| Clinical characteristics of primary ciliary dyskinesia |
| Received:March 18, 2021 Revised:June 11, 2021 Click here to download the full text |
| Citation of this paper:BI Jing,LI Zhuo-zhe,ZHOU Lei,SONG Yuan-lin.Clinical characteristics of primary ciliary dyskinesia[J].Chinese Journal of Clinical Medicine,2021,28(3):348-352 |
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| Author Name | Affiliation | E-mail | | BI Jing | Department of Respiratory and Critial Care Medicine, Zhongshan Hospital, Fudan University, Shanghai Key Laboratory of Pulmonary Inflammation and Injury, Shanghai Institute of Respiratory Diseases, Shanghai 200032, China | | | LI Zhuo-zhe | Department of Respiratory and Critial Care Medicine, Zhongshan Hospital, Fudan University, Shanghai Key Laboratory of Pulmonary Inflammation and Injury, Shanghai Institute of Respiratory Diseases, Shanghai 200032, China | | | ZHOU Lei | Department of Respiratory Medicine, Qingpu Branch, Zhongshan Hospital, Fudan University, Shanghai 201700, China | | | SONG Yuan-lin | Department of Respiratory and Critial Care Medicine, Zhongshan Hospital, Fudan University, Shanghai Key Laboratory of Pulmonary Inflammation and Injury, Shanghai Institute of Respiratory Diseases, Shanghai 200032, China | song.yuanlin@zs-hospital.sh.cn |
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| Abstract:Objective: To explore the clinical characteristics of primary ciliary dyskinesia (PCD). Methods: 20 patients with PCD diagnosed in Zhongshan Hospital, Fudan University from January 2009 to December 2020 were retrospectively summarized. Their demographic data, family history, medical history, clinical symptoms, radiologic images, sputum culture, pulmonary function test, blood gas analysis, semen examination, and the electron microscopic findings of PCD were analyzed. Results: The mean onset age of the disease was (7.25±4.90) years, and the mean age at diagnosis was (25.15±9.80) years. The most common symptoms were recurrent cough (19/20) and expectoration (19/20) since childhood. Other symptoms included sinusitis (14/20) and otitis media (5/20). Eleven patients had situs inversus. All of the 20 patients had bronchiectasis on chest computed tomography (CT) scan, and most of the patients (18/20) had sinusitis on sinuses CT scan. Pulmonary function test indicated obstructive pattern was the most common disorder (8/14). The sperm motility was decreased in 5 cases. The most common structural abnormality of cilia under transmission electron microscopy was outer dynein arms deletion (5/9). Conclusions: PCD patients without situs inversus are easily missed, and PCD should be considered in patients with recurrent cough and expectoration since childhood, especially in patients with sinusitis and otitis media. Electron microscopic examination is still the main diagnostic method of PCD in China. |
| keywords:ciliary motility disorders bronchiectasis Kartagener syndrome |
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