支气管扩张症

最后审阅: 3 一月 2023
最后更新: 14 十月 2022

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
  • 早产
更多 危险因素

诊断性检查

首要检查

  • 高分辨率胸部 CT 扫描
  • 胸部 X 线检查(CXR)
  • 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

  • 原发性纤毛不动综合征(PCD)检查
  • 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
  • 结核病检查
  • 诊断性支气管镜检查及支气管肺泡灌洗
更多 需考虑的检查

治疗流程

初步治疗

初期表现

急症处理

急性加重:轻中度基础疾病

急性加重:重度基础疾病或对初始抗生素治疗无应答/耐药

尽管进行了维持治疗,每年仍然有 3 次或更多次加重

持续性治疗

门诊检查时首次或新分离出铜绿假单胞菌

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), the American Thoracic Society, and the 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

Emeritus Professor of Practice (Respiratory Medicine)

National Heart and Lung Institute

Imperial College London

London

UK

利益声明

PWI declares that he has no competing interests.

Meg Coleman, MBBS, BSc, MRCP

Respiratory Consultant

Honorary Senior Clinical Lecturer

Imperial College Hospitals NHS Trust and National Heart and Lung Institute

London

UK

利益声明

MC has received honoraria from Pfizer and Gilead for delivering educational sessions.

Amanda Messinger, MD, MS

Assistant Professor

Section of Pediatric Pulmonary and Sleep Medicine

Department of Pediatrics

Children's Hospital Colorado

University of Colorado Denver School of Medicine

Aurora

CO

利益声明

AM declares that she 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.

  • 支气管扩张症 images
  • 鉴别诊断

    • 慢性阻塞性肺疾病 (COPD)
    • 哮喘
    • 肺炎
    更多 鉴别诊断
  • 指南

    • 欧洲呼吸学会儿童和青少年支气管扩张管理指南
    • 英国胸科协会关于在呼吸系统疾病成人患者中长期使用大环内酯类药物的指南
    更多 指南
  • 患者教育信息

    何为支气管扩张?

    支气管扩张:如何有效治疗?

    更多 患者教育信息
  • padlock-locked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