Genetic pathogenesis and current trends in diagnosis and therapy of ATP-sensitive potassium channel congenital hyperinsulinism
10.3760/cma.j.issn.2095-428X.2016.08.018
- VernacularTitle:ATP敏感性钾离子通道型先天性高胰岛素血症的遗传发病机制及诊疗进展
- Author:
Zidi XU
;
Yanmei SANG
- Publication Type:Journal Article
- Keywords:
Congenital hyperinsulinism;
ATP-sensitive potassium channel;
Diazoxide;
Pancreatectomy
- From:
Chinese Journal of Applied Clinical Pediatrics
2016;31(8):628-630
- CountryChina
- Language:Chinese
-
Abstract:
ATP-sensitive potassium channel congenital hyperinsulinism (KATP-HI) is the most common and most severe type of congenital hyperinsulinism,accounting for 40％-45％.It is due to the inactivating mutations of the ABCC8 and KCNJ11 gene which encode the ATP-sensitive potassium channel.Diazoxide is the main and preferred therapy for KATP-HI.For KATP-HI children who are unresponsive to medical therapy usually need different degrees of pancreatectomy to maintain normal blood sugar level.
