库欣综合征

最后审阅: 28 四月 2022
最后更新: 17 十一月 2020

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
更多 关键诊断因素

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
其他诊断因素

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
更多 危险因素

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
更多 首要检查

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
  • 大剂量地塞米松抑制试验
  • 垂体 MRI 检查
  • 肾上腺 CT
  • 岩下窦采样
  • 胸部、腹部和盆腔 CT
  • 胸部 MRI
  • 奥曲肽扫描
  • 镓-68 DOTATATE PET/CT
更多 需考虑的检查

治疗流程

持续性治疗

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 the Pituitary Society's President and sits on its Board of Directors. She holds a research grant to the University for Clinical Studies as Principal Investigator for Novartis, Millendo, and Strongbridge, and is an occasional Scientific Consultant for Novartis and Strongbridge. 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.

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