Objective: To analyze the clinical features of cystic fibrosis (CF) associated allergic bronchopulmonary aspergillosis (ABPA) in children. Methods: A retrospective study was performed in 22 children who were diagnosed with CF associated ABPA in Beijing Children's Hospital affiliated to Capital Medical University from March 2010 to March 2020. The clinical features, imaging characteristics, laboratory results and the prognosis were reviewed. Results: A total of 22 cases met the diagnostic criterion, including 12 males and 10 females. The age of diagnosis was (10.4±2.8) years and the age of onset was (5.5±4.4) years. Clinical manifestations included cough and expectoration (22 cases), recurrent wheezing (15 cases), hemoptysis (7 cases), failure to thrive (12 cases), pancreatitis (10 cases), hepatomegaly (7 cases), splenomegaly (4 cases) and steatorrhea (4 cases). CT scans of all the patients showed pulmonary infiltrates and central bronchiectasis, combined with mucoid impaction in 17 cases and high density mucus plug in 12 cases. Eosinophilia was found in 18 patients. Total IgE and serum levels of A. fumigatus-specific IgE were elevated in all 22 patients. Positive culture of sputum or bronchoalvedar lauage fluid for fungus were in 15 cases, with single Aspergillus infection in 8 cases and mixed Aspergillus infection in 3 cases. The predominant bacteria found in the airways were Pseudomonas aeruginosa (17 cases), followed by staphylococcas. aureus (6 cases) and stenotrophomonas. maltophilia (5 cases). Pulmonary function revealed obstructive ventilation dysfunction in 4 cases, mixed dysfunction in 11 cases, and small airway dysfunction in 4 cases. Regarding the treatment, 3 were treated only with systemic corticosteroid, while the remaining 19 cases also received antifungal agents.The follow up continued for 1-7 years, and 6 maintained remission, 10 had recurrent episodes, 1 died, and 5 lost to follow up. Conclusions: CF associated ABPA is extremely rare in China. The overlapping clinical, radiographic, and immunologic features of these two diseases make the diagnosis challenging. Systemic corticosteroids are considered the first-line therapy for these patients, and adjuvant antifungal agents may be helpful. Recurrence rate in our center is high.
目的： 分析儿童囊性纤维化（CF）相关变应性支气管肺曲霉菌病（ABPA）的临床特点。 方法： 回顾性分析首都医科大学附属北京儿童医院2010年3月至2020年3月确诊的22例CF相关ABPA患儿的临床资料、影像学检查、实验室检查及治疗预后等指标。 结果： 确诊CF相关ABPA患儿22例（男12例、女10例），确诊年龄（10.4±2.8）岁，症状初发年龄（5.5±4.4）岁。临床表现为咳嗽咳痰22例，反复喘息15例，咯血7例，生长发育落后12例，胰腺炎10例，肝大7例，脾大及脂肪泻各4例。胸部高分辨CT示22例患儿全部存在肺实质浸润以及弥漫中心型支气管扩张，17例同时伴有管腔内黏液嵌塞，12例存在高密度黏液栓。实验室检查示外周血嗜酸性粒细胞增多18例；血清总IgE>0.5×10(6) U/L 22例；烟曲霉特异性IgE均有不同程度升高。痰或支气管肺泡灌洗液（BALF）真菌培养阳性15例，其中单一曲霉菌感染8例，两种曲霉菌混合感染3例；痰或BALF细菌培养阳性20例，其中最常见的细菌为铜绿假单胞菌（17例），其余依次为金黄色葡萄球菌（6例）及嗜麦芽窄食单胞菌（5例）。肺通气功能示阻塞性通气功能障碍4例，混合性通气功能障碍11例，小气道功能障碍4例。22例患儿均接受系统治疗，单用糖皮质激素治疗3例，激素与抗真菌药物联合治疗19例；随诊1~7年，6例经治疗缓解后处于稳定状态，10例呈现多次复发-缓解，1例死亡，5例失访。 结论： 儿童CF相关ABPA在中国极为罕见，且CF和ABPA具有部分重叠的临床表现、影像学表现以及免疫学特征，诊断难度大，首选全身糖皮质激素作为一线治疗措施，也可以联合应用抗真菌药物，复发率高。.
Keywords: Allergic bronchopulmonary aspergillosis; Child; Clinical feature; Cystic fibrosis.