Bakker R C, Gallas P R, Romijn J A, Wiersinga W M
Department of Endocrinology and Metabolism, Academic Medical Centre, Amsterdam, The Netherlands.
J Endocrinol Invest. 1998 May;21(5):329-33. doi: 10.1007/BF03350337.
The case history of a 56-year-old man is described who suffered from severe adrenocorticotrophic hormone (ACTH)-dependent Cushing's syndrome. The clinical course was complicated by simultaneous infections with Pneumocystis carinii, Staphylococcus aureus, Candida albicans, Aspergillus fumigatus and Herpes simplex, which proved to be fatal. A study of the literature shows that opportunistic infections in endogenous Cushing's syndrome are associated with severe cortisol excess and carry a high mortality. Opportunistic infections are most prevalent in the ectopic ACTH syndrome explained by the very high plasma cortisol concentrations in this condition. Infections with Aspergillus species, Cryptoccus neoformans, Pneumocystis carinii and Nocardia asteroides predominated. Cushing's syndrome with a very high plasma cortisol concentration causes a severe immunocompromized state. Prompt evaluation of the cause of the hypercortisolism, initiation of cortisol lowering therapy, primary prophylaxis for Pneumocystis carinii infection when plasma cortisol exceeds 2500 nmol L-1 and a search for concomitant infectious disease is recommended.
本文描述了一名56岁男性的病例史，该患者患有严重的促肾上腺皮质激素（ACTH）依赖性库欣综合征。临床病程因同时感染卡氏肺孢子虫、金黄色葡萄球菌、白色念珠菌、烟曲霉和单纯疱疹而复杂化，这些感染被证明是致命的。文献研究表明，内源性库欣综合征中的机会性感染与严重的皮质醇过多有关，且死亡率很高。机会性感染在异位ACTH综合征中最为普遍，这是由该病症中非常高的血浆皮质醇浓度所解释的。曲霉菌属、新型隐球菌、卡氏肺孢子虫和星形诺卡菌感染最为常见。血浆皮质醇浓度非常高的库欣综合征会导致严重的免疫功能低下状态。建议迅速评估高皮质醇血症的病因，开始降低皮质醇的治疗，当血浆皮质醇超过2500 nmol/L时对卡氏肺孢子虫感染进行一级预防，并寻找合并的传染病。
