scholarly journals Emergent living related liver transplantation for a patient with severe liver failure due to Wilson's disease

Kanzo ◽  
2004 ◽  
Vol 45 (9) ◽  
pp. 473-478 ◽  
Author(s):  
Masato KATO ◽  
Keiichi KUBOTA ◽  
Junnji KITA ◽  
Mitsugi SIMODA ◽  
Takehiko NEMOTO ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Severe Liver ◽  
Living Related Liver Transplantation ◽  
Living Related

Living-related liver transplantation for Wilson's disease

2005 ◽  
Vol 19 (4) ◽  
pp. 483-486 ◽  
Author(s):  
Sumihito Tamura ◽  
Yasuhiko Sugawara ◽  
Yoji Kishi ◽  
Nobuhisa Akamatsu ◽  
Junichi Kaneko ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Living Related Liver Transplantation ◽  
Living Related

scholarly journals Living-related liver transplantation for Wilson's disease

2005 ◽  
Vol 18 (6) ◽  
pp. 651-656 ◽  
Author(s):  
Xue-hao Wang ◽  
Feng Cheng ◽  
Feng Zhang ◽  
Xiang C. Li ◽  
Lian B. Kong ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Living Related Liver Transplantation ◽  
Living Related

scholarly journals Májátültetés Wilson-kóros betegekben, 1996–2017

2019 ◽  
Vol 160 (51) ◽  
pp. 2021-2025
Author(s):  
Dániel Németh ◽  
Anikó Folhoffer ◽  
Szilvia Bianka László ◽  
László Kóbori ◽  
Dénes Görög ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson’S Disease ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Chronic Liver ◽  
Diagnostic Process ◽  
Decompensated Liver Cirrhosis ◽  
The Mean

Abstract: Introduction: Wilson’s disease is a lethal-without-treatment inherited disorder of copper metabolism. Despite the increased focus on the diagnosis and treatment, liver transplantation is needed in a number of cases even nowadays. Aim: To collect and analyze the data of the Hungarian Wilson’s disease patients who underwent liver transplantation. Method: Data of 24 Wilson’s disease patients who underwent liver transplantation at the Semmelweis University have been analyzed retrospectively. The diagnosis of Wilson’s disease was based on the international score system. The diagnosis of acute liver failure corresponded to the King’s College criteria. All liver transplantations had been performed at the Department of Transplantation and Surgery of Semmelweis University, in 1996 for the first time. Results: The mean age was 26 years, F/M = 13/11. Twelve patients needed urgent liver transplantation for acute liver failure, and 12 underwent transplantation for decompensated liver cirrhosis. One patient had been retransplanted because of chronic rejection. Three patients with acute on chronic liver failure were transplanted via the Eurotransplant program. The mean time on the waiting list was 3 vs 320 days in acute liver failure and chronic liver disease groups, respectively. The overall 5-year survival was 66%, but it was 80% after 2002 indicating both the learning curve effect and the improvement of vigilance in Hungary. Despite difficulties of the diagnostic process, Wilson’s disease was identified in 21/24 patients prior to the transplantation. Conclusion: Liver transplantation is needed in a number of cases of Wilson’s disease. The ideal indication and timing of transplantation may improve the survival of the patients. Orv Hetil. 2019; 160(51): 2021–2025.

scholarly journals Liver Transplantation as a Cure for Neurologically Advanced Wilson’s disease. Learning More from Experience

2020 ◽  
Vol 4 (4) ◽  
Author(s):  
Marina Moguilevitch ◽  
Tracey Straker ◽  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Clinical Findings ◽  
Chronic Liver ◽  
Older Children ◽  
Chronic Liver Failure ◽  
Long Term Follow Up ◽  
Short And Long Term

Clinical presentation and progression of Wilson’s disease can be diverse in different groups of patients. While young children most likely to present with acute or chronic liver failure, older children and adults may exhibit extrahepatic manifestation like neuropsychiatric, cardiac, renal, pancreatic and several others. Diagnosis of Wilson’s disease is based on the combination of specific clinical findings, laboratory and genetic testing. There are different modalities of treatment available for this condition. Liver transplantation has become a acceptable treatment option for acute and chronic liver failure related to Wilson’s disease. It allows for resolution of metabolic disorder and hepatic disease progression. There are no definitive criteria and established outcomes in transplantation of the patients with Wilson’s disease who have different degree of neuropsychiatric presentation. Therefore, it is very important to create specific criteria for selecting of these patients before transplantation and to have short-and long-term follow up system to improve their outcome and survival.

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