Wilson病引起的急性肝功能衰竭 = 13) 未经肝移植的血浆分离法抢救

IF 0.3 Q4 PEDIATRICS Journal of Child Science Pub Date : 2021-01-01 DOI:10.1055/s-0041-1731079
Nida Mirza, R. Bharadwaj, Smita Malhotra, A. Sibal
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引用次数: 0

摘要

摘要Wilson病(WD)是一种铜代谢紊乱,导致铜在人体重要器官(主要是肝脏和大脑)积聚。WD的急性肝功能衰竭预后不良,尤其是在修订的WD预后指数中得分≥11;在这种情况下,紧急肝移植被认为是唯一可以挽救生命的选择。在这里,我们报道了一名WD诱导的肝衰竭和预后评分差的女孩患者,她通过血浆置换术得到了挽救。她还表现出严重的库姆斯阴性溶血性贫血和急性肾损伤。本病例报告强调了除肝移植外的一种辅助方式在WD引起的暴发性肝衰竭治疗中的作用。
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Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis
Abstract Wilson disease (WD) is a disorder of copper metabolism resulting in accumulation of copper in vital organs of the human body, predominantly in the liver and the brain. Acute liver failure in WD has a bad prognosis, especially with a score ≥11 in the revised WD prognostic index; emergency liver transplantation is considered the only life-saving option in this scenario. Here, we reported a girl patient with WD-induced liver failure and poor prognostic score who was rescued by plasmapheresis. She also manifested severe Coombs negative hemolytic anemia and acute kidney injury. This case report highlights the utility of an adjunctive modality besides liver transplantation for the management of fulminant liver failure caused by WD.
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