Mataya Leslie A, Srinivasan Vijay, Rand Elizabeth B, Alcamo Alicia M
Division of Critical Care Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Department of Anesthesiology and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Transl Pediatr. 2021 Oct;10(10):2749-2762. doi: 10.21037/tp-20-375.
Liver disease results in approximately 15,000 pediatric hospitalizations per year in the United States and is a significant burden to child health. Major etiologies of liver failure and indications for transplantation in children include: biliary atresia, metabolic/genetic conditions, toxins, infections, tumors, and immune-mediated liver/biliary injury. Children requiring transplantation are placed on the United Network of Organ Sharing waitlist including those with acute liver failure (ALF) and acute on chronic liver failure (ACLF). ALF is a clinical syndrome in which a previously healthy child develops rapid-onset hepatic dysfunction, and becomes critically ill with multiple organ dysfunction within days. ACLF, by contrast, is generally described as an acute decompensation of pre-existing chronic liver disease (CLD) brought on by a precipitating event, with higher risk of mortality. Children with ALF and ACLF receive multidisciplinary care in pediatric intensive care units (ICUs) due to multiple organ system involvement and high risk of decompensation in these patients. The care of these patients requires a holistic approach that addresses the complex interplay between hepatic and extra-hepatic organ systems. This review will define and describe ALF and ACLF in the pediatric population, and outline the effects of ALF and ACLF on individual organ systems with diagnostic and management considerations in the ICU while awaiting liver transplantation.
在美国,肝脏疾病每年导致约15000名儿童住院,对儿童健康造成重大负担。儿童肝衰竭的主要病因及移植指征包括:胆道闭锁、代谢/遗传疾病、毒素、感染、肿瘤以及免疫介导的肝脏/胆道损伤。需要移植的儿童会被列入器官共享联合网络等待名单,其中包括急性肝衰竭(ALF)和慢性肝病急性肝衰竭(ACLF)患儿。ALF是一种临床综合征,此前健康的儿童会迅速出现肝功能障碍,并在数天内因多器官功能障碍而病情危重。相比之下,ACLF通常被描述为由诱发事件导致的先前存在的慢性肝病(CLD)急性失代偿,死亡率更高。由于这些患者存在多器官系统受累和失代偿的高风险,ALF和ACLF患儿在儿科重症监护病房(ICU)接受多学科护理。对这些患者的护理需要一种整体方法,以解决肝脏和肝外器官系统之间复杂的相互作用。本综述将定义和描述儿科人群中的ALF和ACLF,并概述在等待肝移植期间,ALF和ACLF对各个器官系统的影响以及在ICU中的诊断和管理注意事项。