小结
定义
病史和体格检查
关键诊断因素
- 存在的危险因素
- 面部充血
- 锁骨上脂肪垫
- 紫纹
- 未妊娠
- 月经不调
- 无营养不良
- 无酗酒
- 无生理压力
- 儿童线性生长减速
其他诊断因素
- 女性
- 高血压
- 糖耐量异常和糖尿病
- 过早的骨质疏松和原因不明的骨折
- 体重增加和向心性肥胖
- 痤疮
- 精神症状
- 性欲减退
- 易瘀伤
- 虚弱
- 满月脸
- 颈背部脂肪垫
- 原因不明的肾结石
- 静脉血栓事件
- 多毛症
危险因素
- 外源性皮质类固醇的使用
- 垂体腺瘤
- 肾上腺腺瘤
- 肾上腺癌
- 神经内分泌肿瘤
- 胸腺癌或支气管癌
诊断性检查
首要检查
- 尿妊娠试验
- 血清葡萄糖
- 深夜唾液皮质醇
- 1 mg 过夜地塞米松抑制试验
- 24 小时尿游离皮质醇
- 48 小时 2 mg（小剂量），口服地塞米松抑制试验
需考虑的检查
- 血浆硫酸脱氢表雄酮（DHEAS）水平
- 清晨血浆促肾上腺皮质激素（ACTH）水平
- 垂体 MRI 检查
- 肾上腺 CT
- 大剂量地塞米松抑制试验
- 岩下窦采样（IPSS）
- 胸部、腹部和盆腔 CT
- 胸部 MRI
- 奥曲肽扫描
- 镓-68 DOTATATE PET/CT
治疗流程
库欣病（分泌促肾上腺皮质激素 [ACTH] 的垂体瘤）
异位 ACTH 或 CRH 综合征
单侧肾上腺癌或腺瘤导致的 ACTH 非依赖性库欣综合征
双侧肾上腺疾病（增生或腺瘤）导致的 ACTH 非依赖性库欣综合征
撰稿人
作者
Maria Fleseriu, MD, FACE
Professor of Medicine (Endocrinology) and Neurological Surgery
Director
Pituitary Center
Oregon Health & Science University
Portland
OR
利益声明
MF is on the Pituitary Society's Board of Directors. She holds a research grant to the University for Clinical Studies as Principal Investigator for Recordati and Strongbridge, and is an occasional Scientific Consultant for Recordati, HRA Pharma, and Sparrow. MF is an author of several references cited in this topic.
鸣谢
Dr Maria Fleseriu would like to gratefully acknowledge Dr Ty Carroll and Dr James Findling, previous contributors to this topic.
利益声明
TC is an author of a number of references cited in this topic. He is an investigator in clinical trials sponsored by Corcept. JF is an author of a number of references cited in this topic. He is a consultant for, and investigator in, clinical trials sponsored by Corcept and Novartis.
同行评议者
Paul M. Stewart, FRCP FMedSci
Professor of Medicine
Director of Research
College of Medical and Dental Sciences
University of Birmingham
Honorary Consultant Physician
Queen Elizabeth Hospital
Birmingham
UK
利益声明
PMS declares that he has no competing interests.
Antoine Tabarin, MD
Head
Department of Endocrinology
University Hospital of Bordeaux
Pessac
France
利益声明
AT declares that he has no competing interests.
Liliana Contrersas, MD
Chief
Endocrine Research Department
Instituto de Investigaciones Médicas A. Lanari
University of Buenos Aires and IDIM-CONICET
Buenos Aires
Argentina
利益声明
LC declares that she has no competing interests.
Philip R. Orlander, MD
Professor of Medicine
Director
Division of Endocrinology, Diabetes & Metabolism
University of Texas Medical School
Houston
TX
利益声明
PRO declares that he has no competing interests.
Mouhammed Amir Habra, MD, FACP, FACE
Assistant Professor
Department of Endocrine Neoplasia and Hormonal Disorders
Division of Internal Medicine
University of Texas MD Anderson Cancer Center
Houston
TX
利益声明
MAH declares that he has no competing interests.
鉴别诊断
- 肥胖
- 代谢综合征
更多 鉴别诊断指南
- 偶然发现的肾上腺肿块的诊断、管理和随访
- 美国内分泌外科医师协会关于肾上腺切除术的指南
更多 指南患者教育信息
药源性库欣综合症
内源性库欣综合症
更多 患者教育信息
登录或订阅即可浏览 BMJ Best Practice 临床实践完整内容
内容使用需遵循免责声明