PDF(Pubmed)
Abstract：
Desmopressin is increasingly used for the diagnosis of Cushing disease (CD) since corticotropin-releasing hormone became unavailable. We report the case a 32-year-old man who presented with overt Cushing syndrome. Morning blood cortisol, ACTH, 1 mg dexamethasone suppression test, 24-hour urinary free cortisol, and bedtime salivary cortisol were highly variable, reaching markedly elevated values. Intravenous desmopressin administration produced no ACTH or cortisol increase. Pituitary magnetic resonance imaging, thoracic computed tomography, and DOTATATE positron emission tomography scan identified no lesion. Inferior petrosal sinus sampling (IPSS) with desmopressin stimulation resulted in elevated central-to-peripheral ACTH ratio and prolactin co-secretion, while peripheral ACTH remained stable. No corticotroph tumor was identified on pituitary surgery pathology. Hypercortisolism persisted postoperatively. Cabergoline was initiated, after which the patient rapidly developed transient severe adrenal insufficiency (AI). Bilateral adrenalectomy was performed in view of persistent hypercortisolism. This is an unusual case of petrosal sinus ACTH response to desmopressin without any peripheral response, suggesting a central source of ACTH. Thus, desmopressin should still be used during IPSS in patients with no peripheral response. It is unclear whether the AI episode resulted from a combination of nadir of cyclic hypercortisolism, partial apoplexy, and response to cabergoline of an occult corticotroph tumor.
摘要：
由于促肾上腺皮质激素释放激素不可用，因此去氨加压素越来越多地用于库欣病（CD）的诊断。我们报告了一名32岁的男子，他患有明显的库欣综合征。早上的血皮质醇，ACTH,1mg地塞米松抑制试验，24小时尿游离皮质醇,睡前唾液皮质醇变化很大，达到明显升高的值。静脉施用去氨加压素不会导致ACTH或皮质醇增加。垂体磁共振成像,胸部计算机断层扫描,DOTATATE正电子发射断层扫描未发现病变。去氨加压素刺激的岩下窦采样（IPSS）导致中央与外周ACTH比率升高和催乳素共分泌，而外周ACTH保持稳定。垂体手术病理未发现促肾上腺皮质激素肿瘤。皮质醇增多症术后持续存在。卡麦角林开始了，此后，患者迅速发展为一过性严重肾上腺功能不全（AI）。鉴于持续性皮质醇增多症，进行了双侧肾上腺切除术。这是一个不寻常的情况下，岩窦ACTH对去氨加压素的反应没有任何外周反应，表明ACTH的中心来源。因此，对于无外周反应的患者，在IPSS期间仍应使用去氨加压素。目前尚不清楚AI发作是否源于周期性皮质醇增多症的最低点，部分中风,以及对隐匿性促肾上腺皮质激素肿瘤的卡麦角林的反应。
