Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor
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 liv...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | Russian |
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Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov
2017-06-01
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| Series: | Vestnik Transplantologii i Iskusstvennyh Organov |
| Subjects: | |
| Online Access: | https://journal.transpl.ru/vtio/article/view/750 |
| _version_ | 1797872012967804928 |
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| author | A. R. Monakhov O. M. Tsiroulnikova T. A. Dzhanbekov D. Dzhiner I. E. Pashkova N. P. Mozheiko K. M. Khizroev S. V. Gautier |
| author_facet | A. R. Monakhov O. M. Tsiroulnikova T. A. Dzhanbekov D. Dzhiner I. E. Pashkova N. P. Mozheiko K. M. Khizroev S. V. Gautier |
| author_sort | A. R. Monakhov |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-10T00:52:05Z |
| format | Article |
| id | doaj.art-9311cdeca25c4f5b8cf6bac2eab5832b |
| institution | Directory Open Access Journal |
| issn | 1995-1191 |
| language | Russian |
| last_indexed | 2024-04-10T00:52:05Z |
| publishDate | 2017-06-01 |
| publisher | Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov |
| record_format | Article |
| series | Vestnik Transplantologii i Iskusstvennyh Organov |
| spelling | doaj.art-9311cdeca25c4f5b8cf6bac2eab5832b2023-03-13T10:37:24ZrusFederal Research Center of Transplantology and Artificial Organs named after V.I.ShumakovVestnik Transplantologii i Iskusstvennyh Organov1995-11912017-06-01192414610.15825/1995-1191-2017-2-41-46621Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donorA. R. Monakhov0O. M. Tsiroulnikova1T. A. Dzhanbekov2D. Dzhiner3I. E. Pashkova4N. P. Mozheiko5K. M. Khizroev6S. V. Gautier7V.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 organsV.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 organsV.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 organsV.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs of the Ministry of Healthcare of the Russian Federation;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 organsWilson’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.https://journal.transpl.ru/vtio/article/view/750living donor liver transplantationwilson’s diseasefulminant liver failureав0-incompatible transplantation |
| spellingShingle | A. R. Monakhov O. M. Tsiroulnikova T. A. Dzhanbekov D. Dzhiner I. E. Pashkova N. P. Mozheiko K. M. Khizroev S. V. Gautier Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor Vestnik Transplantologii i Iskusstvennyh Organov living donor liver transplantation wilson’s disease fulminant liver failure ав0-incompatible transplantation |
| title | Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor |
| title_full | Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor |
| title_fullStr | Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor |
| title_full_unstemmed | Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor |
| title_short | Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor |
| title_sort | right lobe liver transplantation in patient with fulminant form of the wilson s disease from ab0 incompatible relative donor |
| topic | living donor liver transplantation wilson’s disease fulminant liver failure ав0-incompatible transplantation |
| url | https://journal.transpl.ru/vtio/article/view/750 |
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