Nathan Anna Marie, de Bruyne Jessie Anne, Eg Kah Peng, Thavagnanam Surendran
Department of Pediatrics, University of Malaya, Kuala Lumpur, Malaysia.
University Malaya Pediatric and Child Health Research Group, University of Malaya, Kuala Lumpur, Malaysia.
Front Pediatr. 2017 Apr 24;5:84. doi: 10.3389/fped.2017.00084. eCollection 2017.
Non-cystic fibrosis bronchiectasis (NCFB) has gained renewed interest, due to its increasing health-care burden. Annual mortality statistics in England and Wales showed that under 1,000 people die from bronchiectasis each year, and this number is increasing by 3% yearly. Unfortunately, there is a severe lack of well-powered, randomized controlled trials to guide clinicians how to manage NCFB effectively. Quality-of-life (QOL) measures in NCFB are an important aspect of clinical care that has not been studied well. Commonly used disease-specific questionnaires in children with NCFB are the St George's Respiratory Questionnaire, Short Form-36, the Leicester Cough Questionnaire, and the Parent Cough-Specific Quality of Life questionnaire (PC-QOL). Of these, only the PC-QOL can be used in young children, as it is a parent-proxy questionnaire. We reviewed pediatric studies looking at QOL in children with NCFB and cystic fibrosis. All types of airway clearance techniques appear to be safe and have no significant benefit over each other. Number of exacerbations and hospitalizations correlated with QOL scores, while symptom subscales correlated with lung function, worse QOL, frequent antibiotic requirements, and duration of regular follow-up in only one study. There was a correlation between QOL and age of diagnosis in children with primary ciliary dyskinesia. Other studies have shown no relationship between QOL scores and etiology of NCFB as well as CT changes. As for treatments, oral azithromycin and yoga have demonstrated some improvement in QOL scores. In conclusion, more studies are required to accurately determine important factors contributing to QOL.
非囊性纤维化支气管扩张症（NCFB）因其日益加重的医疗负担而重新引起了人们的关注。英格兰和威尔士的年度死亡率统计显示，每年有不到1000人死于支气管扩张症，且这一数字每年以3%的速度增长。不幸的是，严重缺乏有力的随机对照试验来指导临床医生如何有效管理NCFB。NCFB的生活质量（QOL）测量是临床护理中一个尚未得到充分研究的重要方面。在患有NCFB的儿童中常用的特定疾病问卷有圣乔治呼吸问卷、简明健康状况调查量表（SF-36）、莱斯特咳嗽问卷以及家长咳嗽特异性生活质量问卷（PC-QOL）。其中，只有PC-QOL可用于幼儿，因为它是一份家长代填问卷。我们回顾了关于NCFB和囊性纤维化患儿生活质量的儿科研究。所有类型的气道清除技术似乎都是安全的，且彼此之间没有显著差异。发作次数和住院次数与生活质量评分相关，而症状子量表仅在一项研究中与肺功能、较差的生活质量、频繁的抗生素需求以及定期随访时间相关。原发性纤毛运动障碍患儿的生活质量与诊断年龄之间存在相关性。其他研究表明，生活质量评分与NCFB的病因以及CT改变之间没有关系。至于治疗方法，口服阿奇霉素和瑜伽已显示出生活质量评分有所改善。总之，需要更多的研究来准确确定影响生活质量的重要因素。
