John Hopkins Aramco Healthcare, Dhahran, Saudi Arabia.
University of Southampton, Southampton, United Kingdom.
Pediatr Pulmonol. 2023 Feb;58(2):475-483. doi: 10.1002/ppul.26213. Epub 2022 Nov 2.
Primary ciliary dyskinesia is a rare genetic disorder characterized by recurrent sinopulmonary infections and worsening obstructive lung disease. Kidney and brain involvement is less common and is associated with overlapping ciliopathies/syndromes. The lungs are impacted early in the course of the disease, so it is vital to monitor lung function and recognize any decline by doing appropriate lung function tests. This systematic review compares different lung function tests and analyzes which one becomes abnormal earlier in the disease.
A systematic review was conducted following the methodology in the "Cochrane Handbook on Systematic Reviews for diagnostic tests." The Preferred Reporting Items for Systematic Review and Meta-Analyses were used to report the results. The risk of bias assessment was done using "The Cochrane Handbook for Systematic Reviews tool for interventional studies." A meta-analysis was not performed due to the small sample size. All studies were analyzed by using Joanna Briggs Institute's critical appraisal tool.
After screening for the duplication of results and applying inclusion and exclusion criteria, 14 studies were assessed by reading the full texts. Out of these, eight were finally included in this systematic review. The total sample size from all studies was 165, including 80 males. All the studies used spirometry as a lung function test, whereas multiple breath washout was used in five studies. Other tests used for comparison were computed tomography (CT), magnetic resonance imaging (MRI), cardiopulmonary exercise testing, 6-min walk test, DLCO, maximal inspiratory pressure, maximal expiratory pressure, and PaO . Lung clearance index (LCI) by multiple breath washout had a stronger association with the structural changes on CT/MRI than spirometry indices like forced expiratory volume in 1 s (FEV1) and forced expiratory flow at 25% to 75% of lung volume (FEF 25-75).
Based on the evidence from this systematic review, LCI becomes abnormal earlier than FEV1 or FEF 25-75 and positively correlates with the findings on high-resolution CT. It has limitations like the lack of reference values and a complex technique to perform the test.
原发性纤毛运动障碍是一种罕见的遗传性疾病，其特征是反复发生的鼻窦和肺部感染以及逐渐恶化的阻塞性肺部疾病。肾脏和脑部受累的情况较为少见，并且与重叠的纤毛病/综合征相关。疾病早期就会对肺部造成影响，因此监测肺部功能并通过适当的肺功能测试识别任何功能下降至关重要。本系统评价比较了不同的肺功能测试，并分析了哪种测试在疾病早期较早出现异常。
按照《Cochrane 诊断测试系统评价手册》中的方法进行了系统评价。采用《系统评价和荟萃分析的 Preferred Reporting Items》来报告结果。使用“Cochrane 干预性研究系统评价工具”对偏倚风险进行评估。由于样本量较小，未进行荟萃分析。所有研究均使用 Joanna Briggs 研究所的批判性评估工具进行分析。
在对结果进行重复筛查并应用纳入和排除标准后，通过阅读全文评估了 14 项研究。其中，8 项最终被纳入本系统评价。所有研究的总样本量为 165 例，包括 80 名男性。所有研究均使用肺活量测定法作为肺功能测试，而 5 项研究则使用了多次呼吸冲洗法。用于比较的其他测试包括计算机断层扫描（CT）、磁共振成像（MRI）、心肺运动测试、6 分钟步行测试、DLCO、最大吸气压力、最大呼气压力和 PaO 。与肺活量测定法的指标（如 1 秒用力呼气量（FEV1）和用力呼出 25%至 75%肺活量时的流量（FEF 25-75））相比，多次呼吸冲洗法的肺清除指数（LCI）与 CT/MRI 的结构变化具有更强的相关性。
基于本系统评价的证据，LCI 比 FEV1 或 FEF 25-75 更早出现异常，并且与高分辨率 CT 的发现呈正相关。它具有一些局限性，例如缺乏参考值和测试技术复杂。
