Pancreatic endocrine and exocrine function in children following near-total pancreatectomy for diffuse congenital hyperinsulinism.

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) pancre

SUBMITTER: Arya VB 

PROVIDER: S-EPMC4026387 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature