title: Three decades of sociomedical experiences from West Swedish Rett females 4-60 years of age.
abstract: Rett syndrome, today known as a worldwide important cause behind severe mental retardation in females, has been seen in Sweden since the 1960s (the senior author). This study gives population representative clinical and sociomedical follow-up data from West Sweden (1/4 of the population in Sweden). The series comprises 54 females diagnosed in 1971-1998, 5-60 years old, median age 20. Mortality in 1971-2000 was 18% (10/54), median age at death 20. Death usually had been sudden and unexpected. The sociomedical follow-up emphasized the adult group. The large majority had usually moved to small group homes, a good and well-accepted environment once the staff had learned the particularities of these so uniquely neurodevelopmentally disabled women.
authors.affiliation: Department of Pediatrics, The Queen Silvia Children's Hospital, Göteborg University, SE-416 85, Gothenburg, Sweden.
authors.forename: B
authors.initials: B
authors.lastname: Hagberg
authors.forename: M
authors.initials: M
authors.lastname: Berg
authors.forename: U
authors.initials: U
authors.lastname: Steffenburg
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D016352:Group Homes; D006801:Humans; D008875:Middle Aged; D009313:National Health Programs / Q000458:organization & administration / Q000706:statistics & numerical data*; D011787:Quality of Health Care; D015518:Rett Syndrome / Q000401:mortality / Q000523:psychology / Q000628:therapy*; D012944:Social Support; D013548:Sweden