Gender dysphoria and gender change in chromosomal females with congenital adrenal hyperplasia

Arch Sex Behav. 2005 Aug;34(4):389-97. doi: 10.1007/s10508-005-4338-5.


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).

Publication types

  • Review

MeSH terms

  • Adrenal Hyperplasia, Congenital / complications*
  • Adrenal Hyperplasia, Congenital / psychology
  • Adrenal Hyperplasia, Congenital / surgery
  • Adult
  • Chromosomes, Human, X
  • Disorders of Sex Development / etiology*
  • Disorders of Sex Development / psychology
  • Female
  • Gender Identity*
  • Gonadal Dysgenesis, 46,XX / complications*
  • Gonadal Dysgenesis, 46,XX / psychology
  • Gonadal Dysgenesis, 46,XX / surgery
  • Humans
  • Identification, Psychological*
  • Male
  • Psychosexual Development*
  • Sex Characteristics
  • Sex Factors
  • Sexual Behavior
  • Time Factors