title: Quality of life in a large cohort of adult Brazilian patients with 46,XX and 46,XY disorders of sex development from a single tertiary centre.
abstract: OBJECTIVE
  Few studies have focused on the quality of life (QoL) of patients with disorders of sex development (DSD). Our aim was to evaluate QoL in DSD patients with defined diagnoses followed until adulthood in a single tertiary centre.
  
  
  METHODS
  Adult patients with DSD (56 patients with 46,XX DSD - 49 with female social sex and 7 with male social sex as well as 88 patients with 46,XY DSD - 54 with female social sex and 34 with male social sex).
  
  
  METHODS
  QoL using WHOQOL-Bref questionnaire.
  
  
  RESULTS
  Both patients with 46,XX DSD and patients with 46,XY DSD had similar QoL scores on the WHOQOL-Bref, comparable to the scores of the Brazilian general population. The chronological age at the start of treatment was negatively and significantly associated with general QoL score. Patients with male social sex DSD had better scores on the psychological domain than patients with female social sex DSD, as found in the Brazilian general population. In addition, among the 46,XY DSD group, the male social sex patients had better QoL compared with the female social sex patients. There was a positive and significant correlation between sexual performance and general QoL, although it explained only 4% of the variability of the general QoL score. The most influencing variables were general health, positive feelings and spirituality, religion and personal beliefs, each of them contributing with 18% of the variability of the general QoL score.
  
  
  CONCLUSIONS
  Our large cohort of adult patients with DSD, which was followed by a multidisciplinary team in a single tertiary centre, had good QoL in adulthood; in addition, late treatment compromised the QoL of patients with DSD, whereas sexual performance has little influence on QoL.
authors: 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: Rita
authors: R
authors: Cassia Amaral
authors: Marlene
authors: M
authors: Inacio
authors: Vinicius N
authors: VN
authors: Brito
authors: Tania A S S
authors: TA
authors: Bachega
authors: Ari A
authors: AA
authors: Oliveira
authors: Sorahia
authors: S
authors: Domenice
authors: Francisco T
authors: FT
authors: Denes
authors: Maria Helena
authors: MH
authors: Sircili
authors: Ivo J P
authors: IJ
authors: Arnhold
authors: Guiomar
authors: G
authors: Madureira
authors: Larissa
authors: L
authors: Gomes
authors: Elaine M F
authors: EM
authors: Costa
authors: Berenice B
authors: BB
authors: Mendonca
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000453:epidemiology* / Q000523:psychology; D000293:Adolescent; D000328:Adult; D001938:Brazil / Q000453:epidemiology; D058490:Disorder of Sex Development, 46,XY / Q000453:epidemiology* / Q000523:psychology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012917:Social Adjustment; D012944:Social Support; D011795:Surveys and Questionnaires; D062606:Tertiary Care Centers; D055815:Young Adult