成功治疗威尔逊氏病导致的急性肝衰竭:偶然还是巧合?

IF 2.5 Q2 GASTROENTEROLOGY & HEPATOLOGY
Valeria Delle Cave, Fabiola Di Dato, Pier Luigi Calvo, Maria Immacolata Spagnuolo, Raffaele Iorio
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引用次数: 0

摘要

背景:急性肝衰竭(ALF)可能是威尔逊氏病(WD)的首发症状,也是最显著的表现。WD引起的ALF(WD-ALF)很难与其他原因引起的肝病区分开来,是肝移植的明确指征。目的:对WD-ALF患者的诊断和治疗方法进行严格评估,以提高他们的原肝存活率:对2018年至2023年两个儿科肝病科的WD-ALF患者进行回顾性分析:在研究期间,16名儿童(9名男性)被诊断为WD,其中2名出现ALF。第一名患儿接受了小剂量D-青霉胺和锌加类固醇的非常规联合治疗,并成功存活,未进行肝移植。第二例患者只接受了支持疗法,需要进行肝移植才能克服 ALF:结论:用小剂量D-青霉胺和锌加类固醇成功治疗了一名WD-ALF患者,这为治疗这种目前只能通过肝移植治疗的疾病提供了新的视角。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Successful treatment of acute liver failure due to Wilson's disease: Serendipity or fortuity?

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.

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来源期刊
World Journal of Hepatology
World Journal of Hepatology GASTROENTEROLOGY & HEPATOLOGY-
CiteScore
4.10
自引率
4.20%
发文量
172
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