遗传性肺间质疾病。
Genetic interstitial lung disease.
机构信息
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Eugene McDermott Center for Human Growth and Development, University of Texas Southwestern Medical Center, Dallas, TX 75390-8591, USA.
出版信息
Clin Chest Med. 2012 Mar;33(1):95-110. doi: 10.1016/j.ccm.2011.11.001. Epub 2011 Dec 6.
Abstract
The interstitial lung diseases (ILDs), or diffuse parenchymal lung diseases, are a heterogeneous collection of more than 100 different pulmonary disorders that affect the tissue and spaces surrounding the alveoli. Patients affected by ILD usually present with shortness of breath or cough; for many, there is evidence of pulmonary restriction, decreased diffusion capacity, and radiographic appearance of alveolar and/or reticulonodular infiltrates. This article reviews the inherited ILDs, with a focus on the diseases that may be seen by pulmonologists caring for adult patients. The authors conclude by briefly discussing the utility of genetic testing in this population.
摘要
间质性肺疾病（ILDs），或弥漫性实质性肺疾病，是一组超过 100 种不同的肺部疾病的统称，这些疾病影响肺泡周围的组织和空间。ILD 患者通常表现为呼吸急促或咳嗽；对许多人来说，有肺部限制、扩散能力下降以及肺泡和/或网状结节状浸润的放射学表现的证据。本文综述了遗传性 ILD，重点介绍了可能由治疗成人患者的肺病专家诊治的疾病。作者最后简要讨论了该人群中基因检测的实用性。
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