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Alpha-1 antitrypsin deficiency-associated liver disease: A review focusing on new assessment tools and therapies α -1抗胰蛋白酶缺乏相关肝病:新的评估工具和治疗方法综述
Pub Date : 2025-07-04 DOI: 10.1016/j.liver.2025.100287
Luca Marzi , Ilaria Ferrarotti , Federica Benini , Andrea Mega , Luisa Siciliani
Alpha-1-antitrypsin deficiency (AATD) is an autosomal codominant genetic disorder, often going undiagnosed. AATD results from malformed or deficient AAT proteins, which predispose individuals to obstructive pulmonary disease and liver disease. The PI*ZZ genotype is the most common and severe, but even milder genotypes like PI*SZ and PI*MZ can lead to lung and liver disease, particularly when combined with metabolic disfunction. The rate ranges of ZZ liver-related mortality are 10 to 40 %. Despite ongoing clinical trials, there is currently no approved therapy for AATD-associated liver disease (AATD-LD), and liver transplantation remains the only curative option. AATD-LD can progress slowly for decades, with contributing factors such as metabolic dysfunction-associated steatotic liver disease, alcohol use, and hepatitis accelerating disease progression. Moreover, these factors complicate the accurate diagnosis of AATD-LD. To date, data on blood markers or non-invasive markers for monitoring and predicting the evolution of AATD-LD are few and not as numerous as for other liver diseases. Moreover, a correct staging of the patient is important not only for the follow-up of the patient but also to evaluate the inclusion of the patient in experimental protocols. This review aims to evaluate non-invasive techniques for monitoring the AATD-LD.
α -1抗胰蛋白酶缺乏症(AATD)是一种常染色体共显性遗传疾病,通常未被诊断出来。AATD是由AAT蛋白畸形或缺陷引起的,这使个体易患阻塞性肺病和肝病。PI*ZZ基因型是最常见和最严重的,但即使是较轻的基因型,如PI*SZ和PI*MZ,也会导致肺部和肝脏疾病,特别是在合并代谢功能障碍的情况下。ZZ肝相关死亡率范围为10% ~ 40%。尽管正在进行临床试验,目前还没有批准的治疗aatd相关肝病(AATD-LD)的方法,肝移植仍然是唯一的治疗选择。AATD-LD可缓慢发展数十年,代谢功能障碍相关的脂肪变性肝病、酒精使用和肝炎等因素可加速疾病进展。此外,这些因素使AATD-LD的准确诊断复杂化。迄今为止,用于监测和预测AATD-LD演变的血液标志物或非侵入性标志物的数据很少,而且不像其他肝脏疾病那样多。此外,患者的正确分期不仅对患者的随访很重要,而且对评估患者在实验方案中的纳入也很重要。本综述旨在评价无创监测AATD-LD的技术。
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引用次数: 0
The impact of functional status on post-liver transplant outcomes in acute-on-chronic liver failure 功能状态对急性慢性肝衰竭患者肝移植后预后的影响
Pub Date : 2025-06-29 DOI: 10.1016/j.liver.2025.100286
David Uihwan Lee , Youngjae Cha , Mohammed Rifat Shaik , Kuntal Bhowmick , Andrew Yi , Andrew Chan , Nishat Anjum Shaik , Zainab Mujahid , Gregory Hongyuan Fan , Keeseok Lee , Sindhura Kolachana , Mohamed Refaat , Raffi Karagozian

Background and aims

Acute-on-Chronic Liver Failure (ACLF) is a severe condition where liver transplantation is often the only definitive treatment. Previous studies have shown an influence of functional status on post-transplant outcomes in patients with advanced chronic liver disease. However, the impact of functional status on outcomes in an ACLF cohort is largely unknown.

Methods

The United Network for Organ Sharing (UNOS) Standard Transplant Analysis and Research (STAR) Database was utilized to study LT patients between 1987 and 2019. Patients were categorized by ACLF grades and further divided within each grade based on their level of assistance—no, some, or total—using KPS scores. The primary outcomes assessed were graft failure and all-cause mortality post-transplant. The secondary outcomes assessed were mortality secondary to specific organ system failures.

Results

Patients without ACLF requiring some (aHR 1.10, 95 %CI 1.04–1.17, p = 0.002) or total assistance (aHR 1.32, 95 %CI 1.22–1.43, p < 0.001) showed increased risk of all-cause mortality. Those needing total assistance also faced a higher risk of graft failure (aHR 1.34, 95 %CI 1.13–1.58, p < 0.001). However, functional status did not significantly impact post-transplant outcomes across all ACLF grades.

