hrp0097p1-91 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2023

Short- and Long-term Outcomes of Diazoxide Unresponsive Infants with Diffuse Hyperinsulinism

Rafferty Deborah , Truong Lisa , Nedrelow Jonathan , Uffman Chip , Pugenent Burton , Sanchez Irene , Thornton Paul

Background: Severe diazoxide unresponsive hyperinsulinism (DUHI) is most often caused by autosomal recessive variants in the KATP channel genes. Because of the limited medical treatments available, many patients are treated with 98% pancreatectomy. This results in a high rate of diabetes by the age of 15 years. Many centers now try to avoid surgery to prevent the inevitable transition to post-surgical diabetes.Objectives:</strong...