title: [MODY diabetes--a monogenic developmental and functional disturbance of pancreatic beta cells].
abstract: MODY (Maturity-Onset Diabetes of the Young) is a group of monogenic disorders characterized by autosomally dominantly inherited diabetes or hyperglycemia typically detected during adolescence or young adulthood. Usually there is at least one person in the family having developed the disease at the age of under 25 years, and the patients are diagnosed with insufficient insulin response to glucose, occasionally also with developmental disturbances of various organs. The most common form is GCK-MODY caused by glucokinase mutations, primarily manifested as elevated fasting glucose levels, usually without the risk of complicating diseases.
authors.affiliation: HYKS, lastenklinikka.
authors.forename: Päivi J
authors.initials: PJ
authors.lastname: Miettinen
authors.forename: Tiinamaija
authors.initials: T
authors.lastname: Tuomi
mesh_terms: D000293:Adolescent; D003924:Diabetes Mellitus, Type 2 / Q000235:genetics* / Q000503:physiopathology*; D005941:Glucokinase / Q000235:genetics*; D006801:Humans; D050417:Insulin-Secreting Cells; D009154:Mutation; D055815:Young Adult
chemical_list: D005941:Glucokinase