Machado Marcio Carlos, de Sa Sandra Valeria, Domenice Sorahia, Fragoso Maria Candida B Villares, Puglia Paulo, Pereira Maria Adelaide Albergaria, de Mendonça Berenice Bilharinho, Salgado Luiz Roberto
Division of Endocrinology and Metabolism, University of Sao Paulo School of Medicine, Sao Paulo, SP, Brazil.
Clin Endocrinol (Oxf). 2007 Jan;66(1):136-42. doi: 10.1111/j.1365-2265.2006.02700.x.
Bilateral inferior petrosal sinus sampling (BIPSS) with corticotrophin-releasing hormone (CRH) stimulation is currently the gold standard test for the differential diagnosis of ACTH-dependent Cushing's syndrome. Reports on the use of desmopressin in this approach are limited. The aim of this study was to evaluate the use of desmopressin during BIPSS in a cohort of patients with ACTH-dependent Cushing's syndrome.
A retrospective case-record study.
Fifty-six patients with confirmed ACTH-dependent Cushing's syndrome underwent BIPSS with desmopressin stimulation when presenting negative pituitary tumour imaging.
Central to peripheral (CEN:PER) ACTH gradient, lateralization of the ACTH source and surgical tumour confirmation were evaluated.
A CEN:PER ACTH gradient was found in 40 patients under basal conditions (CEN:PER >or= 2) and in 47 patients after desmopressin stimulation (CEN:PER >or= 3). Ectopic ACTH-producing tumours (three lung carcinoid tumour, one thymus carcinoid tumour and one thymus hyperplasia) were confirmed in five out of nine patients without the CEN:PER ACTH gradient, and four cases were false negative for Cushing's disease. Lateralization (IPS:IPS >or= 1.4) was observed in 80.8% of patients under basal conditions (38/47) and in 97.8% after desmopressin (46/47), and it was surgically confirmed in 78.7%. There were no false-positive cases. Sensitivity and specificity were 92.1% and 100%, respectively.
Desmopressin improves the differential diagnosis of ACTH-dependent Cushing's syndrome by amplifying the CEN:PER and IPS:IPS ACTH gradients, and is therefore a useful ACTH secretagogue in BIPSS.
促肾上腺皮质激素释放激素（CRH）刺激下的双侧岩下窦采血（BIPSS）目前是促肾上腺皮质激素（ACTH）依赖性库欣综合征鉴别诊断的金标准检测方法。关于去氨加压素在该方法中的应用报道有限。本研究的目的是评估去氨加压素在一组ACTH依赖性库欣综合征患者的BIPSS过程中的应用。
一项回顾性病例记录研究。
56例确诊为ACTH依赖性库欣综合征的患者在垂体肿瘤影像学检查呈阴性时接受了去氨加压素刺激下的BIPSS。
评估中央与外周（CEN:PER）ACTH梯度、ACTH来源的定位以及手术肿瘤确诊情况。
40例患者在基础状态下（CEN:PER≥2）发现CEN:PER ACTH梯度，47例患者在去氨加压素刺激后（CEN:PER≥3）发现该梯度。在9例无CEN:PER ACTH梯度的患者中，5例确诊为异位ACTH分泌肿瘤（3例肺类癌肿瘤、1例胸腺类癌肿瘤和1例胸腺增生），4例库欣病患者为假阴性。基础状态下80.8%（38/47）的患者观察到定位（IPS:IPS≥1.4），去氨加压素刺激后97.8%（46/47）的患者观察到定位，手术确诊率为78.7%。无假阳性病例。敏感性和特异性分别为92.1%和100%。
去氨加压素通过放大CEN:PER和IPS:IPS ACTH梯度改善了ACTH依赖性库欣综合征的鉴别诊断，因此是BIPSS中一种有用的ACTH促分泌剂。
