儿童闭塞性细支气管炎28例

Bronchiolitls obliterans in 28 children

目的了解儿童闭塞性细支气管炎（BO）的临床特点及治疗效果。方法对2001年12月至2012年8月南京医科大学附属南京儿童医院临床诊断的28例BO患儿的临床表现、病因、X线胸片和肺CT、肺功能、纤维支气管镜检查结果、治疗转归进行分析。其中男20例，女8例；年龄4个月～7岁。结果28例BO患儿反复咳嗽、喘息均〉6周，肺部有喘鸣音和湿哕音。病因为感染后BO（PBO）23例，重症渗出性多形性红斑（SJS）后5例。肺cT示28例均存在马赛克征，其中肺不张7例，支气管扩张4例。肺功能表现为阻塞性通气功能障碍16例，混合性通气功能障碍3例。13例行纤维支气管镜术，BALF的中性粒细胞计数均明显增高。患儿均口服激素及阿奇霉素，辅以激素和支气管扩张剂吸入，或白三烯受体拮抗剂口服治疗。随访23例BO患儿5个月～7年，目前尚无死亡，规律治疗的7例患儿临床表现明显改善，肺CT及肺功能有一定程度好转，其余患儿均有不同程度的咳喘反复、加重。结论儿童BO多由感染、sJs引起，其特征性的临床慢性咳嗽及喘息、肺CT马赛克征、肺功能阻塞性通气功能障碍基本可确定诊断。该病目前治疗以激素为主，总体预后不佳。

Objective To recognize the clinical characteristics and treatment of bronchiolitis obliterans （BO） in children. Methods Clinical analysis was done on data of 28 patients with BO diagnosed in Nanjing Children＇s Hospital Affiliated to Nanjing Medical University from Dec. 2001 to Aug. 2012. Clinical manifestation, etiology, chest X-ray, computed tomography （CT）, pulmonary function, flexible bronchoscopy, treatment and prognosis of the children were analyzed. Results Boys were common in all 28 children （20 boys and 8 girls） aged from 4 months to 7 years. All cases presented with persistent cough and wheezing for more than 6 weeks, and crackles as well as wheezing were heard. Twenty-three cases were post-infectious BO （PBO）, and 5 cases occurred after Steven-Johnson syndrome （SJS）. Pulmonary CT showed mosaic perfusion in all cases, atelectasis in 7 cases, and bronchiectasis in 4 cases. Lung function revealed obstructive ventilatory dysfunction in 16 cases, and mixed ventilatory dysfunction in 3 cases. Thirteen cases received flexible bronchoscopy, and the cell of the bronchoalveolar lavage fluid showed increased level of neutrophils. All the cases received oral corticosteroid, azithromycin and inhalation of corticosteroid and bronchodilator, or oral leukotrienes receptor antagonists. After a follow-up in 23 cases for 5 months to 7 years, there was no death occurred. Besides 7 cases experienced regular treatment were ameliorated in clinical presentation, pulmonary imaging and function, the rests experenced persistent symptoms. Conclusions BO in children mostly follows lower respiratory tract infection and SJS. Diagnosis is made according to clinical productive cough and wheezing, mosaic perfusion in pulmonary CT and airway obstructions in pulmonary function. Corticosteroid is mainly used in treatment of BO, and its overall prognosis is poor.