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原发性纤毛运动障碍患者的替代惰性气体清除结果。

Alternative inert gas washout outcomes in patients with primary ciliary dyskinesia.

机构信息

Paediatric Respiratory Medicine, Dept of Paediatrics, Inselspital, Bern University Hospital, University of Bern, Switzerland.

Dept of Paediatric Pulmonology, University Children's Hospital Basel (UKBB), Basel, Switzerland.

出版信息

Eur Respir J. 2017 Jan 25;49(1). doi: 10.1183/13993003.00466-2016. Print 2017 Jan.

DOI:10.1183/13993003.00466-2016
PMID:28122863
Abstract

The lung clearance index (LCI) derived from a nitrogen multiple breath washout test (N-MBW) is a promising tool to assess small airways disease in primary ciliary dyskinesia, but it is difficult to apply in routine clinical settings because of its long measuring time. In this study, we aimed to assess alternative indices derived from shorter washout protocols.49 patients with primary ciliary dyskinesia (mean age 14.7±6.6 years) and 37 controls (mean age 14.3±1.4 years) performed N-MBW and double-tracer gas (DTG) single-breath washout tests. Global (LCI and moment ratio (M/M)), conductive (S) and acinar ventilation inhomogeneity (DTG Slope III (S-DTG)) were determined for each individual. The main outcomes were 1) the ability to detect abnormal lung function from washout indices (>1.64 z-scores) and 2) measurement duration.The prevalence of abnormal values for LCI was 37 out of 47 (79%), for LCI was 34 out of 47 (72%), for M/M was 34 out of 47 (72%), for S was 36 out of 46 (78%) and for S-DTG was 12 out of 35 (34%). Mean±sd duration of measurement was 19.8±11.2 min for LCI, 10.8±4.6 min for LCI and 8.6±2.3 min for SCompared to standard LCI, ventilation inhomogeneity was detected by LCI, moment ratio and S with comparable sensitivity while measurement duration was significantly shorter. Longitudinal studies will show which outcome is most suitable and practical in terms of sensitivity, duration and variability within the course of primary ciliary dyskinesia lung disease.

摘要

肺清除指数(LCI)来源于氮多呼吸冲洗试验(N-MBW),是评估原发性纤毛运动障碍中小气道疾病的一种很有前途的工具,但由于测量时间长,难以在常规临床环境中应用。本研究旨在评估来自较短冲洗方案的替代指数。49 例原发性纤毛运动障碍患者(平均年龄 14.7±6.6 岁)和 37 例对照者(平均年龄 14.3±1.4 岁)进行了 N-MBW 和双示踪气体(DTG)单次呼吸冲洗试验。对每个个体均测定了整体(LCI 和矩比(M/M))、传导性(S)和腺泡通气不均匀性(DTG 斜率 III(S-DTG))。主要结果是 1)从冲洗指数中检测异常肺功能的能力(>1.64 z 分数)和 2)测量时间。LCI 的异常值患病率为 47 例中的 37 例(79%),LCI 为 34 例中的 37 例(72%),M/M 为 34 例中的 37 例(72%),S 为 36 例中的 46 例(78%),S-DTG 为 35 例中的 12 例(34%)。LCI 的测量时间平均值±标准差为 19.8±11.2 分钟,LCI 和 S 的测量时间平均值±标准差分别为 10.8±4.6 分钟和 8.6±2.3 分钟。与标准 LCI 相比,LCI、矩比和 S 检测到通气不均匀性的敏感性相当,但测量时间明显缩短。纵向研究将表明在原发性纤毛运动障碍性肺病的过程中,哪种结果在敏感性、持续时间和变异性方面最适合和实用。

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