Abstract
We report a term male infant presenting on postnatal day 1 with fulminant hepatic failure. Described congenital infection, metabolic disorders, and cardiovascular etiologies of acute neonatal liver failure were assessed and eliminated. A liver biopsy on postnatal day 10 showed neonatal giant cell hepatitis (NGCH) with an unusual degree of fibrosis for this early postnatal age. NGCH is a clinical diagnosis of cholestatic disorders of unknown etiology in the newborn, and, to our knowledge, has not been previously associated with immediate neonatal hepatic failure. The giant cell transformation is a common response to a variety of insults and only rarely occurs beyond the neonatal period. Most cases present with cholestatic jaundice and varying degrees of coagulopathy, and, many, as in this case, show progressive resolution.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$259.00 per year
only $21.58 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
Whitington PF . Fulminant hepatic failure in children In: Suchy FJ, editor Liver Disease in Children St. Louis: Mosby-Year Book 1994 180–213
Shneider BL, Setchell KDR, Whitington PF, Neilson KA, Suchy FJ . Delta-3-oxosteroid 5-beta-reductase deficiency causing neonatal liver failure and hemochromatosis J Pediatr 1994 124: 234–8
Koukoulis G, Mieli-Vergani G, Portmann B . Infantile liver giant cells: immunohistological study of their proliferative state and possible mechanisms of formation Pediatr Dev Pathol 1999 2: 353–9
Balistreri WF . Neonatal giant cell hepatitis In: Hoofnagle JH, Goodman Z, editors Liver Biopsy Interpretation for the 1990's: Clinicopathological Correlations in Liver Disease Chicago: American Association for the Study of Liver Diseases 1991 pp 76–106
Nishinomiya F, Abukawa D, Takada G, Tazawa Y . Relationships between clinical and histological profiles of non-familial idiopathic neonatal hepatitis Acta Paediatr Jpn 1996 38: 242–7
Sandor T, Surinya M, Monus Z . Familial occurrence of giant cell hepatitis in infancy Acta Hepato-Gastroenterol (Stuttgart) 1976 23: 101–4
Moore L, Bourne AJ, Moore DJ, Preston H, Byard RW . Hepatocellular carcinoma following neonatal hepatitis Pediatr Pathol Lab Med 1997 17: 601–10
Clayton PT, Casteels M, Mieli-Vergani G, Lawson AM . Familial giant cell hepatitis with low bile acid concentrations and increased urinary excretion of specific bile alcohols: a new inborn error of bile acid synthesis? Pediatr Res 1995 37: 424–31
Drut R, Gomez MA, Drut RM, Lojo MM . Human papillomavirus–associated neonatal giant cell hepatitis (NGCH) Pediatr Pathol Lab Med 1996 16: 403–12
Drut R, Dut RM, Gomez MA, Rua EC, Lojo MM . Presence of human papillomavirus in extrahepatic biliary atresia J Pediatr Gastroenterol Nutr 1998 27: 530–5
Domiati-Saad R, Dawson DB, Margraf LR, Finegold MJ, Weinberg AG, Rogers BB . Cytomegalovirus and human herpesvirus 6, but not human papillomavirus, are present in neonatal giant cell hepatitis and extrahepatic biliary atresia Pediatr Dev Pathol 2000 3: 367–73
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Correa, K., Nanjundiah, P., Wirtschafter, D. et al. Idiopathic Neonatal Giant Cell Hepatitis Presenting With Acute Hepatic Failure on Postnatal Day One. J Perinatol 22, 249–251 (2002). https://doi.org/10.1038/sj.jp.7210670
Published:
Issue Date:
DOI: https://doi.org/10.1038/sj.jp.7210670
This article is cited by
-
Fatal spontaneous subdural bleeding due to neonatal giant cell hepatitis: a rare differential diagnosis of shaken baby syndrome
Forensic Science, Medicine, and Pathology (2011)