Tyrosinemia and Liver Transplantation: Experience at CHU Sainte-Justine
In: Advances in Experimental Medicine and Biology ISBN: 9783319557793; (2017)
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Zugriff:
Tyrosinemia is a disease of the tyrosine metabolism, affecting mainly liver, kidney and peripheral nerves. Two forms of liver disease caused by a deficiency of FAH are recognised: (1) acute liver failure; (2) chronic liver disease. Since the introduction of NTBC [2-(2-nitro-4-trifluoromethyl benzoyl)-1-3-cyclohexanedione] (nitisinoneR) in the treatment of tyrosinemia, no liver disease has been observed when started in the first weeks of life. Liver transplantation is a good option for the treatment of tyrosinemic patients developing liver nodules, with high suspicion of hepatocarcinoma. In the long-term outcome of the liver transplant, survival was of 90% in tyrosinemic patients.
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Tyrosinemia and Liver Transplantation: Experience at CHU Sainte-Justine
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Autor/in / Beteiligte Person: | Mitchell, Grant A. ; Alvarez, Fernando |
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Quelle: | Advances in Experimental Medicine and Biology ISBN: 9783319557793; (2017) |
Veröffentlichung: | Springer International Publishing, 2017 |
Medientyp: | unknown |
ISBN: | 978-3-319-55779-3 (print) |
DOI: | 10.1007/978-3-319-55780-9_5 |
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