Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor
https://doi.org/10.15825/1995-1191-2017-2-41-46
Abstract
Wilson’s disease is a rare congenital disease caused by deficiency of the copper-transporting P-type ATPase-B enzyme. The course of disease varies widely from the latent form to the acute liver failure which is observed in 5% of Wilson’s disease cases. This clinical case represents experience of liver transplantation as the only curative treatment for patients with fulminant form of Wilson’s disease demonstrating excellent postoperative results. Living donor liver transplantation allows performing the operation in the shortest possible time which is necessary in acute liver failure. The plasmapheresis with plasma exchange AB (IV) allows preparing the patient for transplantation with incompatible blood group.
About the Authors
A. R. Monakhov
V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;
I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation, Department of transplantology and artificial organs
Russian Federation
Russian Federation
O. M. Tsiroulnikova
V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;
I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation, Department of transplantology and artificial organs
Russian Federation
Russian Federation
T. A. Dzhanbekov
V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;
Russian Federation
Russian Federation
D. Dzhiner
I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation, Department of transplantology and artificial organs
Russian Federation
Russian Federation
I. E. Pashkova
V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;
Russian Federation
Russian Federation
N. P. Mozheiko
V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;
Russian Federation
Russian Federation
K. M. Khizroev
V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;
Russian Federation
Russian Federation
S. V. Gautier
V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;
I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation, Department of transplantology and artificial organs
Russian Federation
Russian Federation
References
1. Trocello J-M, Broussolle E, Girardot-Tinant N, Pelosse M, Lachaux A, Lloyd C, Woimant F. Wilson’s disease, 100 years later... Rev. Neurol. (Paris). 2013; 169 (12): 936–943.
2. EASL Clinical Practice Guidelines: Wilson’s disease. J. Hepatol. 2012; 56 (3): 671–685.
3. Catana AM, Medici V. Liver transplantation for Wilson disease. World J. Hepatol. 2012; 4 (1): 5–10.
4. Weiss KH, Schäfer M, Gotthardt DN, Angerer A, Mogler C, Schirmacher P, Schemmer P et al. Outcome and development of symptoms after orthotopic liver thansplantation for Wilson desiase. Clin. Transplant. 2013; 27 (6): 914–922.
5. Schilsky ML. Liver transplantation for Wilson’s disease. Ann. NY Acad. Sci. 2014; 1315: 45–49.
6. Eisenbach C, Sieg O, Stremmel W, Encke J, Merle U. Diagnostic criteria for acute liver failure due to Wilson disease. World J. Gastroenterol. 2007; 13 (11): 1711–1714.
7. Cabeza de Vaca VG, Bellido CB, Martínez JNA, Artacho GS, Gómez LMM, Díaz-Canedo JS, Ruiz FJP, Bravo MAG. Liver transplantation due to fulminant hepatic failure. Transplant. Proc. 2012; 44 (7): 2076–2077.
8. Weiner J, Griesemer A, Island E, Lobritto S, Martinez M, Selvaggi G, Lefkowitch J et al. Longterm Outcomes of Auxiliary Partial Orthotopic Liver Transplantation in Preadolescent Children With Fulminant Hepatic Failure. Liver Transpl. 2016; 22 (4): 485–494.
9. Hyun Sik Chung, Yu Jung Lee, Yun Sung Jo. Proposal for a New Predictive Model of Short-Term Mortality After Living Donor Liver Transplantation due to Acute Liver Failure. Ann. Transplant. 2017; 22: 101–107.
10. Moon D-B, Lee S-G, Kang W-H, Song G-W, Jung D-H, Park G-C, Cho H-D et al. Adult Living Donor Liver Transplantation for Acute-on-Chronic Liver Failure in High-Model for End-Stage Liver Disease Score Patients. Am. J. Transplant. 2017; 20: 1–10.
11. Biolato M, Araneo C, Marrone G, Liguori A, Miele L, Poznani FR, Gasbarrini A, Grieco A. Liver transplantation for drug-induced acute liver failure. Eur. Rev. Med. Pharmacol. Sci. 2017; 21: 37–45.
12. Gautier SV, Akhaladze DG, Tsirulnikova OM, Shevchenko OP. АВ0-Incompatible Living Donor Liver Transplantation in Small Children: the Russian Experience Transplantation. May 2016; 100 (5S): S202.
13. Gautier S, Poptsov V, Tsirulnikova O, Akhaladze D, Shevchenko O. 5 years experience of AB0-incompatible pediatric living donor liver transplantation. Pediatric Transplantation. Special Issue: IPTA 8th Congress on Pediatric Transplantation. Vol. 19, Issue Supplement S1, May 2015: 116.
14. Цирульникова ИЕ. Трансплантация печени детям от АВ0-несовместимых доноров: Автореф. дис. … канд. мед. наук. 2014. Tsirulnikova IE. Transplantatsia pecheni ot AB0-nesovmestimyh donorov: Avtoref. dis. … kand. med. nauk. 2014.
15. Готье СВ, Константинов БА, Цирульникова ОМ. Трансплантация печени. М.: МИА (2008), 246 с. Gautier SV, Konstantinov BA, Tsirulnikova OM. Transplantatsiya pecheni. М.: МIА, 2008: 246.
Review
For citations:
Monakhov A.R., Tsiroulnikova O.M., Dzhanbekov T.A., Dzhiner D., Pashkova I.E., Mozheiko N.P., Khizroev K.M., Gautier S.V. Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor. Russian Journal of Transplantology and Artificial Organs. 2017;19(2):41-46. (In Russ.) https://doi.org/10.15825/1995-1191-2017-2-41-46
Views:
1681
Email this article 