Pediatric Bartter Syndrome Medication

  • Author: Prasad Devarajan, MD; Chief Editor: Craig B Langman, MD   more...
 
Updated: Nov 14, 2011
 

Potassium supplements

Class Summary

Correction of hypokalemia is the most important goal of medical therapy.

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Potassium chloride (Klor-Con, Kaon, K-Tab, K-Dur, Micro-K)

 

Can be administered in various formulations; chloride salt is recommended because of coexisting chloride deficiency. Essential for transmission of nerve impulses, contraction of cardiac muscle, maintenance of intracellular tonicity, skeletal and smooth muscles, and maintenance of normal renal function.

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Prostaglandin synthetase inhibitors

Class Summary

These medications blunt the prostaglandin overproduction, which is responsible for the pressor resistance to angiotensin II and norepinephrine, hyperreninemia and increased sympathoadrenal activity. By inhibiting PGE2 synthesis, they also contribute to the correction of the hemoconcentration defect.

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Indomethacin (Indocin)

 

Nonsteroidal drug with anti-inflammatory, antipyretic, and analgesic properties thought to be mediated by its potent prostaglandin inhibitory effect; ensuing hyporeninemic hypoaldosteronism is thought to be responsible for potassium retention.

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Ibuprofen (Motrin, Ibuprin)

 

Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.

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Potassium-sparing diuretics

Class Summary

These medications enhance the effect of potassium supplementation by decreasing urinary potassium losses.

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Spironolactone (Aldactone)

 

Specific antagonist of aldosterone, primarily by competitively binding to receptors at the aldosterone-dependent sodium-potassium exchange site in the distal convoluted tubule.

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Amiloride (Midamor)

 

Inhibits sodium reabsorption at the distal convoluted tubule, cortical collecting tubule, and collecting duct. This decreases the net negative potential of the tubular lumen and reduces both potassium and hydrogen secretion and their subsequent excretion.

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Contributor Information and Disclosures
Author

Prasad Devarajan, MD  Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of Clinical Nephrology Laboratories, CEO of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine

Prasad Devarajan, MD is a member of the following medical societies: American Heart Association, American Society of Nephrology, American Society of Pediatric Nephrology, National Kidney Foundation, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Uri S Alon, MD  Director of Bone and Mineral Disorders Clinic and Renal Research Laboratory, Children's Mercy Hospital of Kansas City; Professor, Department of Pediatrics, Division of Pediatric Nephrology, University of Missouri-Kansas City School of Medicine

Uri S Alon, MD is a member of the following medical societies: American Federation for Medical Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Adrian Spitzer, MD  Clinical Professor Emeritus, Department of Pediatrics, Albert Einstein College of Medicine

Adrian Spitzer, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Pediatric Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Howard Trachtman, MD  Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine

Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD  The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, Children's Memorial Hospital

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology

Disclosure: NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Abubakr Imam, MD, to the development and writing of the initial version of this article.

References

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  2. Deschênes G, Fila M. Primary molecular disorders and secondary biological adaptations in bartter syndrome. Int J Nephrol. 2011;2011:396209. [Medline]. [Full Text].

  3. Janssen AG, Scholl U, Domeyer C, et al. Disease-Causing Dysfunctions of Barttin in Bartter Syndrome Type IV. J Am Soc Nephrol. Sep 5 2008;[Medline].

  4. Kitanaka S, Sato U, Maruyama K, Igarashi T. A compound heterozygous mutation in the BSND gene detected in Bartter syndrome type IV. Pediatr Nephrol. Feb 2006;21(2):190-3. [Medline].

  5. Zaffanello M, Taranta A, Palma A, et al. Type IV Bartter syndrome: report of two new cases. Pediatr Nephrol. Jun 2006;21(6):766-70. [Medline].

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  7. Garcia-Nieto V, Flores C, Luis-Yanes MI, et al. Mutation G47R in the BSND gene causes Bartter syndrome with deafness in two Spanish families. Pediatr Nephrol. May 2006;21(5):643-8. [Medline].

  8. Kramer BK, Bergler T, Stoelcker B, Waldegger S. Mechanisms of Disease: the kidney-specific chloride channels ClCKA and ClCKB, the Barttin subunit, and their clinical relevance. Nat Clin Pract Nephrol. Jan 2008;4(1):38-46. [Medline].

  9. Seyberth HW. An improved terminology and classification of Bartter-like syndromes. Nat Clin Pract Nephrol. Aug 2008;[Medline].

