Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2013) 33 P21 | DOI: 10.1530/endoabs.33.P21

BSPED2013 Poster Presentations (1) (89 abstracts)

Long-term endocrine and exocrine outcome of medically unresponsive diffuse congenital hyperinsulinism managed with near-total pancreatectomy: 18 years' experience

Ved Bhushan Arya 1 , Syeda Alam 1 , Senthil Senniappan 1 , Sarah E Flanagan 2 , Sian Ellard 2 & Khalid Hussain 1

1UCL Institute of Child Health, Great Ormond Street Hospital NHS Foundation Trust, London, UK; 2Institute of Biomediacal and Clinical Sciences, Exeter, UK.

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.

Volume 33

41st Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

Browse other volumes

Article tools

My recent searches

No recent searches.