Clinicalanalysis Of Chlidren With Idiopathic Pulmonary Fibrosis And Long-term Follow-up Prognosis

Objective: To investigate clinical characteristics,long-term follow-up,and changes of lung function of children with idiopathic pulmonary fibrosis.Methods: Retrospective analysis of clinical data of children with IPF.Results: The median age of diagnosis was 3.9 years(range :0.5 to15.7years).Cough,cyanosis,dyspnea,shortness of breath,velcho sound,weak breath sounds,three concave sign and clubbed fingers are main clinical manifestations.Chest high-resolution computed tomography(HRCT)mainly displayed grid and consolidation shadow,irregular sac-like light-transparent images with honeycomb changes,partial pulmonary interstitial emphysema/emphysema/pneumomediastinum and mediastinal lymphadenectasis.3 cases of lung biopsy showed hyperplasia and consolidation of alveolar space and alveolar septal fibrosis,thickening of alveolar wall,and coexistence of new and old lesions.24 cases were treatedwith any one of or combination of glucocorticoid,N-acetylcysteine and azithromycin.3cases abandoned therapy and 1case die of respiratory and circulatory failure.24 cases continued to be treated according to the original protocol.After 2 to 9 years,24 cases all received telephone followed-up;the longest drug treatment time is 4 years.12 cases were lost to follow-up,7 cases still have exercise limitation,3 cases died,and 2 cases have no exercise limitation.The lung function of 3 cases were followed up for 2 ~3 years.Lung function of 2 cases decreased in the last time;1 case has improvements in FVC% and PFE%,but no improvements in FEV1%.Conclusion: The clinical symptoms of children with IPF are atypical.Chest HRCT is very important in the diagnosis and prognosis of IPF.In the long-term follow-up,some of them have improvements in symptoms;lung function mostly decrease,but a part of index improve.