Clinical analysis of 18 cases with congenital hyperinsulinism without response to diazoxide / 中华实用儿科临床杂志

ABSTRACT

Objective To investigate the clinical features of these children with congenital hyperinsulinism (CHI) who had no response to diazoxide and provide a theoretical foundation for the formulation of CHI treatment strategy.Methods Eighteen patients with CHI who had no response to diazoxide hospitalized in Beijing Children's Hospital from 2008 to 2012 were chosen as research subjects.Their clinical data were analyzed retrospectively.Results There were 18 patients with persistent hypoglycemia after using diazoxide,which indicated that they had no response to diazoxide.Twelve patients of them were born as macrosomia and their onset age was less than 6 months.Half of the children(9/18 cases) even had hypoglycemia in neonatal period.All the manifestations were conformed to the clinical characteristics of ATP-sensitive potassium channel CHI.Four children who were unresponsive to diazoxide received octreotide treatment,and it was effective on them.Four patients had a near-total pancreatectomy.After a long-term followup study,their blood sugar maintained a normal level,and they did not appear serious postoperative complications.Conclusions Children with CHI who have no response to diazoxide are characterized by coming earlier and higher birth weight.Octreotide is proposed in case of non-response to diazoxide.When medical treatment is not efficient in prevention of hypoglycemia,a subtotal pancreatectomy has to be considered as a last resort.