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Endocrine Abstracts (2015) 39 OC6.1 | DOI: 10.1530/endoabs.39.OC6.1

BSPED2015 ORAL COMMUNICATIONS Oral Communications 6 (10 abstracts)

Somatostatin-expressing cells contribute to the pathobiology of atypical congenital hyperinsulinism in infancy

Bing Han 1 , Melanie Newbould 2 , Gauri Batra 2 , Edmund Cheesman 2 , Ross Craigie 2 , Zainab Mohamed 1 , Lindsey Rigby 2 , Raja Padidela 2 , Mars Skae 1 , Karen Cosgrove 2 , Mark Dunne 1 & Indraneel Banerjee 2


1University of Manchester, Manchester, UK; 2Royal Manchester Children’s Hospital, Manchester, UK.


Background: Atypical congenital hyperinsulinism in infancy (CHI-A) represent patients who generally present symptoms of hypoglycaemia later in the neonatal period, are poorly responsive to medical intervention and have no known genetic cause of disease. Our objective was to compare the expression profiles of insulin and somatostatin in islets from patients with CHI-A, diffuse CHI (CHI-D) and age-matched control tissue.

Methods and materials: CHI tissues were obtained following pancreatectomy, and age-matched control tissue following autopsy. CHI-D patients were positive for defects in ABCC8; CHI-A was not associated with defects in CHI-associated genes. Insulin- (INS+) and somatostatin-expressing cells (SOM+) were identified by immunohistochemistry and quantified following digitization of paraffin-embedded tissue samples; Ki67 was used as a marker of cell proliferation and NKX2.2 as a transcription factor which maintains β-cell phenotype in islets with limited expression profile in δ-cells following birth.

Results: We examined islets from CHI-A (n=47) and compared to control (n=50) and CHI-D (n=26) islets. In CHI-A, 49.5% of the islets (n=23) had a quiescent profile associated with condensed cytoplasm, nuclear crowding and reduced numbers of centrally-located INS+ cells. In control and CHI-D, >90% of islets were composed of >70% INS+ cells and <20% SOM+ cells (n=61). In contrast, >70% of quiescent CHI-A islets had <30% INS+ cells and >65% had more than 20% SOM+ cells; with 30% of islets composed of >50% δ-cells (n=20). Surprisingly, ‘quiescent islets’ had twofold higher rates of proliferation than unaffected islets from the same tissue, and >60% δ-cells were positive for NKX2.2; a transcription factor that was only present in a limited number of δ-cells in control islets.

Summary/conclusion: Marked increases in NKX2.2 expression in CHI-A δ-cells combined with increased numbers of SOM+ cells and rates of proliferation imply that an immature δ-cell profile contributes to the pathobiology of CHI.

Volume 39

43rd Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

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