eMedicine Specialties > Pediatrics: General Medicine > Nephrology

Bartter Syndrome: Follow-up

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, Chief Executive Officer of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine
Contributor Information and Disclosures

Updated: Oct 1, 2008

Follow-up

Further Outpatient Care

  • Growth, development, renal function, and serum electrolytes should be evaluated periodically, ideally in a pediatric nephrology clinic. Cases of coexisting growth hormone deficiency that respond to growth hormone therapy with catch-up growth have been reported.

Complications

  • Cardiac arrhythmia and sudden death may result from electrolyte imbalances.
  • Failure to thrive, growth retardation, and developmental delay are common in untreated patients.
  • Chronic hypokalemia results in slow progression to chronic renal insufficiency.
  • A significant decrease in bone mineral density has been documented in patients with both neonatal and classic forms.

Prognosis

  • The effects of prostaglandin synthetase inhibition include an increase in the plasma potassium concentration (however, this rarely exceeds 3.5 mEq/L), a decrease in the magnitude of polyuria, and improved general well being.
  • With treatment, plasma renin and aldosterone levels normalize.
  • Therapy improves the patient's clinical condition and allows catch-up growth.
  • Bone age is usually appropriate for chronological age, and pubertal and intellectual development are normal with treatment.
  • The effectiveness of long-term use of prostaglandin synthetase inhibitors is well established. Some patients may experience a recurrence of hypokalemia, which can be managed by adjusting the indomethacin dose or with potassium supplementation.
  • The disease does not recur in the patient with a transplanted kidney.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Failure to consider Bartter syndrome in patients with hypokalemia, frequent episodes of dehydration, or growth retardation
  • Failure to institute therapy with potassium supplements and prostaglandin synthetase inhibitors in young patients

Special Concerns

  • Special attention should be paid to correcting electrolyte abnormalities when patients undergo surgical procedures.
 
Acknowledgments

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.



More on Bartter Syndrome

Overview: Bartter Syndrome
Differential Diagnoses & Workup: Bartter Syndrome
Treatment & Medication: Bartter Syndrome
Follow-up: Bartter Syndrome
Multimedia: Bartter Syndrome
References
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References

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  2. Birkenhager R, Otto E, Schurmann MJ, et al. Mutation of BSND causes Bartter syndrome with sensorineural deafness and kidney failure. Nat Genet. Nov 2001;29(3):310-4. [Medline].

  3. 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].

  4. 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].

  5. 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].

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Further Reading

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Keywords

Bartter syndrome, Bartter's syndrome, Gitelman syndrome, Gitelman's syndrome, Gullner syndrome, Gullner's syndrome, renal tubular disorder, hypokalemia, hypochloremia, metabolic alkalosis, hyperreninemia, neonatal Bartter syndrome, classic Bartter syndrome, polyuric loop dysfunction, salt-losing tubulopathy, chronic renal failure, maternal polyhydramnios, failure to thrive, strabismus, hypomagnesemia

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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, Chief Executive Officer 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.

Medical Editor

Uri S Alon, MD, Director of Research and Education, Department of Pediatrics, Division of Pediatric Nephrology, Children's Mercy Hospital of Kansas City; Professor, University of Missouri at Kansas City
Uri S Alon, MD is a member of the following medical societies: American Federation for Medical Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Adrian Spitzer, MD, Professor, Department of Pediatrics, Albert Einstein College of Medicine; Director of NIH Training Program, Children's Hospital at Montefiore Medical Center
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.

CME Editor

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, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago
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: Amgen Grant/research funds None; Abbott Honoraria Speaking and teaching; Altus Pharmaceuticals Grant/research funds None; Genzyme Grant/research funds None; Merck Grant/research funds None; NIH Grant/research funds None

 
 
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