Chapter

Monogenic forms of diabetes resulting from β cell dysfunction

Rachel Besser and Andrew Hattersley

in Oxford Textbook of Endocrinology and Diabetes

Second edition

Published on behalf of Oxford University Press

ISBN: 9780199235292
Published online July 2011 | e-ISBN: 9780199608232 | DOI: http://dx.doi.org/10.1093/med/9780199235292.003.1348

Series: Oxford Textbooks

Monogenic forms of diabetes resulting from β cell dysfunction

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Monogenic diabetes refers to diabetes resulting from mutations in a single gene. This chapter discusses monogenic disorders causing β cell dysfunction, which accounts for the majority of cases of monogenic diabetes.

Patients can usually be divided into three clinical categories: maturity-onset diabetes of the young (MODY), which is dominantly inherited familial diabetes; neonatal diabetes, diagnosed under the age of 6 months; and monogenic diabetes syndromes, which are characterized by multiple nonpancreatic features. In each clinical category, there are several aetiological genes that usually result in a discrete clinical phenotype.

Chapter.  4266 words.  Illustrated.

Subjects: Endocrinology and Diabetes

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