Gender Role Behaviour, Sexuality and Sexual Function in Women With Congenital Adrenal Hyperplasia

Abstract

Purpose Females with congenital adrenal hyperplasia (CAH) are exposed to androgens during fetal development resulting in virilization of external genitalia. Gender-atypical behavior has been described in young girls with CAH. Little is known about what effect the disease has on their lives. Material and Methods Sixty-two Swedish women with CAH and age-matched controls completed a 120-item questionnaire, McCoy sexual rating scale and a validated quality of life instrument (PGWB). Surgical outcome was evaluated by clinical examination. The patients were divided into four genotype groups. Results Cases held more male-dominant occupations (30%) compared to controls (13%) (P=0.04), especially women with null mutations. They also reported a greater interest in rough sports (74%) compared to controls (50%) (P=0.007). Eight women with CAH (14%) reported a prime interest in motor vehicles, compared to none of the controls (P=0.002). Non-heterosexual orientation was reported by 19% of women with CAH (P=0.005), 50% of those with null mutations (P=0.0001). The patients in the null genotype group scored lower for sexual function and satisfaction and had more surgical complications. Conclusions Women with CAH display an increased gender-atypical behavior that is correlated to the genotype. This speaks in favor of dose dependent effects of prenatal androgens on the development of higher brain functions. The impact of the disease on upbringing and interpersonal relationships did not correlate with disease severity. Our data also shows that the null genotype group was considerably more affected than the other groups and should be regarded as a subgroup with special needs.