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库欣病的治疗:概述和最新发现。

Medical treatment of Cushing's disease: Overview and recent findings.

机构信息

Department of Medicine.

出版信息

Int J Gen Med. 2009 Dec 29;2:209-17. doi: 10.2147/ijgm.s7545.

DOI:10.2147/ijgm.s7545
PMID:20360906
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2840568/
Abstract

Cushing's disease, due to pituitary adrenocorticotropic hormone (ACTH) hypersecretion, is the most common etiology of spontaneous excess cortisol production. The majority of pituitary tumors causing Cushing's disease measure <1 cm and the excess morbidity associated with these tumors is mostly due to the effects of elevated, nonsuppressible, ACTH levels leading to adrenal steroid hypersecretion. Elevated circulating cortisol levels lead to abnormal fat deposition, hypertension, diabetes, coronary artery disease, osteoporosis, muscle weakness and psychological disturbances. At experienced centers, initial surgical remission rate via transnasal, transphenoidal resection approaches 80% for tumors less than 1 cm, but may be as low as 30% for larger lesions and long-term recurrence in all groups approaches 25%. Residual disease may be managed with more radical surgery, pituitary-directed radiation, bilateral adrenalectomy, or medical therapy. This paper addresses current and novel therapies in various stages of development for Cushing's disease.

摘要

库欣病是由于垂体促肾上腺皮质激素(ACTH)分泌过度引起的,是自发性皮质醇过度产生的最常见病因。大多数导致库欣病的垂体肿瘤小于 1 厘米,这些肿瘤引起的发病率增加主要是由于升高的、不可抑制的、ACTH 水平导致肾上腺类固醇过度分泌所致。循环中皮质醇水平升高会导致异常脂肪沉积、高血压、糖尿病、冠状动脉疾病、骨质疏松症、肌肉无力和心理障碍。在有经验的中心,经鼻蝶入路的初次手术缓解率对于小于 1 厘米的肿瘤为 80%,但对于较大的病变可能低至 30%,并且所有组别的长期复发率接近 25%。残余疾病可以通过更激进的手术、垂体定向放疗、双侧肾上腺切除术或药物治疗来管理。本文介绍了库欣病各阶段的现有和新疗法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28fa/2840568/cc9d939073a0/ijgm-2-209f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28fa/2840568/cc9d939073a0/ijgm-2-209f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28fa/2840568/cc9d939073a0/ijgm-2-209f1.jpg

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本文引用的文献

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Dopamine agonists and valvular heart disease.多巴胺激动剂与心脏瓣膜病
Curr Opin Endocrinol Diabetes Obes. 2009 Aug;16(4):316-20. doi: 10.1097/MED.0b013e32832d9f64.
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Merits and pitfalls of mifepristone in Cushing's syndrome.米非司酮治疗库欣综合征的利弊
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Guidelines for acromegaly management: an update.肢端肥大症管理指南:更新版
漏诊分泌性垂体腺瘤的风险。
Orphanet J Rare Dis. 2016 Oct 6;11(1):135. doi: 10.1186/s13023-016-0516-x.
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Targeting the ERK pathway for the treatment of Cushing's disease.靶向ERK通路治疗库欣病。
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Managing Cushing's disease: the state of the art.库欣病的管理:当前的技术水平
Endocrine. 2014 Sep;47(1):9-20. doi: 10.1007/s12020-013-0129-2. Epub 2014 Jan 11.
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Evidence for orphan nuclear receptor TR4 in the etiology of Cushing disease.TR4 孤儿核受体在库欣病发病机制中的证据。
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Coexpression of dopamine and somatostatin receptor subtypes in corticotroph adenomas.多巴胺与生长抑素受体亚型在促肾上腺皮质激素腺瘤中的共表达。
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Treatment of pituitary-dependent Cushing's disease with the multireceptor ligand somatostatin analog pasireotide (SOM230): a multicenter, phase II trial.多受体配体生长抑素类似物帕瑞肽(SOM230)治疗垂体依赖性库欣病:一项多中心II期试验
J Clin Endocrinol Metab. 2009 Jan;94(1):115-22. doi: 10.1210/jc.2008-1008. Epub 2008 Oct 28.
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