Abstract: 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.

Key words: allergic bronchopulmonary aspergillus;? cystic fibrosis;? asthma