The Clinical Study For Etiology Of87Cases Diffuse Parenchynal Lung Disease/Interstitial Lung Disease In Children

OBJECTIVE:To explore children’s diffuse parenchymal/interstitial lung disease cause of lung disease classification, evaluation of DPLD/ILD procedural diagnostic value of clinical application, and to improve the diagnosis and treatment level of children DPLD/ILD.METHODS:87patients from the first affiliated hospital of guangxi medical university with diffuse parenchymal lung disease/interstitial lung disease In March2009to February2014were included in the study,comply with the set of conditions (chest radiograph of bilateral lung field widely, multiple interstitial or substantial lesions). Reference to put forward by the national children DPLD/ILD cooperation to make the diagnosis, etiology diagnosis program into the clinical characteristics of the groups of cases, lung CT or high resolution CT (HRCT), part of bronchoalveolar lavage fluid (BALF), genetic analysis and pathology were retrospectively analyzed. RESULTS:In strict accordance with diagnostic procedures of the causes for DPLD/ILD raised by pediatric interstitial lung disease cooperative group, This study of87cases of children with DPLD/ILD, etiology diagnosis program by all cases into the group until the end of follow-up, the definite causes were made in79cases,6cases had suspected diagnosis,2case of unknown reason, the causes of pediatric DPLD/ILD in this study were:38cases with diffuse alveolar hemorrhage(DAH),9cases with inborn errors of surfactant metabolism (IESM),8cases with occlusive bronchiolitis obliterans (BO),7cases of diffuse bronchiolitis (DPB),9cases with Idiopathic interstitial pneumonia(IIP),(including5cases with nonspecific interstitial pneumonia (NSIP),1case with acute interstitial pneumonia),3cases with extrinsic allergic alveolitis (EAA),2cases with pulmonary alveolar proteinosis (PAP),2cases with Juvenile rheumatoid arthritis-interstitial lung diseases (JRA-ILD),2cases with Congenital pulmonary vascular dysplasia (CPVD),1case with chorinic eosinophils pneumonia(CEP),2case with diffuse pulmonary lymphangiomatosis (DPL),1case with sarcoidosis,1case with systemic sclerosis (SSc),2case of unknown reason.2.5cases combined with diagnostic IESM by genetic testing.3.10cases were made diagnosed with Clinical radiation-pathology.CONCLUSION:1. The top third causes for children with DPLD/ILD in this group were DAH, IESM, BO.2.The diagnosis of the children DPLD/ILD should be combined with the patient’s medical history, symptoms, physical signs, pulmonary function, HRCT, and so on, can be carried out when necessary genes, bronchoscopy or lung biopsy invasive examination, in order to make clear diagnosis.3. the cause of children DPLD/ILD diagnostic program can clear the basic group of the cause of most of the children.