Letter to the editor: Diagnosis of erythropoietic protoporphyria with severe liver injury-a case report  

作　　者：Debby Wensink Margreet AEM Wagenmakers JH Paul Wilson Janneke G Langendonk 

机构地区：[1]Porphyria Center Rotterdam, Center for lysosomal and metabolic disease, Department of Internal Medicine, Erasmus University Medical Center

出　　处：《World Journal of Gastroenterology》2019年第30期4292-4293,共2页世界胃肠病学杂志（英文版）

摘　　要：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.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.

关 键 词：Erythropoietic protoporphyria LIVER DISEASE BLOOD TRANSFUSION PROTOPORPHYRIN IX Treatment 

分 类 号：R[医药卫生]