Postoperative Outcomes Following Liver Transplantation for Wilson's Disease: A Systematic Review and Meta-Analysis

IF 1.9 4区医学 Q2 SURGERY

Clinical Transplantation Pub Date : 2025-06-02 DOI:10.1111/ctr.70155

Austine Wang, Noor Bakir, Clarissa Ngo, Justin Kang, Felipe Rodriguez, Tyler McKechnie, Cagla Eskicioglu, Maja Segedi, Pablo E. Serrano

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引用次数: 0

Abstract

Background

Liver transplant is indicated in patients with Wilson's disease for acute hepatic failure, advanced cirrhosis, and disease refractory to chelation therapy. This study aims to systematically review data about overall morbidity, hepatic, neuropsychiatric, and survival outcomes following liver transplantation for Wilson disease.

Methods

MEDLINE, Embase, and Central were searched from inception until July 2023. Peer-reviewed articles and published abstracts evaluating patients diagnosed with Wilson's disease and undergoing any type of liver transplant as a result of the disease were eligible for inclusion. A restricted maximum likelihood random effects model was used to generate the pooled proportion of each outcome. The risk of bias for each included observational study was assessed using the Methodological Index for Non-Randomized Studies tool.

Results

A total of 39 studies met all inclusion criteria. All studies were observational. Specific indications for liver transplant were most commonly acute liver failure (36.73%), chronic liver failure (45.02%), and acute-on-chronic liver failure (8.35%). The pooled proportions of mortality at 30 days, 1, and 5 years were 0.10 (95% CI 0.08, 0.13; I² = 16%), 0.11 (95% CI 0.09, 0.14; I² = 37%), and 0.15 (95% CI 0.11, 0.20; I² = 81%), respectively. The postoperative complication with the greatest prevalence was biopsy-proven acute rejection with a pooled proportion of 0.20 (95% CI 0.12, 0.31; I² = 84%). The mean MINORS score for risk of bias for all studies was 8.19.

Conclusion

Overall, reporting quality and consistency of outcomes included in the studies was poor as assessed using the MINORS score. Pooled proportions for 30-day, 1- and 5-year mortality are similar, suggesting most postoperative deaths are acute in nature. Future research should incorporate objective measures and the reporting of standardized parameters to allow more robust comparisons between studies.

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肝豆状核变性术后肝移植的预后：一项系统综述和荟萃分析

肝移植适用于急性肝功能衰竭、晚期肝硬化和对螯合治疗难治性疾病的Wilson病患者。本研究旨在系统地回顾肝豆状核变性患者肝移植后的总体发病率、肝脏、神经精神和生存结果的数据。方法检索MEDLINE、Embase和Central数据库，检索时间为建站至2023年7月。同行评议的文章和发表的摘要评估诊断为威尔逊氏病的患者和因该疾病而接受任何类型的肝移植的患者符合纳入条件。使用限制最大似然随机效应模型生成每个结果的合并比例。每个纳入的观察性研究的偏倚风险使用非随机研究的方法学指数工具进行评估。结果39项研究符合所有纳入标准。所有的研究都是观察性的。肝移植的具体适应症以急性肝衰竭（36.73%）、慢性肝衰竭（45.02%）和急性伴慢性肝衰竭（8.35%）最为常见。30天、1年和5年的总死亡率为0.10 (95% CI 0.08, 0.13；I2 = 16%), 0.11 (95% ci 0.09, 0.14；I2 = 37%), 0.15 (95% CI 0.11, 0.20；I2 = 81%)。术后并发症发生率最高的是活检证实的急性排斥反应，合并比例为0.20 (95% CI 0.12, 0.31；I2 = 84%)。所有研究的平均偏倚风险评分为8.19。总体而言，使用未成年人评分评估的研究结果的报告质量和一致性较差。30天、1年和5年死亡率的合并比例相似，表明大多数术后死亡本质上是急性死亡。未来的研究应纳入客观测量和标准化参数的报告，以便在研究之间进行更有力的比较。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical Transplantation 医学-外科

CiteScore

3.70

自引率

4.80%

发文量

286

审稿时长

2 months

期刊介绍： Clinical Transplantation: The Journal of Clinical and Translational Research aims to serve as a channel of rapid communication for all those involved in the care of patients who require, or have had, organ or tissue transplants, including: kidney, intestine, liver, pancreas, islets, heart, heart valves, lung, bone marrow, cornea, skin, bone, and cartilage, viable or stored. Published monthly, Clinical Transplantation’s scope is focused on the complete spectrum of present transplant therapies, as well as also those that are experimental or may become possible in future. Topics include: Immunology and immunosuppression; Patient preparation; Social, ethical, and psychological issues; Complications, short- and long-term results; Artificial organs; Donation and preservation of organ and tissue; Translational studies; Advances in tissue typing; Updates on transplant pathology;. Clinical and translational studies are particularly welcome, as well as focused reviews. Full-length papers and short communications are invited. Clinical reviews are encouraged, as well as seminal papers in basic science which might lead to immediate clinical application. Prominence is regularly given to the results of cooperative surveys conducted by the organ and tissue transplant registries. Clinical Transplantation: The Journal of Clinical and Translational Research is essential reading for clinicians and researchers in the diverse field of transplantation: surgeons; clinical immunologists; cryobiologists; hematologists; gastroenterologists; hepatologists; pulmonologists; nephrologists; cardiologists; and endocrinologists. It will also be of interest to sociologists, psychologists, research workers, and to all health professionals whose combined efforts will improve the prognosis of transplant recipients.