6533b862fe1ef96bd12c62a5

RESEARCH PRODUCT

Ovarian suppression reduces clinical and endocrine expression of late-onset congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

subject

description

Objective To determine the effectiveness of GnRH-agonist (GnRH-a) treatment in women with late onset congenital adrenal hyperplasia. Design Prospective assessment of GnRH-a treatment in six women with documented late-on-set congenital adrenal hyperplasia who were not preselected. Comparisons were made to previous responses in the same patients receiving dexamethasone. Eight age- and weight-matched ovulatory women served as controls. Setting Academic medical center. Intervention Baseline blood determinations before and after IV ACTH, before and after 6months of GnRH-a treatment. Estrogen and progestin replacement was begun in all women after the 3rd month of treatment. Main Outcome Measures Serum 17-hydroxyprogesterone (17-OHP), gonadotropin, and androgen levels before and after GnRH-a treatment. Responses of 17-OHP and androgens to ACTH assessment of hirsutism using a modified Ferriman-Gallwey score. Results Gonadotropins, estrogen, androgen, and 17-OHP were suppressed with GnRH-a treatment. Levels were similar before and after estrogen and progestin replacement. Responses of 17-OHP after ACTH were blunted but still were elevated compared with responses in controls. Ferriman-Gallwey scores decreased significantly (−8±1; mean±SE). This response was greater than that observed previously with 6months of dexamethasone (−2±0.3). Conclusions Suppression of the ovary with GnRH-a treatment was beneficial in these patients with late-onset congenital adrenal hyperplasia. An ovarian influence on the clinical and biochemical findings of the disorder is suggested.