Hagberg B
Acta Paediatr Scand. 1985 May;74(3):405-8. doi: 10.1111/j.1651-2227.1985.tb10993.x.
The prevalence of Rett's syndrome was studied in a part of southwestern Sweden comprising five counties and the city of Gothenburg. In a population of 315469 children and adolescents, 6-17 years 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 aetiologies 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 aetiological factor to think of in females. This syndrome might well be responsible for one-fourth to one-third of such cases among girls.
在瑞典西南部包括五个郡和哥德堡市的部分地区，对雷特综合征的患病率进行了研究。在6至17岁的315469名儿童和青少年群体中，检测出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，即约为该地区苯丙酮尿症（PKU）患病率的两倍。由于进行性脑部疾病/代谢疾病在瑞典中部该年龄组严重智力迟钝者的病因中占5 - 6%（每10000名儿童中有1.5 - 2.0例），可以得出结论，在该群体中，雷特综合征应被视为女性中需要考虑的一个病因因素。该综合征很可能导致女孩中此类病例的四分之一至三分之一。
