Korean J Pediatr.  2007 Dec;50(12):1231-1240. 10.3345/kjp.2007.50.12.1231.

The clinical manifestations, the short- and long-term outcomes of Bartter syndrome

• Park HW
• Lee JH
• Park YS

• KMID: 2279456
• DOI: http://doi.org/10.3345/kjp.2007.50.12.1231

Abstract

PURPOSE: Bartter syndrome is a renal tubular defect in electrolyte transport characterized by hypokalemia, metabolic alkalosis and other clinical signs and symptoms. The aims of this study were to analyze the clinical manifestations and the short- and long-term outcomes of Bartter syndrome.
METHODS
We retrospectively reviewed clinical history, laboratory finding of blood and urine, renal ultrasonography, and hearing tests of five patients who were diagnosed and managed with Bartter syndrome at Asan Medical Center from April 1992 to May 2007. We also evaluated height and body weight periodically after institution of therapy.
RESULTS
All patients had poor oral intake, failure to thrive and polyuria. Three of them had maternal history of polyhydramnios and premature delivery. The mean age at diagnosis was 11.8 months. All children presented with hypokalemia, metabolic alkalosis, hyperreninemia. Their blood pressures were normal. One patient had nephrocalcinosis on renal ultrasonography and all of them had normal result in hearing tests. After treatment with indomethacin or other prostaglandin inhibitors and potassium supplementation, their clinical features improved with catch-up growth and improvement in the development during long-term follow-up.
CONCLUSION
We emphasize that early diagnosis and proper treatment in patient with Bartter syndrome are related to better prognosis.

Keyword

Bartter syndrome; Failure to thrive; Growth; Treatment outcome

MeSH Terms

Alkalosis
Bartter Syndrome*
Body Weight
Child
Chungcheongnam-do
Diagnosis
Early Diagnosis
Failure to Thrive
Follow-Up Studies
Hearing Tests
Humans
Hypokalemia
Indomethacin
Nephrocalcinosis
Polyhydramnios
Polyuria
Potassium
Prognosis
Prostaglandin Antagonists
Retrospective Studies
Treatment Outcome
Ultrasonography
Indomethacin
Potassium
Prostaglandin Antagonists