Novel protocol including liver biopsy to identify and treat CD8+T-cell predominant acute hepatitis and liver failure
Novel protocol including liver biopsy to identify and treat CD8+T-cell predominant acute hepatitis and liver failure
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DOI：
10.1111/petr.12296
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发表时间：
2014-08-01
影响因子：
1.3
通讯作者：
Cox, Kenneth L.
中科院分区：
文献类型：
--
作者：
McKenzie, Rebecca B.;Berquist, William E.;Cox, Kenneth L.
大多数ALF儿童的病因尚不清楚，通常需要进行肝移植才能生存。一个病人的情况下，促使我们考虑免疫失调可能是不确定的急性肝炎和肝功能衰竭的儿童的原因。我们的研究包括9名儿科患者，他们根据多学科临床协议进行治疗，以识别和治疗免疫介导的急性肝损伤。有炎症证据且活检无活动性感染的患者接受静脉注射免疫球蛋白和甲基强的松龙治疗。7例患者在治疗前或治疗后至少有一个阳性免疫标志物。所有患者都有CD8+ T细胞为主的肝损伤，对免疫治疗完全或部分应答。9名患者中有5名恢复了肝功能，不需要肝移植。其中3名患者随后发生骨髓衰竭，并接受免疫抑制或干细胞移植治疗。本系列强调了这种基于组织的诊断和治疗方法的重要性，可以提高无移植生存率。有必要进一步研究以更好地描述免疫损伤并预测可能从早期和更强的免疫抑制治疗中受益的骨髓衰竭风险患者的子集。
In the majority of children with ALF, the etiology is unknown and liver transplantation is often needed for survival. A patient case prompted us to consider that immune dysregulation may be the cause of indeterminate acute hepatitis and liver failure in children. Our study includes nine pediatric patients treated under a multidisciplinary clinical protocol to identify and treat immune-mediated acute liver injury. Patients with evidence of inflammation and no active infection on biopsy received treatment with intravenous immune globulin and methylprednisolone. Seven patients had at least one positive immune marker before or after treatment. All patients had a CD8+ T-cell predominant liver injury that completely or partially responded to immune therapy. Five of the nine patients recovered liver function and did not require liver transplantation. Three of these patients subsequently developed bone marrow failure and were treated with either immunosuppression or stem cell transplant. This series highlights the importance of this tissue-based approach to diagnosis and treatment that may improve transplant-free survival. Further research is necessary to better characterize the immune injury and to predict the subset of patients at risk for bone marrow failure who may benefit from earlier and stronger immunosuppressive therapy.