RETTS SYNDROME - PREVALENCE AND IMPACT ON PROGRESSIVE SEVERE MENTAL-RETARDATION IN GIRLS
RETTS SYNDROME - PREVALENCE AND IMPACT ON PROGRESSIVE SEVERE MENTAL-RETARDATION IN GIRLS
复制标题
DOI：
10.1111/j.1651-2227.1985.tb10993.x
复制
发表时间：
1985-01-01
期刊：
影响因子：
--
通讯作者：
HAGBERG, B
中科院分区：
文献类型：
--
作者：
HAGBERG, B
在瑞典西南部由5个县和哥德堡市组成的一个地区对雷特综合征的患病率进行了研究。在315469名6 - 17岁的儿童和青少年人群中，发现了10例病例，均为女孩。相应的患病率为每10000名女孩中有0.65例，即大约是同一地区苯丙酮尿症（PKU）患病率的两倍。在瑞典中部这个年龄组的重度智力障碍患者中，进行性脑部疾病和代谢性疾病共同构成病因的5 - 6%（每10000名儿童中有1.5 - 2.0例），由此可以得出结论，在这个群体中，雷特综合征应被视为女性的一个病因因素。在女孩中，这种综合征很可能是此类病因的1/4 - 1/3。
The prevalence of Rett''s syndrome was studied in a part of southwestern Sweden comprising 5 counties and the city of Gothenburg. In a population of 315,469 children and adolescents, 6-17 yr of age, 10 cases were detected, all girls. The corresponding prevalence was 0.65/10,000 girls, i.e., about twice that of phenylketonuria (PKU) in the same area. As progressive brain disorders metabolic diseases together constitute 5-6% (1.5-2.0/10,000 children) of the etiologie among severely mentally retarded persons of this age group in central Sweden, it can be concluded that within this group Rett''s syndrome should be considered as an etiological factor in females. This syndrome might well be responsible for 1/4-1/3 of such among girls.