Генетика моногенных форм сахарного диабета

It is universally recognized that autoimmune type 1 diabetes mellitus (DM) is not the only form of this disease in children. Increasingly more children and adolescents present with DM2, MODY, and rarer syndromal forms of DM. The actual prevalence of DM other than DM1 in children and adolescents is unknown but may be estimated at 10%. Despite rare occurrence of genetic syndromes, they collectively account for almost 5% of DM cases among children. The rapid upgrowth of molecular biology opens up a wide range of possibilities for designating various symptom complexes as nosologically selfconsistent forms. New genetic syndromes associated with DM are annually described. It is important both to adequately identify and treat manifestations and complications of these syndromes in children and to provide relevant medico-genetic counseling and recommendations to the parents. Key words: non-immune diabetes mellitus, MODY, Wolfram syndrome, neonatal, syndromal forms