Lung transplantation for cystic fibrosis: a case report and literature review
10.3969/j.issn.1674-7445.2020.03.012
- VernacularTitle:肺移植治疗囊性纤维化1例并文献复习
- Author:
Ao CHEN
1
;
Qiaoyan LIAN
;
Xin XU
;
Bing WEI
;
Mengyang LIU
;
Guilin PENG
;
Jianheng ZHANG
;
Jianxing HE
;
Chunrong JU
Author Information
1. Guangzhou Medical University, Guangzhou 511436, China
- Publication Type:Research Article
- Keywords:
Cystic fibrosis;
Cystic fibrosis transmembrane conductance regulator protein;
Lung transplantation;
Type Ⅱ respiratory failure;
Sweat test;
Extracorporeal membrane oxygenation;
Gene mutation
- From:
Organ Transplantation
2020;11(3):391-
- CountryChina
- Language:Chinese
-
Abstract:
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.
