Akin Leyla, Adal Erdal, Akin Mustafa Ali, Kurtoglu Selim
Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkey.
J Pediatr Endocrinol Metab. 2012;25(1-2):197-8. doi: 10.1515/jpem.2011.337.
Rett syndrome (RS) is a neurodevelopmental disorder mainly affecting girls. It is characterized by a normal prenatal and perinatal period, apparently normal development for the first 6 months of life, and then a decelaration in head growth, loss of hand and communication skills, psychomotor retardation, as well as the development of sterotyped hand movement and truncal or gait apraxia. It has been shown to be related to mutations in the MECP2 gene located on Xq28. Diabetes mellitus (DM) type 1 may be associated with certain genetic disorders such as Down syndrome, Turner syndrome, and Klinefelter syndrome. In this work, we report the case of a 9-year-old girl with RS who developed DM at the age of 6. To our knowledge, our patient is the third case reported to date of DM associated with Rett syndrome.
雷特综合征(RS)是一种主要影响女孩的神经发育障碍。其特征为产前和围产期正常,出生后头6个月发育明显正常,随后头围生长减速、手部和沟通技能丧失、精神运动发育迟缓,以及出现刻板手部动作和躯干或步态失用症。已证实它与位于Xq28的MECP2基因突变有关。1型糖尿病(DM)可能与某些遗传疾病相关,如唐氏综合征、特纳综合征和克兰费尔特综合征。在本研究中,我们报告了一名9岁患有雷特综合征的女孩,她在6岁时患了糖尿病。据我们所知,我们的患者是迄今为止报道的第三例与雷特综合征相关的糖尿病病例。