Analysis of the clinical characteristics, long-term prognosis and changes of pulmonary function in children with idiopathic pulmonary fibrosis
10.3760/cma.j.cn101070-20200329-00524
- VernacularTitle:儿童特发性肺纤维化临床特征、远期预后及肺功能改变
- Author:
Zhuxia LI
1
;
Yiling TANG
;
Yinlan LEI
;
Jing TAN
;
Xinke DU
;
Chang SHU
Author Information
1. 重庆医科大学附属儿童医院呼吸中心，儿科学重庆重点实验室，儿童发育疾病研究教育部重点实验室，国家儿童健康与疾病临床医学研究中心，儿童发育重大疾病国家国际科技合作基地，重庆 400014
- Keywords:
Idiopathic pulmonary fibrosis;
Clinical characteristics;
Long-term prognosis;
Pulmonary function;
Child
- From:
Chinese Journal of Applied Clinical Pediatrics
2021;36(16):1240-1244
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical characteristics, long-term prognosis and changes of pulmonary function in children with idiopathic pulmonary fibrosis (IPF).Methods:The clinical data, long-term prognosis and changes of pulmonary function of children with IPF admitted to the Department of Pediatric Respiratory Center in Children′s Hospital of Chongqing Medical University from January 2008 to December 2018 were retrospectively analyzed.Results:A total of 28 cases were included, with the median age of 3.9 years (range: 0.5 to 15.7 years). Cough (28 cases, 100.0%), tachypnea (25 cases, 89.3%), cyanosis (19 cases, 67.9%), dyspnea (11 cases, 39.3%), Velcho rales (12 cases, 42.9%), inspiratory three concave sign (11 cases, 39.3%), clubbed fingers and toes (6 cases, 21.4%) and diminished breath sounds (5 cases, 17.9%) were main clinical manifestations.Chest high-resolution computed tomography (HRCT) mainly displayed grid shadow, irregular sac-like light-transparent shadow with ho-neycomb changes and dense shadow, partial (7 cases) pulmonary interstitial emphysema/emphysema/pneumomediastinum.Three 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.In this group, 4 cases did not receive drug therapy due to other reasons (3 cases abandoned therapy, and 1case died of respiratory and circulatory failure during hospitalization). Twenty-four cases were treated with single or combination of oral Prednisone, N-acetylcysteine and Azithromycin.Eleven cases had improved symptoms when discharged, and 13 cases showed no improvement.Twenty-four cases continued to receive oral medication therapy according to the original protocol.Eight cases were followed up for chest HRCT for 3 months to 4 years, the chest HRCT lesions of 7 cases were similar to before, and those of 1 case increased than before.All cases received telephone follow-up for 2 to 7 years; the maximum duration of medication was 4 years.Twelve cases were lost to follow-up, 7 cases had motion limitation, 3 cases died, and 2 cases had no clinical symptom.Three cases were followed up for pulmonary function for 2 to 3 years, among which 2 cases had pulmonary function decreased than before; 1 case had improvements in forced vital capacity as a percentage of the predicted value and peak expiratory flow as a percentage of the predicted value, but decline in forced expiratory volume in the first se-cond as a percentage of the predicted value.Conclusions:The clinical manifestations of children with IPF are lack of specificity.Chest HRCT is of great value in the diagnosis of IPF and preliminary monitoring of the activity of lesion.In the long-term follow-up, some of cases have improvements in symptoms; pulmonary function mostly decreases, but part of indexes can be improved.
