Successful treatment of acute liver failure due to Wilson’s disease: Serendipity or fortuity?  

作　　者：Valeria Delle Cave Fabiola Di Dato Pier Luigi Calvo Maria Immacolata Spagnuolo Raffaele Iorio 

机构地区：[1]Department of Translational Medical Science,Section of Pediatrics,University of Naples Federico II,Naples 80131,Italy [2]Pediatric Gastroenterology Unit,Regina Margherita Children's Hospital,Azienda Ospedaliera-Universitaria Cittàdella Salute e della Scienza,Turin 10126,Italy

出　　处：《World Journal of Hepatology》2024年第8期1111-1119,共9页世界肝病学杂志（英文版）（电子版）

摘　　要：BACKGROUND Acute liver failure(ALF)may be the first and most dramatic presentation of Wilson’s disease(WD).ALF due to WD(WD-ALF)is difficult to distinguish from other causes of liver disease and is a clear indication for liver transplantation.There is no firm recommendation on specific and supportive medical treatment for this condition.AIM To critically evaluate the diagnostic and therapeutic management of WD-ALF patients in order to improve their survival with native liver.METHODS A retrospective analysis of patients with WD-ALF was conducted in two pediatric liver units from 2018 to 2023.RESULTS During the study period,16 children(9 males)received a diagnosis of WD and 2 of them presented with ALF.The first was successfully treated with an unconventional combination of low doses of D-penicillamine and zinc plus steroids,and survived without liver transplant.The second,exclusively treated with supportive therapy,needed a hepatotransplant to overcome ALF.CONCLUSION Successful treatment of 1 WD-ALF patient with low-dose D-penicillamine and zinc plus steroids may provide new perspectives for management of this condition,which is currently only treated with liver transplantation.

关 键 词：Fulminant liver failure Copper CHELATOR Zinc STEROIDS Liver transplantation Autoimmune hepatitis Prognostic score 24 hour-urinary copper excretion Native liver 

分 类 号：R725.8[医药卫生—儿科] R725.7[医药卫生—临床医学] R725.5