title: Quality of Life and Psychological Adjustment of Women Living with 46,XY Differences of Sex Development.
abstract: BACKGROUND
  Progressive care improvement for differences of sex development (DSD), regarding diagnosis communication, psychological, medical and surgical management has been claimed.
  
  
  OBJECTIVE
  To assess clinical management, quality of life (QoL) and the general psychosocial adjustment of individuals with 46,XY DSD. Some differences related to age at diagnosis are investigated.
  
  
  METHODS
  Cross-sectional study using standardized questionnaires.
  
  
  METHODS
  Forty-three Caucasian females with 46,XY DSD (self declared diagnoses: complete androgen insensitivity syndrome, n = 34; complete gonadal dysgenesis, n = 1; 5α-reductase deficiency, n = 4; Leydig cell hypoplasia, n = 1; unknown diagnosis, n = 3; age years: 31.5 ± 9.6 [range 18-57 years]).
  
  
  METHODS
  University Hospitals.
  
  
  METHODS
  Subjects were required to fill in questionnaires (ABCL, WHOQOL, dedicated 17-item questionnaire). Academic and socioeconomic data were compared with those of the Italian population. QoL and psychological data were compared with those of a comparison group (46,XX healthy females: n = 43; age, years: 34.5 ± 9.7, range 22-51 years).
  
  
  RESULTS
  Present sample of women living with 46,XY DSD were well adapted and were higher achievers than controls, both in educational and professional life. They showed good QoL, but they appeared less satisfied in psychological and social areas. They had borderline mean scores and statistically higher scores than the comparison group for depression, anxiety, internalizing and externalizing problems. Younger persons living with a 46,XY DSD showed better psychosocial adjustment than older ones. Younger women showed lower age at diagnosis communication. Psychological support was more often proposed at the time of diagnosis communication to younger individuals, and they undertook it more frequently than older ones.
  
  
  CONCLUSIONS
  Italian people living with 46,XY DSD were well adapted and successful; they reported a good QoL but showed higher degree of psychological distress than the comparison group. Lower psychological distress in younger women could indicate some positive effects of changes in management.
authors.affiliation: Pediatric Endocrinology Unit, Department of Pediatrics, S. Orsola-Malpighi University Hospital of Bologna, Bologna, Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: D'Alberton
authors.affiliation: Pediatric Endocrinology Unit, Department of Pediatrics, S. Orsola-Malpighi University Hospital of Bologna, Bologna, Italy.
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Assante
authors.affiliation: Pediatric Endocrinology Unit, Department of Pediatrics, S. Orsola-Malpighi University Hospital of Bologna, Bologna, Italy.
authors.forename: Maura
authors.initials: M
authors.lastname: Foresti
authors.affiliation: Pediatric Endocrinology Unit, Department of Pediatrics, S. Orsola-Malpighi University Hospital of Bologna, Bologna, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Balsamo
authors.affiliation: Adolescent Medicine, Division of Pediatrics, Department of Obstetrics, Gynecology and Pediatrics, University Hospital, Pisa, Italy.
authors.forename: Silvano
authors.initials: S
authors.lastname: Bertelloni
authors.affiliation: Department of Pediatrics, San Giuseppe Hospital, Empoli, Italy.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Dati
authors.affiliation: Pediatric Endocrinology Unit, Department of Pediatrics, S. Orsola-Malpighi University Hospital of Bologna, Bologna, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Nardi
authors.affiliation: Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.
authors.forename: Maria Letizia
authors.initials: ML
authors.lastname: Bacchi
authors.affiliation: Pediatric Endocrinology Unit, Department of Pediatrics, S. Orsola-Malpighi University Hospital of Bologna, Bologna, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Mazzanti
mesh_terms: D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D058490:Disorder of Sex Development, 46,XY / Q000523:psychology* / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D046468:Sexual Development; D012735:Sexual Dysfunction, Physiological / Q000209:etiology / Q000523:psychology*; D020018:Sexual Dysfunctions, Psychological / Q000209:etiology / Q000523:psychology*; D011795:Surveys and Questionnaires
keywords: DSD; Diagnosis Communication; Differences of Sex Development; Hormone Replacement Therapy; Psychological Adjustment; Psychology; Quality of Life