目的　探讨间质性肺炎病理类型及临床表现。方法　回顾性分析70例经尸体检验确诊为间质性肺炎的患儿的临床及病理资料。结果　男性多于女性，多数为<2岁的婴幼儿；发病急，病情变化快，病程短，临床表现缺乏特异性；常规治疗效果不佳，预后差；12例病因可疑；58例病因不明者病理分型：弥漫性肺泡损伤(DAD)型38例，脱屑性间质性肺炎(DIP)型5例，淋巴细胞性间质性肺炎(LIP)型3例，DAD型合并DIP型 6例，DAD型合并LIP型 2例，DAD型合并呼吸性细支气管炎相关性间质性肺疾病(RB-ILD)型3例，DIP型合并LIP型 1例。结论　儿童间质性肺炎确诊率低；尸体检验有助于明确诊断，为进一步完善病理分型提供参考。

Objective　To better understand pathological types of pediatric interstitial pneumonia and improve clinical diagnosis by analyzing the clinical records and pathological typing of interstitial pneumonia. Methods 70 cases of children diagnosed as interstitial pneumonia by autopsy were retrospectively analyzed. Results The number of males was more than that of females. There was a significant predominance of infants less than 2 years. The clinical features include acute onset, rapid development, short duration and atypical clinical manifestations. Most patients had poor prognosis and curative effect with general therapies. Twelve cases had dubious etiology. Pathologic types of 58 cases with unclear etiology were diffuse alveolar damage type (DAD type, 38/58), desquamative interstitial pneumonia type (DIP type, 5/58), lymphoid interstitial pneumonia type (LIP type, 3/58), DAD type complicating DIP type (6/58), DAD type complicating LIP type (2/58), DAD type complicating respiratory bronchiolotitis-associated interstitial lung disease type (RB-ILD type, 3/58), DIP type complicating LIP type (1/58). Conclusions The confirmed diagnosis rate was relatively low for pediatric interstitial pneumonia. Postmortem examination was helpful for diagnosis and improving clinical diagnosis and pathological typing.