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Liver transplantation for hereditary tyrosinemia type I: analysis of the UNOS database.
- Source :
-
Pediatric transplantation [Pediatr Transplant] 2011 Jun; Vol. 15 (4), pp. 400-5. Date of Electronic Publication: 2011 Apr 19. - Publication Year :
- 2011
-
Abstract
- Patients with HT-1 can develop progressive liver disease and have a high incidence of HCC. LT is indicated in patients with fulminant liver failure, HCC or decompensated chronic liver disease refractory to NTBC. To determine the need for LT and outcomes after LT in children with HT-1. Children with HT-1 who had LT between 10/1987 and 5/2008 were identified from the UNOS database. Of 11,467 children in the UNOS database, 125 (1.1%) required LT secondary to HT-1. Mean age at LT was two and half yr (s.d. ± 3.6 yr). Mean age at LT during the first 10 yr of the study (1.82, s.d. ± 2.86 yr) was significantly lower than in the last decade (3.70, s.d. ± 4.42 yr), p = 0.01. Nearly half of the patients (58, 46.4%) were transplanted between 1988 and 1992. Overall, one- and five-yr patient survival was 90.4% and 90.4%, respectively. LT is a valuable option for children with HT-1 with fulminant liver failure or when medical treatment fails. The rate of LT for children with HT-1 has decreased and age at transplant increased over the last decade most probably reflecting the effect of early diagnosis and treatment with NTBC.<br /> (© 2011 John Wiley & Sons A/S.)
- Subjects :
- Adolescent
Chi-Square Distribution
Child
Databases, Factual
Female
Follow-Up Studies
Graft Rejection
Graft Survival
Humans
Liver Failure physiopathology
Liver Function Tests
Liver Transplantation adverse effects
Liver Transplantation statistics & numerical data
Male
Postoperative Complications epidemiology
Postoperative Complications physiopathology
Retrospective Studies
Risk Assessment
Severity of Illness Index
Treatment Outcome
Tyrosinemias diagnosis
Liver Failure etiology
Liver Failure surgery
Liver Transplantation methods
Tyrosinemias complications
Subjects
Details
- Language :
- English
- ISSN :
- 1399-3046
- Volume :
- 15
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Pediatric transplantation
- Publication Type :
- Academic Journal
- Accession number :
- 21504522
- Full Text :
- https://doi.org/10.1111/j.1399-3046.2011.01497.x