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Monogenic diabetes results from one or more defects in a single gene. The disease may be inherited within families as a dominant, recessive, or non-Mendelian trait or may present as a spontaneous case due to a de novo mutation. Well over 40 different genetic subtypes of monogenic diabetes have been identified to date, each having a typical phenotype and a specific pattern of inheritance.

A familial form of mild diabetes presenting during adolescence or in early adulthood was first described many years ago (1, 2). Even though diabetes presented in young patients, the disease clinically resembled elderly onset non-insulin dependent diabetes and the newly recognized subtype of familial diabetes became known by the acronym MODY (3). As MODY patients passed on the disease to their offspring following an autosomal dominant pattern of inheritance, it was quickly suspected that it might be a monogenic disorder (4). MODY is by far the commonest type of monogenic diabetes. All currently known subtypes of MODY are