Conclusion

Functional status was not a significant predictor of post-transplant outcomes in ACLF patients, regardless of ACLF severity. Poor functional scores in multi-organ failure likely reflect acute critical illness rather than baseline frailty.
背景和目的急性慢性肝衰竭(ACLF)是一种严重的疾病,肝移植通常是唯一的决定性治疗方法。先前的研究表明,功能状态对晚期慢性肝病患者移植后预后的影响。然而,功能状态对ACLF队列结果的影响在很大程度上是未知的。方法利用美国器官共享网络(UNOS)标准移植分析与研究(STAR)数据库对1987 - 2019年肝移植患者进行研究。根据ACLF分级对患者进行分类,并使用KPS评分根据患者的辅助水平(无、部分或全部)在每个等级内进一步划分。评估的主要结果是移植失败和移植后全因死亡率。评估的次要结局是特定器官系统衰竭继发的死亡率。结果无ACLF患者需要部分(aHR 1.10, 95% CI 1.04 ~ 1.17, p = 0.002)或全部辅助(aHR 1.32, 95% CI 1.22 ~ 1.43, p <;0.001)显示全因死亡风险增加。需要全面辅助的患者也面临更高的移植物衰竭风险(aHR 1.34, 95% CI 1.13-1.58, p <;0.001)。然而,在所有ACLF分级中,功能状态对移植后预后没有显著影响。结论无论ACLF严重程度如何,功能状态都不是ACLF患者移植后预后的重要预测因素。多器官衰竭的功能评分差可能反映了急性危重疾病,而不是基线虚弱。
{"title":"The impact of functional status on post-liver transplant outcomes in acute-on-chronic liver failure","authors":"David Uihwan Lee ,&nbsp;Youngjae Cha ,&nbsp;Mohammed Rifat Shaik ,&nbsp;Kuntal Bhowmick ,&nbsp;Andrew Yi ,&nbsp;Andrew Chan ,&nbsp;Nishat Anjum Shaik ,&nbsp;Zainab Mujahid ,&nbsp;Gregory Hongyuan Fan ,&nbsp;Keeseok Lee ,&nbsp;Sindhura Kolachana ,&nbsp;Mohamed Refaat ,&nbsp;Raffi Karagozian","doi":"10.1016/j.liver.2025.100286","DOIUrl":"10.1016/j.liver.2025.100286","url":null,"abstract":"<div><h3>Background and aims</h3><div>Acute-on-Chronic Liver Failure (ACLF) is a severe condition where liver transplantation is often the only definitive treatment. Previous studies have shown an influence of functional status on post-transplant outcomes in patients with advanced chronic liver disease. However, the impact of functional status on outcomes in an ACLF cohort is largely unknown.</div></div><div><h3>Methods</h3><div>The United Network for Organ Sharing (UNOS) Standard Transplant Analysis and Research (STAR) Database was utilized to study LT patients between 1987 and 2019. Patients were categorized by ACLF grades and further divided within each grade based on their level of assistance—no, some, or total—using KPS scores. The primary outcomes assessed were graft failure and all-cause mortality post-transplant. The secondary outcomes assessed were mortality secondary to specific organ system failures.</div></div><div><h3>Results</h3><div>Patients without ACLF requiring some (aHR 1.10, 95 %CI 1.04–1.17, <em>p</em> = 0.002) or total assistance (aHR 1.32, 95 %CI 1.22–1.43, <em>p</em> &lt; 0.001) showed increased risk of all-cause mortality. Those needing total assistance also faced a higher risk of graft failure (aHR 1.34, 95 %CI 1.13–1.58, <em>p</em> &lt; 0.001). However, functional status did not significantly impact post-transplant outcomes across all ACLF grades.</div></div><div><h3>Conclusion</h3><div>Functional status was not a significant predictor of post-transplant outcomes in ACLF patients, regardless of ACLF severity. <em>Poor functional scores in multi-organ failure likely reflect acute critical illness rather than baseline frailty.</em></div></div>","PeriodicalId":100799,"journal":{"name":"Journal of Liver Transplantation","volume":"19 ","pages":"Article 100286"},"PeriodicalIF":0.0,"publicationDate":"2025-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144596636","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Outcomes of SARS-CoV-2 infection in liver transplant recipients of a large volume transplant center in Latin America 拉丁美洲某大容量移植中心肝移植受者SARS-CoV-2感染结局
Pub Date : 2025-06-24 DOI: 10.1016/j.liver.2025.100284
Camila Sotomayor , Daniel García , María Elvira Balcells , Felipe Muñoz , Karen Muñoz , María Pilar Domínguez , Alejandra Cancino , Rodrigo Wolff , Francisco Barrera , Carlos Benítez , Luis Díaz , Eduardo Briceño , Eduardo Viñuela , Pablo Achurra , Patricia Rebolledo , Nicolas Jarufe , María Magdalena Vera , Martin Dib , Jorge A. Martínez

