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免疫重建炎症综合征作为自身免疫性肝炎和急性肝衰竭的一个病因

Immune reconstitution inflammatory syndrome as a cause of autoimmune hepatitis and acute liver failure.

作者信息

Rodrigues Edison Moraes, Fernandes Rogério, Susin Ruth, Fior Bárbara

机构信息

Grupo de Transplante Hepático, Irmandade da Santa Casa de Misericórdia de Porto Alegre - Porto Alegre (RS), Brasil.

Unidade de Terapia Intensiva de Transplantes, Hospital Dom Vicente Scherer, Irmandade da Santa Casa de Misericórdia de Porto Alegre - Porto Alegre (RS), Brasil.

出版信息

Rev Bras Ter Intensiva. 2017 Jul-Sep;29(3):382-385. doi: 10.5935/0103-507X.20170053.

DOI:10.5935/0103-507X.20170053
PMID:29044307
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5632982/
Abstract

Acute liver failure is a rare syndrome with high mortality and is often diagnosed late. Intensivist physicians play fundamental roles in the diagnostic suspicion and the management of the multiple-organic dysfunctions characteristic of this entity. Immune reconstitution inflammatory syndrome is an entity that is characterized by the paradoxical worsening of the patient's previous condition, after the initiation of antiretrovirals, triggered against either pathogens present in the host or autoantigens. Autoimmune hepatitis has recently been described as one of these autoimmune manifestations. The authors report the first case with evolution to acute liver failure and death within a few days after the development of encephalopathy, review the cases of autoimmune hepatitis described and comment on the therapeutic possibilities in this context.

摘要

急性肝衰竭是一种死亡率高的罕见综合征,且常常诊断较晚。重症医学医生在该病症所特有的多器官功能障碍的诊断怀疑及管理中发挥着重要作用。免疫重建炎症综合征是一种在启动抗逆转录病毒治疗后,针对宿主中存在的病原体或自身抗原引发患者先前病情反常恶化的病症。自身免疫性肝炎最近被描述为这些自身免疫表现之一。作者报告了首例在出现脑病后数天内进展为急性肝衰竭并死亡的病例,回顾了已描述的自身免疫性肝炎病例,并对这种情况下的治疗可能性进行了评论。

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本文引用的文献

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Ann Intern Med. 2016 Jul 19;165(2):138-42. doi: 10.7326/M16-0560. Epub 2016 Apr 5.
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Scand J Gastroenterol. 2016 Jul;51(7):814-8. doi: 10.3109/00365521.2016.1157888. Epub 2016 Mar 22.
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Role of liver transplantation in human immunodeficiency virus positive patients.肝移植在人类免疫缺陷病毒阳性患者中的作用。
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EASL Clinical Practice Guidelines: Autoimmune hepatitis.欧洲肝脏研究学会临床实践指南:自身免疫性肝炎
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