多囊肝疾病肝移植受者的等待时间较长，但长期临床结局优于其他病因肝病患者：一项回顾性横断面研究。
Liver transplant recipients with polycystic liver disease have longer waiting times but better long-term clinical outcomes than those with liver disease due to other causes: A retrospective cross-sectional study.
机构信息
Academic Nephrology Unit, Division of Clinical Medicine, Faculty of Health, Sheffield Teaching Hospitals Trust, University of Sheffield and Sheffield Kidney Institute, Sheffield, United Kingdom.
Liver Transplant Unit, Leeds Teaching Hospitals Trust, Leeds, United Kingdom.
出版信息
PLoS One. 2024 Jan 2;19(1):e0294717. doi: 10.1371/journal.pone.0294717. eCollection 2024.
INTRODUCTION
Liver transplantation is the only curative option for patients with polycystic liver disease (PLD). In the United Kingdom, these patients are listed on the variant syndrome list due to their preserved liver function reflected in the United Kingdom End-stage Liver Disease (UKELD) score. The transplantation and survival rates for this patient group in the UK have not been previously reported.
METHODS
A retrospective cross-sectional analysis of patients receiving liver transplantation between 2010 and 2017 was performed using the NHS blood and transplantation database. This database contains the demographic, clinical parameters, indication for transplantation and follow-up of all patients in UK-based transplant centres. Basic statistics was performed using SPSS version 27.
RESULTS
5412 recipients received elective liver allografts in the study period. 1.6% (100) of recipients had PLD as their primary indication for transplantation with 60 receiving liver only allografts and 40 receiving combined liver-kidney allografts. PLD patients had a >3-fold longer mean waiting time for transplantation compared to non-PLD patients, 508 days v 154 days respectively. PLD patients receiving combined liver-kidney allografts had a longer waiting time than those receiving a liver only allograft, 610 days v 438 days respectively. There were comparable patient survival rates for people with PLD and non-PLD primary indications at 30 days (94.0% vs 97.6%) and 1 year (92.0% vs 93.2%) but improved survival rates at 5 years (81.3% vs 76.5%). There were also comparable allograft survival rates for people with PLD and non-PLD primary indications at 30 days (93.9% vs 95.3%) and 1 year (91.9% vs 91.2%) but improved survival rates at 5 years (82.5% vs 77.3%). Transplant centre-level analysis identified variation in the proportion of liver transplantations for people with PLD as their primary listed indication.
CONCLUSIONS
Patients with PLD wait significantly longer for liver transplantation compared to other indications. However, transplanted PLD patients demonstrate better longer-term patient and liver allograft survival rates compared to transplanted non-PLD patients. The unexpected variation between individual UK centres transplanting for PLD deserves further study.
简介
肝移植是多囊肝病（PLD）患者的唯一治愈选择。在英国，由于这些患者的英国终末期肝病（UKELD）评分反映了其保留的肝功能，因此他们被列入变体综合征名单。该患者群体在英国的移植和存活率以前没有报告过。
方法
使用 NHS 血液和移植数据库对 2010 年至 2017 年间接受肝移植的患者进行回顾性横断面分析。该数据库包含英国移植中心所有患者的人口统计学、临床参数、移植适应症和随访信息。使用 SPSS 版本 27 进行基本统计。
结果
在研究期间，5412 名接受者接受了选择性肝同种异体移植。1.6%（100 名）的接受者将 PLD 作为他们的主要移植适应症，其中 60 名接受肝单独同种异体移植，40 名接受肝肾联合同种异体移植。与非 PLD 患者相比，PLD 患者的平均移植等待时间长了 3 倍以上，分别为 508 天和 154 天。接受肝肾联合同种异体移植的 PLD 患者的等待时间比接受单独肝同种异体移植的患者长，分别为 610 天和 438 天。在 30 天（94.0%对 97.6%）和 1 年（92.0%对 93.2%）时，PLD 和非 PLD 主要适应症患者的患者生存率相当，但在 5 年时（81.3%对 76.5%）生存率更高。在 30 天（93.9%对 95.3%）和 1 年（91.9%对 91.2%）时，PLD 和非 PLD 主要适应症患者的同种异体移植存活率相当，但在 5 年时（82.5%对 77.3%）存活率更高。移植中心水平分析发现，PLD 患者作为主要列入适应症的肝移植比例存在差异。
结论
与其他适应症相比，PLD 患者等待肝移植的时间明显更长。然而，与接受非 PLD 患者的移植相比，接受移植的 PLD 患者的患者和肝移植存活率在长期内更好。英国个别中心对 PLD 进行移植的意外差异值得进一步研究。
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