儿童间质性肺疾病的分类和诊断程序

摘要

儿童间质性肺疾病是一组庞大而异质性的肺疾病,以弥漫性渗出和气体交换障碍为特征.儿童间质性肺疾病的分类主要为:婴儿特有的间质性肺疾病;原发于肺部的间质性肺疾病,包括特发性间质性肺炎;伴肺部浸润的系统性疾病;已知原因的疾病.儿童间质性肺疾病诊断应先根据病史、临床表现以及影像学检查,确定是否为间质性肺疾病;进一步通过病原学、血清学检查等寻找病因,如病因仍不明确则需通过侵袭性检查获得诊断和病理分型.%Interstitial lung disease (ILD) in infants and children comprises a large and heterogeneous group of respiratory disorders characterized by diffuse infiltrates and disordered gas exchange. The classification of the ILD in children are the following: disorders unique to infancy; primary lung disease, including idiopathic interstitial pneumonia; systemic disorders with pulmonary involvement; ILD of specific (known) cause. The first step in diagnosis approach is to confirm the ILD based on history, clinical presentation, and imaging examination. Further confirmatory causes can be identified by pathogenic and serological examination. If the causes are still not clear the invasive examination is needed to make the diagnosis and pathological type.