Abstract: Cystic fibrosis （CF） is a rare disease caused by a mutation of the gene encoding the cystic fibrosis transmembrane conductance regulator （CFTR）. One case of repeated severe pulmonary infection in children with CF of Han nationality was reported in this paper. The main pathogen of the respiratory tract was Pseudomonas aeruginosa. After the child developed bronchiectasis, the main treatment options during hospitalization were intravenous antibiotics and bronchoalveolar lavage. At follow-up, the main treatment options were inhaled amikacin and oral n-acetylcysteine. After treatment, the patient has a good prognosis.

Key words: cystic fibrosis, recurrent lung infections, pseudomonas aeruginosa, child

摘要： 囊性纤维化（cystic fibrosis， CF）是由编码囊性纤维化跨膜传导调节因子（cystic fibrosis transmembrane conductance regulator， CFTR）的基因突变引起的罕见疾病。文章报告1例反复肺部严重感染的汉族CF患儿，铜绿假单胞菌是该例CF患儿的主要呼吸道病原体。该例患儿出现支气管扩张后，住院期间主要治疗方案为静脉使用抗生素和支气管肺泡灌洗。 随访中主要治疗方案为吸入阿米卡星和口服N-乙酰半胱氨酸。经过治疗，该患儿预后较好。

关键词: 囊性纤维化, 反复肺部感染, 铜绿假单胞菌, 儿童