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Erythropoietic protoporphyria with severe liver dysfunction and acute pancreatitis

✍ Scribed by Koga Komatsu; Tooru Shimosegawa; Midori Uchi; Yoshisuke Maruhama; Takayoshi Toyota

Publisher
Springer Japan
Year
2000
Tongue
English
Weight
146 KB
Volume
35
Category
Article
ISSN
0944-1174

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Erythropoietic protoporphyria (EPP) is a disease of the heme metabolism due to a deficiency of ferrochelatase, leading to accumulation of protoporphyrin (PPIX) in the erythrocyte (red blood cell [RBC]). The major clinical manifestation in EPP is photosensitivity; however, in a small number of patien