背景:肺加重(PEx)在囊性纤维化(CF)患者中具有重要的临床意义。PEx使用了多个定义,本范围审查旨在确定文献中报道的不同定义,并确定哪些体征和症状通常用于定义它们。
方法:使用Embase进行搜索,MEDLINE,科克伦图书馆,Scopus和CINAHL.纳入1990年1月至2022年12月以英文发表的所有报告涉及CF患者PEx定义的临床试验或前瞻性观察性研究的出版物。然后提取数据进行定性主题分析。
结果:共确定了14039条记录,一旦重复被删除,就会筛选7647个标题和摘要,898份全文审查,377份符合纳入标准。148种出版物中使用了现有的定义。在75%的论文中,使用了客观定义,25%的人使用主观定义,细分为基于治疗的定义(76%)和涉及临床医生判断的定义(24%)。客观定义分为三组:基于体征和症状的组合(50%),这些基于预定义的体征和症状组合以及急性治疗的开始(47%)和涉及不同临床特征的评分,每个评分都有特定的权重(3%).定义中报告的最常见的体征和症状是,按顺序,痰液生产,咳嗽,肺功能,体重/食欲,呼吸困难,胸部X光改变,胸部的声音,发烧,疲劳或嗜睡和咯血。
结论:我们在文献报道的CF患者中发现了PEx定义的实质性差异。需要发展国际商定的,标准化和验证的特定年龄定义。这样的定义将允许进行研究和有效的荟萃分析之间的比较,并且在CF护理中的高效调节剂治疗时代尤其重要。
BACKGROUND: Pulmonary exacerbations (PExs) are clinically important in people with cystic fibrosis (CF). Multiple definitions have been used for PEx, and this scoping review aimed to identify the different definitions reported in the literature and to ascertain which signs and symptoms are commonly used to define them.
METHODS: A search was performed using Embase, MEDLINE, Cochrane Library, Scopus and CINAHL. All publications reporting clinical trials or prospective observational studies involving definitions of PEx in people with CF published in English from January 1990 to December 2022 were included. Data were then extracted for qualitative thematic analysis.
RESULTS: A total of 14 039 records were identified, with 7647 titles and abstracts screened once duplicates were removed, 898 reviewed as full text and 377 meeting the inclusion criteria. Pre-existing definitions were used in 148 publications. In 75% of papers, an objective definition was used, while 25% used a subjective definition, which subcategorised into treatment-based definitions (76%) and those involving clinician judgement (24%). Objective definitions were subcategorised into three groups: those based on a combination of signs and symptoms (50%), those based on a predefined combination of signs and symptoms plus the initiation of acute treatment (47%) and scores involving different clinical features each with a specific weighting (3%). The most common signs and symptoms reported in the definitions were, in order, sputum production, cough, lung function, weight/appetite, dyspnoea, chest X-ray changes, chest sounds, fever, fatigue or lethargy and haemoptysis.
CONCLUSIONS: We have identified substantial variation in the definitions of PEx in people with CF reported in the literature. There is a requirement for the development of internationally agreed-upon, standardised and validated age-specific definitions. Such definitions would allow comparison between studies and effective meta-analysis to be performed and are especially important in the highly effective modulator therapy era in CF care.