Clinical treatment for MODY diabetes
DOI:
https://doi.org/10.47196/diab.v48i3.190Keywords:
monogenic, mody, glucokinase, HNF-1Abstract
The term MODY (Maturity Onset Diabetes in Youngs) comes from the old DM classification that subdivided it into those that started in youth from those that started in adulthood. At present, they are framed within those tables characterized by "genetic defects in beta cell function". It is a monogenic form of the disease whose common denominator is the hyposecretion of insulin as the primary trigger factor. Currently 13 MODY subtypes have been identified. Although MODY represents approximately 1-2% of patients with DM, it is estimated that a large percentage of cases are undiagnosed. Regarding the relative frequency, MODY 2 and MODY 3 represent around 60-80% of cases, and MODY 1 10% of them. In general, patients with MODY are characterized by having: 1) DM of onset in young age, generally under 25 years; 2) strong family influence; 3) without stigmas of insulin resistance; 4) insulin-independence; 5) absence of autoantibodies related to autoimmune DM. The diagnosis of MODY brings with it prognostic, therapeutic, and genetic counseling implications. Those patients with a glucokinase mutation (MODY 2) usually do not develop chronic micro and macrovascular complications and generally do not require pharmacological treatment, while those with HNF-1α mutations (MODY 3) have a tendency to microvascular complications and possess the characteristic of present hyperresponsiveness to low doses of sulfonylureas, sometimes even have severe hypoglycemia.
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