronism •Renal artery stenosis •Renin secreting tumor •Liddle syndrome •11β-HSD deficiency •11β-Hydroxylase deficiency •17α-OH/17,20-lyase deficiency • Licorice abuse Chloride Resistant Metabolic Alkalosis Normal Blood PressureNormal Blood Pressure
  14. 14. BartterBartter SyndromeSyndrome • Autosomal recessive renal tubular disorderAutosomal recessive renal tubular disorder characterized by hypokalemia,characterized by hypokalemia, hypocholermia, metabolic alkalosis,hypocholermia, metabolic alkalosis, hyperreninemia with normal blood pressure,hyperreninemia with normal blood pressure, decreased pressor responsiveness to infuseddecreased pressor responsiveness to infused angiotensin II, and hyperplasia of theangiotensin II, and hyperplasia of the juxtaglomerular complex.juxtaglomerular complex.Bartter FC, Et al., Am J Med 33:811-828, 1962Bartter FC, Et al., Am J Med 33:811-828, 1962..
  15. 15. Bartter SyndromeBartter Syndrome ClassificationClassification O Neonatal BartterNeonatal Bartter syndrome.syndrome. O Classic Bartter syndrome.Classic Bartter syndrome. O Gitelman syndrome.Gitelman syndrome.
  16. 16. Lumen Ca2+ , Mg2+ , Na+ , NH4 + Na+ 2Cl- K+ K+ Lumen (+) voltage Ca++ furosemide TRPV4 ROMK KCNJ1 ATP Cl- 3Na+ 2K+ ClC-Kb Barttin Blood Paracellin-1 +8 mV Cl- ClC-Ka CaSR NKCC2 SLC12A1 Thick ascending limb, Loop of HenleThick ascending limb, Loop of Henle
  17. 17. ATP 3Na+ 2K+ Na+ Cl- Ca++ Na+ Ca++ ECaC1 TRPV5 Cl- ClC-Kb Barttin NCCT thiazide NCX1 Ca++ PMCA1B Distal convoluted tubuleDistal convoluted tubule Lumen Blood -10 mV Mg++ TRPM6
  18. 18. PathophysiologyPathophysiology
  19. 19. Lumen Ca2+ , Mg2+ , Na+ , NH4 + Na+ 2Cl- K+ K+ Lumen (+) voltage Ca++ furosemide TRPV4 ROMK KCNJ1 ATP Cl- 3Na+ 2K+ ClC-Kb Barttin Blood Paracellin-1 +8 mV Cl- ClC-Ka ? CaSR NKCC2 SLC12A1 Thick ascending limb, Loop of HenleThick ascending limb, Loop of Henle
  20. 20. Lumen Ca2+ , Mg2+ , Na+ , NH4 + Na+ 2Cl- K+ K+ Lumen (+) voltage Ca++ furosemide TRPV4 ROMK KCNJ1 ATP Cl- 3Na+ 2K+ ClC-Kb Barttin Blood Paracellin-1 +8 mV Cl- ClC-Ka ? CaSR NKCC2 SLC12A1 Bartter syndromeBartter syndrome type I type II type III type IV type V
  21. 21. ATP 3Na+ 2K+ Na+ Cl- Ca++ Na+ Ca++ ECaC1 TRPV5 Cl- ClC-Kb Barttin NCCT thiazide NCX1 Ca++ PMCA1B Gitelman syndromeGitelman syndrome Lumen Blood Mg++ TRPM6
  22. 22. Bartter SyndromeBartter Syndrome ClassificationClassification O Neonatal Bartter syndrome. O Classic Bartter syndrome. O Gitelman syndrome. Bartter Syndrome Genotype-Phenotype Correlations Genetic Type Defective Gene Clinical Type Bartter type I NKCC2 Neonatal Bartter type II ROMK Neonatal Bartter type III CLCNKB Classic Bartter type IV BSND Neonatal with deafness Bartter type V CLCNKB and CLCNKA Neonatal with deafness Gitelman syndrome NCCT Gitelman syndrome
  23. 23. A) Hypokalemic metabolic alkalosis. B) Hypercalciuria. C) Activated RAAS + Normal blood pressure. D) Diminished serum PGE2 level. All the following are true ofAll the following are true of Bartter Syndrome EXCEPTBartter Syndrome EXCEPT Na+ 2Cl- K+ ↓K, volume contraction & ↑ANII →↑ entrarenal BGE2 → vicious cercal → growth retardation and hyperplasia, juxtaglomerular complex Lumen Ca2+ , Mg2+ , Na+ , NH4 + Na+ 2Cl- K+ K+ Lumen (+) voltage C a ++ furosemide TRPV4 ROMK KCNJ1 ATP Cl- 3Na+ 2K+ ClC-Kb Barttin Bloo d Paracellin-1 +8 mV Cl- ClC- Ka ? CaSR NKCC2 SLC12A1
  24. 24. The following Lab abnormalities are presentThe following Lab abnormalities are present in patients with Gitelman syndrome:in patients with Gitelman syndrome: A) Hypercalciuria B) Hypomagnesemia C) Increased urinary prostaglandins D) Normal plasma renin activity ATP 3Na+ 2K+ Na+ Cl- Ca++ Na+ Ca++ ECaC1 TRPV5 Cl- ClC-Kb Barttin NCCT thiazide NCX1 Ca++ PMCA1B Lumen Blood -10 mV Mg++ TRPM6
  25. 25. All the following are true aboutAll the following are true about neonatal Bartter syndrome EXCEPTneonatal Bartter syndrome EXCEPT A) Presents in neonatal period. B) Associated with nephrocalcinosis. C) Associated with oligohydraminos. D) May be associated with deafness.
