A retrospective clinical analysis of 68 patients with congenital hyperinsulinism

Abstract

Objective To analyze the clinical characteristics and treatment strategy in 68 patients with congenital hyperinsulinism(CHI).Methods Sixty-eight patients with CHI hospitalized from February,2002 to December,2011 were recruited.Clinical data of the patients were collected and the clinical characteristics and treatment methods were retrospectively analyzed.Results Among the 68 patients,46 were treated with diazoxide as a trial for 7-10 days.36 of these patients were responsive to diazoxide,while the other 10 patients were unresponsive.1 patient who was unresponsive to diazoxide was treated with octreotide as a trial for 12 days with some effect,but eventually the treatment was stopped due to serious gastrointestinal side effects.5 patients were treated with subtotal pancreatectomy,achieving in nearly normal blood glucose levels after surgery in 4 cases,and the rest 1 patient having diabetes mellitus 1 month after operation.Conclusions (1) Diazoxide is the first choice in treating CHI with relatively high efficacy and safety.(2) Octreotide is the second-line medicine to treat CHI.For the patients unresponsive to diazoxide,octreotide may be chosen as a trial to control serious hypoglycemia.(3) For the patients unresponsive to diazoxide,subtotal pancreatectomy is one of the effective methods to control hypoglycemia.Since there is no effective way to discriminate the pathological types of CHI,the choice of pancreatectomy remains optional. Key words: Hyperinsulinism, congenital; Diazoxide; Pancreatectomy