Unique presentation of neonatal liver failure:A case report  

作　　者：Eman Al Atrash Amer Azaz Samar Said Mohammad Miqdady 

机构地区：[1]Department of Pediatrics,Division of Pediatric Gastroenterology,Sheikh Khalifa Medical City,Abu Dhabi 971,United Arab Emirates [2]Department of Pathology,Mayo Clinic,Rochester,MN 55905,United States

出　　处：《World Journal of Clinical Pediatrics》2024年第2期221-226,共6页世界临床儿科杂志

摘　　要：BACKGROUND 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.CASE SUMMARY 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.CONCLUSION Neonatal liver failure in the absence of hepatocellular regeneration potentially reflects an acquired or inborn defect in the regulation of hepatic regeneration.

关 键 词：LIVER HYPERBILIRUBINEMIA Le foie vide Neonatal hemochromatosis Case report 

分 类 号：R722.1[医药卫生—儿科]