Department of Pediatrics, St. Louis Children's Hospital, One Children's Place, St Louis, MO 63110, USA.
Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Box 65, 225 E Chicago Avenue, Chicago, IL 60611, USA.
Clin Liver Dis. 2022 Aug;26(3):461-471. doi: 10.1016/j.cld.2022.03.007. Epub 2022 Jun 25.
Pediatric acute liver failure (PALF) is a complex, unpredictable, often rapidly progressive, potentially devastating clinical syndrome that occurs in infants, children, and adolescents without pre-existing liver disease. PALF is characterized by acute onset of hepatocellular injury and liver-based coagulopathy, frequently accompanied by hepatic encephalopathy. Etiologies include drug and toxin exposures, metabolic and genetic disorders, infections, and immune-mediated disease. PALF management primarily involves early contact with and consideration of transfer to a pediatric liver transplant center and intensive supportive multidisciplinary clinical care, with targeted therapies available for a subset of causes. Outcomes include survival with native liver, death, and liver transplantation. Efforts to develop reliable clinical prognostic tools to predict PALF outcomes early in the course of disease have not yet been fulfilled, and the possibility remains that some transplanted PALF patients might have survived without transplantation.
小儿急性肝衰竭（PALF）是一种复杂、不可预测、常迅速进展、潜在破坏性的临床综合征，发生于无既往肝脏疾病的婴儿、儿童和青少年。PALF 的特征为急性肝细胞损伤和肝性凝血功能障碍，常伴有肝性脑病。病因包括药物和毒素暴露、代谢和遗传疾病、感染以及免疫介导的疾病。PALF 的治疗主要包括早期接触并考虑转至儿科肝移植中心和强化支持性多学科临床治疗，针对部分病因可采用靶向治疗。结局包括存活、死亡和肝移植。目前尚未开发出可靠的临床预后工具来早期预测 PALF 结局，仍有可能一些接受移植的 PALF 患者在未经移植的情况下存活。
