Vilar L, Freitas M C, Naves L A, Canadas V, Albuquerque J L, Botelho C A, Egito C S, Arruda M J, Silva L M, Arahata C M, Agra R, Lima L H C, Azevedo M, Casulari L A
Division of Endocrinology, Hospital das Clínicas, Pernambuco Federal University, Recife, Brazil.
J Endocrinol Invest. 2008 Nov;31(11):1008-13. doi: 10.1007/BF03345640.
To evaluate the role of non-invasive dynamic tests in the diagnosis and differential diagnosis of Cushing's syndrome (CS).
We studied laboratory features of 74 patients with endogenous CS, subdivided as follows: 46 (62.1%) with Cushing's disease (CD), 21 (28.3%) with an adrenal tumor, and 7 (9.5%) with ectopic ACTH syndrome (EAS).
In 100% of cases of CS we found serum cortisol levels greater than 1.8 microg/dl after low-dose dexamethasone suppression tests (LDDST), as well as elevation of midnight serum or salivary cortisol. However, urinary free cortisol was normal in 11.5% of patients. ACTH levels were suppressed in patients with adrenal tumors, normal or high in CD and invariably increased in EAS. After the 8-mg overnight dexamethasone suppression test (HDDST), serum cortisol suppression >50% was observed in 79.5% of cases of CD and in 28.6% of subjects with EAS, whereas cortisol suppression >80% was only found in CD. After stimulation with CRH or desmopressin an ACTH rise > or =35% occurred in 86.5% of individuals with CD and 14.3% of those with EAS, whereas an ACTH rise > or =50 achieved 100% specificity. Moreover, the combination of serum cortisol suppression >50% after HDDST and an ACTH increase > or =35% after the administration of CRH or desmopressin only occurred in CD.
Our findings demonstrate that LDDST had 100% sensitivity for the diagnosis of CS and that HDDST and stimulation tests with CRH or desmopressin may be very useful for confirmation of CS etiology when analyzed together or when more stringent cut-offs are used.
评估非侵入性动态试验在库欣综合征(CS)诊断及鉴别诊断中的作用。
我们研究了74例内源性CS患者的实验室特征,这些患者分为以下几类:46例(62.1%)库欣病(CD)患者,21例(28.3%)肾上腺肿瘤患者,7例(9.5%)异位促肾上腺皮质激素(ACTH)综合征(EAS)患者。
在所有CS病例中,低剂量地塞米松抑制试验(LDDST)后血清皮质醇水平均高于1.8μg/dl,午夜血清或唾液皮质醇也升高。然而,11.5%的患者尿游离皮质醇正常。肾上腺肿瘤患者的ACTH水平被抑制,CD患者的ACTH水平正常或升高,EAS患者的ACTH水平总是升高。8毫克过夜地塞米松抑制试验(HDDST)后,79.5%的CD病例和28.6%的EAS患者血清皮质醇抑制率>50%,而仅在CD患者中发现皮质醇抑制率>80%。促肾上腺皮质激素释放激素(CRH)或去氨加压素刺激后,86.5%的CD患者和14.3%的EAS患者ACTH升高≥35%,而ACTH升高≥50%具有100%的特异性。此外,HDDST后血清皮质醇抑制率>50%与CRH或去氨加压素给药后ACTH升高≥35%仅在CD患者中同时出现。
我们的研究结果表明,LDDST对CS诊断的敏感性为100%,HDDST以及CRH或去氨加压素刺激试验在联合分析或使用更严格的临界值时,对于确定CS病因可能非常有用。