Tsigos C, Papanicolaou D A, Chrousos G P
Developmental Endocrinology Branch, National Institutes of Health, Bethesda, MD 20892, USA.
Baillieres Clin Endocrinol Metab. 1995 Apr;9(2):315-36. doi: 10.1016/s0950-351x(95)80354-8.
Excess endogenous glucocorticoid production, whether ACTH-dependent or ACTH-independent, results in the classic clinical and biochemical picture of Cushing's syndrome. The diagnosis requires demonstration of an increased cortisol secretion rate, best achieved using determination of urinary free cortisol as an index. In mild cases, distinction from the hypercortisolism of pseudo-Cushing states may prove difficult. If the physician is in doubt, a dexamethasone/CRH test should be performed. Primary adrenal Cushing's syndrome can be diagnosed on the basis of undetectable plasma ACTH and the results of adrenal imaging procedures. ACTH-dependent Cushing's syndrome can be differentiated using an oCRH test and imaging procedures. In the presence of a discrete pituitary lesion on imaging, a standard oCRH test with results consistent with such a lesion is sufficient to proceed to transsphenoidal surgery. In the absence of such a lesion or if the oCRH test is equivocal, simultaneous BIPSS with oCRH administration should be performed to distinguish between a pituitary or ectopic source. Surgical ablation is the treatment of choice for all types of Cushing's syndrome. In the 5% of cases with Cushing's disease in whom transsphenoidal surgery fails and in the 5% of cases in whom the disease recurs, repeat transsphenoidal surgery or radiation therapy in association with mitotane treatment are reasonable alternatives. Bilateral adrenalectomy effectively cures hypercortisolism if resection of the ACTH-secreting tumour is unsuccessful and radiation/medical therapy fails.
内源性糖皮质激素分泌过多，无论是否依赖促肾上腺皮质激素（ACTH），都会导致库欣综合征典型的临床和生化表现。诊断需要证明皮质醇分泌率增加，最好通过测定尿游离皮质醇作为指标来实现。在轻度病例中，与假性库欣状态的皮质醇增多症相鉴别可能很困难。如果医生有疑问，应进行地塞米松/促肾上腺皮质激素释放激素（CRH）试验。原发性肾上腺库欣综合征可根据无法检测到的血浆ACTH以及肾上腺成像检查结果进行诊断。依赖ACTH的库欣综合征可通过oCRH试验和成像检查进行鉴别。如果成像显示有明确的垂体病变，且标准oCRH试验结果与此类病变相符，则足以进行经蝶窦手术。如果没有此类病变或oCRH试验结果不明确，则应同时进行双侧岩下窦采血（BIPSS）并注射oCRH，以区分垂体或异位来源。手术切除是所有类型库欣综合征的首选治疗方法。在5%经蝶窦手术失败的库欣病病例以及5%疾病复发的病例中，再次经蝶窦手术或联合米托坦治疗的放射治疗是合理的替代方案。如果切除分泌ACTH的肿瘤不成功且放射/药物治疗失败，双侧肾上腺切除术可有效治愈皮质醇增多症。
