Bilton Diana, Stanford Gemma
aDepartment of Adult Cystic Fibrosis, Royal Brompton Hospital bNational Heart and Lung Institute, Imperial College cDepartment of Physiotherapy, Royal Brompton Hospital, London, UK.
Curr Opin Pulm Med. 2014 Nov;20(6):601-6. doi: 10.1097/MCP.0000000000000104.
This review examines the evidence for use of agents that improve sputum clearance in cystic fibrosis.
The basic defect in cystic fibrosis causes abnormal airway surface liquid leading to mucus plugging and subsequent infection and inflammation in the airways. Agents that improve clearance of secretions should improve lung function, reduce infective exacerbations and may enhance survival. There are two main varieties of mucoactive therapy, namely mucolytic therapy, which reduces viscosity in order to improve clearance, and hyperosmolar therapy, which increases the airway surface liquid volume and increases mucociliary clearance. The development of mannitol as a hyperosmolar agent in dry powder form has heightened the debate regarding the optimal approach to sputum clearance. The evidence for use of individual agents is discussed, as well as a practical approach to combining therapies.
Dornase alfa, a mucolytic agent, and hypertonic saline and mannitol, both hyperosmolar agents, have all been shown to benefit patients with cystic fibrosis. The optimal combination of therapies has not been identified, but a combination of a hyperosmolar agent combined with a mucolytic provides the most logical approach to improving lung function and reducing exacerbations.
本综述探讨了用于改善囊性纤维化患者痰液清除的药物的证据。
囊性纤维化的基本缺陷导致气道表面液体异常,进而导致黏液阻塞以及随后气道内的感染和炎症。改善分泌物清除的药物应能改善肺功能、减少感染性加重发作,并可能提高生存率。黏液活性治疗主要有两种类型,即黏液溶解疗法,通过降低黏稠度来改善清除;以及高渗疗法,通过增加气道表面液体量和提高黏液纤毛清除功能来实现。甘露醇作为干粉形式的高渗剂的研发加剧了关于痰液清除最佳方法的争论。文中讨论了使用单一药物的证据以及联合治疗的实用方法。
黏液溶解剂多纳培南、高渗剂高渗盐水和甘露醇均已被证明对囊性纤维化患者有益。尚未确定最佳的联合治疗方案,但高渗剂与黏液溶解剂联合使用为改善肺功能和减少病情加重提供了最合理的方法。