Endocrine Abstracts

Congenital hyperinsulinism (CHI) is a cause of severe and persistent hypoglycaemia due to unregulated insulin secretion from pancreatic β-cells. Mutations in the ABCC8 and KCNJ11 genes are the most common cause of medically unresponsive CHI. Histologically there are three major subgroups, focal, diffuse and atypical. The pathophysiology of focal CHI is complex and involves a two hit process with the patient firstly inheriting a paternal ABCC8/KCNJ11 m...

Background: Congenital hyperinsulinism (CHI) is cause of severe hyperinsulinaemic hypoglycaemia in the neonatal and infancy periods. Histologically there are three major subgroups, diffuse, focal and atypical. Patients with diffuse CHI who are medically unresponsive will require a near total pancreatectomy. This has traditionally been performed using an open surgical approach.Aims: To report our experience of laparoscopic near total pancreatectomy for me...

Introduction: Hyperinsulinaemic hypoglycaemia (HH) is the most common cause of severe and persistent hypoglycaemia in neonates. The treatment of diazoxide unresponsive HH involves pancreatectomy. Mammalian target of rapamycin (mTOR) is a protein kinase that regulates cellular proliferation. We aimed to evaluate the efficacy of mTOR inhibitor Sirolimus and assess mTOR expression in the pancreas of infants with severe HH.Methods: Four infants with severe, ...