Liver transplantation in children with metabolic diseases: The studies of pediatric liver transplantation experience

Authors

  • Ronen Arnon,

    1. Departments of Pediatrics and the Recanati-Miller Transplant Institute, Department of Surgery, Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY, USA
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  • Nanda Kerkar,

    1. Departments of Pediatrics and the Recanati-Miller Transplant Institute, Department of Surgery, Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY, USA
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  • Michael K. Davis,

    1. Pediatric Hepatology and Liver Transplantation, Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Florida and Shands Children’s Hospital, Gainesville, FL, USA
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  • Ravinder Anand,

    1. The EMMES Corporation, Rockville, MD, USA
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  • Wanrong Yin,

    1. The EMMES Corporation, Rockville, MD, USA
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  • Regino P. González-Peralta,

    1. Pediatric Hepatology and Liver Transplantation, Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Florida and Shands Children’s Hospital, Gainesville, FL, USA
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  • for the SPLIT Research Group

    1. Departments of Pediatrics and the Recanati-Miller Transplant Institute, Department of Surgery, Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY, USA
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Nanda Kerkar, Departments of Pediatrics and the Recanati-Miller Transplant Institute, Department of Surgery, Mount Sinai Medical Center, Mount Sinai School of Medicine, One Gustave, L. Levy Place, Box 1104, New York, NY 10029, USA
Tel.: 212-659-8060
Fax: 212-241-2064
E-mail: Nanda.Kerkar@mountsinai.orgGrant Support: This work was supported by the National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health (U01-DK061693-01A1), and unrestricted grants from Astellas Pharma US and Roche Laboratories.

Abstract

Arnon R, Kerkar N, Davis MK, Anand R, Yin W, González-Peralta RP for the SPLIT Research Group. Liver transplantation in children with metabolic diseases: The studies of pediatric liver transplantation experience.
Pediatr Transplantation 2010: 14:796–805. © 2010 John Wiley & Sons A/S.

Abstract:  Metabolic diseases are the second largest indication for LT in children after BA. There are limited data on the long-term post-transplant outcome in this unique group of patients. Therefore, our aim was to assess post-liver transplant outcomes and to evaluate risk factors for mortality and graft loss in children with metabolic disorders in comparison to those with non-metabolic diagnoses. We reviewed all patients enrolled in the SPLIT registry. Between 1995 and 2008, 446 of 2997 (14.9%) children enrolled in SPLIT underwent liver transplant for metabolic diseases. One-yr and five-yr patient survival for children with metabolic diseases was 94.6% and 88.9% and for those with other diseases 90.7% and 86.1% (log-rank p = 0.05), respectively. One-yr and five-yr graft survival for children with metabolic disorders was 90.8% and 83.8%, and for those with other diseases 85.4% and 78.0% (log-rank p = 0.005), respectively. Children with metabolic diseases were less likely to experience gastrointestinal complications (5.6% vs. 10.7%, p = 0.001), portal vein thrombosis (2.9% vs. 5.2%, p = 0.04), and reoperations within 30 days post-transplant (33.4% vs. 37.8%, p = 0.05) than those with other indications. In conclusion, children who underwent liver transplant for metabolic disease had similarly excellent patient survival as, and better graft survival than, those who received a liver allograft for other indications.

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