Introduction and Objectives

The COVID-19 pandemic significantly affected liver transplantation (LT) worldwide, with higher mortality observed in patients with chronic diseases. However, the impact of COVID-19 on transplant recipients, particularly those on immunosuppressive therapy, has been variably reported.
Our center’s historical 90-day LT mortality is 8%, and with waiting list mortality exceeding 35%, we kept our program operational, pausing elective Living Donor Liver Transplant (LDLT) for three months to minimize donor risk.
This study evaluates LT outcomes during the pandemic, particularly SARS-CoV-2 infection-related mortality, in 104 patients transplanted during the first COVID-19 wave.

Materials and Methods

We conducted a retrospective review of patients who underwent LT between January 1, 2020, and December 31, 2021, at our center in Santiago, Chile. All recipients tested negative for SARS-CoV-2 pre-transplant, and COVID-19 cases were tracked postoperatively.

Results

Among 104 adult patients, 84% were elective cases, 15% emergency, and 1% re-transplant. The mean age was 56.2 ±12.5; 56% male. The most frequent indications were NAFLD (41%), hepatocarcinoma (23%), autoimmune hepatitis (16%), and alcoholic liver disease (14%). Deceased donors provided 79.8% of the grafts, while living donors accounted for 20.2%. The mean MELD score was 22.5± 9.5. Nineteen recipients (18.3%) acquired postoperative RT-PCR-confirmed SARS-CoV-2 infection; 73.6% were symptomatic, and 26.3 % had early infections. Most had mild symptoms of COVID-19, requiring only symptomatic treatment (10/19; 52.6%). One patient required non-invasive mechanical ventilation (5.3%), and 3 required invasive mechanical ventilation (3/19;15.8%), with mortality in all of them. The overall 90-day post-transplant mortality rate in the cohort was 7.7%. Among non-infected patients, it was 5.9%, while in recipients with SARS-CoV-2 infection, it reached 15.8%. In early infected patients, mortality was 40% (2/5).

Conclusions

In conclusion, while SARS-CoV-2 infection significantly affected LT recipients, the post-transplant mortality in infected patients remained lower than waiting list mortality.