  10. Walsh SB, Unwin E, Vargas-Poussou R, Houillier P, Unwin R. Does hypokalaemia cause nephropathy? an observational study of renal function in patients with Bartter or Gitelman syndrome. QJM. Nov 2011;104(11):939-44. [Medline].

  11. Chaudhuri A, Salvatierra O Jr, Alexander SR, Sarwal MM. Option of pre-emptive nephrectomy and renal transplantation for Bartter's syndrome. Pediatr Transplant. Mar 2006;10(2):266-70. [Medline].

  12. Bichet DG, Fujiwara TM. Reabsorption of sodium chloride--lessons from the chloride channels. N Engl J Med. Mar 25 2004;350(13):1281-3. [Medline].

  13. Estevez R, Boettger T, Stein V, et al. Barttin is a Cl- channel beta-subunit crucial for renal Cl- reabsorption and inner ear K+ secretion. Nature. Nov 29 2001;414(6863):558-61. [Medline].

  14. Hebert SC. Bartter syndrome. Curr Opin Nephrol Hypertens. Sep 2003;12(5):527-32. [Medline].

  15. Miyamura N, Matsumoto K, Taguchi T, et al. Atypical Bartter syndrome with sensorineural deafness with G47R mutation of the beta-subunit for ClC-Ka and ClC-Kb chloride channels, barttin. J Clin Endocrinol Metab. Feb 2003;88(2):781-6. [Medline]. [Full Text].

  16. Rodriguez-Soriano J, Vallo A, Aguirre M. Bone mineral density and bone turnover in patients with Bartter syndrome. Pediatr Nephrol. Aug 2005;20(8):1120-5. [Medline].

  17. Rodriguez-Soriano J, Vallo A, Perez de Nanclares G, et al. A founder mutation in the CLCNKB gene causes Bartter syndrome type III in Spain. Pediatr Nephrol. Jul 2005;20(7):891-6. [Medline].

  18. Scheinman SJ, Guay-Woodford LM, Thakker RV, Warnock DG. Genetic disorders of renal electrolyte transport. N Engl J Med. Apr 15 1999;340(15):1177-87. [Medline].

  19. Schlingmann KP, Konrad M, Jeck N, et al. Salt wasting and deafness resulting from mutations in two chloride channels. N Engl J Med. Mar 25 2004;350(13):1314-9. [Medline].

  20. Shalev H, Ohali M, Kachko L, Landau D. The neonatal variant of Bartter syndrome and deafness: preservation of renal function. Pediatrics. Sep 2003;112(3 Pt 1):628-33. [Medline]. [Full Text].

  21. Starremans PG, Kersten FF, Knoers NV, et al. Mutations in the human Na-K-2Cl cotransporter (NKCC2) identified in Bartter syndrome type I consistently result in nonfunctional transporters. J Am Soc Nephrol. Jun 2003;14(6):1419-26. [Medline]. [Full Text].

  22. Vaisbich MH, Fujimura MD, Koch VH. Bartter syndrome: benefits and side effects of long-term treatment. Pediatr Nephrol. Aug 2004;19(8):858-63. [Medline].

 
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Normal transport mechanisms in the thick ascending limb of the loop of Henle. Reabsorption of sodium chloride is achieved with the sodium-chloride potassium-chloride cotransporter, which is driven by the low intracellular concentrations of sodium, chloride, and potassium. Low concentrations are maintained by the basolateral sodium pump (sodium-potassium adenosine triphosphatase), basolateral chloride channel (ClC-kb), and apical potassium channel (ROMK).
Type I neonatal Bartter syndrome. Mutations in the sodium-chloride potassium-chloride cotransporter gene result in defective reabsorption of sodium, chloride, and potassium.
Type II neonatal Bartter syndrome. Mutations in the ROMK gene result in an inability to recycle potassium from the cell back into the tubular lumen, with resultant inhibition of the sodium-chloride potassium-chloride cotransporter.
Classic Bartter syndrome. Mutations in the ClC-kb chloride channel lead to an inability of chloride to exit the cell, with resultant inhibition of the sodium-chloride potassium-chloride cotransporter.
Table. Bartter Syndrome Genotype-Phenotype Correlations
Bartter Syndrome Genotype-Phenotype Correlations
Genetic Type Defective Gene Clinical Type
Bartter type INKCC2Neonatal
Bartter type IIROMKNeonatal
Bartter type IIICLCNKBClassic
Bartter type IVBSNDNeonatal with deafness
Bartter type VCLCNKB and CLCNKANeonatal with deafness
Gitelman syndromeNCCTGitelman syndrome
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