1. Novel protocol including liver biopsy to identify and treat CD8+ T-cell predominant acute hepatitis and liver failure.
- Author
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McKenzie RB, Berquist WE, Nadeau KC, Louie CY, Chen SF, Sibley RK, Glader BE, Wong WB, Hofmann LV, Esquivel CO, and Cox KL
- Subjects
- Adolescent, Anemia, Aplastic etiology, Anemia, Aplastic therapy, Child, Child, Preschool, Female, Hepatitis immunology, Humans, Immunohistochemistry, Immunosuppression Therapy adverse effects, Immunosuppressive Agents therapeutic use, Inflammation, Liver immunology, Liver surgery, Liver Failure, Acute immunology, Liver Transplantation, Male, Retrospective Studies, Stem Cell Transplantation, Treatment Outcome, Biopsy, CD8-Positive T-Lymphocytes cytology, Hepatitis therapy, Liver pathology, Liver Failure, Acute therapy
- Abstract
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., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
- Published
- 2014
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