新冠肺炎大流行对全球肝移植(LT)有显著影响,慢性疾病患者的死亡率更高。然而,COVID-19对移植受者的影响,特别是对那些接受免疫抑制治疗的移植受者的影响,已有不同的报道。我们中心历史上90天肝移植死亡率为8%,而等待名单死亡率超过35%,我们保持了我们的项目运作,暂停选择性活体肝移植(LDLT)三个月,以尽量减少供体风险。本研究评估了在第一次COVID-19浪潮期间移植的104例患者在大流行期间的肝移植结果,特别是SARS-CoV-2感染相关的死亡率。材料和方法我们在智利圣地亚哥的中心对2020年1月1日至2021年12月31日期间接受肝移植的患者进行了回顾性研究。所有受者移植前SARS-CoV-2检测均为阴性,术后追踪COVID-19病例。结果104例成人患者中,择期占84%,急诊占15%,再移植占1%。平均年龄56.2±12.5岁;56%的男性。最常见的适应症是NAFLD(41%)、肝癌(23%)、自身免疫性肝炎(16%)和酒精性肝病(14%)。死者供者占移植总量的79.8%,而活体供者占20.2%。MELD平均评分为22.5±9.5分。19例(18.3%)术后获得经rt - pcr确诊的SARS-CoV-2感染;有症状者占73.6%,早期感染占26.3%。大多数COVID-19症状轻微,只需要对症治疗(10/19;52.6%)。无创机械通气1例(5.3%),有创机械通气3例(3/19,15.8%),均有死亡率。移植后90天总死亡率为7.7%。在未感染的患者中,这一比例为5.9%,而在SARS-CoV-2感染的患者中,这一比例达到15.8%。早期感染患者死亡率为40%(2/5)。结论虽然SARS-CoV-2感染对肝移植受者有显著影响,但感染患者的移植后死亡率仍低于等候名单死亡率。
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引用次数: 0
Current knowledge about immunotherapy response after liver transplantation of patients with liver cancer 肝癌患者肝移植后免疫治疗反应的现状
Pub Date : 2025-06-06 DOI: 10.1016/j.liver.2025.100285
W. Gaya Shivega , Xin Wei Wang , Shay Behrens
Hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (iCCA) are the two main liver cancers responsible for cancer deaths worldwide. Multiple etiologies exist driving these diseases; however, there are limited effective treatments to date. Recent studies have demonstrated improved outcomes in patients with advanced disease treated with immune checkpoint inhibition (ICI). Further, as these patients undergo liver transplantation, it’s critical to have an understanding of the impact of ICI on the immune system post-transplantation. In this review, we will provide an overview on ICI therapy in liver cancer, ICI utilization in the peri-transplantation setting, and discuss molecular predictions to immunotherapy response.
肝细胞癌(HCC)和肝内胆管癌(iCCA)是世界范围内导致癌症死亡的两种主要肝癌。这些疾病存在多种病因;然而,迄今为止有效的治疗方法有限。最近的研究表明,免疫检查点抑制(ICI)可以改善晚期疾病患者的预后。此外,由于这些患者接受肝移植,了解ICI对移植后免疫系统的影响至关重要。在这篇综述中,我们将提供肝癌中ICI治疗的概述,ICI在移植周围环境中的应用,并讨论免疫治疗反应的分子预测。
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引用次数: 0
Rapid defatting of macrosteatotic liver grafts: A radiologic proof-of-concept study 大脂肪变性肝移植物的快速脱脂:一项放射学概念验证研究
Pub Date : 2025-05-30 DOI: 10.1016/j.liver.2025.100283
Pierre De Mathelin, Thierry Artzner, Baptiste Michard, Philippe Bachellier, François Faitot, Pietro Addeo
None
没有一个
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引用次数: 0
Psychological screening and follow-up care for living liver donors: 5-year prospective cohort data from a single academic center 活体肝供者的心理筛查和随访护理:来自单一学术中心的5年前瞻性队列数据
Pub Date : 2025-05-13 DOI: 10.1016/j.liver.2025.100281
Leonieke Kranenburg , Alicia Chorley , Emma Massey , Hayo ter Burg , Robert Minnee , Markus Boehnert

