Valizadeh Majid, Rahmani Fatemeh, Nikoohemmat Mohammad, Ramezani Ahmadi Amirhossein, Hosseinpanah Farhad, Niroomand Mahtab, Khameneh Bagheri Arash, Grossman Ashley, Abiri Behnaz
Obesity Research Center, Research Institute for Endocrine Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Clinical Research and Development Center, Shahid Modarres Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Endocr Res. 2024 Aug-Nov;49(4):232-242. doi: 10.1080/07435800.2024.2381720. Epub 2024 Jul 20.
Cushing's syndrome (CS) poses diagnostic challenges, particularly in distinguishing pituitary-dependent Cushing's syndrome, Cushing's disease (CD), from the ectopic ACTH syndrome (EAS). This study evaluated the diagnostic value of the desmopressin stimulation test (DST) in patients with ACTH-dependent CS in helping this discrimination.
Twenty-three ACTH-dependent CS patients underwent sequential DST, bilateral inferior petrosal sinus sampling (BIPSS), and transsphenoidal surgery (TSS). Two definitions of a positive DST results were applied. Diagnostic performance was assessed using sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and likelihood ratios. To avoid bias from predetermined criteria, we generated univariate receiver-operating characteristic (ROC) curves, plotting sensitivity against 1-specificity at various percentage cortisol and ACTH response levels.
Against BIPSS, DST demonstrated robust sensitivity (Definition 1: 90.0%, Definition 2: 76.2%) and overall accuracy (Definition 1: 87.0%, Definition 2: 73.9%). PPV was high (Definition 1: 95.0%, Definition 2: 94.1%), but NPV indicated potential false negatives. Compared to TSS, DST showed good sensitivity (Definition 1: 90.9-77.3%) and PPV (100.0%) but limited NPV (16.7%). The likelihood ratios emphasized the diagnostic value of the test. Notably, against TSS, DST showed perfect discriminatory power (AUC 1.000 for percent ACTH, 0.983 for percent cortisol).
The desmopressin test shows promise in accurately identifying the underlying cause of ACTH-dependent CS, potentially reducing the reliance on invasive procedures and providing a practical solution for managing complex cases. Further research with larger cohorts is required to validate the utility of the DST in routine clinical practice.
库欣综合征(CS)带来了诊断挑战,尤其是在区分垂体依赖性库欣综合征即库欣病(CD)与异位促肾上腺皮质激素(ACTH)综合征(EAS)方面。本研究评估了去氨加压素刺激试验(DST)在促肾上腺皮质激素(ACTH)依赖性CS患者中对这种鉴别诊断的价值。
23例ACTH依赖性CS患者依次接受了DST、双侧岩下窦采血(BIPSS)和经蝶窦手术(TSS)。应用了两种DST阳性结果的定义。使用敏感性、特异性、阳性预测值(PPV)、阴性预测值(NPV)和似然比评估诊断性能。为避免预定标准带来的偏差,我们生成了单变量受试者操作特征(ROC)曲线,在不同的皮质醇和ACTH反应水平百分比下绘制敏感性与1 - 特异性的关系。
与BIPSS相比,DST显示出较高的敏感性(定义1:90.0%,定义2:76.2%)和总体准确性(定义1:87.0%,定义2:73.9%)。PPV较高(定义1:95.0%,定义2:94.1%),但NPV显示存在潜在假阴性。与TSS相比,DST显示出良好的敏感性(定义1:90.9 - 77.3%)和PPV(100.0%),但NPV有限(16.7%)。似然比强调了该试验的诊断价值。值得注意的是,与TSS相比,DST显示出完美的鉴别能力(ACTH百分比的AUC为1.000,皮质醇百分比的AUC为0.983)。
去氨加压素试验在准确识别ACTH依赖性CS的潜在病因方面显示出前景,可能减少对侵入性检查的依赖,并为处理复杂病例提供切实可行的解决方案。需要更大样本队列的进一步研究来验证DST在常规临床实践中的效用。