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Monogenic diabetes syndromes
All children diagnosed with diabetes in the first 6 months of life should have genetic testing Maturity-onset diabetes of the young should be considered in individuals who have mild stable fasting hyperglycemia and multiple family members with diabetes not characteristic of type 1 or type 2 diabetes. Because a diagnosis of maturity onset diabetes of the young may impact therapy and lead to identification of other affected family members, consider referring individuals with diabetes not typical of  type 1 or type 2 diabetes and occurring in successive generations (suggestive of an autosomal dominant pattern of inheritance) to a specialist for further evaluation

Maturity-Onset Diabetes of the Young Moody’s characterized by impaired insulin secretion with minimal or no defects in /insulin action. It is inherited in an autosomal dominant pattern. Abnormalities at six genetic loci on different chromosomes have been identified to date. The most common form (MODY 3) is associated with mutations on chromosome 12 in a hepatic transcription factor referred to as hepatocyte nuclear factor (HNF)-1a and also referred to as transcription factor-1 (TCF-1). The second most common form (MODY 2) is associated with mutations in the glucosidase gene on chromosome 7p and results in a defective glucosidase molecule. Glucosidase converts glucose to glucose-6-phosphate, the metabolism of which, in turn, stimulates insulin secretion by the b-cell. The less common forms of MODY result from mutations in other.
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