Sapunar J, Triantafilo Y, Arteaga E, Rojas A, Campino C, López J M, Rodríguez J A
Departamento de Endocrinología, Metabolismo y Nutrición, Hospital Clínico, Universidad Católica de Chile.
Rev Chil Obstet Ginecol. 1991;56(3):213-6.
Late-onset adrenal enzymatic deficiencies are a common cause of hirsutism. They can produce a PCO syndrome-like clinical course, underscoring the multi-causality of this condition. We report a 18 year old woman with secondary amenorrhea and hirsutism, whose serum levels of testosterone and DHEA-S were 175 ng/dl and 7.3 micrograms/ml and the LH/FSH ratio was 5.9. Dexamethasone decreased testosterone and DHEA-S levels, while the ACTH stimulation test increased the 17 hydroxypregnenolone serum concentration and the 17 hydroxypregnenolone/17 hydroxyprogesterone ratio to values considered diagnostic of 3 beta hydroxysteroid dehydrogenase deficiency.
迟发性肾上腺酶缺乏是多毛症的常见原因。它们可导致类似多囊卵巢综合征的临床病程，突显了该病症的多病因性。我们报告一名18岁继发性闭经和多毛症女性，其血清睾酮和硫酸脱氢表雄酮水平分别为175 ng/dl和7.3微克/毫升，促黄体生成素/促卵泡激素比值为5.9。地塞米松降低了睾酮和硫酸脱氢表雄酮水平，而促肾上腺皮质激素刺激试验使血清17-羟孕烯醇酮浓度及17-羟孕烯醇酮/17-羟孕酮比值升高至被认为可诊断3β-羟类固醇脱氢酶缺乏的值。
