Psychiatric manifestations in young females with congenital adrenal hyperplasia in Taiwan

Background: Congenital adrenal hyperplasia (CAH) is a syndrome of prenatal and/or postnatal androgen excess secondary to genetic deficits in the cytochrome p450 enzymes of the cortisol synthesis pathway. Women with CAH may suffer from different degrees of androgenization. This study documented psychiatric manifestations in young women born with CAH in Taiwan. Methods: From July 1, 2005 to February 31, 2006 the psychiatric morbidity, gender identity and sex-typed behavior during childhood of 11 young women with CAH were assessed either using questionnaire-based semi-structured interviews or self-reported questionnaires. Results: Of the 11 young women (mean age 15.3 ± 5.6 years), 3 (27.3%) had salt-wasting CAH and 8 (72.7%) had simple-virilizing CAH. Two patients without prior gender assignment (delayed diagnosis) were evaluated for primary amenorrhea at age 19 and 24 years, respectively. Four of the eleven participants (36.4%) had had an axis I psychiatric diagnosis within the past year. The subjects with more atypical sex-typed behavior in childhood tended to have a higher risk of minor psychiatric illness (r = -0.706, p < 0.05). Conclusions: This study examined the impact of CAH on the patients' psychological well-being. Psychiatric needs were found to be unmet. Earlier psychiatric evaluation and intervention for these patients is suggested.