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Clin Respir J. 2014 Oct;8(4):425-30. doi: 10.1111/crj.12089. Epub 2014 Jan 10.

Primary ciliary dyskinesia complicated with diffuse panbronchiolitis: a case report and literature review.

Author information

1
Shanghai Institute of Respiratory Diseases, Zhongshan Hospital, Shanghai Medical College of Fudan University, Shanghai, China; Department of Respiratory Medicine, Huai'an First People's Hospital, Nanjing Medical University, Huai'an, China.

Abstract

BACKGROUND:

Fifty percent of patients with primary ciliary dyskinesia (PCD) have situs inversus. Diffuse panbronchiolitis (DPB) might be one of the characteristic features of the lung in PCD.

METHODS:

We reported a case of PCD without situs inversus, yet complicated with DPB, and did literature review.

RESULTS:

A 34-year-old nonsmoking Chinese woman with 6-year primary infertility suffered from recurrent episodes of respiratory tract infections since childhood. Lung auscultation revealed end-inspiratory coarse crackles. Pulmonary function tests demonstrated mild obstructive ventilation functional impairment. Lung biopsy showed respiratory bronchiolitis. Nasal mucosa cilia showed the absence of both outer and inner dynein arms of the microtubules. Saccharin test was positive. Chest images showed bronchiectasis and bronchiolitis but no situs inversus. Paranasal sinus computed tomography (CT) showed maxillary sinusitis and ethmoid sinusitis. A culture of bronchoalveolar lavage fluid was positive for Pseudomonas aeruginosa. Her conditions improved in clinical symptoms and CT images after 2 months of treatment with azithromycin. Literature review revealed that very rare patients were diagnosed as PCD complicated with diffuse DPB, and all of them had situs inversus.

CONCLUSIONS:

The association of DPB might be one of the characteristic features of the lung in PCD. Further studies on the concurrence of these two diseases are suggested so as to elucidate the mechanism of both.

KEYWORDS:

case report; clinical profiles; diffuse panbronchiolitis; literature review; primary ciliary dyskinesia

PMID:
24308375
PMCID:
PMC4237194
DOI:
10.1111/crj.12089
[Indexed for MEDLINE]
Free PMC Article

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