Neonatal giant cell hepatitis: Histological and etiological findings

Michael Torbenson, John Hart, Maria Westerhoff, Ruba K. Azzam, Abeer Elgendi, Haikaeli C. Mziray-Andrew, Grace E. Kim, Ann Scheimann

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Background: Neonatal giant cell hepatitis (NGCH) is an important diagnostic consideration in infants who present with jaundice. In this study, we examined 2 separate tertiary care center cohorts of individuals with NGCH to better understand the potential etiologies and their histological correlates. Methods: All liver biopsies (1984 to 2007) with a histological diagnosis of NGCH were reviewed from 2 tertiary care centers. Cases diagnosed at the time of biopsy as biliary atresia, paucity of intrahepatic bile ducts, total parenteral nutrition associated liver injury, or α-1-antitrypsin deficiency were excluded. Liver biopsies were examined for cholestasis, giant cell change, extramedullary hematopoiesis, inflammation, and fibrosis. Follow-up clinical and laboratory findings were reviewed. Results: Sixty-two cases of NGCH were identified (73% male) for analysis. The average age at liver biopsy was 2 months. Giant cell change affected on average 36% of hepatocytes (range, 5%-90%). Extramedullary hematopoiesis was common (74% of cases), often prominent, and included both myelopoiesis and erythropoiesis. Despite the term "hepatitis" in "neonatal giant cell hepatitis," portal and lobular inflammation was mild-to-absent in 95% of cases. Lobular cholestasis ranged from mild-to-moderate and demonstrated predominately a canalicular pattern (84% of cases). Bile ducts appeared hypoplastic (32% of cases) but were not absent or reduced in numbers. In contrast, another 18% of cases showed at least mild focal ductular proliferation. Portal or pericellular fibrosis was present in 30% of cases and was advanced in 8%. Subsequent clinical follow-up identified the following etiologies: Idiopathic (49%), hypopituitarism (16%), biliary atresia (8%), Alagille syndrome (6%), bile salt defects (6%), as well as several other entities present at 5% or less. Of note, the biopsy findings did not readily distinguish between the different etiologies with the exception that bile duct hypoplasia was more common in cases of hypopituitarism. Conclusions: In this series of cases, pan-hypopituitarism was the most common recognizable clinical association with neonatal giant cell hepatitis. However, most cases of neonatal giant cell hepatitis remain idiopathic and histological features do not readily distinguish among the various etiologies.

Original languageEnglish (US)
Pages (from-to)1498-1503
Number of pages6
JournalAmerican Journal of Surgical Pathology
Volume34
Issue number10
DOIs
StatePublished - Oct 1 2010
Externally publishedYes

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Giant Cells
Hepatitis
Hypopituitarism
Biopsy
Extramedullary Hematopoiesis
Biliary Atresia
Liver
Cholestasis
Bile Ducts
Tertiary Care Centers
Fibrosis
Alagille Syndrome
Myelopoiesis
Inflammation
Intrahepatic Bile Ducts
Total Parenteral Nutrition
Erythropoiesis
Jaundice
Bile Acids and Salts
Hepatocytes

Keywords

  • hypopituitarism
  • neonatal giant cell hepatitis
  • paucity of intrahepatic bile ducts

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Torbenson, M., Hart, J., Westerhoff, M., Azzam, R. K., Elgendi, A., Mziray-Andrew, H. C., ... Scheimann, A. (2010). Neonatal giant cell hepatitis: Histological and etiological findings. American Journal of Surgical Pathology, 34(10), 1498-1503. https://doi.org/10.1097/PAS.0b013e3181f069ab

Neonatal giant cell hepatitis : Histological and etiological findings. / Torbenson, Michael; Hart, John; Westerhoff, Maria; Azzam, Ruba K.; Elgendi, Abeer; Mziray-Andrew, Haikaeli C.; Kim, Grace E.; Scheimann, Ann.

In: American Journal of Surgical Pathology, Vol. 34, No. 10, 01.10.2010, p. 1498-1503.

Research output: Contribution to journalArticle

Torbenson, M, Hart, J, Westerhoff, M, Azzam, RK, Elgendi, A, Mziray-Andrew, HC, Kim, GE & Scheimann, A 2010, 'Neonatal giant cell hepatitis: Histological and etiological findings', American Journal of Surgical Pathology, vol. 34, no. 10, pp. 1498-1503. https://doi.org/10.1097/PAS.0b013e3181f069ab
Torbenson, Michael ; Hart, John ; Westerhoff, Maria ; Azzam, Ruba K. ; Elgendi, Abeer ; Mziray-Andrew, Haikaeli C. ; Kim, Grace E. ; Scheimann, Ann. / Neonatal giant cell hepatitis : Histological and etiological findings. In: American Journal of Surgical Pathology. 2010 ; Vol. 34, No. 10. pp. 1498-1503.
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