Metabolic alkalosis
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Metabolic alkalosis
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Metabolic alkalosis
- 1. Metabolic Alkalosis:Metabolic Alkalosis: the Missing Puzzle Piecethe Missing Puzzle Piece By Prof. Ashraf Abdel Basset Professor of Pediatrics
- 3. History:History: O A three-year old previously healthy female presented with recurrent episodes of quadriparesis.
- 4. Examination:Examination: Apparently healthy female. Her blood pressure was 90/60. Apart from generalized hypotonia, her neurological examination was unremarkable.
- 6. 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.
- 7. 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.
- 8. So….So…. The main abnormality is “hypokalemicThe main abnormality is “hypokalemic metabolic alkalosis withmetabolic alkalosis with ↑↑ urine chloride”.urine chloride”.
- 10. 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
- 11. Distal convoluted tubuleDistal convoluted tubule Na+ 2Cl- K+
- 12. 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
- 13. Back to Our PatientBack to Our Patient What is the most likely diagnosisWhat is the most likely diagnosis??
- 14. 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
- 15. 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
- 16. 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..
- 17. Bartter SyndromeBartter Syndrome ClassificationClassification O Neonatal BartterNeonatal Bartter syndrome.syndrome. O Classic Bartter syndrome.Classic Bartter syndrome. O Gitelman syndrome.Gitelman syndrome.
- 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. 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
- 22. 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
- 23. 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
- 24. 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
- 25. 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
- 26. 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
- 27. 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
- 28. 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.
- 30. 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
- 31. 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
- 32. Back to Our PatientBack to Our Patient Urinary Ca: 6 mg/kg/d Serum Mg: 2 mEq/ml
- 33. Neonatal BartterNeonatal Bartter SyndromeSyndrome Classic BartterClassic Bartter SyndromeSyndrome GitelmanGitelman SyndromeSyndrome
- 35. 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 ….
- 37. Hypokalemic metabolic alkalosisHypokalemic metabolic alkalosis CL, Mg,CL, Mg, Renin,Renin, AldosteroneAldosterone Ca
- 38. 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.
- 39. 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.
- 40. ESPNT-14th Congress-Common Pitfalls in the Practice of Pediatric Nephrology
Notas del editor
- Generating the lumen-positive electrical charge that drives the paracellular reabsorption of Na+, Ca2+ and Mg2+ in this segment( Na absorption depends on NKCC2 with about half of the sodium taking the transcellular route and half taking a paracellular route by cation selective intercellular pathways. The absorbed Na—via counter-current multiplication—contribute to medullary interstitial hypertonicity which is the osmotic driving force for water absorption along CD. All Cl is absorbed through transcellular route. K which enter through NKCC2 recycles back to the tubular urine through renal outer medullary potassium (ROMK) channels. Luminal K secretion in addition establishes a lumen positive transepithelial voltage gradient that provides a driving force for paracellular transport of cations like sodium, calcium, and magnesium. Basolateral sodium release from the cell is by the sodium pump. basolateral chloride exit by two highly homologous ClC-K-type chloride channel proteins (ClC-Ka and ClC-Kb) associate with their beta subunit barttin.
- Generating the lumen-positive electrical charge that drives the paracellular reabsorption of Na+, Ca2+ and Mg2+ in this segment( Na absorption depends on NKCC2 with about half of the sodium taking the transcellular route and half taking a paracellular route by cation selective intercellular pathways. The absorbed Na—via counter-current multiplication—contribute to medullary interstitial hypertonicity which is the osmotic driving force for water absorption along CD. All Cl is absorbed through transcellular route. K which enter through NKCC2 recycles back to the tubular urine through renal outer medullary potassium (ROMK) channels. Luminal K secretion in addition establishes a lumen positive transepithelial voltage gradient that provides a driving force for paracellular transport of cations like sodium, calcium, and magnesium. Basolateral sodium release from the cell is by the sodium pump. basolateral chloride exit by two highly homologous ClC-K-type chloride channel proteins (ClC-Ka and ClC-Kb) associate with their beta subunit barttin.
- DCT1 cells express an apical magnesium conductance (TRPM6), whereas DCT2 cells provide an apical calcium conductance formed by ECaC (TRPV5). Impairment of DCT1 cell function by mutations of NCCT or ClC-Kb might shift the DCT1/DCT2 cell ratio in favor of DCT 2 cells, resulting in increased magnesium and decreased calcium excretion. 2 types of cells are present DCT1, DCT2. NaCl absorption in the DCT1 occurs almost exclusively by the transcellular route mediated by the sodium chloride co-transporter NCCT. The absorbed Na does not contribute to the urinary concentrating mechanisms. No apical K conductance TRPM6 allow apical conductance of Mg The basolateral conductance of Na and Cl is the same as TAL. DCT2 express apical epithelial Na channel(e Na c) and apical conductance of Ca by E Ca C (TRPV5).