目的 分析囊性纤维化（CF）并发变应性支气管肺曲霉菌病（ABPA）的临床特征。方法 回顾分析1例确诊为 CF并发ABPA患儿的临床资料，分析近10年PubMed、中国知网、万方数据库中报道CF并发ABPA的资料。结果 11岁患儿， 男，曾诊断哮喘，因持续咳嗽、喘息入院，初步确诊ABPA。基因检测显示患儿CFTR基因变异，汗液氯离子浓度93 mmol/L。 最终确诊CF并发ABPA。以“ABPA、CF、儿童”为关键词检索PubMed、中国知网、万方数据库近10年文献，病例报道 7例，国内2例，国外5例。患儿基因变异不同，临床表现亦不同。中国报道CF并发ABPA病例与欧美国家不同，均为先发 现ABPA，经过基因检查、汗液氯离子试验确诊CF。结论 CF患儿应监测是否存在ABPA，及早发现、治疗。

Objective? To analyse the characteristics of allergic bronchopulmonary aspergillosis (ABPA) in children with cystic fibrosis (CF). Methods? The clinical data of a child with ABPA and CF was retrospectively analyzed and related literatures in database within 10 years were analyzed. Results? An 11 -year-old boy was diagnosed with asthma. He was admitted to the hospital because of “cough, wheezing”, and ABPA was confirmed. The genetic test identified mutation in CFTR gene and the concentration of chloride ions in sweat was 93 mmol/L. The final diagnosis was ABPA with CF. Seven cases of ABPA with CF were reported in detail in PubMed, CNKI, and Wanfang data. Five children born in European and American countries mostly were diagnosed CF by screening at early age as manifested repeated infections, respiratory symptoms, and pancreatic insufficiency. The clinical manifestation is varied among different gene mutations. Two cases of ABPA with CF reported in China were first diagnosed with ABPA, and CF was confirmed by gene examination and sweat chloride ion test. Conclusion? The disease process and clinical manifestations of CF complicated with ABPA in children are different related to the different mutations. The clinical manifestations of CF children in China are not typical and genetic testing should be conducted for children diagnosed with ABPA. Children with CF should be monitored for the presence of ABPA.