Diagnostic and treatment progress of neonatal congenital hyperinsulinism

Abstract

Congenital hyperinsulinism (CHI) is a complex heterogeneous syndrome caused by gene mutations which lead to abnormal secretion of insulin from pancreatic β-cells. The clinical manifestations of neonatal CHI are not typical, and the brain damage is severe. Early recognition, diagnosis and reasonable management of neonatal CHI is very important, but difficult. In recent years, the research progresses of different aspects of CHI, such as molecular genetics, pathology, imaging technology, drug therapy and surgical treatment have been completely changed the clinical management of neonatal CHI. This article reviews recent research progress of the diagnosis and treatment of neonatal CHI. Key words: Persistent hyperinsulinemia hypoglycemia of infancy; Diagnosis; Therapeutics; Infant, newborn