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BOOK REVIEW
Year : 2012  |  Volume : 136  |  Issue : 4  |  Page : 685

Monogenic hyperinsulinemic hypoglycemia disorders


Department of Endocrinology, Postgraduate Institute of Medical Education & Research, Chandigarh 160 012, India

Date of Web Publication8-Nov-2012

Correspondence Address:
Anil Bhansali
Department of Endocrinology, Postgraduate Institute of Medical Education & Research, Chandigarh 160 012
India
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Source of Support: None, Conflict of Interest: None


PMID: 23168715

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How to cite this article:
Bhansali A. Monogenic hyperinsulinemic hypoglycemia disorders. Indian J Med Res 2012;136:685

How to cite this URL:
Bhansali A. Monogenic hyperinsulinemic hypoglycemia disorders. Indian J Med Res [serial online] 2012 [cited 2019 Aug 21];136:685. Available from: http://www.ijmr.org.in/text.asp?2012/136/4/685/103290

Monogenic hyperinsulinemic hypoglycemia disorders, C.A. Stanley, D.D. De Leon, Volume Editors (Frontiers in Diabetes, Vol. 21, M Porta, F. M. Matschinsky, (Series Editors) (Karger, Basel) 2012. 196 pages.

ISBN 978-3-8055-9943-6

In 20 th century, endocrinology was confined to discovery of insulin, invention of radio-immunoassay and something about hormones and endocrine disorders. However, the last three decades have witnessed enormous growth in molecular endocrinology, diagnostic modalities and invention of newer drugs in the management of various endocrine disorders. This book on Monogenic Hyperinsulemenic Hypoglycemia Disorders is a piece of excellent work by experts in this field, provides newer insights in congenital hyperinsulinism. Recognition of eight different genetic defects prominent among them being SUR1, Kir 6.2, SCHAD mutations involved in inappropriate secretion of insulin has provided in-depth knowledge in understanding the least understood subject of congenital hyperinsulinism. Elaborate description of glutamate dehydrogenase (GDH) and their activating mutation and short-chain-3 hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) makes worthy to learn these rare causes of congenital hyperinsulinism. Understanding of the molecular mechanism of focal hyperinsulinism (FoHI) by 'two hits' theory representing the imbalance between genes of tumour suppression and β-cell proliferation explains FoHI. Newer diagnostic modalities for pre-operative localization of focal hyperinsulinism like DOPA-PET scan and treatment modalities targeting different channelopathies are important advances in this field. New vistas in the management also includes GLP-1 receptor antagonist exendin which inhibits the insulin secretion and corrects fasting hypoglycaemia in SUR1 -/- mice suggesting their potential role in K ATP channel mediated hyperinsulinism. Use of green tea as it is enriched in flavonoids, has also been shown to be effective in glutamate dehydrogenase mediated hypoglycaemia.

Overall, this book will be very useful for paediatricians, paediatric endocrinologists and all clinical endocrinologists, who are actively involved in the management of endocrine emergencies. This will also inspire the young researchers to unravel the 'endocrine pancreas'.




 

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