Aim

This study reports on the outcomes of psychological screening and care for all living donor candidates who entered our center’s program since the start in 2018, and is the first study to evaluate the use of the ELPAT Psychosocial Assessment Tool (EPAT) for this population.

Methods

All donor candidates were screened using the ELPAT Psychosocial Assessment Tool (EPAT), consisting of a structured interview with set topics and a combination of validated questionnaires. Reports of the interviews were retrieved form the medical records and analysed per topic. Data from the questionnaires were analysed with existing cut-off scores. An independent samples t-test was used to compare means of related versus unrelated donors. Data on additional pre- or post donation psychological treatment was retrieved from the medical records.

Results

137 donor candidates underwent psychology screening for living liver donation. Over half of them had sought professional mental health support and/or used psychotropic drugs in the past. However, the average scores for current anxiety and depression as measures by the questionnaires were low. Unrelated donor candidates had statistically significant lower scores on emotional support and anxiety. Of all candidates, 2 were declined for psychological reasons; 53 eventually donated part of their liver, and of these, 15 received additional psychological treatment.

Discussion

The EPAT is a useful tool for living liver donor screening, covering all important psychological domains. Providing psychological treatment on indication proved to be a feasible way for this group to deal with potential psychological complaints during the process.
目的本研究报告了自2018年开始进入本中心项目的所有活体供体候选人的心理筛查和护理结果,是第一项评估ELPAT社会心理评估工具(EPAT)在该人群中的使用情况的研究。方法使用ELPAT心理社会评估工具(EPAT)筛选所有供体候选人,包括设定主题的结构化访谈和有效问卷的组合。访谈报告从医疗记录中检索,并按主题进行分析。调查问卷的数据用现有的分值进行分析。独立样本t检验用于比较相关和非相关供体的平均值。从医疗记录中检索捐赠前后额外心理治疗的数据。结果137例活体肝捐献候选人接受了心理筛查。其中一半以上的人过去曾寻求过专业的心理健康支持和/或使用过精神药物。然而,问卷调查显示,当前焦虑和抑郁的平均得分很低。无血缘关系的捐赠者候选人在情感支持和焦虑方面得分明显较低。在所有候选人中,有2人因心理原因被拒绝;53人最终捐献了部分肝脏,其中15人接受了额外的心理治疗。EPAT是活体肝供者筛选的有用工具,涵盖所有重要的心理领域。根据适应证提供心理治疗是该群体处理过程中潜在心理投诉的可行方法。
{"title":"Psychological screening and follow-up care for living liver donors: 5-year prospective cohort data from a single academic center","authors":"Leonieke Kranenburg ,&nbsp;Alicia Chorley ,&nbsp;Emma Massey ,&nbsp;Hayo ter Burg ,&nbsp;Robert Minnee ,&nbsp;Markus Boehnert","doi":"10.1016/j.liver.2025.100281","DOIUrl":"10.1016/j.liver.2025.100281","url":null,"abstract":"<div><h3>Aim</h3><div>This study reports on the outcomes of psychological screening and care for all living donor candidates who entered our center’s program since the start in 2018, and is the first study to evaluate the use of the ELPAT Psychosocial Assessment Tool (EPAT) for this population.</div></div><div><h3>Methods</h3><div>All donor candidates were screened using the ELPAT Psychosocial Assessment Tool (EPAT), consisting of a structured interview with set topics and a combination of validated questionnaires. Reports of the interviews were retrieved form the medical records and analysed per topic. Data from the questionnaires were analysed with existing cut-off scores. An independent samples <em>t</em>-test was used to compare means of related versus unrelated donors. Data on additional pre- or post donation psychological treatment was retrieved from the medical records.</div></div><div><h3>Results</h3><div>137 donor candidates underwent psychology screening for living liver donation. Over half of them had sought professional mental health support and/or used psychotropic drugs in the past. However, the average scores for current anxiety and depression as measures by the questionnaires were low. Unrelated donor candidates had statistically significant lower scores on emotional support and anxiety. Of all candidates, 2 were declined for psychological reasons; 53 eventually donated part of their liver, and of these, 15 received additional psychological treatment.</div></div><div><h3>Discussion</h3><div>The EPAT is a useful tool for living liver donor screening, covering all important psychological domains. Providing psychological treatment on indication proved to be a feasible way for this group to deal with potential psychological complaints during the process.</div></div>","PeriodicalId":100799,"journal":{"name":"Journal of Liver Transplantation","volume":"19 ","pages":"Article 100281"},"PeriodicalIF":0.0,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144098523","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Graft versus host disease after liver transplantation: A single center case series 肝移植后移植物抗宿主病:单中心病例系列
Pub Date : 2025-05-09 DOI: 10.1016/j.liver.2025.100282
Erin Horsfall , Peter Browett , Amanda Charlton , Edward Gane

Background

Graft-versus-host disease (GVHD) after liver transplantation (LT) is a rare and usually fatal complication. Recent advances in diagnosis and treatment have improved outcomes. Understanding pre and post-transplant risk factors, early clinical features, and targeted treatment strategies are vital for optimal management.

Methods

We conducted a retrospective case series of GVHD after LT in the New Zealand Liver Transplant Unit (NZLTU). Patients were identified from a prospectively maintained database and clinical data were analyzed to assess risk factors, clinical presentations, treatments, and outcomes.

Results

Among the 873 LT recipients, six (0.7 %) developed GVHD. The median time of GVHD was 37 days post transplantation, usually presenting with skin and gastrointestinal involvement. Initial management involved immunosuppression reduction and corticosteroid therapy, with refractory cases being treated with ruxolitinib or basiliximab. The overall mortality rate was 66 %. Liver re-transplantation was pursued in one case following GVHD remission, which was complicated by GVHD recurrence after liver re-transplantation, a first-ever reported clinical case.

