Shen Y L, Chen Q H, Tang X L, Xu H, Li H M, Zhao S Y
Department No. 2 of Respiratory Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Zhonghua Er Ke Za Zhi. 2020 Aug 2;58(8):646-652. doi: 10.3760/cma.j.cn112140-20200426-00435.
To analyze the clinical features of cystic fibrosis (CF) associated allergic bronchopulmonary aspergillosis (ABPA) in children. 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. 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 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 (17 cases), followed by (6 cases) and (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. 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.
分析儿童囊性纤维化（CF）相关变应性支气管肺曲霉病（ABPA）的临床特征。对2010年3月至2020年3月在首都医科大学附属北京儿童医院确诊为CF相关ABPA的22例患儿进行回顾性研究。回顾其临床特征、影像学特点、实验室检查结果及预后情况。共22例符合诊断标准，其中男12例，女10例。诊断年龄为（10.4±2.8）岁，发病年龄为（5.5±4.4）岁。临床表现包括咳嗽咳痰（22例）、反复喘息（15例）、咯血（7例）、生长发育迟缓（12例）、胰腺炎（10例）、肝大（7例）、脾大（4例）及脂肪泻（4例）。所有患者胸部CT均显示肺部浸润及中心性支气管扩张，17例合并黏液嵌塞，12例合并高密度黏液栓。18例患者出现嗜酸性粒细胞增多。22例患者总IgE及血清IgE水平均升高。15例痰或支气管肺泡灌洗液真菌培养阳性，其中单纯曲霉菌感染8例，混合曲霉菌感染3例。气道内主要病原菌为（17例），其次为（6例）和（5例）。肺功能检查显示4例为阻塞性通气功能障碍，11例为混合性通气功能障碍，4例为小气道功能障碍。治疗方面，3例仅接受全身糖皮质激素治疗，其余19例同时接受抗真菌药物治疗。随访1～7年，6例病情缓解，10例复发，1例死亡,5例失访。CF相关ABPA在中国极为罕见。这两种疾病在临床、影像学及免疫学方面的重叠特征使得诊断具有挑战性。全身糖皮质激素被认为是这些患者的一线治疗药物，辅助抗真菌药物可能有益。本中心复发率较高。
