Liver Transplantation for Metabolic Liver Disease: Experience at a Living Donor Dominant Liver Transplantation Center
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초록
Purpose: Metabolic liver disease (MLD) often progresses to life-threatening conditions. This study intends to describe the outcomes of liver transplantation (LTx) for MLD at a living donor-dominant transplantation center where potentially heterozygous carrier grafts are employed. Methods: We retrospectively evaluated the medical records of 54 patients with MLD who underwent LTx between November 1995 and February 2012 at Asan Medical Center in Seoul, Korea. The cumulative graft and patient survival rates were analyzed according to patient age, and living or deceased donor LTx. Recurrence of the original disease was also investigated.Results: The post-transplant cumulative patient survival rates at one, five, and 10 years were 90.7%, 87.5% and 87.5%, and the graft survival rates were 88.8%, 85.5%, and 85.5%, respectively. There were no differences in the patient survival rates according to the recipient age, human leukocyte antigen matching, and living or deceased donor LTx. There were also no differences in the patient survival rates between the MLD and the non-MLD groups for children. Recurrence of the original metabolic disease was not observed in any patient during the follow-up period.Conclusion: Our results suggest that the living donor-dominant transplantation program is well-tolerated in MLD with-out recurrence of the original MLD using all types of transplantation.
Abstract
Purpose: Metabolic liver disease (MLD) often progresses to life-threatening conditions. This study intends to describe the outcomes of liver transplantation (LTx) for MLD at a living donor-dominant transplantation center where potentially heterozygous carrier grafts are employed. Methods: We retrospectively evaluated the medical records of 54 patients with MLD who underwent LTx between November 1995 and February 2012 at Asan Medical Center in Seoul, Korea. The cumulative graft and patient survival rates were analyzed according to patient age, and living or deceased donor LTx. Recurrence of the original disease was also investigated.Results: The post-transplant cumulative patient survival rates at one, five, and 10 years were 90.7%, 87.5% and 87.5%, and the graft survival rates were 88.8%, 85.5%, and 85.5%, respectively. There were no differences in the patient survival rates according to the recipient age, human leukocyte antigen matching, and living or deceased donor LTx. There were also no differences in the patient survival rates between the MLD and the non-MLD groups for children. Recurrence of the original metabolic disease was not observed in any patient during the follow-up period.Conclusion: Our results suggest that the living donor-dominant transplantation program is well-tolerated in MLD with-out recurrence of the original MLD using all types of transplantation.
참고문헌 (25)
- Shneider BL, "Pediatric liver transplantation in metabolic di'sease: clinical decision making", Pediatr Transplant, 6, pp.25~29 (2002)
- Oh SH, "Long-term outcomes of pediatric living donor liver transplantation at a single institution", Pediatr Transplant, 14, pp.870~878 (2010)
- Oh SH, "Clinical experience of more than 200 cases of pediatric liver transplantation at a single center: improved patient survival", Transplant Proc, 44, pp.484~486 (2012)
- Arnon R, "Liver transplantation in children with metabolic diseases: the studies of pediatric liver transplantation experience", Pediatr Transplant, 14, pp.796~805 (2010)
- Bellary S, "Liver transplantation for Wilson's disease", J Hepatol, 23, pp.373~381 (1995)
- Sternlieb I, "The role of radiocopper in the diagnosis of Wilson's disease", Gastroenterology, 77, pp.138~142 (1979)
- Asonuma K, "Living related liver transplantation from heterozygote genetic carriers to children with Wilson's disease", Pediatr Transplant, 3, pp.201~205 (1999)
- 김진택, "윌슨병 환아에서 이형 접합 보인자 공여간을 이용한 혈연간 생체 부분 간이식", Pediatric Gastroenterology, Hepatology & Nutrition, 6(2), pp.161~166 (2003), 대한소아소화기영양학회
- Roche-Sicot J, "Acute intravascular hemolysis and acute liver failure associated as a first manifestation of Wilson's disease", Ann Intern Med, 86, pp.301~303 (1977)
- Hwang S, "Lessons learned from 1,000 living donor liver transplantations in a single center: how to make living donations safe", Liver Transpl, 12, pp.920~927 (2006)
- Kim BS, "A case of ornithine transcarbamylase deficiency successfully treated with protein restriction and living related liver transplantation", J Korean Pediatr Soc, 42, pp.868~873 (1999)
- Kayler LK, "Liver transplantation in children with metabolic disorders in the United States", Am J Transplant, 3, pp.334~339 (2003)
- Sze YK, "Pediatric liver transplantation for metabolic liver disease: experience at King's College Hospital", Transplantation, 87, pp.87~93 (2009)
- Morioka D, "Living donor liver transplantation for pediatric patients with inheritable metabolic disorders", Am J Transplant, 5, pp.2754~2763 (2005)
- Lee BH, "Distinct clinical courses according to presenting phenotypes and their correlations to ATP7B mutations in a large Wilson's disease cohort.", Liver Int, 31, pp.831~839 (2011)
- Stracciari A, "Effect of liver transplantation on neurological manifestations in Wilson disease", Arch Neurol, 57, pp.384~386 (2000)
- Marin C, "Liver transplantation in Wilson's disease:are its indications established?", Transplant Proc, 39, pp.2300~2301 (2007)
- Medici V, "Liver transplantation for Wilson's disease:The burden of neurological and psychiatric disorders", Liver Transpl, 11, pp.1056~1063 (2005)
- Kassam N, "Liver transplantation for neuropsychiatric Wilson disease", Can J Gastroenterol, 12, pp.65~68 (1998)
- Yoshitoshi EY, "Long-term outcomes for 32 cases of Wilson's disease after living-donor liver transplantation", Transplantation, 87, pp.261~267 (2009)
- Franco LM, "Hepatocellular carcinoma in glycogen storage disease type Ia: a case series", J Inherit Metab Dis, 28, pp.153~162 (2005)
- Mazariegos G, "Liver transplantation for pediatric metabolic disease", Mol Genet Metab, 111, pp.418~427 (2014)
- Häberle J, "Suggested guidelines for the diagnosis and management of urea cycle disorders", Orphanet J Rare Dis, 7, pp.32~ (2012)
- Angelis M, "En bloc heterotopic auxiliary liver and bilateral renal transplant in a patient with homozygous protein C deficiency", J Pediatr, 138, pp.120~122 (2001)
- Jiyoung Ryu, "Intractable metabolic acidosis in a child with propionic acidemia undergoing liver transplantation -a case report-", Korean Journal of Anesthesiology, 65(3), pp.257~261 (2013), 대한마취통증의학회