Faculty of Medicine, University of Coimbra, Portugal.
Department of Pediatric Surgery, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, Portugal; Pediatric Liver Transplantation, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, Portugal.
Transplant Rev (Orlando). 2021 Dec;35(4):100631. doi: 10.1016/j.trre.2021.100631. Epub 2021 Jun 1.
Liver transplantation (LT) remains the standard of care in the treatment of acute pediatric liver failure (PALF) for the replacement of a severely damaged native liver in patients who are unlikely to recover. However, this is burdened by the consequences of long-term immunosuppression. Auxiliary partial liver orthotopic transplantation (APOLT) has emerged as a possible improved approach, by providing a graft that assures liver function until the regeneration of the native liver occurs, and then allows for possible progression to immunosuppression withdrawal. No previous systematic review has assessed APOLT for PALF. The aim of this work is to provide information on survival, postoperative complications, and withdrawal of immunosuppression after APOLT for PALF.
The study was carried out according to the recommendations of the preferred report items for systematic reviews and meta-analyzes (PRISMA). We searched several electronic databases until October 31st, 2020, using the search terms "acute liver failure", "auxiliary liver transplant" and the MESH term "liver failure, acute". All types of clinical publications that presented results on APOLT for PALF, in English or Portuguese, and restricted to humans and for children under 18 years old were included. The following exclusion criteria were applied: "follow-up time <6 months", "does not report complications" and "does not report immunosuppression regimen (double vs triple)". Demographic data, clinical characteristics at the time of surgery and postoperative results were analyzed.
A total of 14 references (including 45 patients) were selected, including 3 case series (6-20 patients) and 11 case reports. Of the 45 subjects, 33 (73.3%) were male and 12 (26.7%) female. In most cases (n = 30; 66.7%), the cause of PALF was undetermined. All patients underwent APOLT. Their median age was 9 (range 0.6-17) years. In the postoperative period, the immunosuppression regimen was double in 34 (75.6%) and triple in 11 (24.4%) individuals. The main postoperative complications were rejection and infection. Over a follow-up period of 6 months to 14 years, 10 (22.2%) patients died. The main cause of death was sepsis (70%). Six (13.3%) patients were retransplanted. Of the survivors (n = 35), 68.6% achieved complete withdrawal from the immunosuppression regimen.
Based on current published evidence, APOLT for the treatment of PALF is a safe option, with an acceptable rate of complications and mortality. It has the great advantage of providing an immunosuppression-free life in the majority (68.6%) of survivors.
肝移植（LT）仍然是治疗急性儿科肝衰竭（PALF）的标准治疗方法，适用于那些不太可能恢复的患者，用严重受损的自体肝脏替代。然而，这是长期免疫抑制的后果。辅助部分肝原位移植（APOLT）作为一种可能的改进方法出现了，它提供了一个移植物，在自体肝再生之前保证肝功能，然后允许可能进展到免疫抑制剂的撤除。以前没有系统评价评估过 APOLT 在 PALF 中的应用。本研究的目的是提供关于 PALF 患者接受 APOLT 后的存活率、术后并发症和免疫抑制剂撤药的信息。
该研究按照系统评价和荟萃分析的首选报告项目（PRISMA）的建议进行。我们使用了 "急性肝衰竭"、"辅助性肝移植 "和 "肝衰竭，急性" 的 MeSH 术语，在 2020 年 10 月 31 日之前在几个电子数据库中进行了搜索。纳入了所有以英语或葡萄牙语发表的关于 APOLT 在 PALF 中的应用的临床研究，包括病例系列研究（6-20 例患者）和病例报告，限制为人类和 18 岁以下的儿童。应用了以下排除标准："随访时间<6 个月"、"不报告并发症" 和 "不报告免疫抑制剂方案（双 vs 三）"。分析了人口统计学数据、手术时的临床特征和术后结果。
共选择了 14 篇参考文献（包括 45 例患者），包括 3 个病例系列研究（6-20 例患者）和 11 个病例报告。在 45 名患者中，33 名（73.3%）为男性，12 名（26.7%）为女性。在大多数情况下（n=30；66.7%），PALF 的病因未明。所有患者均行 APOLT 治疗。他们的中位年龄为 9 岁（范围 0.6-17 岁）。在术后期间，免疫抑制剂方案为双的有 34 例（75.6%），三的有 11 例（24.4%）。主要的术后并发症是排斥和感染。在 6 个月至 14 年的随访期间，10 例（22.2%）患者死亡。主要死因是败血症（70%）。6 例（13.3%）患者进行了再次移植。在幸存者（n=35）中，68.6%的患者完全停止了免疫抑制剂治疗。
根据目前已发表的证据，APOLT 治疗 PALF 是一种安全的选择，并发症和死亡率可接受。它有一个很大的优势，即在大多数（68.6%）幸存者中提供无免疫抑制剂的生活。
