Bronchiectasis Assessment in Primary Ciliary Dyskinesia: A Non-Invasive Approach Using Forced Oscillation Technique.
Bronchiectasis Assessment in Primary Ciliary Dyskinesia: A Non-Invasive Approach Using Forced Oscillation Technique.
复制标题
原发性睫状运动障碍中的支气管扩张评估：一种使用强制振荡技术的非侵入性方法。
DOI：
10.3390/diagnostics13132287
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发表时间：
2023-07-06
期刊：
影响因子：
3.6
通讯作者：
Mosquera, Ricardo A.
中科院分区：
文献类型：
--
作者：
De Jesus-Rojas, Wilfredo;Reyes-Pena, Luis;Muniz-Hernandez, Jose;de Porras, Patricia Quiles Ruiz;Melendez-Montanez, Jesus;Ramos-Benitez, Marcos J.;Mosquera, Ricardo A.
关键词：
原发性纤毛运动障碍（PCD）是一种常染色体隐性遗传病，由运动性纤毛功能障碍引起，可引起慢性上呼吸道和下呼吸道感染，导致支气管扩张。然而，需要额外的工具来监测PCD中支气管扩张的进展。强迫振荡技术（FOT）是一种不依赖于努力的肺功能测试，可用于评估呼吸力学。在这项回顾性研究中，我们旨在描述6名成人PCD患者和1名患有RSPH4A （c.921+3_921+6delAAGT（含电子））突变的儿童患者的呼吸阻抗（阻力（Rrs）和电抗（Xrs））的影像学表现。我们比较了高分辨率胸部计算机断层扫描（CT）的x线表现和FOT结果。我们的研究结果表明，FOT测量的呼吸阻抗可能是检测和监测(RSPH4A （c.921+3_921+6delAAGT（内含子））突变的PCD患者支气管扩张进展的有价值的工具。然而，需要进一步的研究来验证这些结果，并确定其他基因突变的PCD患者支气管扩张监测的敏感性和特异性。
Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder that results from the dysfunction of motile cilia, which can cause chronic upper and lower respiratory infections leading to bronchiectasis. However, there is a need for additional tools to monitor the progression of bronchiectasis in PCD. The forced oscillation technique (FOT) is an effort-independent lung function test that can be used to evaluate respiratory mechanics. In this retrospective study, we aimed to describe the radiographic findings associated with respiratory impedance (resistance (Rrs) and reactance (Xrs)) measured by FOT in six adult PCD patients and one pediatric with the (RSPH4A (c.921+3_921+6delAAGT (intronic)) founder mutation. We compared the radiographic findings on a high-resolution chest computed tomography (CT) scan with the FOT results. Our findings suggest that respiratory impedance measured by FOT may be a valuable tool for detecting and monitoring the progression of bronchiectasis in PCD patients with the (RSPH4A (c.921+3_921+6delAAGT (intronic)) founder mutation. However, further research is necessary to validate these results and determine the sensitivity and specificity of bronchiectasis monitoring in PCD patients with other genetic mutations.
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DOI：
10.2147/copd.s173686
发表时间：
2018
影响因子：
2.8
作者：
Ribeiro CO;Faria ACD;Lopes AJ;de Melo PL
通讯作者：
de Melo PL
影响因子：
1.9
作者：
Taniuchi, Namiko;Hino, Mitsunori;Gemma, Akihiko
通讯作者：
Gemma, Akihiko
DOI：
10.1183/20734735.020514
发表时间：
2015-03
期刊：
Breathe (Sheffield, England)
影响因子：
--
作者：
Brashier B;Salvi S
通讯作者：
Salvi S
影响因子：
2.6
作者：
Mirra V;Werner C;Santamaria F
通讯作者：
Santamaria F
影响因子：
4.6
作者：
Yamamoto, Yuji;Miki, Keisuke;Kida, Hiroshi
通讯作者：
Kida, Hiroshi