This is rare
form of type 2 diabetes inherited in an autosomal dominant fashion and
presenting at young age. It comprises <5% of childhood diabetes and <1%
of all type of diabetes. It is also known as Mason-type diabetes after the index
family. The diagnostic criteria include diagnosis of type 2 diabetes with onset
before age of 25 years in at least two family members, with evidence of
vertical transmission throughout at least three generations.
All 6 types
of MODY are characterized by defects in insulin secretion rather than insulin
action, but sometimes insulin resistance is seen due to glucotoxicity. The
defect consists of mutation in gene in β cells, encoding glucokinase (MODY 2)
and other transcription factors leading to (MODY 1-6) which leads to defective
insulin action, pancreatic dysfunction, etc.
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