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. 2023 Oct 24;10(11):1727.
doi: 10.3390/children10111727.

Clinical Characteristics and Immune Responses in Children with Primary Ciliary Dyskinesia during Pneumonia Episodes: A Case-Control Study

Danli Lu  1   2   3   4   5 , Wenhao Yang  1   2   3   4   5 , Rui Zhang  1 , Yan Li  1 , Tianyu Cheng  1   2   3   4   5 , Yue Liao  1 , Lina Chen  1   2   3   4   5 , Hanmin Liu  1   2   3   4   5
Affiliations

Clinical Characteristics and Immune Responses in Children with Primary Ciliary Dyskinesia during Pneumonia Episodes: A Case-Control Study

Danli Lu et al. Children (Basel). .

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).

Conclusion: PCD is linked to immune system impairment, which significantly contributes to our understanding of the pathophysiology of this entity.

Keywords: imaging examination; immunology; inflammation; pathogen; pneumonia; primary ciliary dyskinesia.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Flowchart of patient enrollment.
Figure 2
Figure 2
TEM images of the ultrastructure of the ciliary axonemes from a normal child and patients with PCD: (a) Normal ciliary axonemes in a healthy individual, indicated by the green arrow. (b) The lack of ODA and IDA in the patient, indicated by the red arrows. (c) The lack of CP in the patient, indicated by the red arrows.
Figure 3
Figure 3
Imaging features in the two groups. * p < 0.05.
Figure 4
Figure 4
Lobe involvement in the two groups. * p < 0.05.
Figure 5
Figure 5
Comparison CT scan of the two groups and typical imaging features of PCD. (a) CT shows patchy pulmonary shadows in a control-group patient, especially in the left lobes. (b) Chest CT of a patient with PCD shows the bronchiectasis and compensated emphysema; the red arrow points to signs of pulmonary emphysema. (c) Chest CT of a patient with PCD shows interstitial inflammation. (d) Chest CT shows mosaic attenuation in a patient with PCD. (e) Nasopharynx CT shows sinusitis in a control-group patient. (f) Nasopharynx CT shows sinusitis in a patient with PCD. The red arrow in (e,f) points to signs of nasosinusitis.
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