ADULT HEIGHT AND MENSTRUAL HISTORY AMONG PATIENTS WITH CLASSICAL 21-HYDROXYLASE DEFICIENCY ADRENAL-HYPERPLASIA - CORRELATION WITH GROWTH AND THERAPY DURING DEVELOPMENT

Study Objective: To assess the outcome variables of menstrual and repr oductive history and height among adult females with 21-hydroxylase co ngenital adrenal hyperplasia based upon parameters of adequacy of gluc ocorticoid suppressive therapy, parental heights, and growth during in fancy, childhood, and adolescence. Design and Participants: Forty-thre e women and adolescents with 21-hydroxylase deficiency were evaluated using interviews, medical record reviews, and physical examination. Pa tients were categorized by menstrual regularity into three groups: A-r egular menses (n = 15), B-irregular menses (n = 9), and C-regular mens es that subsequently became irregular (n = 10). For comparison, a grou p of nine currently pubertal, premenarchal girls with classical 21-hyd roxylase deficiency (group D) were similarly evaluated. Age of diagnos is, growth parameters, hormonal indices, age of puberty and menarche o nset, and relative glucocorticoid dosages were compared among the grou ps. Results: Adult height, corrected for midparental height, was talle r in A compared with B and C (p < 0.03), although actual height at age 3 years and at the onset of puberty did not differ. Hormonal indices were least suppressed in B and best suppressed in D. Group C was bette r suppressed than B during puberty and adulthood. Age at diagnosis, co ncurrent mineralocorticoid therapy, glucocorticoid or equivalent dosag e/M2, skeletal age Z scores, pubertal onset, and menarche age did not differ among the groups. Two women (group A) had normal pregnancies. C onclusion: Although the small numbers preclude significant differences in many of the analyses, there is a definite trend for Group A with t he tallest height and regular menses to have had tighter control with lower androgen levels in infancy, childhood, puberty, and during adult hood. Results also suggest that adequate suppression during puberty wi ll permit the onset of regular menses as the group that developed irre gular menses after initial regular cycles (C) was better suppressed du ring puberty than the group that never menstruated regularly. Follow-u p of group D, the younger, more recently treated patients, should prov ide better evidence as to whether tighter control will result in talle r adult height and regular menses.