Acute on chronic liver failure (ACLF) is a unique syndrome that afflicts patients with chronic liver disease and results in high short-term mortality, in the setting of organ system failures. Given this prognosis, there is an urgent need to understand risk factors for this condition, for appropriate medical management of organ failures, and for selection criteria for patients who may benefit from liver transplantation (LT). Although several definitions exist to identify ACLF, all of them are designed to identify patients with uniquely high mortality. Currently, management of severe ACLF relies on best supportive care for specific organ failures. Thromboelastography should guide the evaluation of coagulation pathways and hyperfibrinolysis in ACLF; prophylactic blood product transfusions and thrombopoetin agonists are not recommended. Combination therapy with terlipressin and albumin has been shown to be efficacious in the management of the hepatorenal syndrome but should be administered with caution in patients with ACLF-3. Recent data have characterized the role of beta-blockers and transjugular intrahepatic portosystemic shunt placement in the management of ACLF. Investigational therapies such as extracorporeal liver support and hepatocyte stem cell therapies have shown promise; larger scale studies may better define the subpopulations of patients with ACLF mostly likely to benefit from these evolving therapeutics. Regarding LT in ACLF, data suggest that even patients with 3 or more organ system failures may have a 1-year survival >80%. However, further efforts are needed to understand the predictors of post-LT survival to facilitate LT criteria for this condition.

中文翻译：

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慢性肝衰竭的严重急性治疗：器官衰竭的管理、研究性治疗以及肝移植的作用。

慢性肝衰竭急性发作 (ACLF) 是一种独特的综合征，在器官系统衰竭的情况下，会影响慢性肝病患者并导致较高的短期死亡率。鉴于这种预后，迫切需要了解这种情况的危险因素，对器官衰竭进行适当的医疗管理，以及可能受益于肝移植（LT）的患者的选择标准。尽管存在多种识别 ACLF 的定义，但所有这些定义都是为了识别死亡率极高的患者。目前，严重 ACLF 的治疗依赖于针对特定器官衰竭的最佳支持治疗。血栓弹力图应指导 ACLF 凝血途径和纤溶亢进的评估；不建议预防性输注血液制品和血小板生成素激动剂。特利加压素和白蛋白联合治疗已被证明可有效治疗肝肾综合征，但 ACLF-3 患者应谨慎使用。最近的数据描述了 β 受体阻滞剂和经颈静脉肝内门体分流术在 ACLF 治疗中的作用。体外肝脏支持和肝细胞干细胞疗法等研究疗法已显示出希望；更大规模的研究可能会更好地定义最有可能受益于这些不断发展的治疗方法的 ACLF 患者亚群。关于 ACLF 中的 LT，数据表明，即使患有 3 个或更多器官系统衰竭的患者，其 1 年生存率也可能 >80%。然而，需要进一步努力了解 LT 后生存的预测因素，以促进针对这种情况的 LT 标准。