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Horm Metab Res 2014; 46(03): 157-170
DOI: 10.1055/s-0034-1367063
Review
© Georg Thieme Verlag KG Stuttgart · New York

Hyperinsulinaemic Hypoglycaemia

V. B. Arya
1   London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust and The Institute of Child Health, University College London, London, UK
,
Z. Mohammed
1   London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust and The Institute of Child Health, University College London, London, UK
,
O. Blankenstein
2   Department of Endocrinology, Charité-University Medicine, Berlin, Germany
,
P. De Lonlay
3   Reference Center for Inherited Metabolic Diseases, Université Paris-Descartes, Institute Imagine, Paris, France
,
K. Hussain
1   London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust and The Institute of Child Health, University College London, London, UK
› Author Affiliations
Further Information

Publication History

received 02 September 2013

accepted 09 January 2014

Publication Date:
20 February 2014 (online)

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Abstract

Insulin secretion from pancreatic β-cells is tightly regulated to maintain fasting blood glucose level between 3.5–5.5 mmol/l. In hyperinsulinaemic hypoglycaemia (HH) insulin secretion becomes unregulated so that insulin secretion persists despite low blood glucose levels. HH can be due to a large number of causes and recent advances in genetics have begun to provide novel insights into the molecular mechanisms of HH. Defects in key genes involved in regulating insulin secretion have been linked to HH. The most severe forms of HH are clinically observed in the newborn period whereas in adults an insulinoma is the commonest cause of HH. This review provides an overview on the molecular mechanisms leading to HH in children and adults, it describes the clinical presentation and diagnosis, and finally the treatment options for the different forms of HH are discussed.

Key words

congenital hyperinsulinism - hyperinsulinaemic hypoglycaemia - insulinoma
 

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