Sepe Angela, Villella Valeria R, Cimbalo Chiara, Castaldo Alice, Nunziata Francesco, Corcione Adele, Bona Gianni, Maiuri Luigi, Raia Valeria
Department of Translational Medical Sciences, University Federico II, Naples, Italy.
Department of Pediatrics, University Federico II, Naples, Italy.
Minerva Pediatr. 2019 Aug;71(4):371-375. doi: 10.23736/S0026-4946.19.05509-9. Epub 2019 Feb 13.
Structural lung disease begins very early in children with cystic fibrosis (CF), often in the first three months of life. Inhaled medications represent an attractive therapeutic approach in CF that are routinely used as early intervention strategies. Two aerosolized solutions, hypertonic saline and dornase alfa, have significant potential benefits by improving mucociliary clearance, with minimal associated side-effects. In particular, they favor rehydration of airway surface liquid and cleavage of extracellular DNA in the airways, respectively, consequently reducing rate of pulmonary disease exacerbations. Indirect anti-inflammatory effects have been documented for both drugs, addressing each of the three interrelated elements in the vicious cycle of lung disease in CF: airway obstruction, inflammation and infection. This short review aimed to summarize the main papers that support potential clinical impact of inhaled solutions on pulmonary disease in CF.
结构性肺病在患有囊性纤维化(CF)的儿童中很早就开始出现,通常在生命的头三个月。吸入药物是CF中一种有吸引力的治疗方法,常被用作早期干预策略。两种雾化溶液,高渗盐水和重组人脱氧核糖核酸酶,通过改善黏液纤毛清除功能具有显著的潜在益处,且相关副作用最小。特别是,它们分别有利于气道表面液体的再水化和气道中细胞外DNA的裂解,从而降低肺部疾病加重的发生率。这两种药物都有间接抗炎作用,针对CF肺部疾病恶性循环中的三个相互关联的因素:气道阻塞、炎症和感染。这篇简短的综述旨在总结支持吸入溶液对CF肺部疾病潜在临床影响的主要论文。