医学
肝移植
凝血病
肝病
重症监护医学
脑病
病因学
肝性脑病
疾病
肝衰竭
移植
儿科
内科学
肝硬化
作者
Sakil Kulkarni,Catherine A. Chapin,Estella M. Alonso,David A. Rudnick
标识
DOI:10.1016/j.cld.2022.03.007
摘要
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.
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