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Monogenic forms of diabetes resulting from β cell dysfunction 

Monogenic forms of diabetes resulting from β cell dysfunction
Monogenic forms of diabetes resulting from β cell dysfunction

Rachel Besser

and Andrew Hattersley

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date: 18 May 2022

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.

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