Chen Wei, Shao Changzhou, Song Yuanlin, Bai Chunxue
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.
Clin Respir J. 2014 Oct;8(4):425-30. doi: 10.1111/crj.12089. Epub 2014 Jan 10.
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.
We reported a case of PCD without situs inversus, yet complicated with DPB, and did literature review.
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.
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.
原发性纤毛运动障碍（PCD）患者中有50%存在内脏反位。弥漫性泛细支气管炎（DPB）可能是PCD肺部的特征性表现之一。
我们报告了1例无内脏反位但合并DPB的PCD病例，并进行了文献复习。
一名34岁不吸烟的中国女性，有6年原发性不孕史，自幼反复出现呼吸道感染。肺部听诊可闻及吸气末粗湿啰音。肺功能检查显示轻度阻塞性通气功能障碍。肺活检显示呼吸性细支气管炎。鼻黏膜纤毛显示微管内外动力蛋白臂均缺失。糖精试验阳性。胸部影像显示支气管扩张和细支气管炎，但无内脏反位。鼻窦计算机断层扫描（CT）显示上颌窦炎和筛窦炎。支气管肺泡灌洗 fluid培养铜绿假单胞菌阳性。阿奇霉素治疗2个月后，其临床症状和CT影像有所改善。文献复习显示，极罕见患者被诊断为PCD合并弥漫性DPB，且所有患者均有内脏反位。
DPB的关联可能是PCD肺部的特征性表现之一。建议对这两种疾病的并发情况进行进一步研究，以阐明两者的机制。
