Doull Iolo
Department of Paediatric Respiratory Medicine and Paediatric Cystic Fibrosis Centre, Children's Hospital for Wales, Cardiff CF14 4XN, United Kingdom.
Paediatr Respir Rev. 2020 Sep;35:95-98. doi: 10.1016/j.prrv.2020.04.001. Epub 2020 Apr 9.
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.
囊性纤维化（CF）调节剂疗法的证据基础持续扩大，很可能多达90%的CF患者能从中受益。然而，在全球范围内，获得基本CF护理和调节剂疗法的机会存在显著不平等。对于婴幼儿，现在有吸入高渗盐水的证据基础。越来越多的证据表明，CF中的结构性肺病并非纯粹由感染引起，黏液潴留和炎症也是关键因素，并且阿奇霉素对长期感染铜绿假单胞菌的患者有价值的进一步证据。最后，运动对你有好处，但气道清除对黏液清除更有效。
