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- Title
Late Onset 21-Hydroxyiase Deficiency in a Girl Mimicking True Sexual Precocity.
- Authors
Kalter-Leibovici, O.; Dickerman, Z.; Zamir, R.; Weiss, I.; Kaufman, H.; Laron, Z.
- Abstract
Complete sexual precocity as a presenting symptom of untreated classical congenital adrenal hyperplasia (CAH) is rare and has never been reported in the late onset type. A six-year-old girl presented with early breast and pubic hair development and 17 β-estradiol (E2) levels of 75 pg/ml. The diagnosis of late onset 21-hydroxylase deficiency was confirmed by the levels of (17-OHP) (basal level 425 ng/dl; following ACTH stimulation - 3250 ng/dl) and the HLA type of B14 DR1. Following treatment with 20-25 mg/m² of hydrocortisone acetate the plasma E2 level rapidly fell to < 10 pg/ml. It is suggested that the premature breast development was induced by increased adrenal estrogen production or increased peripheral aromatization of the high levels of adrenal androgens.
- Publication
Journal of Pediatric Endocrinology & Metabolism, 1989, Vol 3, Issue 2, p121
- ISSN
0334-018X
- Publication type
Article
- DOI
10.1515/jpem.1989.3.2.121