Uncontrolled insulin secretion from a childhood pancreatic beta-cell adenoma is not due to the functional loss of ATP-sensitive potassium channels.

Abstract:

:We report the case of an 8-year-old child who presented with severe hyperinsulinaemic hypoglycaemia due to a pancreatic islet cell adenoma. In vivo, there was no beneficial response to the hyperglycaemia-inducing agent diazoxide and as a consequence the child underwent a subtotal pancreatectomy. In vitro studies of adenomatous beta-cells revealed no operational defects in ATP-sensitive potassium channel activity and appropriate responses to diazoxide. In comparison with patients with focal adenomatous hyperplasia, genetic analysis of the isolated adenoma showed no loss of heterozygosity for chromosome 11p15 and expression of the cyclin-dependent kinase inhibitor p57(kip2). This case illustrates that the excess insulin secretion from an infantile adenoma has an aetiology different from that observed in hyperinsulinism in infancy.

journal_name

Endocr Relat Cancer

journal_title

Endocrine-related cancer

authors

Hussain K,Cosgrove KE,Shepherd RM,Chapman JC,Swift SM,Smith VV,Kassem SA,Glaser B,Lindley KJ,Aynsley-Green A,Dunne MJ

doi

10.1677/erc.0.0090221

subject

Has Abstract

pub_date

2002-12-01 00:00:00

pages

221-6

issue

4

eissn

1351-0088

issn

1479-6821

journal_volume

9

pub_type

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