MODY

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Maturity onset diabetes of the young (MODY) is a heterogeneous group of monogenic disorders distinct from type 1 (insulin-dependent) and type 2 (noninsulin-dependent) diabetes (1,2).

there is β cell dysfunction characterised by non-ketotic diabetes and absence of pancreatic auto-antibodies (3)

The condition was first described by Tattersall in 1974–1975, a familial form of non-insulin dependent diabetes which showed autosomal dominant inheritance which presented before the age of 25 years (1,2). The molecular genetic basis of MODY was subsequently recognised in the 1990s and mutations in 10 different genes have been identified so far (2).

Prevalence of MODY is unknown but the minimum UK population prevalence of all MODY subtypes is estimated to be 68-108 cases per million.

the prevalence of MODY subtypes differ from country to country

in countries such as France, Spain, and Italy where glucose testing of asymptomatic people is done routinely, glucokinase (GCK) mutations are commonly diagnosed while in countries where random blood glucose tests are seldom done hepatocyte nuclear factor 1α (HNF1A) is commonly diagnosed.

in the UK, most common causes of mutations of MODY are

glucokinase (GCK) - 32%
hepatocyte nuclear factor 1α (HNF1A) - 52%
hepatocyte nuclear factor 4α (HNF4A) - 10% (3)

Reference:

Last edited 09/2020 and last reviewed 02/2021

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