Ovarian suppression reduces clinical and endocrine expression of late-onset congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
OBJECTIVE
To determine the effectiveness of GnRH-agonist (GnRH-a) treatment in women with late onset congenital adrenal hyperplasia.


METHODS
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.


METHODS
Academic medical center.


METHODS
Baseline blood determinations before and after i.v. ACTH, before and after 6 months of GnRH-a treatment. Estrogen and progestin replacement was begun in all women after the 3rd month of treatment.


METHODS
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 6 months 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.
Carmina
E
E
Cattedra di Endocrinologia, Universita de Palermo, Italy.
Lobo
R A
RA
D019326:17-alpha-Hydroxyprogesterone; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D000328:Adult; D017668:Age of Onset; D000728:Androgens / Q000097:blood; D003907:Dexamethasone / Q000627:therapeutic use; D004702:Endocrine Glands / Q000187:drug effects* / Q000503:physiopathology; D005260:Female; D006062:Gonadotropins / Q000097:blood; D006628:Hirsutism / Q000209:etiology; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood; D010053:Ovary / Q000187:drug effects*; D011446:Prospective Studies; D017329:Triptorelin Pamoate / Q000627:therapeutic use*
D000728:Androgens; D006062:Gonadotropins; D006908:Hydroxyprogesterones; D017329:Triptorelin Pamoate; D019326:17-alpha-Hydroxyprogesterone; D003907:Dexamethasone