Published online Jun 9, 2024. doi: 10.5409/wjcp.v13.i2.92263
Revised: February 13, 2024
Accepted: March 14, 2024
Published online: June 9, 2024
Processing time: 138 Days and 24 Hours
Acute fulminant liver failure rarely occurs in the neonatal period. The etiologies include viral infection (15%), metabolic/genetic disease (10%), hematologic disorders (15%), and ischemic injury (5%). Gestational alloimmune liver disease usually manifests as severe neonatal liver failure, with extensive hepatic and extrahepatic iron overload, sparing the reticuloendothelial system. Empty liver failure is a rare cause of liver failure where a patient presents with liver failure in the neonatal period with no hepatocytes in liver biopsy.
A 5-week-old male presented with jaundice. Physical examination revealed an alert but deeply icteric infant. Laboratory data demonstrated direct hyperbilirubinemia, a severely deranged coagulation profile, normal transaminase, and normal ammonia. Magnetic resonance imaging of the abdomen was suggestive of perinatal hemochromatosis. Liver biopsy showed histiocytic infiltration with an absence of hepatocytes. No hemosiderin deposition was identified in a buccal mucosa biopsy.
Neonatal liver failure in the absence of hepatocellular regeneration potentially reflects an acquired or inborn defect in the regulation of hepatic regeneration.
Core Tip: We report a rare case of liver failure in which a term infant, with no history of perinatal complication, presented at age of 4-wk with an insidious onset of liver failure. We speculated that a severe liver insult may have occurred sub-clinically in the first week of life which was not detected. Regardless of the etiology of the marked hepatocyte destruction, there appears to be a. complete absence of hepatocellular regeneration, indicating a possible acquired or inborn defect in the regulation of hepatic regeneration.