Archives of Sexual Behavior

, Volume 34, Issue 4, pp 389–397 | Cite as

Gender Dysphoria and Gender Change in Chromosomal Females with Congenital Adrenal Hyperplasia

  • Arianne B. DessensEmail author
  • Froukje M. E. Slijper
  • Stenvert L. S. Drop


This article reviews the literature on studies and case reports on gender identity and gender identity problems, gender dysphoria, and gender change in chromosomal females with congenital adrenal hyperplasia, raised male or female. The large majority (94.8%) of the patients raised female (N= 250) later developed a gender identity as girls and women and did not feel gender dysphoric. But 13 (5.2%) patients had serious problems with their gender identity. This percentage is higher than the prevalence of female-to-male transsexuals in the general population of chromosomal females. Among patients raised male, serious gender identity problems were reported in 4 (12.1%) out of 33 patients. From these observations, we conclude that the assignment to the female gender as a general policy for 46,XX patients with CAH appears justified, even in severely masculinized 46,XX newborns with CAH (Prader stage IV or V).

Key Words

congenital adrenal hyperplasia gender identity gender dysphoria 


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Copyright information

© Springer Science + Business Media, Inc. 2005

Authors and Affiliations

  • Arianne B. Dessens
    • 1
    Email author
  • Froukje M. E. Slijper
    • 1
  • Stenvert L. S. Drop
    • 1
  1. 1.Pediatrics Department, Division of Pediatric Endocrinology, Erasmus Medical CentreSophia Children’s HospitalRotterdamThe Netherlands

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