Clinical analysis of 12 cases with congenital hyperinsulinism with spontaneous remission

Abstract

Objective To provide a more reasonable therapeutic schedule for congenital hyperinsulinism (CHI) children and to analyze the clinical characteristics and follow-up data of the 12 cases with CHI whose symptoms alleviated spontaneously. Methods We choosed 12 patients with CHI whose symptoms alleviated spontaneously, hospitalized in Beijing Children's Hospital from December 2008 to January 2014,as research subjects. The clinical data and post follow-up data were analyzed retrospectively. Results The 12 patients can maintain normal blood glucose for at least three months without drug treatment or after stopping the drug, which suggest that the group of children with CHI could relieve itself. The hypoglycemia relieved in 4 patients only by strengthening the feeding, and the other 8 children relieved gradually after diazoxide and (or) octreotide treatment. The onset age of the 12 children were all less than one year, and most of them younger than six months at onset (10/12). Most of them were appropriate for gestational age (7/12), 4 patients of them were born as macrosomia and the other one was small for gestional age. Seven cases of them alleviated in less than one year old (7/12), and the latest age of alleviation was 6 years. Four children were found carrying the mutation of ABCC8 gene. Conclusion Some children with CHI have the tendency of spontaneous remission, and the follow-up time should be extended. Especially for children with CHI who are not responsive to diazoxide, surgical treatment should be conside red cautiously to avoid serious surgical complications . Key words: Congenital hyperinsulinism; ATP-sensitive potassium channel; Diazoxide