支气管扩张症

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最后审阅： 4 九月 2022
最后更新： 07 四月 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
  • 咳嗽
  • 咳痰
  • 湿啰音、高音调吸气性喘鸣和干啰音
  • 呼吸困难
  • 发热
更多 关键诊断因素

其他诊断因素

  • 疲劳
  • 咯血
  • 鼻窦炎
  • 体重减轻
  • 喘息
  • 胸膜炎性胸痛
  • 杵状指
其他诊断因素

危险因素

  • 囊性纤维化
  • 宿主免疫缺陷
  • 既往感染
  • 支气管气道的先天性疾病
  • 原发性纤毛运动障碍
  • α-1 抗胰蛋白酶缺乏
  • 结缔组织病
  • 炎症性肠病
  • 误吸性或吸入性损伤
  • 局部支气管阻塞
  • 高、瘦的白人女性，年龄≥60 岁
更多 危险因素
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诊断性检查

首要检查

  • 胸部 X 线检查（CXR
  • 高分辨率胸部 CT 扫描
  • 全血细胞计数（FBC
  • 痰培养和药敏
  • 血清 α-1 抗胰蛋白酶表型和水平
  • 血清免疫球蛋白
  • 汗液氯化物检测
  • 类风湿因子
  • 烟曲霉菌特异性 IgE 或皮肤点刺试验
  • 血清抗 HIV 抗体
  • 鼻呼出气一氧化氮检测 (NNO)
  • 肺功能检查 (pulmonary function test, PFT)
更多 首要检查

需考虑的检查

  • 支气管活检和纤毛电子显微镜镜检
  • 囊性纤维化跨膜调节（CFTR）蛋白基因突变检测
  • 吞咽功能检查
  • 食管 pH 值监测
  • 6 分钟步行试验
更多 需考虑的检查
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治疗流程

急症处理

初期表现

持续性治疗

急性加重：轻中度基础疾病

急性加重：重度基础疾病或对口服抗生素无反应/耐药

尽管进行了维持治疗，每年仍然有 3 次或更多次加重

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撰稿人

作者

Anne E. O'Donnell, MD

Professor of Medicine

Chief, Division of Pulmonary, Critical Care and Sleep Medicine

The Nehemiah and Naomi Cohen Chair in Pulmonary Disease Research

Georgetown University Medical Center

Washington

DC

利益声明

AEOD has received scientific consulting fees from the following companies: Bayer, Xellia, Horizon, Grifols, Insmed, and Electromed. AEOD is the Principal Investigator for trials sponsored by the following companies (research funding provided directly to Georgetown University): Bayer, Insmed, Aradigm, Parion, Zambon. AEOD has been a faculty member at the annual meetings of CHEST (American College of Chest Physicians), American Thoracic Society, and European Respiratory Society. AEOD is the author of a reference cited in this topic.

鸣谢

Dr Anne E. O'Donnell would like to gratefully acknowledge Dr Pamela J. McShane and Dr Sangeeta M. Bhorade, previous contributors to this topic.

利益声明

PJM and SMB declare that they have no competing interests.

同行评议者

Philip W. Ind, BA (Cantab), MB BChir, MA (Cantab), FRCP

Consultant Physician

Honorary Senior Lecturer

Imperial College Healthcare Trust

Hammersmith Hospital

London

UK

利益声明

PWI declares that he has no competing interests.

Ware Kuschner, MD

Associate Professor of Medicine

Stanford University

Stanford

Staff Physician

US Department of Veterans Affairs

Palo Alto Health Care System

Palo Alto

CA

利益声明

WK declares that he has no competing interests.

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