Waters Valerie, Stanojevic Sanja, Ratjen Felix
a Division of Infectious Diseases, Department of Pediatrics, Hospital for Sick Children , University of Toronto , Toronto , Canada.
b Division of Respiratory Medicine, Department of Pediatrics, Hospital for Sick Children , University of Toronto , Toronto , Canada.
Expert Rev Respir Med. 2016 Nov;10(11):1221-1228. doi: 10.1080/17476348.2017.1246963. Epub 2016 Oct 20.
Cystic fibrosis (CF) is a disease characterized by recurrent flares of respiratory symptoms, known as pulmonary exacerbations (PExs), which have a cumulative, detrimental effect on lung function decline and overall mortality. Although much research has been done on the effects of PExs in adults with CF, considerably less is known about these events in young children with CF. Areas covered: This review describes the typical presentation of PExs in children and their impact on long-term clinical outcomes. Traditional and new monitoring techniques, such as pulmonary function testing using multiple breath washout, radiographic modalities and microbiological screening methods are reviewed. Finally, the choice, administration and duration of antimicrobial treatment as well as the potential use of antiviral therapy is discussed. Expert commentary: Although it is now well recognized that a significant proportion of patients do not recover their lung function following PExs, to date, little progress has been made to improve outcomes in this group. Additional therapies, to complement antimicrobials, may be required to treat infection and inflammation during PExs. Trials of anti-inflammatories such as corticosteroids or other novel drugs need to be done in the setting of PExs with the goal of complete lung function recovery for all individuals with CF.
囊性纤维化（CF）是一种以呼吸道症状反复发作为特征的疾病，这些发作被称为肺部加重（PExs），对肺功能下降和总体死亡率具有累积性的有害影响。尽管已经对PExs对成年CF患者的影响进行了大量研究，但对于患有CF的幼儿中的这些情况了解得要少得多。涵盖领域：本综述描述了儿童PExs的典型表现及其对长期临床结局的影响。对传统和新的监测技术进行了综述，例如使用多次呼气洗脱的肺功能测试、影像学检查方法和微生物筛查方法。最后，讨论了抗菌治疗的选择、给药方式和持续时间以及抗病毒治疗的潜在用途。专家评论：尽管现在人们已经充分认识到相当一部分患者在PExs后肺功能无法恢复，但迄今为止，在改善该群体的结局方面进展甚微。可能需要补充抗菌药物的其他疗法来治疗PExs期间的感染和炎症。需要在PExs的背景下进行诸如皮质类固醇或其他新药等抗炎药的试验，目标是让所有CF患者的肺功能完全恢复。
