Progress in the treatment of idiopathic pulmonary fibrosis in children
10.3760/cma.j.issn.1673-4912.2014.11.016
- VernacularTitle:儿童特发性肺纤维化治疗进展
- Author:
Xiaoni ZHANG
- Publication Type:Journal Article
- Keywords:
Pulmonary fibrosis;
Idiopathic;
Treatment;
Children
- From:
Chinese Pediatric Emergency Medicine
2014;21(11):728-731
- CountryChina
- Language:Chinese
-
Abstract:
Idiopathic pulmonary fibrosis (IPF)is a chronic progressive interstitial lung disease.The prevalence of IPF is increasing year by year,and the diagnostic process is complex,the mortality rate is more than 50％ in children.It has been demonstrated by multicenter,randomized,placebo-controlled trials that most of the clinical assays performed with corticosteroids,immunomodulators and antifibrotic drugs have shown inconclusive results for the treatment of IPF.To date,only pirfenidone has been proved to have significant efficacy in controlled clinical trials in the treatment of IPF.There has been an exponential increase in the number of compounds entering clinical trials for IPF in the last decade.The therapeutic use of new stem cell translantation will have a broad prospect in IPF.
