Rubbo Bruna, Kant Avni, Zhang Kewei, Allegorico Annalisa, Basilicata Simona, Boon Mieke, Borrelli Melissa, Calogero Claudia, Carr Siobhán B, Carroll Mary, Constant Carolina, Castillo Corullón Silvia, Corvol Harriet, Cutrera Renato, Dillenhöfer Stefanie, Emiralioglu Nagehan, Eralp Ela, Eryilmaz Polat Sanem, Gardner Laura, Gokdemir Yasemin, Harris Amanda, Hogg Claire, Karadag Bulent, Kobbernagel Helene, Koerner-Rettberg Cordula, Kouis Panayiotis, Lorent Natalie, Marcou Markella, Mathin June K, Martinu Vendula, Moreno-Galdó Antonio, Morgan Lucy, Nielsen Kim G, Omran Heymut, Ozcelik Ugur, Pohunek Petr, Raidt Johanna, Robinson Phil, Rovira-Amigo Sandra, Santamaria Francesca, Schlegtendal Anne, Tamalet Aline, Thouvenin Guillaume, Ullmann Nicola, Walker Woolf, Yiallouros Panayiotis, Kuehni Claudia E, Latzin Philipp, Beydon Nicole, Lucas Jane S
Clinical and Experimental Sciences, University of Southampton Faculty of Medicine, Southampton, UK.
Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.
ERJ Open Res. 2024 Sep 30;10(5). doi: 10.1183/23120541.00253-2024. eCollection 2024 Sep.
Respiratory pathogens are frequently isolated from airway samples in primary ciliary dyskinesia (PCD) patients. Few studies have investigated associations between these pathogens and lung function, with current management based on evidence from cystic fibrosis. We investigated the association between commonly isolated respiratory pathogens and lung function in PCD patients.
Using a cross-sectional design, we prospectively collected clinical and concurrent microbiology data from 408 participants with probable or confirmed PCD, aged ≥5 years, from 12 countries. We used Global Lung Function Initiative 2012 references to calculate forced expiratory volume in 1 s (FEV) z-scores. For 351 patients (86%) with complete data, we assessed the association of the four most frequently isolated pathogens with lung function by fitting multilevel linear models with country as random intercept, adjusted for age at diagnosis, age at lung function, use of antibiotic prophylaxis and body mass index z-scores.
Individuals with growth in culture had significantly lower FEV z-scores (β= -0.87, 95% CI -1.40- -0.34), adjusted for presence of , methicillin-sensitive and , and for covariates. When stratified by age, associations remained strong for adults but not for children. Results were similar when ciliary defects by transmission electron microscopy were included in the models and when restricting analysis to only confirmed PCD cases.
We found that was associated with worse lung function in individuals with PCD, particularly adults. These findings suggest that it is prudent to aim for eradication in the first instance, and to treat exacerbations promptly in colonised patients.
在原发性纤毛运动障碍（PCD）患者的气道样本中经常分离出呼吸道病原体。很少有研究调查这些病原体与肺功能之间的关联，目前的管理基于囊性纤维化的证据。我们调查了PCD患者中常见分离出的呼吸道病原体与肺功能之间的关联。
采用横断面设计，我们前瞻性地收集了来自12个国家的408名年龄≥5岁、可能或确诊为PCD的参与者的临床和同期微生物学数据。我们使用全球肺功能倡议2012年的参考值来计算1秒用力呼气量（FEV）z评分。对于351名（86%）有完整数据的患者，我们通过拟合以国家为随机截距的多级线性模型，评估了四种最常分离出的病原体与肺功能的关联，并对诊断时年龄、肺功能检测时年龄、抗生素预防的使用情况和体重指数z评分进行了调整。
经调整 、甲氧西林敏感 和 的存在情况以及协变量后，培养结果呈阳性的个体的FEV z评分显著更低（β = -0.87，95%可信区间 -1.40 - -0.34）。按年龄分层时，成人的关联仍然很强，而儿童则不然。当模型中纳入透射电子显微镜检测的纤毛缺陷情况以及将分析仅限于确诊的PCD病例时，结果相似。
我们发现 与PCD个体，尤其是成人的肺功能较差有关。这些发现表明，首先力求根除 是谨慎的做法，对于定植该病原体的患者应及时治疗病情加重情况。
