Lung function decline is mitigated following liver transplantation in people with cystic fibrosis: A retrospective cohort study.
Lung function decline is mitigated following liver transplantation in people with cystic fibrosis: A retrospective cohort study.
复制标题
囊性纤维化患者肝移植后肺功能下降得到缓解：一项回顾性队列研究。
DOI：
10.1097/lvt.0000000000000232
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发表时间：
2024
期刊：
影响因子：
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通讯作者：
Stephenson,AnneL
中科院分区：
文献类型：
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作者：
Albaiz,FaisalA;Ramos,KathleenJ;Sykes,Jenna;Stanojevic,Sanja;Ma,Xiayi;Quon,BradleyS;Marshall,BruceC;Cromwell,ElizabethA;Ostrenga,JoshuaS;Faro,Albert;Elbert,Alexander;Goss,ChristopherH;Stephenson,AnneL
目前缺乏关于囊性纤维化（pwCF）患者肝移植（LT）后健康结果的文献。我们的目的是评估肝移植对pwCF患者肺功能的影响。我们对1987年至2019年在美国和加拿大接受肝移植的pwCF患者进行了回顾性队列研究。同时进行肺肝移植和肝移植前进行肺移植的个体被排除在外。我们分析了肝移植前和肝移植后预测1秒用力呼气量、体重指数、肺恶化率和肝移植后总生存率的百分比。共纳入402例肝移植受者。移植的中位年龄为14.9岁，69.7%的移植是在18岁以下的儿童中进行的。LT后预测1秒内用力呼气量百分比的下降率从每年预测的- 2.2%下降到- 0.7%，每年预测的差异为1.5% （95% CI, 0.8, 2.2; p< 0.001）。肝移植后，体重指数下降率降低，肺加重发生率减少（术前0.6 vs后0.4；比率比0.7,p< 0.01）。移植后中位生存时间为13.9年，5年总生存率为77.6%。肝移植前肺功能较高的患者肝移植后死亡风险较低，非F508缺失基因型患者生存率较差。pwCF中的LT最常见于儿童和青少年，并与肺功能和营养状况下降的速度较慢以及肺恶化的减少有关。
There is paucity of literature on the health outcomes following liver transplantation (LT) in people with cystic fibrosis (pwCF). We aim to evaluate changes in lung function following LT in pwCF. We performed a retrospective cohort study of pwCF who underwent LT between 1987 and 2019 in the United States and Canada. Simultaneous lung-liver transplants and individuals who had lung transplant prior to LT were excluded. We analyzed pre-LT and post-LT percent predicted forced expiratory volume in 1 second, body mass index, rates of pulmonary exacerbation, and post-LT overall survival. A total of 402 LT recipients were included. The median age of transplant was 14.9 years and 69.7% of the transplants were performed in children less than 18 years old. The rate of decline in percent predicted forced expiratory volume in 1 second was attenuated after LT from− 2.2% to− 0.7% predicted per year with a difference of 1.5% predicted per year (95% CI, 0.8, 2.2; p< 0.001). Following LT, the rate of decline in body mass index was reduced, and there were fewer pulmonary exacerbations (0.6 pre vs. 0.4 post; rate ratio 0.7, p< 0.01). The median survival time post-transplant was 13.9 years and the overall probability of survival at 5 years was 77.6%. Those with higher lung function pre-LT had a lower risk of death post-LT, and those with genotypes other than F508 deletion had worse survival. LT in pwCF occurs most often in children and adolescents and is associated with a slower rate of decline in lung function and nutritional status, and a reduction in pulmonary exacerbations.