论文部分内容阅读
儿童间质性肺疾病是儿童呼吸系统的重要疾病，包括一大类疾病谱，发病率呈增加趋势，有较高的致死率，且10%的病例为家族性患病。可以为原发性肺部疾病，又可以是全身性系统性疾病的肺部表现，与基因突变和环境因素密切相关。本文就儿童间质性肺疾病的病因、病理、临床特征，诊治新进展作一综述。“,”Interstitial lung disease(ILD) in infants and children is a major disease of respiratory system with higher morbidity than before.It comprises a large spectrum of respiratory disorders that are associated with high mortality.It is an important disease threatening the lives of children and 10% of cases are familial disease. Either it is the primary lung disease , related to genetic mutations , and environmental factors are closely related , there may be a pulmonary manifestations of systemic disease. In this paper, the etiology , pathology, clinical features , diagnosis approaches, as wel as treatment progress of ILD are reviewed.