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Medical and surgical treatment of neonatal hemochromatosis: Single center experience
Author(s) -
Heffron Thomas,
Pillen Todd,
Welch David,
Asolati Massimo,
Smallwood Gregory,
Hagedorn Phil,
Fasola Carlos,
Solis David,
Rodrigues John,
DePaolo Jill,
Spivey James,
Martinez Enrique,
Henry Stuart,
Romero Rene
Publication year - 2007
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2006.00675.x
Subject(s) - medicine , sepsis , single center , chelation therapy , pediatrics , liver transplantation , surgery , liver function , liver disease , survival rate , jaundice , transplantation , thalassemia
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 function 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.