Neonatal hemochromatosis: Report of successful orthotopic liver transplantation

Elizabeth B. Rand*, Daniel T. McClenathan, Peter F. Whitington

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

44 Scopus citations

Abstract

Neonatal hemochromatosis is clinically characterized by profound hepatic insufficiency and death in the newborn period. Hemosiderosis develops in utero with iron deposition in a particular organ distribution with sparing of the reticuloendothelial system. Neonatal hemochromatosis has been established as a distinct form of giant cell hepatitis for more than three decades, but no consensus has yet been reached as to whether the iron deposition represents a primary disease or a secondary response (1,2). Neonatal hemochromatosis has pathologic similarities to hereditary hemochromatosis as seen in adults, but the human leukocyte antigen associations are absent (3-7). Many of the reported cases of neonatal hemochromatosis are in sibling pairs, but the mechanism of transmission is unknown (3, 4,7).

Original languageEnglish (US)
Pages (from-to)325-329
Number of pages5
JournalJournal of pediatric gastroenterology and nutrition
Volume15
Issue number3
DOIs
StatePublished - Oct 1992

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Gastroenterology

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