Convincing Features
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Maturity-onset diabetes of the young (MODY) was first reported in 1974 as mild familial diabetes with dominant inheritance. Classically, MODY was characterized by autosomal dominant inheritance, onset before 45 years of age, the absence of βcell autoimmunity, absence of insulin resistance, and sustained β-cell function.
However, the new diagnostic criteria set forth in the Practice Guideline for MODY in 20084 include the onset before 25 years of age in one family member, the presence of diabetes in two consecutive generations, the absence of β-cell autoantibodies, and sustained endogenous insulin secretion. The preserved β-cell function is indicated by the lack of need for insulin treatment or a serum C-peptide level of >200 pmol/L even after 3 years of insulin treatment.
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