作　　者： ; ; ; ; ; ;

机构地区： 广州医科大学

出　　处： 《器官移植》 2020年第3期391-394,423,共5页

摘　　要： 目的探讨肺移植治疗囊性纤维化(CF)的疗效和预后。方法回顾性分析行同种异体双肺移植术治疗1例终末期CF受者的临床资料,对CF受者的临床特点、诊断方法及治疗策略进行总结。结果本例受者幼年发病,反复咳嗽、咳浓痰30年,肺部反复感染合并急性加重、慢性鼻窦炎及极重度营养不良,肺移植术前因呼吸肌无力难以脱离有创呼吸机,长期入住重症监护室(ICU)。影像学检查提示双肺多发囊柱状支气管扩张伴感染,经汗液测试和基因检测进一步确诊为CF。该受者2017年8月17日接受双肺移植术后经过康复治疗,肺功能逐渐恢复正常,康复后至投稿日已2年余,享有与同龄健康人同等的生活质量。结论肺移植术作为终末期CF的有效治疗手段,不仅能挽救患者生命,而且还能显著提高患者的生活质量。 Objective To investigate the clinical efficacy and prognosis of lung transplantation in the treatment of cystic fibrosis(CF).Methods Clinical data of one patient with end-stage CF undergoing allogeneic bilateral lung transplantation were retrospectively analyzed.Clinical characteristics,diagnostic methods and treatment strategies of the CF recipient were summarized.Results The recipient had suffered from relevant symptoms since childhood including repeated cough and purulent sputum for 30 years,complicated with recurrent pulmonary infection combined with acute exacerbation,chronic sinusitis and extremely severe malnutrition.Prior to lung transplantation,the patient had to depend upon the invasive ventilator due to respiratory muscle weakness,and admitted to intensive care unit(ICU)for a long time.Imaging examination revealed multiple cystic columnar bronchiectasis accompanied with infection in bilateral lungs.The diagnosis of CF was further confirmed by sweat test and gene detection.The recipient underwent bilateral lung transplantation on August 17,2017 and received rehabilitation treatment.The lung function was gradually restored to normal.The recipient had obtained the same quality of life to the healthy counterparts since the date of manuscript submission(over 2 years).Conclusions Lung transplantation is an efficacious treatment for end-stage CF,which can not only save patients’lives,but also significantly improve the quality of life of patients.

关 键 词： 囊性纤维化 囊性纤维化跨膜电导调节蛋白 肺移植 型呼吸衰竭 汗液测试 体外膜肺氧合 基因突变

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