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库欣综合征评估的个体化方法

AN INDIVIDUALIZED APPROACH TO THE EVALUATION OF CUSHING SYNDROME.

作者信息

Sharma Susmeeta T

出版信息

Endocr Pract. 2017 Jun;23(6):726-737. doi: 10.4158/EP161721.RA.

Abstract

UNLABELLED

Cushing syndrome (CS) is caused by chronic exposure to excess glucocorticoids. Early recognition and treatment of hypercortisolemia can lead to decreased morbidity and mortality. The diagnosis of CS and thereafter, establishing the cause can often be difficult, especially in patients with mild and cyclic hypercortisolism. Surgical excision of the cause of excess glucocorticoids is the optimal treatment for CS. Medical therapy (steroidogenesis inhibitors, medications that decrease adrenocorticotropic hormone [ACTH] levels or glucocorticoid antagonists) and pituitary radiotherapy may be needed as adjunctive treatment modalities in patients with residual, recurrent or metastatic disease, in preparation for surgery, or when surgery is contraindicated. A multidisciplinary team approach, individualized treatment plan and long-term follow-up are important for optimal management of hypercortisolemia and the comorbidities associated with CS.

ABBREVIATIONS

ACTH = adrenocorticotropic hormone; BIPSS = bilateral inferior petrosal sinus sampling; CBG = corticosteroid-binding globulin; CD = Cushing disease; CRH = corticotropin-releasing hormone; CS = Cushing syndrome; Dex = dexamethasone; DST = dexamethasone suppression test; EAS = ectopic ACTH syndrome; FDA = U.S. Food & Drug Administration; HDDST = high-dose DST; IPS/P = inferior petrosal sinus to peripheral; MRI = magnetic resonance imaging; NET = neuroendocrine tumor; PET = positron emission tomography; UFC = urinary free cortisol.

摘要

未标注

库欣综合征(CS)由长期暴露于过量糖皮质激素引起。早期识别和治疗高皮质醇血症可降低发病率和死亡率。CS的诊断以及随后确定病因往往具有挑战性,尤其是在轻度和周期性高皮质醇血症患者中。手术切除过量糖皮质激素的病因是CS的最佳治疗方法。对于有残留、复发或转移性疾病的患者、在术前准备时或手术禁忌时,可能需要药物治疗(类固醇生成抑制剂、降低促肾上腺皮质激素[ACTH]水平的药物或糖皮质激素拮抗剂)和垂体放疗作为辅助治疗方式。多学科团队方法、个性化治疗方案和长期随访对于高皮质醇血症及与CS相关的合并症的最佳管理至关重要。

缩写

ACTH = 促肾上腺皮质激素;BIPSS = 双侧岩下窦采血;CBG = 皮质类固醇结合球蛋白;CD = 库欣病;CRH = 促肾上腺皮质激素释放激素;CS = 库欣综合征;Dex = 地塞米松;DST = 地塞米松抑制试验;EAS = 异位ACTH综合征;FDA = 美国食品药品监督管理局;HDDST = 大剂量DST;IPS/P = 岩下窦与外周血;MRI = 磁共振成像;NET = 神经内分泌肿瘤;PET = 正电子发射断层扫描;UFC = 尿游离皮质醇

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