MODY
Pronunciation: MOH-dee
Also written as: MODY — Maturity-Onset Diabetes of the Young
Maturity-Onset Diabetes of the Young, a monogenic form of diabetes caused by mutations in genes affecting beta-cell function.
Full Definition
Maturity-Onset Diabetes of the Young (MODY) represents a heterogeneous group of monogenic diabetes disorders characterized by autosomal dominant inheritance, early onset (typically before age 25), and primary defects in pancreatic beta-cell function. MODY accounts for 1-2% of all diabetes cases and is caused by mutations in at least 14 different genes, with HNF1A, GCK, and HNF4A being the most common. Unlike type 1 diabetes, MODY patients retain some insulin production and typically do not require insulin initially. Accurate diagnosis is crucial as treatment varies significantly between MODY subtypes, with some responding well to sulfonylureas while others may require insulin from onset.
Usage
Usage note: Always use all capitals for the acronym; capitalize each word in the full form.
In Context
- "Genetic testing confirmed MODY-2 due to a glucokinase mutation, explaining the mild fasting hyperglycemia." — Genetic counseling report
- "The family history of diabetes across three generations suggested possible MODY requiring genetic evaluation." — Pediatric endocrinology consultation