The authors declare no conflicts of interest. “
“Bartter syndrome (BS) encompasses

a group of rare genetic, autosomal recessive, renal tubular diseases characterized by urinary loss of sodium, potassium, and chloride; hypokalemic metabolic alkalosis; high plasma levels of renin HSP inhibitor and aldosterone; and high levels of prostaglandins (PGs) in blood and urine as a secondary phenomenon. Clinically patients present polyuria, polydipsia, failure to thrive, life-threatening episodes of dehydration, episodes of fever, and normal or low blood pressure. Frequently, pediatricians are the first professionals to attend to these patients and it is therefore important to be aware of this condition, since prognosis is better with earlier diagnosis and treatment. There are different types of BS, and

clinical and laboratorial variability depends on the affected tubular carrier.1 and 2 According to the affected region, some differences can be observed in the management of the disease, for instance, type II BS is associated with very mild hypokalemia, whereas RO4929097 order in type IV BS, treatment with indomethacin is much less effective.3 The present study aimed to describe the results of a long-term follow-up of BS patients treated with different drugs. This retrospective study, based on a prospective protocol, enrolled patients with clinical and laboratorial diagnosis of BS from 1993 until 2012, and adherent to the treatment, which was evaluated by adherence to scheduled

clinic appointments and serum bicarbonate and SPTLC1 potassium levels. Genetic analysis is not available in this service. The protocol was initially based on electrolytes supplementation (potassium and, in some cases, sodium), spironolactone, and the non-selective non-steroidal anti-inflammatory drug (nsNSAID), indomethacin. However, during the period of indomethacin treatment (1993 to 2003), six of 12 (50%) patients presented significant gastrointestinal symptoms;4 and since 2003, it was decided to adopt a selective NSAID (sNSAID), celecoxib, in order to avoid gastrointestinal compromise. Patients who developed proteinuria were converted to an angiotensin conversion enzyme inhibitor (ACEi), enalapril, in replacement to NSAID. This conversion was made during hospitalization, since patients can potentially develop serious hypotension with ACEi. The following variables were evaluated during treatment with each drug: Z-score for weight and stature, glomerular filtration rate (GFR) through creatinine clearance (using Schwartz’s formula, since urinary collection of 24 hours is difficult, especially in polyuric patients),5 average potassium supplementation, and serum levels of potassium and bicarbonate. The presence of proteinuria, gastrointestinal (GI) complaints, and findings of upper digestive endoscopy (UDE) were also evaluated.

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