Effect of non-steroidal anti-inflammatory drugs in children with Bartter syndrome

Abstract

Introduction Bartter syndrome (BS) is a congenital salt-wasting tubulopathy with an induced expression of cyclooxygenase-2 in the macula densa leading to hyperreninemia. Renin Angiotensin Aldosterone system (RAAs) activation leads to hypokalaemic alkalosis. NSAIDs are now currently used in BS, however few studies have investigated the effect of NAIDs on RAAs activation, biological parameters and treatment modifications. Material and methods We included 19 patients with BS treated with NAIDs between 1994 and 2016. We assessed renin and aldosterone serum levels, serum electrolytes, calcium, phosphorus, Vitamin D and PTH before and after treatment initiation. We also recorded modifications in sodium and potassium supplements and adverse events. Results Median age and weight at treatment initiation were 7 [4–49] months and 6175 [4360–13,675] grams respectively. Serum renin and aldosterone levels significantly decreased from 1532 [952–2638] to 226 [132–704] pg/mL (P Conclusion In this report, we confirm the major benefit of NSAIDs treatment in patients with BS. We also assess that monitoring renin serum level is of interest to adapt patients’ treatments. Finally, we demonstrate a beneficial effect of NSAIDs both on the decrease of the calciuria and the decrease of bone turn-over.