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Liver transplantati...
Liver transplantation for hereditary transthyretin amyloidosis : after 20 years still the best therapeutic alternative?
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- Ericzon, Bo-Göran (author)
- Karolinska Institutet
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Wilczek, Henryk E. (author)
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Larsson, Marie (author)
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- Wijayatunga, Priyantha (author)
- Umeå universitet,Statistik
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Stangou, Arie (author)
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Pena, João Rodrigues (author)
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Furtado, Emanuel (author)
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Barroso, Eduardo (author)
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Daniel, Jorge (author)
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Samuel, Didier (author)
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Adam, Rene (author)
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Karam, Vincent (author)
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Poterucha, John (author)
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Lewis, David (author)
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Ferraz-Neto, Ben-Hur (author)
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Cruz, Márcia Waddington (author)
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Munar-Ques, Miguel (author)
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Fabregat, Juan (author)
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Ikeda, Shu-Ichi (author)
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Ando, Yukio (author)
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Heaton, Nigel (author)
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Otto, Gerd (author)
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- Suhr, Ole (author)
- Umeå universitet,Medicin
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(creator_code:org_t)
- 2015
- 2015
- English.
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In: Transplantation. - 0041-1337 .- 1534-6080. ; 99:9, s. 1847-1854
- Related links:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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http://kipublication...
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Abstract
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- BACKGROUND: Until recently, liver transplantation (Ltx) was the only available treatment for hereditary transthyretin (TTR) amyloidosis; today, however, several pharmacotherapies are tested. Herein, we present survival data from the largest available database on transplanted hereditary TTR patients to serve as a base for comparison.METHODS: Liver transplantation was evaluated in a 20-year retrospective analysis of the Familial Amyloidosis Polyneuropathy World Transplant Registry.RESULTS: From April 1990 until December 2010, data were accumulated from 77 liver transplant centers. The Registry contains 1940 patients, and 1379 are alive. Eighty-eight Ltx were performed in combination with a heart and/or kidney transplantation. Overall, 20-year survival after Ltx was 55.3%. Multivariate analysis revealed modified body mass index, early onset of disease (<50 years of age), disease duration before Ltx, and TTR Val30Met versus non-TTR Val30Met mutations as independent significant survival factors. Early-onset patients had an expected mortality rate of 38% that of the late-onset group (P < 0.001). Furthermore, Val30Met patients had an expected mortality rate of 61% that of non-TTR Val30Met patients (P < 0.001). With each year of duration of disease before Ltx, expected mortality increased by 11% (P < 0.001). With each 100-unit increase in modified body mass index at Ltx, the expected mortality decreased to 89% of the expected mortality (P < 0.001). Cardiovascular death was markedly more common than that observed in patients undergoing Ltx for end-stage liver disease.CONCLUSIONS: Long-term survival after Ltx, especially for early-onset TTR Val30Met patients, is excellent. The risk of delaying Ltx by testing alternative treatments, especially in early-onset TTR Val30Met patients, requires consideration.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Gastroenterologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Gastroenterology and Hepatology (hsv//eng)
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- ref (subject category)
- art (subject category)
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- By the author/editor
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Ericzon, Bo-Göra ...
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Wilczek, Henryk ...
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Larsson, Marie
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Wijayatunga, Pri ...
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Stangou, Arie
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Pena, João Rodri ...
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show more...
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Furtado, Emanuel
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Barroso, Eduardo
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Daniel, Jorge
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Samuel, Didier
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Adam, Rene
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Karam, Vincent
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Poterucha, John
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Lewis, David
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Ferraz-Neto, Ben ...
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Cruz, Márcia Wad ...
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Munar-Ques, Migu ...
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Fabregat, Juan
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Ikeda, Shu-Ichi
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Ando, Yukio
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Heaton, Nigel
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Otto, Gerd
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Suhr, Ole
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show less...
- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Gastroenterology ...
- Articles in the publication
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Transplantation
- By the university
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Umeå University
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Karolinska Institutet