Abstract
Serum levels of 11-deoxycortisol were determined in 182 hirsute women. Three patients presented high basal 11-deoxycortisol levels and an exaggerated response of this steroid to ACTH stimulation. A fourth patient had normal basal 11-deoxycortisol but was hyperresponsive to ACTH stimulation. Therefore diagnosis of late-onset 11 β-hydroxylase deficiency was made in 4 out of 182 hirsute women with a prevalence of 2.2% in the group studied. In these patients, clinical findings and other hormonal patterns were not different from those of other women suffering from hirsutism.
Similar content being viewed by others
References
Gabrilove J.L., Shama E.C., Dorfman R.I. Adrenocortical 11 β-hydroxylase deficiency and virilism first manifest in the adult woman. N. Engl. J. Med. 272: 1189, 1965.
Chathelineau G., Brerault J.L., Fiet J., Julien R., Dreux C., Canivet J. Adrenocortical 11 β-hydroxylase defect in adult women with postmenarchal onset of symptoms. J. Clin. Endocrinol. Metab. 51: 287, 1980.
Zachmann M., Tassinari D., Prader A. Clinical and biochemical variability of congenital adrenal hyperplasia due to 11 β-hydroxylase deficiency. J. Clin. Endocrinol. Metab. 56: 222, 1983.
Yen S.S.C. Chronic anovulation caused by peripheral endocrine disorders. In: Yen S.S.C, Jaffe R.B. (Eds.), Reproductive endocrinology. Saunders C., Philadelphia, 1986, p. 441.
Guthrie G.P., Wilson E.A., Quillen D.L., Jawad M.S. Adrenal androgen excess and defective 11 β-hydroxylation in women with idiopathic hirsutism. Arch. Intern. Med. 142: 729, 1982.
Bouallouche A., Brerault J.L., Fiet J., Julien R., Vermeulen C., Clathelineau G. Evidence for adrenal and for ovarian dysfunction as a possible etiology of idiopathic hirsutism. Am. J. Obstet. Gynecol. 147: 57, 1983.
Hatch R., Rosenfield R.L., Kim M.H., Trodway D. Hirsutism: implications, etiology and treatment. Am. J. Obstet. Gynecol. 140: 815, 1981.
Carmina E., Rosato F., Jannì A. Increased DHEAS levels in PCO syndrome: evidence for the existence of two subgroups of patients. J. Endocrinol. Invest. 9: 5, 1986.
Carmina E., Malizia G., Jannì A. Prevalence of late-onset 21 -hydroxylase deficiency in hirsutism of Western Sicily. J. Endocrinol. Invest. 10 (Suppl.): 75, 1987.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Carmina, E., Malizia, G., Pagano, M. et al. Prevalence of late-onset 11 β-hydroxylase-deficiency in hirsute patients. J Endocrinol Invest 11, 595–598 (1988). https://doi.org/10.1007/BF03350188
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/BF03350188