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Abstract：
OBJECTIVE: This study explored the clinical features and immune responses of children with primary ciliary dyskinesia (PCD) during pneumonia episodes.
METHODS: The 61 children with PCD who were admitted to hospital because of pneumonia were retrospectively enrolled into this study between April 2017 and August 2022. A total of 61 children with pneumonia but without chronic diseases were enrolled as the control group. The clinical characteristics, levels of inflammatory indicators, pathogens, and imaging features of the lungs were compared between the two groups.
RESULTS: The PCD group had higher levels of lymphocytes (42.80% versus 36.00%, p = 0.029) and eosinophils (2.40% versus 1.25%, p = 0.020), but lower neutrophil counts (3.99 versus 5.75 × 109/L, p = 0.011), percentages of neutrophils (46.39% versus 54.24%, p = 0.014), CRP (0.40 versus 4.20 mg/L, p < 0.001) and fibrinogen (257.50 versus 338.00 mg/dL, p = 0.010) levels. Children with PCD and children without chronic diseases were both most commonly infected with Mycoplasma pneumoniae (24.6% versus 51.9%). Children with PCD had significantly more common imaging features, including mucous plugging (p = 0.042), emphysema (p = 0.007), bronchiectasis (p < 0.001), mosaic attenuation (p = 0.012), interstitial inflammation (p = 0.015), and sinusitis (p < 0.001).
CONCLUSIONS: PCD is linked to immune system impairment, which significantly contributes to our understanding of the pathophysiology of this entity.
METHODS: The 61 children with PCD who were admitted to hospital because of pneumonia were retrospectively enrolled into this study between April 2017 and August 2022. A total of 61 children with pneumonia but without chronic diseases were enrolled as the control group. The clinical characteristics, levels of inflammatory indicators, pathogens, and imaging features of the lungs were compared between the two groups.
RESULTS: The PCD group had higher levels of lymphocytes (42.80% versus 36.00%, p = 0.029) and eosinophils (2.40% versus 1.25%, p = 0.020), but lower neutrophil counts (3.99 versus 5.75 × 109/L, p = 0.011), percentages of neutrophils (46.39% versus 54.24%, p = 0.014), CRP (0.40 versus 4.20 mg/L, p < 0.001) and fibrinogen (257.50 versus 338.00 mg/dL, p = 0.010) levels. Children with PCD and children without chronic diseases were both most commonly infected with Mycoplasma pneumoniae (24.6% versus 51.9%). Children with PCD had significantly more common imaging features, including mucous plugging (p = 0.042), emphysema (p = 0.007), bronchiectasis (p < 0.001), mosaic attenuation (p = 0.012), interstitial inflammation (p = 0.015), and sinusitis (p < 0.001).
CONCLUSIONS: PCD is linked to immune system impairment, which significantly contributes to our understanding of the pathophysiology of this entity.
摘要：
目的：本研究探讨了肺炎发作期原发性纤毛运动障碍（PCD）患儿的临床特征和免疫反应。
方法：在2017年4月至2022年8月期间，对61例因肺炎入院的PCD患儿进行回顾性研究。共纳入61例肺炎但无慢性疾病患儿作为对照组。临床特点,炎症指标的水平，病原体,比较两组肺部影像学特征。
结果：PCD组淋巴细胞水平较高（42.80％对36.00％，p=0.029)和嗜酸性粒细胞(2.40%对1.25%，p=0.020),但中性粒细胞计数较低（3.99对5.75×109/L，p=0.011),中性粒细胞百分比(46.39%对54.24%，p=0.014),CRP（0.40对4.20mg/L，p<0.001）和纤维蛋白原（257.50对338.00mg/dL，p=0.010)水平。PCD患儿和无慢性疾病患儿均最常感染肺炎支原体(24.6%对51.9%)。儿童PCD有明显更常见的影像学特征，包括粘液堵塞（p=0.042），肺气肿（p=0.007），支气管扩张（p<0.001），马赛克衰减（p=0.012），间质性炎症（p=0.015），和鼻窦炎（p<0.001）。
结论：PCD与免疫系统损伤有关，这极大地有助于我们对该实体的病理生理学的理解。
方法：在2017年4月至2022年8月期间，对61例因肺炎入院的PCD患儿进行回顾性研究。共纳入61例肺炎但无慢性疾病患儿作为对照组。临床特点,炎症指标的水平，病原体,比较两组肺部影像学特征。
结果：PCD组淋巴细胞水平较高（42.80％对36.00％，p=0.029)和嗜酸性粒细胞(2.40%对1.25%，p=0.020),但中性粒细胞计数较低（3.99对5.75×109/L，p=0.011),中性粒细胞百分比(46.39%对54.24%，p=0.014),CRP（0.40对4.20mg/L，p<0.001）和纤维蛋白原（257.50对338.00mg/dL，p=0.010)水平。PCD患儿和无慢性疾病患儿均最常感染肺炎支原体(24.6%对51.9%)。儿童PCD有明显更常见的影像学特征，包括粘液堵塞（p=0.042），肺气肿（p=0.007），支气管扩张（p<0.001），马赛克衰减（p=0.012），间质性炎症（p=0.015），和鼻窦炎（p<0.001）。
结论：PCD与免疫系统损伤有关，这极大地有助于我们对该实体的病理生理学的理解。
