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库欣病。

Cushing's disease.

机构信息

Department of Endocrinology, Diabetes and Nutrition, University Hospital (CHU) of Bordeaux and University of Bordeaux, France.

Department of Endocrinology, Diabetes and Nutrition, University Hospital (CHU) of Bordeaux and University of Bordeaux, France; INSERM U1215, NeuroCentre Magendie, University of Bordeaux, France.

出版信息

Presse Med. 2021 Dec;50(4):104091. doi: 10.1016/j.lpm.2021.104091. Epub 2021 Oct 27.

DOI:10.1016/j.lpm.2021.104091
PMID:34718112
Abstract

Cushing's disease (CD) is the most prevalent cause of endogenous hypercortisolism. CD is responsible for multiple co-morbidities and increased mortality. Accurate and prompt diagnosis and optimal treatment are essential to improve the prognosis of CD. However, the diagnosis of CD is probably one of the most difficult in endocrinology and, therefore, diagnostic workup should be performed in an experienced center. Transsphenoidal surgery performed by an expert surgeon is the only therapeutic option that can offer definitive cure and remains the first-line treatment in most patients. Second-line treatments include pharmacotherapy, pituitary radiotherapy and bilateral adrenalectomy. The second-line therapeutic strategy is complex, must be individualized and performed in a multidisciplinary expert center. Symptomatic treatments of persisting co-morbidities after remission, which are responsible for increased mortality and impaired quality of life is an important part of medical management.

摘要

库欣病(CD)是最常见的内源性皮质醇增多症的原因。CD 导致多种合并症和死亡率增加。准确和及时的诊断及优化治疗对于改善 CD 的预后至关重要。然而,CD 的诊断可能是内分泌学中最困难的诊断之一,因此,诊断检查应在有经验的中心进行。由专家外科医生进行的经蝶窦手术是唯一能够提供明确治愈效果的治疗方法,并且仍然是大多数患者的一线治疗方法。二线治疗包括药物治疗、垂体放疗和双侧肾上腺切除术。二线治疗策略复杂,必须个体化,并在多学科专家中心进行。缓解后持续存在的合并症的对症治疗是导致死亡率增加和生活质量下降的重要原因,也是医学管理的重要组成部分。

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