儿童变应性支气管肺曲霉病8例

Analysis of allergic bronchopulmonary aspergiUosis in 8 children

目的总结儿童变应性支气管肺曲霉病（ABPA）病例的临床特点，提高对该病的认识，以期早期诊断及治疗。方法以首都医科大学附属北京儿童医院2010年3月至2013年12月确诊的所有ABPA病例为研究对象，收集病例的临床资料、实验室检查、影像学检查及治疗预后等资料，并进行描述性分析。结果确诊ABPA患儿8例，均为学龄期儿童，年龄为7岁2个月-13岁8个月。8例患儿中最显著的临床表现为咳嗽（8例）、咯痰（8例）、喘息（6例）、发热（4例）及咯血（3例）。8例患儿中有6例外周血嗜酸性粒细胞计数明显升高，2例嗜酸性粒细胞计数正常；7例患儿血清总IgE水平显著升高（1．000～5．000[U／L），1例患儿血清IgE水平轻度升高。血清变应原检测结果显示所有患儿对包括户尘螨、真菌等多种致敏原过敏，8例患儿对烟曲霉均有不同程度过敏，但仅有2例患儿痰和／或支气管灌洗液真菌培养烟曲霉阳性。影像学检查均有明显的肺部浸润表现，7例患儿具有典型中心型支气管扩张表现。肺功能表现有阻塞性通气功能障碍或混合性通气功能障碍。诊断ABPA后有7例患儿进行治疗，包括口服糖皮质激素、抗真菌治疗（伊曲康唑或伏立康唑口服）及其他对症治疗。治疗后患儿临床症状缓解，血清总IgE水平及嗜酸性粒细胞计数下降。结论儿童ABPA相对少见，临床表现缺乏特异性，但血清总IgE水平显著升高、血嗜酸性粒细胞计数升高、烟曲霉特异性IgE／IgG抗体阳性及中心型支气管扩张等表现相对特异，是ABPA的主要诊断标准。ABPA对全身糖皮质激素及抗真菌药物治疗反应较好。

Objective To summarize the clinical features of the allergic bronchopulmonary aspergillosis （ABPA） in children,in order to improve the understanding for ABPA and make early diagnosis and treatment of the disease. Methods A retrospective study was performed on ABPA patients diagnosed in Department of Respiratory Medicine, Beijing Children＇s Hospital Affiliated to Capital Medical University from March 2010 to December 2013. The clinical features, laboratory results, image characteristics and the prognosis information were reviewed. Results Eight ABPA cases met the diagnostic criterion. All patients were school -age children （7 years and 2 months to 13 years and 8 months old）. Cough （8 cases）,productive sputum （8 cases）,wheezing （5 cases）,fever （4 cases） and hemoptysis （ 3 cases） were the main clinical features. Six of the 8 patients showed eosinophilia. IgE level was elevated in 7 patients （ 1. 000 -5. 000 IU/L）. All 8 patients were allergic to aspergillus fumigatus ,while only 2 cases were positive in sputum culture for aspergillus fumigatus. CT scans showed pulmonary opacities in all 8 cases ,while 7 patients had typical central bronchiectasis. Seven patients were treated with the regimen, which included glucocorticosteroid, antifungal agents （ voriconazole or itraconazole） and regular bronchoscope. The symptoms of all treated patients relieved, the total serum IgE level and eosinophil cell count decreased spontaneously after the therapy. Conclusions ABPA is rare in children and the clinical features are non - specific. If the patient has elevated total IgE level in serum and eosinophilia, especially in patients with underling diseases,ABPA should be suspected. The positive result of specific antibodies to aspergillus fumigatus and central bronchiectasis on the radiology may give the suggestive diagnosis. ABPA patients generally have good response to the therapy of glucocorticosteroid and antifungal agents.