双侧岩下窦静脉采血在库欣综合征诊断中应用的专家共识(2023)  

Expert Consensus on Bilateral Inferior Petrosal Sinus Sampling in the Diagnosis of Cushing's Syndrome(2023)

作　　者：中国罕见病联盟下丘脑垂体疾病学组 北京协和医院垂体疾病创新诊疗中心 朱惠娟 卢琳 张晓波[3] 吕晓虹 张丁月 陈适[4] Chinese Medical Association Rare Diseases Branch,Hypothalamic and Pituitary Disease Group of China Alliance of Rare Diseases(CARD);Innovation Center of Pituitary Diseases,Peking Union Medical College Hospital;ZHU Huijuan;LU Lin;ZHANG Xiaobo(不详;Department of Endocrinology,Key Laboratory of Endocrinology of National Health Commission,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Radiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]不详 [2]中国医学科学院北京协和医院内分泌科、国家卫生健康委员会内分泌重点实验室,北京100730 [3]中国医学科学院北京协和医院放射科,北京100730 [4]北京协和医院内分泌科

出　　处：《罕见病研究》2023年第3期365-376,共12页Journal of Rare Diseases

基　　金：中央高水平医院临床科研业务费(2022-PUMCH-A-015,2022-PUMCH-B-016,2022-PUMCH-B-114);国家重点研发计划(2022YFC3600203)。

摘　　要：库欣综合征(CS)是由多种病因导致的以向心性肥胖、高血压、血糖升高和骨质疏松等为主要表现的临床症候群。对于促肾上腺皮质激素(ACTH)依赖性库欣综合征的患者,在常规影像学检查定位不清的情况下,进行双侧岩下窦静脉采血(BIPSS)是判断ACTH来源的金标准。但BIPSS的适应证、禁忌证、操作流程、注意事项及结果判定在不同医疗中心存在差异,且目前国际上和国内尚无关于BIPSS的临床指南和专家共识发表。为进一步完善BIPSS在疑难CS患者诊断流程中的操作规范,中国罕见病联盟下丘脑垂体疾病学组、北京协和医院垂体疾病创新诊疗中心组织国内内分泌科、放射介入科、神经外科和检验科等相关领域的专家制订该共识,旨在为临床实践中CS鉴别诊断流程使用BIPSS提供规范性的方法学指导。Cushing's syndrome(CS)is a clinical syndrome caused by a variety of causes,with main manifestations exhibited by central obesity,purple skin striae,hypertension,and diabetes.In patients with adrenocorticotropic hormone(ACTH)-dependent CS,the result of bilateral inferior petrosal sinus sampling(BIPSS)is the gold standard for determining the source of ACTH in the absence of routine imaging findings.However,the indications,contraindications,operating procedures,precautions and outcome judgments of BIPSS differ from one medical center to another,and there are currently no international and domestic clinical guidelines and expert consensus on BIPSS.In order to further improve the operation specifications of BIPSS in the diagnosis process of difficult CS,the Hypothalamic and Pituitary Disease Group of the China Alliance for Rare Diseases and the Innovation Center of Pituitary Diseases of Peking Union Medical College Hospital organized experts in endocrinology,interventional radiology,neurosurgery and laboratory in China to formulate this consensus.This consensus aims to provide standardized methodological guidance for the use of BIPSS in the differential diagnostic process of CS in clinical practice.

关 键 词：库欣综合征 双侧岩下窦静脉采血 库欣病 异位ACTH分泌综合征 专家共识 

分 类 号：R586.2[医药卫生—内分泌]