Study Objective: To assess the outcome variables of menstrual and reproductive history and height among adult females with 21-hydroxylase congenital adrenal hyperplasia based upon parameters of adequacy of glucocorticoid suppressive therapy, parental heights, and growth during infancy, childhood, and adolescence. Design and Participants: Forty-three women and adolescents with 21-hydroxylase deficiency were evaluated using interviews, medical record reviews, and physical examination. Patients were categorized by menstrual regularity into three groups: A - regular menses (n = 15), B - irregular menses (n = 9), and C - regular menses that subsequently became irregular (n = 10). For comparison, a group of nine currently pubertal, premenarchal girls with classical 21-hydroxylase deficiency (group D) were similarly evaluated. Age of diagnosis, growth parameters, hormonal indices, age of puberty and menarche onset, and relative glucocorticoid dosages were compared among the groups. Results: Adult height, corrected for midparental height, was taller 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 better suppressed than B during puberty and adulthood. Age at diagnosis, concurrent mineralocorticoid therapy, glucocorticoid or equivalent dosage/M2, skeletal age Z scores, pubertal onset, and menarche age did not differ among the groups. Two women (group A) had normal pregnancies. Conclusion: Although the small numbers preclude significant differences in many of the analyses, there is a definite trend for Group A with the tallest height and regular menses to have had tighter control with lower androgen levels in infancy, childhood, puberty, and during adulthood. Results also suggest that adequate suppression during puberty will permit the onset of regular menses as the group that developed irregular menses after initial regular cycles (C) was better suppressed during puberty than the group that never menstruated regularly. Follow-up of group D, the younger, more recently treated patients, should provide better evidence as to whether tighter control will result in taller adult height and regular menses.
All Science Journal Classification (ASJC) codes
- Pediatrics, Perinatology, and Child Health
- Obstetrics and Gynecology