MATURITY-ONSET DIABETES OF THE YOUNG (MODY) - 3 CASE-REPORTS AND NEW PERSPECTIVES

Maturity-onset diabetes of the young (MODY) is a rare form of juvenile diabetes mellitus that presents with hyperglycaemia in the absence of ketosis. We present three cases of all of whom had pedigrees with in multiple generations. All our patients presented in adolescence with e vidence of insulin resistance. Two patients were relatively overweight . All three patients were readily controlled on diet alone. None had a ny clinical evidence of diabetic complications in early adulthood. Rec ently there has been a marked increase in our understanding of MODY. G enetic linkage and mutational analyses have identified three subtypes (MODY1, 2 and 3) that are all transmitted in an autosomal dominant fas hion. The pathophysiology of the MODY subtypes is variable with both i ncreased and decreased insulin levels being seen. A failure to recogni se MODY will result in a lack of appropriate therapy and the potential for diabetic complications.