Original Article

J Korean Soc Pediatr Endocrinol. 2005; 10(1): 64-70.

The Behavioral and Psychic Masculinizing Scores and Its Related Factors in Female Patients with Congenital Adrenal Hyperplasia Caused by 21-Hydroxylase Deficiency.

Il Tae Hwang, Seong Gon Ryu, and Kyung Hee Yi

¹Department of Pediatrics, College of Medicine, Hallym University, Korea. ithwang83@hallym.or.kr
²Department of Psychiatrics, College of Medicine, Hallym University, Korea.
³Department of Pediatrics, Kwang Myung Sung Ae Hospital, Seoul, Korea.

OBJECTIVE: We analyzed the degree of behavioral and psychic masculinization as well as factors influencing the masculinization in female patients with CAH caused by 21-hydroxylase deficiency. METHODS: We studied 26 patients with salt-wasting (SW) type, 29 patients with simple virilizing (SV) type and 30 control patients, who were older than 10 years of age. Study participants were given a questionnaire containing 55 items (20 masculine items, 20 feminine items, 15 neutral items) in order to evaluate the degree of feminity and masculinity.
RESULTS
SW and SV girls revealed significantly higher masculine scores and lower feminine scores than the control groups (P<0.05). Feminine scores were lower in the SW group compared to those in the SV group (P<0.05). Neutral scores showed no significant difference among three groups. Masculine, feminine, bisexual and undifferentiated personality characteristics exhibit 57.6%, 3.8%, 7.6%, and 30.7% in SW group, respectively; 31%, 17.2%, 20.6%, and 30%, respectively in SV group; 3.3%, 50.0%, 20.0% and 26.6%, respectively in control group. Masculine scores were negatively correlated with age at diagnosis and treatment (r=-0.516, P<0.05), but not with the levels of 17-hydroxyprogesterone at the time of diagnosis and study, advancement of bone age, age at clitoplasty, age at onset of puberty and age at menarche.
CONCLUSIONS
Female patients with 21-hydroxylase deficiency, who reveal high masculinizing score, should be closely monitored for the adverse effects of high degree of masculinization, such as polycystic ovarian syndrome. Additionally, it is important to treat adequately after early diagnosis to minimize the degree of masculinization and to develop the prenatal mode of treatment to interrupt the secretion of adrenal androgen.

Keywords : 21-hydroxylase deficiency, Masculinization, Androgen