Conclusions

GVHD after LT is associated with significant morbidity and mortality. Prompt recognition, early intervention, and close monitoring are crucial to improve patient outcomes. Early treatment with ruxolitinib should be considered in the treatment of GVHD after LT.
背景:肝移植术后移植物抗宿主病(GVHD)是一种罕见且通常致命的并发症。诊断和治疗方面的最新进展改善了结果。了解移植前后的危险因素、早期临床特征和有针对性的治疗策略对优化治疗至关重要。方法我们对新西兰肝移植中心(NZLTU)肝移植后GVHD的回顾性病例系列进行了研究。从前瞻性维护的数据库中识别患者,并分析临床数据以评估风险因素、临床表现、治疗和结果。结果873例肝移植受者中,6例(0.7%)发生GVHD。GVHD的中位时间为移植后37天,通常表现为皮肤和胃肠道受累。最初的治疗包括减少免疫抑制和皮质类固醇治疗,难治性病例用ruxolitinib或basiliximab治疗。总死亡率为66%。1例GVHD缓解后并发GVHD再移植,这是首次报道的临床病例。结论肝移植后静脉血栓淤积与发病率和死亡率显著相关。及时识别、早期干预和密切监测对改善患者预后至关重要。LT后GVHD的治疗应考虑早期应用ruxolitinib治疗。
{"title":"Graft versus host disease after liver transplantation: A single center case series","authors":"Erin Horsfall ,&nbsp;Peter Browett ,&nbsp;Amanda Charlton ,&nbsp;Edward Gane","doi":"10.1016/j.liver.2025.100282","DOIUrl":"10.1016/j.liver.2025.100282","url":null,"abstract":"<div><h3>Background</h3><div>Graft-versus-host disease (GVHD) after liver transplantation (LT) is a rare and usually fatal complication. Recent advances in diagnosis and treatment have improved outcomes. Understanding pre and post-transplant risk factors, early clinical features, and targeted treatment strategies are vital for optimal management.</div></div><div><h3>Methods</h3><div>We conducted a retrospective case series of GVHD after LT in the New Zealand Liver Transplant Unit (NZLTU). Patients were identified from a prospectively maintained database and clinical data were analyzed to assess risk factors, clinical presentations, treatments, and outcomes.</div></div><div><h3>Results</h3><div>Among the 873 LT recipients, six (0.7 %) developed GVHD. The median time of GVHD was 37 days post transplantation, usually presenting with skin and gastrointestinal involvement. Initial management involved immunosuppression reduction and corticosteroid therapy, with refractory cases being treated with ruxolitinib or basiliximab. The overall mortality rate was 66 %. Liver re-transplantation was pursued in one case following GVHD remission, which was complicated by GVHD recurrence after liver re-transplantation, a first-ever reported clinical case.</div></div><div><h3>Conclusions</h3><div>GVHD after LT is associated with significant morbidity and mortality. Prompt recognition, early intervention, and close monitoring are crucial to improve patient outcomes. Early treatment with ruxolitinib should be considered in the treatment of GVHD after LT.</div></div>","PeriodicalId":100799,"journal":{"name":"Journal of Liver Transplantation","volume":"19 ","pages":"Article 100282"},"PeriodicalIF":0.0,"publicationDate":"2025-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144071613","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The impact of recipient age on the post-liver transplant prognosis of simultaneous liver and kidney transplantation 受者年龄对同期肝肾移植术后预后的影响
Pub Date : 2025-04-28 DOI: 10.1016/j.liver.2025.100275
D.U. Lee , K.J. Lee , Y.J. Cha , G.H. Fan , M.R. Shaik , K. Bhowmick , H. Chou , C. Sun , H. Chou , R. Malik

Background & Aims

Simultaneous liver and kidney transplant (SLKT) can be a life-saving procedure for those with liver failure and concomitant kidney disease. This study evaluates the post-transplant outcomes and prognosis based on the recipients’ age using the UNOS-STAR database.

Methods

Using the UNOS-STAR registry (2005–2019), patients who underwent SLKT were selected. They were stratified by the recipients’ each age cohort. Those under the age of 18, retransplantation, and with prior liver transplantation were excluded. Iterative Cox regression was performed on multiple mortality endpoints to assess the prognostic effects.

Results

From 5748 SLKT patients, there were five age groups of recipients: ages 18–39 (n = 371), quadragenarian (n = 759), quinquagenarian (n = 2112), sexagenarian (n = 2202), and septuagenarian (n = 183). The median follow-up time was 3.03 years (25–75 % IQR: 1.02–7.00). Assessing the primary endpoints, the sexagenarian recipients had a significantly lower incidence of graft failure compared to the controls (aHR 0.36, 95 % CI 0.19–0.68, p = 0.002). The septuagenarian recipients experienced a higher incidence of all-cause mortality compared to the controls (aHR 2.08, 95 % CI 1.34–3.21, p = 0.001). However, the quadragenarian and quinquagenarian recipients did not have significant differences in all-cause mortality and graft failure. Evaluating the end-organ outcomes, there were no significant differences obtained among the recipients with various age cohorts.

