摘要
对首都医科大学附属北京儿童医院呼吸科2018年6月收治的1例囊性纤维化接受肺移植手术患儿资料进行回顾性分析。患儿,女,10岁,间断咳嗽、咯黄痰4年余,杵状指阳性,肺部高分辨CT(HRCT)提示支气管扩张和黏液嵌塞,鼻窦HRCT提示鼻窦炎。患儿囊性纤维化跨膜调节因子(CFTR)基因复合杂合突变,汗液试验阳性,免疫球蛋白E(IgE)和嗜酸性粒细胞增高,烟曲霉特异性IgE阳性。诊断囊性纤维化、变应性支气管肺曲霉菌病、鼻窦炎,予抗感染、糖皮质激素及对症治疗,之后21个月内反复出现呼吸困难、呼吸衰竭,肺功能进行性下降,于2020年3月接受双肺移植手术,目前术后1年3个月余,恢复良好。
Objective A case of cystic fibrosis admitted in the Respiratory Department of Beijing Children′s Hospital,Capital Medical University in June 2018 and underwent lung transplantation later was analyzed retrospectively.A 10-year-old girl had intermittent productive cough for more than 4 years with clubbed-finger.The lung high resolution CT(HRCT)showed bronchiectasis and mucus impaction,and the nasal sinus HRCT showed sinusitis.She had cystic fibrosis transmembrane conductance regulator(CFTR)gene complex heterozygous mutation and positive sweat test.The immunoglobulin E(IgE)level and eosinophil count increased,and aspergillus fumigatus-specific IgE was positive.She was diagnosed as cystic fibrosis,allergic bronchopulmonary aspergillosis and sinusitis.Anti-infection,glucocorticoid and symptomatic treatment were given.During the following 21 months,the child had repeated respiratory difficulties and respiratory failure.Her lung function declined.Bilateral lung transplantation was performed in March 2020.She had a good recovery at more than 1 year and 3 months postoperatively.
作者
王梓竹
王昊
陈兰勤
贺建新
徐保平
申昆玲
Wang Zizhu;Wang Hao;Chen Lanqin;He Jianxin;Xu Baoping;Shen Kunling(Respiratory Department,Beijing Children′s Hospital,Capital Medical University,China National Clinical Research Center of Respiratory Diseases,National Center for Children′s Health,Beijing 100045,China)
出处
《中华实用儿科临床杂志》
CAS
CSCD
北大核心
2022年第3期216-218,共3页
Chinese Journal of Applied Clinical Pediatrics
基金
北京市医院管理中心儿科学科协同发展中心专项课题(XTZD20180104)。
关键词
囊性纤维化
肺移植
儿童
Cystic fibrosis
Lung transplantation
Child
