diaendoСахарный диабетDiabetes mellitus2072-03512072-0378Endocrinology research centre10.14341/2072-0351-6246diaendo-6246Research ArticleСтатьиArticlesГенетика моногенных форм сахарного диабетаGenetics of monegenic forms of diabetes mellitusКураеваТамара ЛеонидовнаKuraevaTamara Leonidovnakuraevatl@mail.ruЗильберманЛюбовь ИосифовнаZil'bermanLyubov' Iosifovnakuraevatl@mail.ruТитовичЕлена ВитальевнаTitovichElena Vital'evnakuraevatl@mail.ruПетерковаВалентина АлександровнаPeterkovaValentina Alexandrovnakuraevatl@mail.ruФГУ Эндокринологический научный центр, МоскваРоссияEndocrinological Research Centre, MoscowRussian Federation201115032011141№1 (2011)2027Copyright © Кураева Т.Л., Зильберман Л.И., Титович Е.В., Петеркова В.А., 20112011Кураева Т.Л., Зильберман Л.И., Титович Е.В., Петеркова В.А.Kuraeva T.L., Zil'berman L.I., Titovich E.V., Peterkova V.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.dia-endojournals.ru/jour/article/view/6246

В настоящее время стало очевидным, что сахарный диабет (СД) в детском возрасте не ограничивается аутоиммунным 1 типом. Все чаще у детей и подростков удается идентифицировать диабет 2 типа, MODY тип, а также более редкие синдромальные формы диабета. Истинная распространенность диабета не 1 типа? в детском и подростковом возрасте неизвестна, предполагается, что она может достигать 10%. Хотя генетические синдромы встречаются редко, все вместе они составляют приблизительно 5% среди всех детей с СД. Активное развитие молекулярной биологии в наш век открывает широкие возможности для определения различных симптомокомплексов в нозологически самостоятельные формы. Каждый год появляются описания новых генетических синдромов, ассоциированных с СД. Важность этих синдромов для пациентов заключается в грамотной идентификации и лечении составляющих проявлений синдрома и осложне- ний, а для членов семей - в возможности получить медико-генетическую консультацию и соответствующие рекомендации

It is universally recognized that autoimmune type 1 diabetes mellitus (DM) is not the only form of this disease in children. Increasingly more children andadolescents present with DM2, MODY, and rarer syndromal forms of DM. The actual prevalence of DM other than DM1 in children and adolescentsis unknown but may be estimated at 10%. Despite rare occurrence of genetic syndromes, they collectively account for almost 5% of DM cases amongchildren. The rapid upgrowth of molecular biology opens up a wide range of possibilities for designating various symptom complexes as nosologically selfconsistentforms. New genetic syndromes associated with DM are annually described. It is important both to adequately identify and treat manifestationsand 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

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The authors declare that there are no conflicts of interest present.