Conclusion

This study demonstrates the advanced age of transplant recipients with SLKT was associated with increased all-cause mortality. Therefore, certain groups of SLKT recipients had differential impacts based on the chronicle ages.
背景,目的:肝肾同步移植(SLKT)对于肝功能衰竭和伴随肾脏疾病的患者可能是一种挽救生命的手术。本研究使用UNOS-STAR数据库评估基于受者年龄的移植后结果和预后。方法使用UNOS-STAR注册表(2005-2019),选择接受SLKT的患者。他们按受助人的每个年龄段进行分层。排除年龄在18岁以下、再移植和既往肝移植的患者。对多个死亡率终点进行迭代Cox回归,以评估预后影响。结果5748例SLKT患者中,接受治疗的患者分为5个年龄组:18-39岁(371例)、四岁(759例)、五岁(2112例)、六十岁(2202例)和七十岁(183例)。中位随访时间为3.03年(25 - 75% IQR: 1.02-7.00)。评估主要终点,与对照组相比,60岁以上受体的移植物衰竭发生率显著降低(aHR 0.36, 95% CI 0.19-0.68, p = 0.002)。与对照组相比,70多岁的受体患者的全因死亡率更高(aHR 2.08, 95% CI 1.34-3.21, p = 0.001)。然而,四岁和五岁的受体在全因死亡率和移植物衰竭方面没有显著差异。在评估终末器官结果时,不同年龄组的受者之间没有显著差异。结论:该研究表明,SLKT移植受者的高龄与全因死亡率增加有关。因此,某些SLKT接受者群体的影响是不同的。
{"title":"The impact of recipient age on the post-liver transplant prognosis of simultaneous liver and kidney transplantation","authors":"D.U. Lee ,&nbsp;K.J. Lee ,&nbsp;Y.J. Cha ,&nbsp;G.H. Fan ,&nbsp;M.R. Shaik ,&nbsp;K. Bhowmick ,&nbsp;H. Chou ,&nbsp;C. Sun ,&nbsp;H. Chou ,&nbsp;R. Malik","doi":"10.1016/j.liver.2025.100275","DOIUrl":"10.1016/j.liver.2025.100275","url":null,"abstract":"<div><h3>Background &amp; Aims</h3><div>Simultaneous liver and kidney transplant (SLKT) can be a life-saving procedure for those with liver failure and concomitant kidney disease. This study evaluates the post-transplant outcomes and prognosis based on the recipients’ age using the UNOS-STAR database.</div></div><div><h3>Methods</h3><div>Using the UNOS-STAR registry (2005–2019), patients who underwent SLKT were selected. They were stratified by the recipients’ each age cohort. Those under the age of 18, retransplantation, and with prior liver transplantation were excluded. Iterative Cox regression was performed on multiple mortality endpoints to assess the prognostic effects.</div></div><div><h3>Results</h3><div>From 5748 SLKT patients, there were five age groups of recipients: ages 18–39 (<em>n</em> = 371), quadragenarian (<em>n</em> = 759), quinquagenarian (<em>n</em> = 2112), sexagenarian (<em>n</em> = 2202), and septuagenarian (<em>n</em> = 183). The median follow-up time was 3.03 years (25–75 % IQR: 1.02–7.00). Assessing the primary endpoints, the sexagenarian recipients had a significantly lower incidence of graft failure compared to the controls (aHR 0.36, 95 % CI 0.19–0.68, <em>p</em> = 0.002). The septuagenarian recipients experienced a higher incidence of all-cause mortality compared to the controls (aHR 2.08, 95 % CI 1.34–3.21, <em>p</em> = 0.001). However, the quadragenarian and quinquagenarian recipients did not have significant differences in all-cause mortality and graft failure. Evaluating the end-organ outcomes, there were no significant differences obtained among the recipients with various age cohorts.</div></div><div><h3>Conclusion</h3><div>This study demonstrates the advanced age of transplant recipients with SLKT was associated with increased all-cause mortality. Therefore, certain groups of SLKT recipients had differential impacts based on the chronicle ages.</div></div>","PeriodicalId":100799,"journal":{"name":"Journal of Liver Transplantation","volume":"19 ","pages":"Article 100275"},"PeriodicalIF":0.0,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105912","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Initial hepatic artery reperfusion for complex portal vein thrombosis in living donor liver transplantation: A case report 活体肝移植并发复杂门静脉血栓的初始肝动脉再灌注1例
Pub Date : 2025-04-15 DOI: 10.1016/j.liver.2025.100276
Takako Yamada Fujii , Koichi Tanaka , Takuya Kimura
{"title":"Initial hepatic artery reperfusion for complex portal vein thrombosis in living donor liver transplantation: A case report","authors":"Takako Yamada Fujii ,&nbsp;Koichi Tanaka ,&nbsp;Takuya Kimura","doi":"10.1016/j.liver.2025.100276","DOIUrl":"10.1016/j.liver.2025.100276","url":null,"abstract":"","PeriodicalId":100799,"journal":{"name":"Journal of Liver Transplantation","volume":"18 ","pages":"Article 100276"},"PeriodicalIF":0.0,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143869762","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pure living donor liver transplant for liver and polycystic kidney disease, outcomes and experiences from an Asian transplant unit 纯活体供肝移植治疗肝脏和多囊肾疾病,来自亚洲移植单位的结果和经验
Pub Date : 2025-04-11 DOI: 10.1016/j.liver.2025.100272
Darren W. Chua , Horng-ren Yang , Ping Chun Li , Sheng-Hsien Chen , Shih Chao Hsu , Te-Hong Chen , Hsueh-Chou Lai , Ching-Feng Wu , You-Cian Lin , Long-Bin Jeng

Background

Adult polycystic liver disease (PLD) is a rare genetic disorder. In instances of intractable symptoms from mass effect or recurrent cyst-related complications, liver transplant affords the best therapeutic solution.

