Liver transplantation in Wilson’s disease: Long-term experience of single center
Liver transplantation in Wilson’s disease: Long-term experience of single center
Aim: Liver transplantation (LT) is the most effective treatment method for preventing progressive and lethal complications of Wilson’sdisease (WD). Despite the deceased donor liver transplantation (DDLT) and living donor liver transplantation (LDLT) are performedin many centers for WD, a limited number of reports were published, about long-term results. The aim of this study is to share thelong-term outcomes of single center.Material and Methods: Patients who underwent LT for the WD, between 1997 and 2017 were included. Patient’s survival data, deathcauses, neurological symptoms and follow-up data were analyzed retrospectively.Results: Eighteen patients (8DDLT, 10LDLT) with the median age of 17.11±9.88 (6-43) were included. Donor relationship was familialin all LDLT patients. Median follow-up time was 80.57±67.59 (0.23-240.9) months and the median survival time was 173.74±25.13months. Two patients (11.1%) died in the perioperative period (0-90 days) and totally 3 patients (16.6%) died in the postoperative0-12 month’s period. The survival rates of the patient’s at 1-, 5- and 10 years were 83.3%, 75% and 67.3%, respectively. After LT,neurological symptoms disappeared in 6 of 7 patients and no improvement was observed in one patient. During follow-up period,chronic rejection was seen in 5 patients, 3 patients were treated with medical procedure and 2 patients died due to chronic rejection.Conclusions: Our findings are consistent with the literature, long-term survival is achieved in patients with no mortality, in thepostoperative 0-12 month period and after LT, neurological symptoms are disappeared in most of the patients; according to thisdata, LT is an effective treatment method for the WD and complications.
___
- 1. Pabón V, Dumortier J, Gincul R, et al. Long-term results of
liver transplantation for Wilson’s disease. Gastroenterol.
Clin. Biol 2008;32:378-81.
- 2. Catana AM, Medici V. Liver transplantation for Wilson
disease. World J. Hepatol 2012;4:5-10.
- 3. Haberal M, Moray G, Karakayali H, et al. Liver Transplantation
for Wilson ’ s Cirrhosis : One Center ’ s Experience. Transpl.
Proc 1999;31:3160-1.
- 4. Lin L-J, Wang D-X, Ding NN, et al. Comprehensive analysis
on clinical features of Wilson’s disease: an experience over
28 years with 133 cases. Neurol. Res 2014;36:157-63.
- 5. Ferenci P, Czlonkowska A, Stremmel W, et.al. EASL
clinical practice guidelines: wilson’s disease. J. Hepatol
2012;56:671-85.
- 6. Steindl P, Ferenci P, Dienes HP, et al. Wilson’s disease
in patients presenting with liver disease: a diagnostic
challenge. Gastroenterol 1997;113:212-18.
- 7. Lorincz MT. Neurologic Wilson’s disease. Ann. N Y. Acad. Sci
2010;1184:173-87.
- 8. Pfister ED, Karch A, Polak WG, et sl. Predictive factors for
survival in children liver transplanted for Wilson disease: a
cohort study using ELTR data. Liver Transplant 2018.
- 9. Medici V, Mirante VG, Fassati LR, et al. Liver transplantation
for Wilson’s disease: The burden of neurological and
psychiatric disorders. Liver Transplant 2005;11:1056-63.
- 10. Stracciari A, Tempestini A, Borghi A, et al. Effect of liver
transplantation on neurological manifestations in Wilson
disease. Arch Neurol 2000;57:384–86.
- 11. Eghtesad B, Nezakatgoo N, Geraci LC, et al. Liver
transplantation for Wilson’s disease: a single-center
experience. Liver Transpl Surg 1999;5:467-74.
- 12. Yagci MA, Tardu A, Karagul S, et al. Influence of Liver
Transplantation on Neuropsychiatric Manifestations of
Wilson Disease. Transplant. Proc. 2015;47:1469-73.
- 13. Członkowska A, Tarnacka B, Möller JC, et al. Unified wilson’s
disease rating scale - a proposal for the neurological
scoring of wilson’s disease patients. Neurol Neurochir Pol
2007;41:1-12.
- 14. Erol I, Alehan F, Ozcay F, et al. Neurologic complications of
liver transplantation in pediatric patients with the hepatic
form of wilson’s disease. J. Child Neurol 2008;23:293-300.
- 15. Öcal R, Öcal S, Kırnap M, et al. Complications of liver
transplant in adult patients with the hepatic form of wilson
disease. Exp Clin Transplant 2018;16:38-40.
- 16. Arnon R, Annunziato R, Schilsky M, et al. Liver transplantation
for children with Wilson disease: Comparison of outcomes
between children and adults. Clin. Transplant 2011;25:52-
60.
- 17. Haberal M, Akdur A, Moray G, et al. Auxiliary partial orthotopic
living liver transplant for wilson disease. Exp Clin Transplant
2017;15:182-84.
- 18. Ozcay F, Us B, Baskin E, et al. Long term follow-up of
glomerular and tubular functions in liver transplanted
patients with Wilson’ s disease. Pediatr Transplant
2008;12:785-89.
- 19. Rodriguez-Castro KI, Hevia-Urrutia FJ, Sturniolo GC.
Wilson’s disease: A review of what we have learned. World
J. Hepatol 2015;7:2859-870.
- 20. Cheng F, Li GQ, Zhang F, et al. Outcomes of living-related
liver transplantation for Wilson’s disease: a single-center
experience in China. Transplant 2009;87:751-57.
- 21. Yoshitoshi EY, Takada Y, Oike F, et al. Long-term outcomes
for 32 cases of Wilson’s disease after living-donor liver
transplantation. Transplant 2009;87:261-67.
- 22. Sevmis S, Karakayali H, Aliosmanoglu I, A, et al. Liver
Transplantation for Wilson’s Disease. Transplant. Proc
2008;40:228-30.
- 23. Savas N, Canan O, Ozcay F, et al. Hepatocellular carcinoma in
Wilson ’ s disease : A rare association in childhood. Pediatr
Transplant 2006;10:639-43.
- 24. Reyes CV. Hepatocellular Carcinoma in Wilson Disease
–related Liver Cirrhosis. Gastroenterol Hepatol (NY)
2008;4:435-37.
- 25. Lankarani KB, Malek-Hosseini SA, Nikeghbalian S, et al.
Fourteen years of experience of liver transplantation for
Wilson’s disease; A report on 107 cases from Shiraz, Iran.
PLoS One 2016;11:1-11.
- 26. Guillaud O, Dumortier J, Sobesky R, et al. Long term results
of liver transplantation for Wilson’s disease: Experience in
France. J. Hepatol 2014;60:579-89.