Abstract
MODY (Maturity-onset Diabetes of the Young) represents a specific form of non-insulin-dependent diabetes mellitus (NIDDM) which comprises about 5 % of all patients with NIDDM. The disease is characterised by an early onset before the age of 25 years, by autosomal dominant inheritance and by variable clinical phenotype.
Five subtypes of MODY (MODY 1-5) have been identified until now. Their classification is based on gene mutations in five known MODY genes (genes for hepatal nuclear factor - 1a [HNF - 1a], hepatal nuclear factor - 4a [ HNF- 4a], hepatal nuclear factor - 1b [HNF-1b], glucokinase [GCK ] and for insulin promotor factor - 1[IPF-1]).
The identification of a gene mutation not only determines the MODY subtype but also predicts the prognosis of the patient and contributes to therapeutical decisions.