Maturity onset diabetes of the young
Maturity onset diabetes of the young (MODY) is a rare hereditary form of diabetes mellitus. Six types have been described.
Signs, symptoms and diagnosis
As it occurs very infrequently, most cases are initially ascribed to diabetes mellitus type 1, as it occurs in young people (teenagers) who do not exhibit the phenotype of type 2 diabetes (obesity, hypertension and related conditions). As oral medication (e.g. sulfonylureas, metformin or thiazolidinediones) are ineffective in type 1 diabetes, MODY patients generally end up on insulin therapy.
Pathophysiology
The six recognised forms of MODY are all due to ineffective insulin production in pancreatic β-cells. They are inherited in an autosomal dominant fashion, and most patients therefore have other members of the family with diabetes; penetrance differs between the types (from 40% to 90%).
