Radetti G, Cacciari E, Zachmann M
Department of Paediatrics, General Hospital of Bolzano, Italy.
Eur J Pediatr. 1990 Aug;149(11):762-4. doi: 10.1007/BF01957274.
The case of a 17-year-old male with isolated hypersecretion of dehydroepiandrosterone sulphate (DHEAS) is described. Cushing syndrome, congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency, an androgen producing adrenal tumour, and elevated plasma DHEAS due to an increased renal threshold were excluded. Selective renal vein catheterization confirmed bilateral, isolated adrenal DHEAS hypersecretion. The dexamethasone suppression suggests a functional nature of this alteration. This condition has to be considered in the differential diagnosis of excessive adrenal androgen production in males, or of hirsutism in females.
本文描述了一名17岁男性,其存在硫酸脱氢表雄酮(DHEAS)分泌过多的情况。已排除库欣综合征、因3β-羟基类固醇脱氢酶缺乏导致的先天性肾上腺皮质增生、分泌雄激素的肾上腺肿瘤以及因肾阈值升高导致的血浆DHEAS升高。选择性肾静脉插管证实双侧肾上腺存在孤立性DHEAS分泌过多。地塞米松抑制试验提示这种改变具有功能性。在男性肾上腺雄激素分泌过多或女性多毛症的鉴别诊断中必须考虑这种情况。