Liver Transplantation for Neonatal Hemochromatosis: Analysis of the UNOS Database
Division of Pediatric Hepatology, Mount Sinai Medical Center, New York
Division of Pediatric Gastroenterology, Mount Sinai Medical Center, New York
Psychology Department, Fordham University, Bronx
Recanati/Miller Transplantation Institute, Mount Sinai Medical Center, New York
Meeting: 2013 American Transplant Congress
Abstract number: 59
Neonatal hemochromatosis (NH) is a rare condition of unclear etiology and remains the most common identifiable cause of acute liver failure in the neonate. Liver transplantation (LT) is the only definitive treatment option for neonates with NH who have failed medical therapy. Purpose: To determine the outcomes of LT in neonates with NH. Methods: Infants with NH who underwent LT between 1987 and 2009 were selected from the UNOS database. Results: 32 infants with NH with a total of 34 transplants were identified. Clinical data is shown in Table 1. One and five year patient and graft survival was 75%, 68.8%, 62.5%, 56.3% respectively. Ten neonates (31.3%) died during the study period, most commonly due to infection (4, 40%). Seventeen neonates (53.1%) lost their graft, most commonly caused by a vascular thrombosis (5, 29.4%) and primary non function (3, 17.6%). Two neonates underwent retransplantation during the study period. There were no statistically significant risk factors identified for graft loss or death. One and five year patient and graft survival for NH were not significantly different when compared to the same age-weight recipients with other causes of acute liver failure (ALF) (n=194). There was no statistical difference in the outcome before and after the PELD era (after 2002). Conclusion: Given the severe clinical presentation of NH, LT for neonates with NH has a relatively high rate of graft loss and death. However, the outcomes remain comparable to same age-weight recipients with other causes of ALF.
Recipient Characteristics | Mean (SD) or % |
Age* (Years) | 0 |
Gender (% Female) | 40.6 |
Ethnicity (% White) | 75.0 |
Ethnicity (% African-American) | 12.5 |
Weight (kg) | 3.4 (0.8) |
Albumin Gr/L | 2.9 (0.8) |
Creatinine mg% | 0.5 (0.5) |
Bilirubin mg% | 21.4 (11.8) |
ALT IU/ml | 141 (416) |
Life Support (% Yes) | 43.8 |
Transplant Characteristics | |
Status 1 (% Yes) | 87.5 |
Cold Ischemic Time (Hours) | 8.5 (3.4) |
Liver Type: Whole (%) | 50.0 |
Liver Type: Technical Variant (%) | 50.0 |
Time between listing and transplant (Days) | 13.8 (12.2) |
Donor Characteristics | |
Donor Age (Years) | 7.2 (10.5) |
Donor Type: Cadaveric (%) | 93.7 |
Donor Type: Living (%) | 6.3 |
To cite this abstract in AMA style:
Sheflin-Findling S, Annunziato R, Ragusa R, Chu J, Kerkar N, Arnon R. Liver Transplantation for Neonatal Hemochromatosis: Analysis of the UNOS Database [abstract]. Am J Transplant. 2013; 13 (suppl 5). https://atcmeetingabstracts.com/abstract/liver-transplantation-for-neonatal-hemochromatosis-analysis-of-the-unos-database/. Accessed October 9, 2024.« Back to 2013 American Transplant Congress