Acetyl salicylic acid treatment in neonatal Bartter syndrome—a commentary letter
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Martin Kِmhoff
- چاپ و سال / کشور: 2011
Description
Antenatal Bartter syndrome is an autosomal recessive disorder caused by loss-of-function mutations in genes encoding for the renal outer medullary potassium channel (RomK) or the sodium–potassium-2-chloride co-transporter (NKCC2) [1]. Given that NKCC2 is sensitive to inhibition by furosemide and requires RomK for proper functioning, defects in either transport protein severely impair salt reabsorption in the thick ascending loop of Henle, thereby mimicking chronic administration of furosemide. Apart from the occurrence of transient hyperkalemia in patients with mutant RomK, which reflects their requirement to secrete potassium into the urine by the distal tubules, the phenotypes of NKCC2- and RomK-deficient patients are undistinguishable: polyuria shortly after birth with isosthenuria, excessive renal prostaglandin E2 production, and hyper-reninism with hypokalemic alkalosis.
Pediatr Nephrol (2011) 26:1341–1342 DOI 10.1007/s00467-011-1922-x Received: 27 April 2011 / Revised: 28 April 2011 / Accepted: 2 May 2011 / Published online: 31 May 2011
