儿童闭塞性细支气管炎26例临床研究

目的 分析儿童闭塞性细支气管炎(bronchiolitis obliterans,BO)的临床特点、影像学特点、实验室检查、治疗方法与临床转归.方法 2009年6月-2011年4月期间广州医学院第一附属医院儿科病房诊断为BO的住院患儿26例.分析BO患儿病原学及临床特点、危险因素、影像学特点、实验室检查、治疗方法及治疗反应.结果 26例中男18例,女8例,发病年龄4.5个月～8岁;病程(6.2±3.5)个月,随诊时间在2个月～2年.临床表现为不同程度的持续喘息(26例,100％),反复咳嗽(24例,92％)、运动不耐受(22例,85％)、气促(21例,81％)、三凹征(20例,77％)、痰鸣(16例,62％)、肺部反复湿哕音(10例,38％)、唇周紫绀(3例,12％).未见杵状指(趾).18例(69％)病原学检测阳性,支原体11例(42％),呼吸道合胞病毒4例(15％),副流感病毒、流感病毒甲型、乙型各2例(8％),博卡病毒1例(4％),其中混合感染阳性者占8例(31％).胸部X线平片检查未见异常者16例(62％),肺炎样改变10例(38％),其中仅1例疑似肺间质改变.所有病例的胸部高分辨CT(HRCT)均有肺部空气潴留、肺灌注不良的马赛克征.19例患儿检测了抗中性粒细胞胞浆抗体(ANCA),其中PANCA阳性10例(53％),CANCA阳性8例(42％).所有患儿均使用口服、全身激素与低剂量阿奇霉素治疗,其中13例(50％)治疗后咳嗽、喘息的严重程度及发作频率有明显改善,喘息、三凹征改善的天数为(7.1±4.8)d;13例(50％)激素效果不理想,或HRCT无改变者加用甲氨蝶呤后喘息、三凹征改善,时间为(16.4±11.0)d.HRCT下病灶修复时间滞后于症状改善时间,HRCT最长随访时间是1.5年,大部分病例肺部病灶仅部分吸收好转,尚无一例病灶完全吸收.结论 呼吸道感染是儿童BO发生的重要病因,临床以长期持续的喘息、咳嗽、活动不耐受、气促、三凹征为主要表现,但缺乏特异性,胸部X线平片不能提供诊断的信息,HRCT提示的典型马赛克征是诊断BO的重要征象.约50％患儿ANCA阳性,提示BO患儿可能存在免疫损伤.口服全身激素及甲氨蝶呤可改善临床症状,但整体治疗效果不理想.

Clinical characteristics of bronchiolitis obliterans in pediatric patients

Objective To analyze the clinical characteristics,image findings,laboratory examination,the therapeutic methods and clinical outcomes of bronchiolitis obliterans (BO) in pediatric patients.Method Twenty-six pediatric patients with BO were reported.All data were collected from cases who were hospitalized in the Department of Pediatrics,First Affiliated Hospital of Guangzhou Medical College from June 1st,2009 to the April 30th,2011,and infectious agents,clinical manifestations,risk factors,changes in imageology,laboratory examination,therapeutic methods and treatment responses were analyzed.Result The ranges of age at onset was 4.5 months-8 years in 26 cases ( 18 boys and 8 girls).The course of disease was (6.2 ± 3.5 ) months.The period of followed-up ranged from 2 to 24 months.The common clinical characteristics were persistent wheezing of different severity (26 cases,100％ ),cough (24 cases,92％ ),intolerance to exercise ( 22 cases,85％ ),short of breath (21 cases,81％ ),retraction:(20 cases,77％ ),wheezy phlegm (16 cases,62％ ),keeping with crackles ( 10 cases,38％ ),cyanosis around the mouth (3 cases,12％ ) and no clubbed fingers (toes).In 18 cases the etiology was detected,mycoplasma (11 cases,42％),respiratory syncuial virus (4 cases,15％),parainfluenza virus (2 cases,8％),influenza virus A (2 cases,8％ ) and influenza virus B (2 cases,8％ ),human bocavirus (HBoV) (1case,4％). There were 8 cases (31％) with combined infection. Chest X-ray in 10 cases indicated changes suggestive of bronchopneumonia (38％),in only 1 case there was an image of interstitial pneumonia disease (4％).All the patients were diagnosed by high-resolution computerized tomography (HRCT).All cases were demonstrated to have air retention,poor blood perfusion in lung,just like “Westemark sign” with HRCT.In 19 cases antineutrophil cytoplasmic antibody (ANCA) was determined and 10 patients (53％)were positive for P-ANCA,and 8 cases (42％) were positive for C-ANCA. All patients received oral corticosteroid and low doses azithromycin.In 13 cases (50％) the treatment effectively reduced the severity of disease and the frequency of cough and wheezing.The average number of days for symptom improvement was (7.1 ± 4.8) days.Conclsion Respiratory infection plays an important role in BO in children.The chronic and persistent wheezing,cough,intolerance to exercises,short breath,retraction were the main clinical manifestations. But these symptoms are non-specific. Chest X-ray can not provide enough information for diagnosis.Classical “Westemark sign” with HRCT is an important sign.ANCA with a high positive rate ( approximately 50％ ) suppose immuno-lesion in BO.Oral corticosteroid and methotrexate may relieve clinical symptoms.