Methods

This is a retrospective case series of patients who underwent isolated pure living donor liver transplantation (LDLT) for adult polycystic kidney disease (PCKD) associated PLD from 2012- 2022. Short-term peri‑operative outcomes including renal sequelae and long-term survival were analysed.

Results

From 2012–2022, a total of 9 patients underwent LDLT for PLD. The median explanted liver weight was 6950 g (IQR 5653–7700) with a median blood loss of was 5450mls (IQR 2400–9875). There was 1 mortality due to acute antibody-mediated rejection (11.1 %). Of the four patients with existing chronic kidney disease (CKD), 2 (50 %) developed end-stage renal failure and was renal replacement therapy (RRT) dependent at 3 months. The median overall survival in our series was 63.9 months with a 5-year survival of 88.9 %.

Conclusions

Despite its technical challenges, LDLT affords an excellent long-term prognosis with acceptable major morbidity. Pre-operative CKD appears to be a risk factor for early renal deterioration.
背景成人多囊肝病(PLD)是一种罕见的遗传性疾病。方法这是一项回顾性病例系列研究,研究对象是2012-2022年期间因成人多囊肾病(PCKD)相关多囊肝病而接受离体纯活体肝移植(LDLT)的患者。结果从2012年到2022年,共有9名患者接受了LDLT治疗。切除肝脏的中位重量为 6950 克(IQR 5653-7700),中位失血量为 5450 毫升(IQR 2400-9875)。有一名患者因急性抗体介导的排斥反应而死亡(11.1%)。在4名患有慢性肾病(CKD)的患者中,有2人(50%)发展为终末期肾衰竭,3个月后需要接受肾脏替代治疗(RRT)。在我们的系列研究中,总生存期的中位数为 63.9 个月,5 年生存率为 88.9%。术前慢性肾脏病似乎是导致早期肾功能恶化的一个危险因素。
{"title":"Pure living donor liver transplant for liver and polycystic kidney disease, outcomes and experiences from an Asian transplant unit","authors":"Darren W. Chua ,&nbsp;Horng-ren Yang ,&nbsp;Ping Chun Li ,&nbsp;Sheng-Hsien Chen ,&nbsp;Shih Chao Hsu ,&nbsp;Te-Hong Chen ,&nbsp;Hsueh-Chou Lai ,&nbsp;Ching-Feng Wu ,&nbsp;You-Cian Lin ,&nbsp;Long-Bin Jeng","doi":"10.1016/j.liver.2025.100272","DOIUrl":"10.1016/j.liver.2025.100272","url":null,"abstract":"<div><h3>Background</h3><div>Adult polycystic liver disease (PLD) is a rare genetic disorder. In instances of intractable symptoms from mass effect or recurrent cyst-related complications, liver transplant affords the best therapeutic solution.</div></div><div><h3>Methods</h3><div>This is a retrospective case series of patients who underwent isolated pure living donor liver transplantation (LDLT) for adult polycystic kidney disease (PCKD) associated PLD from 2012- 2022. Short-term peri‑operative outcomes including renal sequelae and long-term survival were analysed.</div></div><div><h3>Results</h3><div>From 2012–2022, a total of 9 patients underwent LDLT for PLD. The median explanted liver weight was 6950 g (IQR 5653–7700) with a median blood loss of was 5450mls (IQR 2400–9875). There was 1 mortality due to acute antibody-mediated rejection (11.1 %). Of the four patients with existing chronic kidney disease (CKD), 2 (50 %) developed end-stage renal failure and was renal replacement therapy (RRT) dependent at 3 months. The median overall survival in our series was 63.9 months with a 5-year survival of 88.9 %.</div></div><div><h3>Conclusions</h3><div>Despite its technical challenges, LDLT affords an excellent long-term prognosis with acceptable major morbidity. Pre-operative CKD appears to be a risk factor for early renal deterioration.</div></div>","PeriodicalId":100799,"journal":{"name":"Journal of Liver Transplantation","volume":"18 ","pages":"Article 100272"},"PeriodicalIF":0.0,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143864091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Journal of Liver Transplantation
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