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Medical and surgical treatment of neonatal hemochromatosis: Single center experience
Pediatric transplantation
  • Thomas Heffron
  • Todd Pillen
  • David Welch
  • Massimo Asolati
  • Gregory Smallwood, Philadelphia College of Osteopathic Medicine
  • Phil Hagedorn
  • David Solis
  • et al.
Document Type
Article
Publication Date
1-1-2007
Abstract
NH is a rare disorder of iron storage in newborns resulting in rapid liver failure. Outcomes are dismal with 20-30% survival. We report our experience in eight children with NH. Assessment of liver f0nction included admission PT and serum levels of FV and FVII. Medical treatment (antioxidant cocktail) was started in all patients, with chelation therapy in six. Of these six, three survived with medical treatment alone. The other three underwent liver transplant. One died 158 days after transplant to sepsis: two are well more than five yr after transplant. The two neonates who did not receive chelation therapy, died to multi-organ failure and sepsis. In summary, five children (62.5%) survived long-term. In the three transplanted, one- and five-yr-survival was 66%. Older children with compromised synthetic liver function (FVII levels ≤ 15%) required liver replacement for survival. Early referral to a tertiary care center is essential to increase survival of these children with a rare and otherwise fatal disease. Single center experience of children with NH is here presented. Potentials for survival improvement with of medical and surgical treatment are examined. © 2007 Blackwell Munksgaard.
Comments

This article was published in Pediatric transplantation, Volume 11, Issue 4, Pages 374-378.

The published version is available at http://dx.doi.org/10.1111/j.1399-3046.2006.00675.x.

Copyright © 2007 Wiley.

Citation Information
Thomas Heffron, Todd Pillen, David Welch, Massimo Asolati, et al.. "Medical and surgical treatment of neonatal hemochromatosis: Single center experience" Pediatric transplantation Vol. 11 Iss. 4 (2007) p. 374 - 378
Available at: http://works.bepress.com/gregory_smallwood/48/