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Advances in medical management of acute liver failure in children: promoting native liver survival
The Lancet Child & Adolescent Health ( IF 36.4 ) Pub Date : 2022-08-02 , DOI: 10.1016/s2352-4642(22)00190-0
Akash Deep 1 , Emma C Alexander 2 , Yonca Bulut 3 , Emer Fitzpatrick 4 , Serge Grazioli 5 , Nigel Heaton 6 , Anil Dhawan 7
Affiliation  

Paediatric acute liver failure (PALF) is defined as a biochemical evidence of acute liver injury in a child with no previous history of chronic liver disease characterised by an international normalised ratio (INR) of 1·5 or more unresponsive to vitamin K with encephalopathy, or INR of 2·0 or more with or without encephalopathy. PALF can rapidly progress to multiorgan dysfunction or failure. Although the transplant era has substantially changed the outlook for these patients, transplantation itself is not without risks, including those associated with life-long immunosuppression. Consequently, there has been an increased focus on improving medical management to prioritise bridging of patients to native liver survival, which is possible due to improved understanding of the underlying pathophysiology of multiorgan involvement in PALF. In this Review, we discuss recent advances in the medical management of PALF with an aim of reducing the need for liver transplantation. The Review will focus on the non-specific immune-mediated inflammatory response, extracorporeal support devices, neuromonitoring and neuroprotection, and emerging cellular and novel future therapeutic options.



中文翻译:

儿童急性肝衰竭的医疗管理进展:促进天然肝存活

小儿急性肝功能衰竭 (PALF) 被定义为既往无慢性肝病史的儿童出现急性肝损伤的生化证据,其特征是国际标准化比率 (INR) 为 1·5 或更高,对维生素 K 无反应并伴有脑病,或 INR 为 2·0 或更高,伴有或不伴有脑病。PALF 可迅速发展为多器官功能障碍或衰竭。尽管移植时代极大地改变了这些患者的前景,但移植本身并非没有风险,包括与终生免疫抑制相关的风险。因此,人们越来越关注改善医疗管理,以优先考虑将患者桥接到天然肝脏存活,这可能是由于对 PALF 中多器官受累的潜在病理生理学的了解有所提高。在这篇评论中,我们讨论了 PALF 医疗管理的最新进展,旨在减少对肝移植的需求。该评论将重点关注非特异性免疫介导的炎症反应、体外支持装置、神经监测和神经保护,以及新兴的细胞和新的未来治疗选择。

更新日期:2022-08-02
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