DOI QR코드

DOI QR Code

Liver Transplantation for Metabolic Liver Disease: Experience at a Living Donor Dominant Liver Transplantation Center

  • Kim, Jun Suk (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Kim, Kyung Mo (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Oh, Seak Hee (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Kim, Hyun Jin (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Cho, Jin Min (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Yoo, Han-Wook (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Namgoong, Jung-Man (Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Kim, Dae Yeon (Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Kim, Ki-Hun (Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Hwang, Shin (Division of Hepato-Biliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Sung-Gyu (Division of Hepato-Biliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine)
  • Received : 2014.10.02
  • Accepted : 2014.12.18
  • Published : 2015.03.30

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 without recurrence of the original MLD using all types of transplantation.

Keywords

References

  1. Shneider BL. Pediatric liver transplantation in metabolic di'sease: clinical decision making. Pediatr Transplant 2002;6:25-9. https://doi.org/10.1034/j.1399-3046.2002.1p057.x
  2. Oh SH, Kim KM, Kim DY, Lee YJ, Rhee KW, Jang JY, et al. Long-term outcomes of pediatric living donor liver transplantation at a single institution. Pediatr Transplant 2010;14:870-8. https://doi.org/10.1111/j.1399-3046.2010.01357.x
  3. Oh SH, Kim KM, Kim DY, Song SM, Kim T, Hwang S, et al. Clinical experience of more than 200 cases of pediatric liver transplantation at a single center: improved patient survival. Transplant Proc 2012;44:484-6. https://doi.org/10.1016/j.transproceed.2012.02.002
  4. Arnon R, Kerkar N, Davis MK, Anand R, Yin W, Gonzalez-Peralta RP; SPLIT Research Group. Liver transplantation in children with metabolic diseases: the studies of pediatric liver transplantation experience. Pediatr Transplant 2010;14:796-805. https://doi.org/10.1111/j.1399-3046.2010.01339.x
  5. Bellary S, Hassanein T, Van Thiel DH. Liver transplantation for Wilson's disease. J Hepatol 1995;23:373-81. https://doi.org/10.1016/0168-8278(95)80194-4
  6. Sternlieb I, Scheinberg IH. The role of radiocopper in the diagnosis of Wilson's disease. Gastroenterology 1979;77:138-42.
  7. Asonuma K, Inomata Y, Kasahara M, Uemoto S, Egawa H, Fujita S, et al. Living related liver transplantation from heterozygote genetic carriers to children with Wilson's disease. Pediatr Transplant 1999;3:201-5. https://doi.org/10.1034/j.1399-3046.1999.00014.x
  8. Kim JT, Chang SH, Choi BH, Kim KM, Yoo HW, Lee YJ, et al. Living-related liver transplantation with heterozygote carrier graft in children with wilson disease. Korean J Pediatr Gastroenterol Nutr 2003;6:161-6.
  9. Roche-Sicot J, Benhamou JP. Acute intravascular hemolysis and acute liver failure associated as a first manifestation of Wilson's disease. Ann Intern Med 1977;86:301-3. https://doi.org/10.7326/0003-4819-86-3-301
  10. Hwang S, Lee SG, Lee YJ, Sung KB, Park KM, Kim KH, et al. Lessons learned from 1,000 living donor liver transplantations in a single center: how to make living donations safe. Liver Transpl 2006;12:920-7. https://doi.org/10.1002/lt.20734
  11. Kim BS, Kim KM, Yoo HW, Lee SG. A case of ornithine transcarbamylase deficiency successfully treated with protein restriction and living related liver transplantation. J Korean Pediatr Soc 1999;42:868-73.
  12. Kayler LK, Rasmussen CS, Dykstra DM, Punch JD, Rudich SM, Magee JC, et al. Liver transplantation in children with metabolic disorders in the United States. Am J Transplant 2003;3:334-9. https://doi.org/10.1034/j.1600-6143.2003.00058.x
