囊性纤维化并发变应性支气管肺曲霉病1例报告及文献复习

Cystic fibrosis complicated with allergic bronchopulmonary aspergillosis:A case report and literature review

囊性纤维化(CF)是由囊性纤维化跨膜传导调节因子(CFTR)功能缺陷引起的一种常染色体隐性遗传疾病。变应性支气管肺曲霉病(ABPA)是最严重的烟曲霉相关疾病之一,多发生在CF及支气管哮喘患者中。CF并发ABPA可累及全身多个系统,主要为呼吸道相关症状,包括咳嗽、咳痰、喘息、咯血等,影像学上表现为肺部炎症浸润和中央(近端)支气管扩张等,可有胰腺炎、脂肪泻、肝脾肿大等其他系统表现,临床症状复杂多样。本文通过对1例以反复咳嗽、咳痰,加重伴咯血为首发症状的CF并发ABPA患者的诊疗经过进行总结并复习相关文献,旨在提高临床医师对CF并发ABPA患者的重视及对此类疾病的敏感性。

Cystic fibrosis(CF)is an autosomal recessive genetic disease caused by the functional defect of cystic fibrosis transmembrane conductance regulator(CFTR).Allergic bronchopulmonary aspergillosis(ABPA)is one of the most serious aspergillus fumigatus related diseases,which mostly occurs in patients with CF and asthma.CF complicated with ABPA can affect multiple systems throughout the body.The clinical symptoms are complex and diverse,mainly related to respiratory symptoms,including cough,expectoration,wheezing,hemoptysis,and so on.Imaging manifestations include pulmonary inflammatory infiltration and central(proximal)bronchiectasis.Other system manifestations involve pancreatitis,steatorrhea,hepatosplenomegaly,etc.This article will summarize the diagnosis and treatment of a CF patient with ABPA whose first symptoms were repeated cough,expectoration,and aggravated hemoptysis.The relevant literature will also be reviewed,so as to raise clinicians′attention to CF patients with ABPA and to improve their sensitivity to such diseases.