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MODY

ORPHA:552· ICD-10 E34.8

Definition

MODY (maturity-onset diabetes of the young) is a rare, familial, clinically and genetically heterogeneous form of diabetes characterized by young age of onset (generally 10-45 years of age) with maintenance of endogenous insulin production, lack of pancreatic beta-cell autoimmunity, absence of obesity and insulin resistance and extra-pancreatic manifestations in some subtypes.

Prevalence
1-5 / 10 000
Inheritance
Autosomal dominant, Not applicable
Age of onset
Adolescent, Adult, Childhood