Maturity Onset Diabetes of the Young

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Timsit J, Bellanné-Chantelot C, Dubois-Laforgue D, Velho G
Diagnosis and management of maturity-onset diabetes of the young. [Journal Article, Review]
Treat Endocrinol 2005; 4(1):9-18.

Maturity-onset diabetes of the young (MODY) is a dominantly inherited form of non-ketotic diabetes mellitus. It results from a primary defect of insulin secretion, and usually develops at childhood, adolescence, or young adulthood. There have been a number of cases where individuals were diagnosed as have diabetes as old as in their 30s. 1-5% of individuals with diabetes have this form of diabetes. MODY is different in what diabetes is thought of in metabolic and clinical features. All MODY genes have not been identified, but mutations in six genes cause the majority of the MODY cases. MODY2 and MODY3 are the most frequent. Patients with MODY2 have mild, asymptomatic, and stable hyperglycemia that is present from birth. They rarely develop microvascular disease, and seldom require pharmacologic treatment of hyperglycemia. In patients with MODY3, severe hyperglycemia usually occurs after puberty, and may lead to the diagnosis of type 1 diabetes. Sensitivity to sulfonylureas may be retained in MODY3 patients. Diabetic retinopathy and nephropathy frequently occur in patients with MODY3, making frequent follow-up mandatory. By contrast, other risk factors are not present in patients with MODY and the frequency of cardiovascular disease is not increased. This clinical spectrum of MODY is wider than initially described. A family history of diabetes is not always present. Molecular diagnosis is important in terms of prognosis, family screening, and therapy.