title: Quality of life of patients with 46,XX and 46,XY disorders of sex development.
abstract: Disorders of sex development (DSD) result from abnormalities in the complex process of sex determination and differentiation. An important consideration to guide the assignment of social sex in newborns with ambiguous genitalia is the quality of life (QoL) of these patients in adulthood. The rarity of most DSD conditions makes it difficult to conduct a long-term follow-up of affected patients through adulthood. This review of papers on the QoL of DSD patients evaluated in developing and developed countries by qualitative and quantitative instruments revealed a large spectrum of QoL, ranging from very poor to similar to, or even better than, the normal population. A more adequate QoL was found in patients from tertiary centres, indicating that the medical care of DSD patients should be multidisciplinary and carried out by specialized teams.
authors.affiliation: Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular LIM/42, Disciplina de Endocrinologia, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Rita Cassia
authors.initials: RC
authors.lastname: Amaral
authors.forename: Marlene
authors.initials: M
authors.lastname: Inacio
authors.forename: Vinicius N
authors.initials: VN
authors.lastname: Brito
authors.forename: Tania A S S
authors.initials: TA
authors.lastname: Bachega
authors.forename: Sorahia
authors.initials: S
authors.lastname: Domenice
authors.forename: Ivo J P
authors.initials: IJ
authors.lastname: Arnhold
authors.forename: Guiomar
authors.initials: G
authors.lastname: Madureira
authors.forename: Larissa
authors.initials: L
authors.lastname: Gomes
authors.forename: Elaine M F
authors.initials: EM
authors.lastname: Costa
authors.forename: Berenice B
authors.initials: BB
authors.lastname: Mendonca
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000453:epidemiology / Q000503:physiopathology / Q000523:psychology; D000312:Adrenal Hyperplasia, Congenital / Q000453:epidemiology / Q000503:physiopathology / Q000523:psychology; D000328:Adult; D058490:Disorder of Sex Development, 46,XY / Q000453:epidemiology / Q000503:physiopathology / Q000523:psychology; D012734:Disorders of Sex Development / Q000453:epidemiology / Q000503:physiopathology / Q000523:psychology; D005260:Female; D006801:Humans; D008297:Male; D011788:Quality of Life