Ancion G, Hennen G
Service de Biochimie, Université de Liège.
Rev Med Liege. 2000 Oct;55(10):929-34.
The diagnosis of Cushing's syndrome remains a challenge in clinical endocrinology. Cushing's syndromes are usually classified as dependent or independent from ACTH. In the first class are Cushing's disease, the ectopic corticotropin syndrome and the rare ectopic CRH syndrome. These ACTH-dependent Cushing's syndromes usually present diffusely hyperplastic adrenal glands. In the second class, are cortisol producing unilateral adrenocortical adenomas or carcinomas. New entities have recently emerged as bilateral adrenal hyperplasia not dependent from ACTH; their etiopathogenies are heterogeneous with illicit expressions at the adrenal level of functional receptors to various ligands: GIP, catecholamines, lutropin... The knowledge of such entities has to be taken into consideration in the diagnostic and management of ACTH independent Cushing syndromes.
库欣综合征的诊断在临床内分泌学中仍然是一项挑战。库欣综合征通常分为依赖促肾上腺皮质激素（ACTH）或不依赖ACTH两类。第一类包括库欣病、异位促肾上腺皮质激素综合征和罕见的异位促肾上腺皮质激素释放激素（CRH）综合征。这些依赖ACTH的库欣综合征通常表现为肾上腺弥漫性增生。第二类是分泌皮质醇的单侧肾上腺皮质腺瘤或癌。最近出现了一些新的类型，即不依赖ACTH的双侧肾上腺增生；其病因多种多样，涉及肾上腺水平上各种配体（如胃抑肽、儿茶酚胺、促黄体生成素等）功能受体的异常表达。在诊断和管理不依赖ACTH的库欣综合征时，必须考虑到这些新类型。
