Infantile giant cell hepatitis with autoimmune hemolytic anemia
Poddighe D. Madiyeva A. Talipova D. Umirbekova B.
2021Baishideng Publishing Group Co
World Journal of Hepatology
2021#13Issue 4411 - 420 pp.
Giant cell hepatitis (GCH) is characterized by large and multinucleated (syncytial) hepatocytes in the context of liver inflammation. Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity. The etiology is unknown; concomitant viral infections (as potential trigger factors) have been identified in few patients. The pathogenesis reportedly relies upon immune-mediated/ autoimmune mechanisms. This condition should be considered in any infant developing Coombs-positive anemia; indeed, anemia usually precedes the development of hepatitis. The clinical course is usually aggressive without the appropriate immunosuppressive therapy, which may include steroids, conventional immunosuppressors (e.g., azathioprine and cyclophosphamide as first-line treatments), intravenous immunoglobulin, and biologics (rituximab). Improvements in medical management (including the availability of rituximab) have significantly reduced the mortality of this condition in the last decade.
Autoimmune hemolytic anemia , Giant cell hepatitis , Hyperbilirubinemia , Infantile hepatitis , Jaundice , Rituximab
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Department of Medicine, Nazarbayev University School of Medicine, Nur-Sultan, 010000, Kazakhstan
Department of Pediatrics, National Research Center for Maternal and Child Health, Nur-Sultan, 010000, Kazakhstan
Department of Medicine
Department of Pediatrics
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