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Liver transplantation for erythropoietic protoporphyria

✍ Scribed by Mion, François B.C.; Faure, Jean-Louis; Berger, Françoise; McGregor, Brigitte; Perrot, Henri; Paliard, Pierre


Book ID
123056634
Publisher
Elsevier Science
Year
1992
Tongue
English
Weight
1021 KB
Volume
16
Category
Article
ISSN
0168-8278

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In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin, primarily in the bone marrow, resulting in increased biliary excretion of this heme precursor. Some patients will develop progressive liver disease that may ultimately require liver transplantation. However, exc

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Liver transplantation is an established lifesaving treatment for patients with severe protoporphyric liver disease, but disease recurrence in the graft occurs for the majority of recipients. Severe burn injuries may occur when protective light filters are not used with surgical luminaires. Motor neu