弥漫性泛细支气管炎一例及文献综述

Diffuse panbronchiolitis in a child:case report and literature review

目的探讨儿童弥漫性泛细支气管炎的诊断和治疗。方法总结1例弥漫性泛细支气管炎患儿的临床、影像学表现以及病理改变,并复习相关文献。结果患儿临床表现为长期咳嗽、咯痰伴喘息,逐渐出现呼吸困难。双肺闻及较多细湿哕音及喘鸣音,杵状指可疑。胸部 X 线片:两肺透光度增强,左下肺见多发结节及网状影。胸部高分辨 CT:两肺广泛分布小叶中心性细小结节影,左下肺局部小支气管壁增厚,双下叶和右中叶少许支气管轻微扩张。副鼻窦科瓦位提示副鼻窦炎。支气管镜肺活检病理提示支气管上皮破坏,管壁可见大量淋巴细胞、少量泡沫状组织细胞、中性粒细胞浸润,有淋巴滤泡形成,周围肺泡壁有少许淋巴细胞、组织细胞浸润,肺泡壁组织增生不明显,偶见局部纤维化并突入肺泡腔。常规肺功能提示混合性通气功能障碍,支气管舒张试验阳性。PaO_265 mm Hg。根据临床、影像表现、病理改变以及目前弥漫性泛细支气管炎的诊断标准,确诊为弥漫性泛细支气管炎。给予小剂量红霉素[5～10 mg/(kg·d)]治疗,患儿病情明显好转。结论弥漫性泛细支气管炎可发生于中国儿童。主要诊断依据为慢性咳嗽、咯痰伴喘息,胸部 CT 表现为两肺弥漫性分布的小结节影,为细支气管中心性或小叶中心性,伴有副鼻窦炎。小剂量红霉素治疗可控制病情。

Objective Diffuse panbronchiolitis （DPB） is a chronic progressive disease of the lower respiratory tract, which is prevalent in Asian population. So far, many DPB cases have been found in adults in China, To our knowledge, no pediatric DPB case has ever been reported in China. We describe the first pediatric DPB case in Chinese literature and the second case in the English-language literature. Method The clinical manifestations, characteristic imaging and histological features of this DPB case were summarized. Results The patient was a 13-year old girl complained of chronic productive cough with wheezing. Chest auscultation revealed fine moist rales and wheezing over both lung fields. The chest X-ray showed small nodules and reticular opacities in left lower lobe. High-resolution thorax computerized tomography （HRCT） demonstrated bilateral diffuse small centrilobuler nodules and bronchial wall thickening or bronchiectasis in some parts of the lungs. Histopathological examination of transbronchial biopsy specimen revealed lymphocytes and foamy histocytes infiltrated in the walls of bronchi, respiratory bronchioles and adjacent alveoli. Lymphoid follicles were present around some bronchi. Sinus radiographs revealed sinusitis. Lung function studies showed obstruction and restriction. PaO2 was 65 mm Hg. The diagnosis of DPB was made according to the current diagnostic criteria. Low-dose erythromycin [ 5 - 10 mg/（ kg·d ）] was effective. Conclusion DPB could occur in children in China. The major diagnostic clues may include the following： （1） persistent cough, sputum, and dyspnea; （2） coexistent chronic sinusitis; （3） bilateral diffuse small nodular opacities on HRCT. Low-dose erythromycin was effective in treatment of the case with DPB.