Pancreatic Endocrine and Exocrine Function in Children Following Near-total Pancreatectomy for Diffuse Congenital Hyperinsulinism

Overview

Journal PLoS One

Specialties General Medicine
Science

Date 2014 May 21

PMID 24840042

Citations 39

Authors

Ved Bhushan Arya

Senthil Senniappan

Huseyin Demirbilek

Syeda Alam

Sarah E Flanagan

Sian Ellard

Khalid Hussain

Affiliations

Soon will be listed here.

Abstract

Context: Congenital hyperinsulinism (CHI), the commonest cause of persistent hypoglycaemia, has two main histological subtypes: diffuse and focal. Diffuse CHI, if medically unresponsive, is managed with near-total pancreatectomy. Post-pancreatectomy, in addition to persistent hypoglycaemia, there is a very high risk of diabetes mellitus and pancreatic exocrine insufficiency.

Setting: International referral centre for the management of CHI.

Patients: Medically unresponsive diffuse CHI patients managed with near-total pancreatectomy between 1994 and 2012.

Intervention: Near-total pancreatectomy.

Main Outcome Measures: Persistent hypoglycaemia post near-total pancreatectomy, insulin-dependent diabetes mellitus, clinical and biochemical (faecal elastase 1) pancreatic exocrine insufficiency.

Results: Of more than 300 patients with CHI managed during this time period, 45 children had medically unresponsive diffuse disease and were managed with near-total pancreatectomy. After near-total pancreatectomy, 60% of children had persistent hypoglycaemia requiring medical interventions. The incidence of insulin dependent diabetes mellitus was 96% at 11 years after surgery. Thirty-two patients (72%) had biochemical evidence of severe pancreatic exocrine insufficiency (Faecal elastase 1<100 µg/g). Clinical exocrine insufficiency was observed in 22 (49%) patients. No statistically significant difference in weight and height standard deviation score (SDS) was found between untreated subclinical pancreatic exocrine insufficiency patients and treated clinical pancreatic exocrine insufficiency patients.

Conclusions: The outcome of diffuse CHI patients after near-total pancreatectomy is very unsatisfactory. The incidence of persistent hypoglycaemia and insulin-dependent diabetes mellitus is very high. The presence of clinical rather than biochemical pancreatic exocrine insufficiency should inform decisions about pancreatic enzyme supplementation.

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