Maturity onset diabetes of the young, MODY
Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 25 November 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.
● 1-2% of all cases of diabetes (Diabetes Metab Syndr Obes 2012;5:101)
● Also called monogenic diabetes (Wikipedia)
● Type 2 diabetes-like condition which occurs in 2+ generations, with autosomal-dominant inheritance (Diabetes Care 2011;34:1878)
● Autosomal dominant, but not a single entity - mutations in 9 genes identified to date (see diagram below)
● Common genes affected are Hepatic Nuclear Factor 1 or 4 alpha, glucokinase
● Onset before age 25, normal weight, mild hypoglycemia
● No GAD antibodies, no insulin resistance, no beta cell loss but impaired beta cell function
Single gene mutations resulting in monogenic diabetes / MODY
End of Pancreas > Diabetes mellitus > Maturity onset diabetes of the young, MODY
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