Case Report
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Pediatr. Jun 9, 2024; 13(2): 92263
Published online Jun 9, 2024. doi: 10.5409/wjcp.v13.i2.92263
Unique presentation of neonatal liver failure: A case report
Eman Al Atrash, Amer Azaz, Samar Said, Mohammad Miqdady
Eman Al Atrash, Amer Azaz, Mohammad Miqdady, Department of Pediatrics, Division of Pediatric Gastroenterology, Sheikh Khalifa Medical City, Abu Dhabi 971, United Arab Emirates
Samar Said, Department of Pathology, Mayo Clinic, Rochester, MN 55905, United States
Author contributions: Al Atrash E wrote the manuscript; Miqdady M and Azzaz A revised the manuscript; Said S provided the pathology slides with the caption; All authors have read and approve the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read CARE Checklist (2016), and the manuscript was prepared and revised according to CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Eman Al Atrash, MBBS, Pediatric Gastroenterology Fellow, Department of Pediatrics, Division of Pediatric Gastroenterology, Sheikh Khalifa Medical City, Karama Street, Abu Dhabi 971, United Arab Emirates. emanalatrash8@gmail.com
Received: January 20, 2024
Revised: February 13, 2024
Accepted: March 14, 2024
Published online: June 9, 2024
Processing time: 138 Days and 24 Hours
Abstract
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.

Keywords: Liver; Hyperbilirubinemia; Le foie vide; Neonatal hemochromatosis; Case report

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.