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Clinical outcomes and survival following lung transplantation in patients with Alpha-1 antitrypsin deficiency.
Respiratory Medicine ( IF 4.3 ) Pub Date : 2020-09-02 , DOI: 10.1016/j.rmed.2020.106145
Leonard Riley 1 , Jorge Lascano 1
Affiliation  

Objective

The primary intention of our study is to describe disease-specific outcomes in patients with alpha-1 antitrypsin deficiency (AATD) following lung transplantation (LT).

Methods

We reviewed the Organ Procurement and Transplant Network database to identify AATD patients who have undergone LT in the United States.

Results

Two thousand two hundred and thirteen patients with AATD underwent LT between March 1992 and September 2019. A total of 1556 patients received LT with a median age at listing was 51 years. The median time spent on the LT waitlist was 263 days. The median ischemic time was 4.75 h. The Kaplan-Meier survival analysis following LT for AATD patients at 1-, 5-, and 10 years was 82%, 56%, and 34%, at 1-, 5-, and 10 years, respectively. The median survival time post-LT is 6.4 years (Interquartile range 5.6–6.8 years). The post-LT survival was significantly better in double LT compared to single LT (Median 7.7 vs 4.4 years, p < 0.001). Increasing age, presence of CMV mismatch, reintubation prior to discharge, and requiring treatment for rejection within one year of transplantation did impact post-LT mortality.

Conclusion

The median survival after LT in AATD is 6.4 years and is similar to other lung diseases. When compared to usual COPD LT, AATD patients have increased post-LT mortality due to infections and liver disease. Recipients of a double lung transplant had a favorable outcome compared to single lung transplant.



中文翻译:

Alpha-1抗胰蛋白酶缺乏症患者肺移植后的临床结局和生存率。

目的

我们研究的主要目的是描述肺移植(LT)后患有α-1抗胰蛋白酶缺乏症(AATD)的患者的疾病特异性结果。

方法

我们审查了器官采购和移植网络数据库,以识别在美国接受过LT的AATD患者。

结果

在1992年3月至2019年9月之间,对213例AATD患者进行了LT。共有1556例接受LT的患者中位年龄为51岁。在LT等待列表上花费的平均时间为263天。中位缺血时间为4.75小时。AATD患者于1年,5年和10年进行LT后的Kaplan-Meier生存分析分别为1年,5年和10年为82%,56%和34%。LT后的中位生存时间为6.4年(四分位间距为5.6–6.8年)。与单次LT相比,两次LT的LT后生存率明显更好(中位7.7 vs 4.4年,p  <0.001)。年龄的增长,CMV不匹配的存在,出院前的再插管以及在移植后一年内需要接受排斥反应的治疗确实影响了LT术后的死亡率。

结论

AATD LT患者的中位生存期为6.4年,与其他肺部疾病相似。与普通COPD LT相比,AATD患者由于感染和肝脏疾病而导致LT后死亡率增加。与单肺移植相比,双肺移植的接受者有良好的预后。

更新日期:2020-09-02
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