Changes over time in sex assignment for disorders of sex development

Kolesinska, Z. et al. (2014) Changes over time in sex assignment for disorders of sex development. Pediatrics, 134(3), e710-e715. (doi: 10.1542/peds.2014-1088)

Full text not currently available from Enlighten.


BACKGROUND AND 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.<p></p> 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.<p></p> 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.<p></p> 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.

Item Type:Articles
Glasgow Author(s) Enlighten ID:Rodie, Dr Martina and Bryce, Dr Jillian and Jiang, Mr Jipu and Sinnott, Richard O. and Ahmed, Professor Syed Faisal
Authors: Kolesinska, Z., Ahmed, S.F., Niedziela, M., Bryce, J., Molinska-Glura, M., Rodie, M., Jiang, J., Sinnott, R.O., Hughes, I.A., Darendeliler, F., Hiort, O., van der Zwan, Y., Cools, M., Guran, T., Holterhus, P.-M., Bertelloni, S., Lisa, L., Arlt, W., Krone, N., Ellaithi, M., Balsamo, A., Mazen, I., Nordenstrom, A., Lachlan, K., Alkhawari, M., Chatelain, P., and Weintrob, N.
College/School:College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
Journal Name:Pediatrics
Publisher:American Academy of Pediatrics
ISSN (Online):1098-4275

University Staff: Request a correction | Enlighten Editors: Update this record

Project CodeAward NoProject NamePrincipal InvestigatorFunder's NameFunder RefLead Dept
458221EuroDSD - European Disorders of Sex DevelopmentRichard SinnottEuropean Commission (EC)FP7 HEALTH 2014IT - E-SCIENCE