  13. Sze YK, Dhawan A, Taylor RM, Bansal S, Mieli-Vergani G, Rela M, et al. Pediatric liver transplantation for metabolic liver disease: experience at King's College Hospital. Transplantation 2009;87:87-93. https://doi.org/10.1097/TP.0b013e31818bc0c4
  14. Morioka D, Kasahara M, Takada Y, Corrales JP, Yoshizawa A, Sakamoto S, et al. Living donor liver transplantation for pediatric patients with inheritable metabolic disorders. Am J Transplant 2005;5:2754-63. https://doi.org/10.1111/j.1600-6143.2005.01084.x
  15. Lee BH, Kim JH, Lee SY, Jin HY, Kim KJ, Lee JJ, et al. Distinct clinical courses according to presenting phenotypes and their correlations to ATP7B mutations in a large Wilson's disease cohort. Liver Int 2011;31:831-9. https://doi.org/10.1111/j.1478-3231.2011.02503.x
  16. Stracciari A, Tempestini A, Borghi A, Guarino M. Effect of liver transplantation on neurological manifestations in Wilson disease. Arch Neurol 2000;57:384-6. https://doi.org/10.1001/archneur.57.3.384
  17. Marin C, Robles R, Parrilla G, Ramirez P, Bueno FS, Parrilla P. Liver transplantation in Wilson's disease: are its indications established? Transplant Proc 2007;39:2300-1. https://doi.org/10.1016/j.transproceed.2007.06.039
  18. Medici V, Mirante VG, Fassati LR, Pompili M, Forti D, Del Gaudio M, et al; Monotematica AISF 2000 OLT Study Group. Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders. Liver Transpl 2005;11:1056-63. https://doi.org/10.1002/lt.20486
  19. Kassam N, Witt N, Kneteman N, Bain VG. Liver transplantation for neuropsychiatric Wilson disease. Can J Gastroenterol 1998;12:65-8. https://doi.org/10.1155/1998/414236
  20. Yoshitoshi EY, Takada Y, Oike F, Sakamoto S, Ogawa K, Kanazawa H, et al. Long-term outcomes for 32 cases of Wilson's disease after living-donor liver transplantation. Transplantation 2009;87:261-7. https://doi.org/10.1097/TP.0b013e3181919984
  21. Franco LM, Krishnamurthy V, Bali D, Weinstein DA, Arn P, Clary B, et al. Hepatocellular carcinoma in glycogen storage disease type Ia: a case series. J Inherit Metab Dis 2005;28:153-62. https://doi.org/10.1007/s10545-005-7500-2
  22. Mazariegos G, Shneider B, Burton B, Fox IJ, Hadzic N, Kishnani P, et al. Liver transplantation for pediatric metabolic disease. Mol Genet Metab 2014;111:418-27. https://doi.org/10.1016/j.ymgme.2014.01.006
  23. Haberle J, Boddaert N, Burlina A, Chakrapani A, Dixon M, Huemer M, et al. Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet J Rare Dis 2012;7:32. https://doi.org/10.1186/1750-1172-7-32
  24. Angelis M, Pegelow CH, Khan FA, Verzaro R, Tzakis AG. En bloc heterotopic auxiliary liver and bilateral renal transplant in a patient with homozygous protein C deficiency. J Pediatr 2001;138:120-2. https://doi.org/10.1067/mpd.2001.109199
  25. Ryu J, Shin YH, Ko JS, Gwak MS, Kim GS. Intractable metabolic acidosis in a child with propionic acidemia undergoing liver transplantation-a case report-. Korean J Anesthesiol 2013;65:257-61. https://doi.org/10.4097/kjae.2013.65.3.257

Cited by

  1. Complete Recovery of Oxysterol 7α-Hydroxylase Deficiency by Living Donor Transplantation in a 4-Month-Old Infant: the First Korean Case Report and Literature Review vol.33, pp.51, 2015, https://doi.org/10.3346/jkms.2018.33.e324
  2. Pediatric metabolic liver diseases: Evolving role of liver transplantation vol.11, pp.6, 2015, https://doi.org/10.5500/wjt.v11.i6.161
  3. Liver transplantation for pediatric inherited metabolic liver diseases vol.13, pp.10, 2015, https://doi.org/10.4254/wjh.v13.i10.1351
  4. Safety and recommendations for vaccinations of children with inborn errors of metabolism vol.35, pp.None, 2015, https://doi.org/10.1016/j.ejpn.2021.10.002