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World J Gastroenterol ; 25(30): 4292-4293, 2019 08 14.
Artigo em Inglês | MEDLINE | ID: mdl-31435180

RESUMO

Erythropoietic protoporphyria (EPP) is an extremely rare disease which is often unrecognized as diagnosis. In the recent article Lui et al describe a patient with a new diagnosis of EPP with severe liver injury. Approximately 5%-20% of patients with EPP develop liver manifestations. The most severe complication of EPP is an hepatic crisis, which is a medical emergency requiring urgent treatment. Intensive treatment should consist of (exchange) transfusions and preferably in a center that performs liver transplantations.


Assuntos
Protoporfiria Eritropoética , Transfusão Total , Humanos , Doenças Raras
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