Division of Gastroenterology, Hepatology and Nutrition, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA.
Department Pediatric Hepatology, Ann and Robert H Lurie Children's Hospital, Chicago, Illinois, USA.
J Pediatr Gastroenterol Nutr. 2022 Jan 1;74(1):138-158. doi: 10.1097/MPG.0000000000003268.
Pediatric acute liver failure (PALF) is a rare, rapidly progressive clinical syndrome with significant morbidity and mortality. The phenotype of PALF manifests as abrupt onset liver dysfunction, which can be brought via disparate etiology. Management is reliant upon intensive clinical care and support, often provided by the collaborative efforts of hepatologists, critical care specialists, and liver transplant surgeons. The construction of an age-based diagnostic approach, the identification of a potential underlying cause, and the prompt implementation of appropriate therapy can be lifesaving; however, the dynamic and rapidly progressive nature of PALF also demands that diagnostic inquiries be paired with monitoring strategies for the recognition and treatment of common complications of PALF. Although liver transplantation can provide a potential life-saving therapeutic option, the ability to confidently determine the certainness that liver transplant is needed for an individual child has been hampered by a lack of adequately tested clinical decision support tools and accurate predictive models. Given the accelerated progress in understanding PALF, we will provide clinical guidance to pediatric gastroenterologists and other pediatric providers caring for children with PALF by presenting the most recent advances in diagnosis, management, pathophysiology, and associated outcomes.
儿科急性肝衰竭(PALF)是一种罕见的、迅速进展的临床综合征,具有显著的发病率和死亡率。PALF 的表型表现为肝功能突然异常,其病因各异。治疗依赖于强化的临床护理和支持,通常由肝病学家、重症监护专家和肝移植外科医生的共同努力提供。建立基于年龄的诊断方法、确定潜在的根本原因,并及时实施适当的治疗方法可能是救命的;然而,PALF 的动态和迅速进展性质也要求诊断探究与监测策略相匹配,以识别和治疗 PALF 的常见并发症。尽管肝移植可以提供潜在的救命治疗选择,但由于缺乏经过充分测试的临床决策支持工具和准确的预测模型,因此确定个体儿童是否需要肝移植的确定性能力受到了阻碍。鉴于对 PALF 的理解取得了加速进展,我们将通过介绍在诊断、管理、病理生理学和相关结果方面的最新进展,为儿科胃肠病学家和其他儿科提供者提供照顾 PALF 患儿的临床指导。