  26. 26. BS Type I BS Type IIIBS Type I BS Type III GSGS • Age at presentationAge at presentation NeonatalNeonatal ≤ 2 years old≤ 2 years old • Growth retardationGrowth retardation SevereSevere Mild-moderateMild-moderate • PolyuriaPolyuria PresentPresent PresentPresent May be presentMay be present • NephrocalcinosisNephrocalcinosis PresentPresent +/-+/- • PolyhydramniosPolyhydramnios PresentPresent May be presentMay be present • ChondrocalcinosisChondrocalcinosis AbsentAbsent AbsentAbsent May be presentMay be present • Carpal pedal spasmCarpal pedal spasm AbsentAbsent AbsentAbsent May be presentMay be present • SN Hearing LossSN Hearing Loss May be presentMay be present AbsentAbsent AbsentAbsent Clinical picture ….Clinical picture …. Absent or mildAbsent or mild AbsentAbsent AbsentAbsent Above 5 yearsAbove 5 years
  27. 27. BS Type I BS Type IIIBS Type I BS Type III GSGS • HypokalemiaHypokalemia PresentPresent PresentPresent PresentPresent • Metabolic alkalosisMetabolic alkalosis PresentPresent PresentPresent PresentPresent • HyperreninemiaHyperreninemia PresentPresent PresentPresent PresentPresent • HyperaldosteronemiaHyperaldosteronemia PresentPresent PresentPresent PresentPresent • Urinary calciumUrinary calcium Very HighVery High HighHigh • HypomagnesemiaHypomagnesemia Absent or mildAbsent or mild Absent or mildAbsent or mild • Urinary prostaglandinsUrinary prostaglandins HighHigh HighHigh Biochemical markers ….Biochemical markers …. LowLow PresentPresent NormalNormal
  28. 28. Back to Our PatientBack to Our Patient Urinary Ca: 6 mg/kg/d Serum Mg: 2 mEq/ml
  29. 29. Neonatal BartterNeonatal Bartter SyndromeSyndrome Classic BartterClassic Bartter SyndromeSyndrome GitelmanGitelman SyndromeSyndrome
  30. 30. BS Type I BS Type III GS • Oral K supplements Usually required Usually required Usually required • K sparing diuretics Indicated Indicated Indicated • NSAIDs Used with caution Indicated Not indicated • NaCl Usually required Recommend Recommend • ACE-inhibitors Indicated Indicated Indicated • Oral Mg supplements Not required Not required Usually required • Growth Hormone May be beneficial May be beneficial May be beneficial Treatment ….
  31. 31. Hypokalemic metabolic alkalosisHypokalemic metabolic alkalosis CL, Mg,CL, Mg, Renin,Renin, AldosteroneAldosterone Ca
  32. 32. O Hypokalemic metabolic alkalosis is not an uncommon disorder among children. O Patients are often asymptomatic but they may develop serious neurologic or respiratory symptoms.
  33. 33. O Do not forget to exclude hypokalemic metabolic alkalosis on dealing with a case of unexplained paresis or paralysis. O The golden point during dealing with hypokalemia is not giving K supplementation, but knowing the cause to treat it.
  34. 34. ESPNT-14th Congress-Common Pitfalls in the Practice of Pediatric Nephrology

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