伴有周期性激素生成的异位库欣综合征——一例提示发病机制的病例
Ectopic Cushing's syndrome with periodic hormonogenesis--a case suggesting a pathogenetic mechanism.
作者信息
Estopiñán V, Varela C, Riobo P, Dominguez J R, Sancho J
机构信息
Hospital Ramón y Cajal, Madrid, Spain.
出版信息
Postgrad Med J. 1987 Oct;63(744):887-9. doi: 10.1136/pgmj.63.744.887.
Abstract
We report on a case of ectopic Cushing's syndrome due to a thymic carcinoid tumour with periodic hormonogenesis. Periods of hormonal production averaged 27 days. Prior to bilateral adrenalectomy, mean (s.d) values of ACTH and cortisol were 202.1 (50.3) pg/ml and 46 (14.7) micrograms/dl, ACTH rising to 3996 +/- 425 pg/ml (P less than 0.01) and cortisol falling to 6.3 +/- 1.5 micrograms/dl (P less than 0.01) in the immediate postoperative period. During the late postoperative period (2-13 months following surgery) ACTH levels fell to 509.3 (123.8) pg/ml (P less than 0.01), but remaining even higher (P less than 0.01) than before adrenalectomy. The pattern of ACTH in the present case suggests the existence of a negative feedback exerted by the cortisol over tumoral ACTH.
摘要
我们报告一例因胸腺类癌伴周期性激素生成导致的异位库欣综合征病例。激素产生周期平均为27天。双侧肾上腺切除术前，促肾上腺皮质激素（ACTH）和皮质醇的平均（标准差）值分别为202.1（50.3）pg/ml和46（14.7）μg/dl，术后即刻ACTH升至3996±425 pg/ml（P<0.01），皮质醇降至6.3±1.5μg/dl（P<0.01）。术后晚期（术后2 - 13个月），ACTH水平降至509.3（123.8）pg/ml（P<0.01），但仍高于肾上腺切除术前水平（P<0.01）。本例中ACTH的变化模式提示皮质醇对肿瘤性ACTH存在负反馈作用。
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