Congenital adrenal hyperplasia I: Gender-related behavior and attitudes in female patients and sisters

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Abstract

Thirty-five female patients with congenital adrenal hyperplasia (CAH) were compared to a group of 16 healthy sisters in regard to gender-related behavioral patterns, present attitudes, and plans for the future. A semi-structured interview with the subjects, ages 11 to 41 yr, and their mothers concentrated on four to five age stages. Results of retrospective data from single items as well as from several related composite scales (“interests and behavior,” “appearance,” “overall scores”) revealed significant group differences: Both in mother-assessment and self-assessment, CAH patients showed a “more masculine” orientation than their sisters, but this was far from consistent across all age stages, especially for single items. Unexpectedly, the gender-behavior differences between CAH patients and sisters did not hold for certain items and scales of “social behavior” (e.g., assertiveness, dominance, acceptance in peer groups) and, in contrast to some of the existing literature, also not for “high-energy expenditure.” With regard to expectations for the future, CAH patients had less of a “wish to have their own children” and a higher preference for “having a career versus staying at home.”

Age, socioeconomic status, intelligence, and presence or absence of a sister as possibly intervening psychosocial/demographic factors could not explain the group differences in behavior. Degree of genital masculinization (Prader stages) or “onset and quality” of therapy as measures of pre- and postnatal androgenization, respectively, could also not account for the degree of the “more masculine” orientation in the CAH group. Nevertheless, the overall results are compatible with earlier findings on the masculinizing effects of prenatal androgens on behavior in humans and point to a time period after sexual differentiation of the genitalia and before birth as the most likely one for the effects of prenatal hormones on behavioral masculinization in humans.

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