Cystic fibrosis 2019: Year in review

被引:6
|
作者
Doull, Iolo [1 ,2 ]
机构
[1] Childrens Hosp Wales, Dept Paediat Resp Med, Cardiff CF14 4XN, Wales
[2] Childrens Hosp Wales, Paediat Cyst Fibrosis Ctr, Cardiff CF14 4XN, Wales
关键词
Cystic fibrosis; Elexacaftor; Hypertonic saline; Azithromycin; INHALED HYPERTONIC SALINE; TEZACAFTOR-IVACAFTOR; PSEUDOMONAS-AERUGINOSA; AZITHROMYCIN; LUMACAFTOR; TRIAL;
D O I
10.1016/j.prrv.2020.04.001
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
The evidence base for modulator therapies in cystic fibrosis (CF) has continued to expand, and it is likely that up to 90% of people with CF could benefit. Worldwide there are however marked inequalities of access to basic CF care and modulator therapies. For infants and young children there is now an evidence base for inhaled hypertonic saline. There is increasing evidence that structural lung disease in CF is not due purely to infection and that mucus retention and inflammation are also key, and further evidence of the value of azithromycin in those chronically infected with Pseudomonas aeruginosa. Finally, exercise is good for you, but airway clearance is better for mucus clearance. (C) 2020 Elsevier Ltd. All rights reserved.
引用
收藏
页码:95 / 98
页数:4
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