Endocrine Abstracts

Introduction: Diffuse congenital hyperinsulinism (CHI) is a major cause of severe hypoglycaemia. One treatment option is near-total pancreatectomy, which carries a risk of diabetes mellitus (DM) and pancreatic exocrine insufficiency.

Objective: We report our centre’s experience on 36 consecutive medically unresponsive diffuse CHI children managed with near-total pancreatectomy.

Methods: Following near-total pancreatectomy, these children underwent regular 24-h blood glucose profile, controlled fast and oral glucose tolerance tests to assess glucose homeostasis. Clinical and biochemical evidence (faecal elastase 1) of pancreatic exocrine insufficiency was also evaluated.

Results: From 1994 to 2012, 36 children (male/female, 16/20) underwent near-total pancreatectomy for medically unresponsive diffuse CHI. The mean (±S.D.) birth weight (g) and gestational age (weeks) was 4080±730 and 38±2 respectively. The patients presented with severe hyperinsulinaemic hypoglycaemia at a median age of 1 day. Molecular genetic analysis revealed ABCC8/KCNJ11 mutation in 31 patients. The median age at pancreatectomy was 0.17 years. The diagnosis of diffuse disease was reached either by pancreatic venous sampling or ¹⁸F DOPA PET–CT. Histology confirmed diffuse disease in all patients. Hypoglycaemia persisted post-pancreatectomy in 16 patients (44%), necessitating either total-pancreatectomy, treatment with octreotide, diazoxide and/or frequent high carbohydrate feeds. Over a mean (±S.D..) follow-up period of 8 (±4.75) years, 18 children developed DM. The cumulative risk of DM after 5 and 10 years post-pancreatectomy was 74 and 86% respectively. Twenty-six patients (72%) showed biochemical evidence of pancreatic exocrine insufficiency, only seventeen (47%) of which developed clinical pancreatic exocrine insufficiency necessitating pancreatic enzyme replacement.

Conclusions: Near-total pancreatectomy for CHI is associated with high risk of DM, however not all patients with post-pancreatectomy go onto develop clinical or biochemical evidence of pancreatic exocrine insufficiency. There seems to be a poor correlation between biochemical and clinical pancreatic exocrine insufficiency.