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Revista Paulista de Pediatria

Print version ISSN 0103-0582

Abstract

MAIA, Marta Liliane A. et al. Bartter's syndrome: evaluation of statural growth and metabolic profile. Rev. paul. pediatr. [online]. 2011, vol.29, n.2, pp. 146-151. ISSN 0103-0582.  http://dx.doi.org/10.1590/S0103-05822011000200003.

OBJECTIVE: Bartter's syndrome is one of the most important inherited diseases that cause chloride leak. The objective of this study was to report the follow-up of ten patients with the syndrome. METHODS: This observational study was based on the review of medical charts reporting the metabolic features, creatinine clearance, nutritional and anthropometric assessment of ten patients with Bartter's syndrome followed at the Nephrology Service of the Universidade Federal de São Paulo, in their first and last medical appointments, after a mean follow-up period of 43 months (3-76 months). During the follow-up, the management included the administration of potassium (100%) and magnesium (60%) supplements, non-steroidal anti-inflammatory agents (90%), angiotensin-converting enzyme inhibitors (40%) and spironolactone (50%). Statistical analysis was performed comparing the results of first versus last clinical appointment by non-parametric Wilcoxon test. RESULTS: Improvement of serum electrolytes and statural growth after the treatment was observed but only serum potassium [3.05mEq/L versus 3.25 mEq/L (p=0.01)] and weigh-for-age Z-score [initial median -2.47 versus -1.35 (p=0.02)] improved significantly. Out of the ten patients studied, two presented decrease of creatinine clearance with chronic kidney disease at stage 2 at the end of the follow-up. These patients had already started the follow-up with decreased creatinine clearance. CONCLUSIONS: There is a need of early treatment of patients with Bartter's syndrome in order to improve their electrolytes and nutritional condition without compromising the creatinine clearance. 

Keywords : metabolic alkalosis; failure to thrive; polyuria; hypokalemia; developmental disabilities; Bartter syndrome.

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