Abstract:

Objective    To better understand the spectrum of the disease and to explore the clinical significance of diagnostic procedures in clinical application.Method    The medical records of 349 children with diffuse parenchymal lung disease /interstitial lung disease admitted to Beijing Children’s hospital from July 2001 to September 2011 were studied retrospectively. The diagnostic approaches were applied，including the history ， physical examination ， non-invasive examination and invasive examination. Result    The 321 cases were made specific diagnosis through the diagnostic procedures and 28 cases were unclassified. The classification of the 349 cases were as follows:146 cases were  with primary lung diseases，14 cases were with lung disease secondary to systemic disease， 161 cases had the lung disease of  known causes， and the 28 cases were unclassified.The 160/349（45.85%） cases were diagnosed based on the noninvasive examination， including all 147 cases of bronchiolitis obliterans，3 cases of  hypersensitive pneumonitis，2 cases secondary to aspiration，2 cases of systemic lupus erythematosus， 2 cases of juvenile rheumatoid arthritis，1 case infected by CMV， 1 case of Langerhans cell histocytosis，1 case of undifferentiated connective tissue disease and 1 case of Wegener's granulomatosis. The others were diagnosed by invasive examination， including 2 cases of Langerhans cell histocytosis and 1 case of dermatomyositis by skin biopsies， 1 case of Langerhans cell histocytosis by lymphaden biopsy ，all 113 cases of idiopathic pulmonary hemosiderosis， 2 cases of eosinophilic pneumonia and 2 cases of hypersensitive pneumonitis by bronchoalveolar lavage fluid；40 cases were diagnosied by the lung biopsy， including 23 cases of idiopathic interstitial pneumonia. Conclusion    Diffuse parenchymal lung disease in children is a heterogeneous group of respiratory disorders . Bronchiolitis obliterans， idiopathic pulmonary hemosiderosis and idiopathic interstitial pneumonia are the most common disease in the diffuse parenchymal lung disease of the children. The procedural diagnosis method could make specific diagnosis for 321/349（91.98%） cases with parenchymal lung disease in this study. The 45.85% of whem are diagnosied by the noninvasive examination，and the other cases such as the idiopathic pulmonary hemosiderosis and the idiopathic interstitial pneumonia were confirmed by the invasive method.

摘要：

目的    探讨儿童弥漫性实质性肺疾病间质性肺疾病 （DPLD/ZRD）的分类和疾病谱，评价儿童DPLD/ILD的程序性诊断的临床应用价值。方法    回顾性分析北京儿童医院内科病房2001年7月至2011年9月收治的349例DPLD患儿的临床资料， 对其分类和程序性诊断方法进行总结和评估。结果    349例DPLD/ILD患儿最终特异性诊断321例（91.98%，321/349）321/349例（91.98%），未明确分型28例（8.02%），分类为：（1）原发性肺部疾病146例（41.84%）；（2）伴肺浸润的系统疾病14例（4.01%）；（3）已知原因的DPLD161例（46.13%）；（4）未明确分型DPLD 28例（8.02%）。非侵入性检查获得特异性诊断的有160/349例（45.85%），其中包括闭塞性细支气管炎（BO）147例、过敏性肺泡炎3例、吸入性因素致DPLD2例、系统性疾病的肺损害7例、CMV感染1例。侵入性检查获得特异性诊断的有161/349例（46.13 %），其中3例皮肤活检和1例淋巴结活检诊断的系统性疾病，支气管肺泡灌洗液细胞分析诊断的2例过敏性肺炎和2例嗜酸性肺炎。支气管肺泡灌洗液找到肺含铁血黄素细胞诊断的特发性肺含铁血黄素沉着症113例，肺组织病理确定诊断的间质性肺疾病40例，其中包括23例特发性间质性肺炎。结论    儿童DPLD是一组异质性疾病，疾病谱庞杂，儿童以BO、IPH、IIP为最常诊断的疾病。程序性诊断能明确本组大多数病例的诊断，非侵入性检查能使160/349例（45.85%）的病例得到特异性诊断，部分诊断不明161/349例（46.13 %）仍需侵入性检查而诊断，如IIP则需通过肺活检的组织病理检查进行诊断和分类。