title: Changes over time in sex assignment for disorders of sex development.
abstract: OBJECTIVE
  It is unclear whether the proportion of infants with a disorder of sex development who are raised as male or female has changed over time. The temporal trends in sex assignment of affected cases entered in the International Disorder of Sex Development (I-DSD) Registry were studied.
  
  
  METHODS
  Cases of disorders of sex development reported as partial androgen insensitivity syndrome (PAIS; n = 118), disorder of gonadal development (DGD; n = 232), and disorder of androgen synthesis (DAS; n = 104) were divided into those who were born before 1990, 1990-1999, and after 1999. External appearance of the genitalia was described by the external masculinization score.
  
  
  RESULTS
  The median (5th-95th percentile) external masculinization scores of those infants with PAIS, DGD, and DAS who were raised as boys were 6 (2-9), 6 (3-9), and 6 (1-12), respectively, and were significantly higher than in those raised as girls (2 [0-6], 2 [0-7], and 0 [0-5], respectively); this difference was maintained in the 3 temporal birth cohorts (P < .01). Of the 118 cases in the pre-1990 cohort, 41 (35%) were raised as boys; of the 148 cases in the 1990-1999 cohort, 60 (41%) were raised as boys; and of the 188 cases in the post-1999 cohort, 128 (68%) were raised as boys.
  
  
  CONCLUSIONS
  Although there is an association between the external appearance of the genitalia and the choice of sex assignment, there are clear temporal trends in this practice pointing toward an increased likelihood of affected infants being raised as boys. The impact of this change in practice on long-term health outcomes requires additional focus.
authors.affiliation: Poznan University of Medical Sciences, Poznan, Poland;
authors.forename: Zofia
authors.initials: Z
authors.lastname: Kolesinska
authors.affiliation: University of Glasgow, Glasgow, United Kingdom; faisal.ahmed@glasgow.ac.uk.
authors.forename: S Faisal
authors.initials: SF
authors.lastname: Ahmed
authors.affiliation: Poznan University of Medical Sciences, Poznan, Poland;
authors.forename: Marek
authors.initials: M
authors.lastname: Niedziela
authors.affiliation: University of Glasgow, Glasgow, United Kingdom;
authors.forename: Jillian
authors.initials: J
authors.lastname: Bryce
authors.affiliation: Poznan University of Medical Sciences, Poznan, Poland;
authors.forename: Marta
authors.initials: M
authors.lastname: Molinska-Glura
authors.affiliation: University of Glasgow, Glasgow, United Kingdom;
authors.forename: Martina
authors.initials: M
authors.lastname: Rodie
authors.affiliation: University of Glasgow, Glasgow, United Kingdom;
authors.forename: Jipu
authors.initials: J
authors.lastname: Jiang
authors.affiliation: University of Melbourne, Melbourne, Australia;
authors.forename: Richard O
authors.initials: RO
authors.lastname: Sinnott
authors.affiliation: University of Cambridge, Cambridge, United Kingdom;
authors.forename: Ieuan A
authors.initials: IA
authors.lastname: Hughes
authors.affiliation: Istanbul University, Istanbul, Turkey;
authors.forename: Feyza
authors.initials: F
authors.lastname: Darendeliler
authors.affiliation: University of Lübeck, Lübeck, Germany;
authors.forename: Olaf
authors.initials: O
authors.lastname: Hiort
authors.affiliation: Sophia Children's Hospital/Erasmus MC, Rotterdam, Netherlands;
authors.forename: Yvonne
authors.initials: Y
authors.lastname: van der Zwan
authors.affiliation: University Hospital Ghent, Ghent University, Ghent, Belgium;
authors.forename: Martine
authors.initials: M
authors.lastname: Cools
authors.affiliation: Marmara University, Istanbul, Turkey;
authors.forename: Tulay
authors.initials: T
authors.lastname: Guran
authors.affiliation: University Hospital Schleswig-Holstein, Kiel, Germany;
authors.forename: Paul-Martin
authors.initials: PM
authors.lastname: Holterhus
authors.affiliation: University Hospital Pisa, Pisa, Italy;
authors.forename: Silvano
authors.initials: S
authors.lastname: Bertelloni
authors.affiliation: Institute of Endocrinology, Prague, Czech Republic;
authors.forename: Lidka
authors.initials: L
authors.lastname: Lisa
authors.affiliation: Centre for Endocrinology, Diabetes, and Metabolism, University of Birmingham, Birmingham, United Kingdom;
authors.forename: Wiebke
authors.initials: W
authors.lastname: Arlt
authors.affiliation: Centre for Endocrinology, Diabetes, and Metabolism, University of Birmingham, Birmingham, United Kingdom;
authors.forename: Nils
authors.initials: N
authors.lastname: Krone
authors.affiliation: Ahfad University for Women, Omdurman, Sudan; Al-Neelain Medical Research Centre, Khartoum, Sudan;
authors.forename: Mona
authors.initials: M
authors.lastname: Ellaithi
authors.affiliation: University Hospital Bologna, Bologna, Italy;
authors.forename: Antonio
authors.initials: A
authors.lastname: Balsamo
authors.affiliation: National Research Centre, Cairo, Egypt;
authors.forename: Inas
authors.initials: I
authors.lastname: Mazen
authors.affiliation: Karolinska Institutet, Stockholm, Sweden;
authors.forename: Anna
authors.initials: A
authors.lastname: Nordenstrom
authors.affiliation: University of Southampton, Southampton, United Kingdom;
authors.forename: Katherine
authors.initials: K
authors.lastname: Lachlan
authors.affiliation: Amiri Hospital, Kuwait, Kuwait;
authors.forename: Mona
authors.initials: M
authors.lastname: Alkhawari
authors.affiliation: University Claude Bernard Lyon 1, Lyon, France;
authors.forename: Pierre
authors.initials: P
authors.lastname: Chatelain
authors.affiliation: Pediatric Endocrinology and Diabetes Unit, Dana Children's Hospital, Tel Aviv, Israel; Sourasky Medical Center, Tel Aviv, Israel; and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Naomi
authors.initials: N
authors.lastname: Weintrob
mesh_terms: D000293:Adolescent; D000328:Adult; D015331:Cohort Studies; D012734:Disorders of Sex Development / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D005783:Gender Identity; D006801:Humans; D008297:Male; D012042:Registries; D013997:Time Factors; D055815:Young Adult
keywords: DSD; atypical; differences; gender; genitalia; intersex