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KMID : 1103920120180040411
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Korean Journal of Hepatology
2012 Volume.18 No. 4 p.411 ~ p.416
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Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria
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Park Pyoung-Jae
Hwang Shin
Choi Young-Il
Yu Young-Dong
Park Gil-Chun
Jung Sung-Won
Yoon Sam-Youl
Song Gi-Won
Ha Tae-Yong
Lee Sung-Gyu
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Abstract
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Erythropoietic protoporphyria (EPP) is an inherited disorder of the heme metabolic pathway that is characterized by accumulation of protoporphyrin in the blood, erythrocytes, and tissues, and cutaneous manifestations of photosensitivity, all resulting from abnormalities in ferrochelatase (FECH) activity due to mutations in the FECH gene. Protoporphyrin is excreted by the liver, and excess protoporphyrin leads to cholelithiasis with obstructive episodes and chronic liver disease, finally progressing to liver cirrhosis. Patients with end-stage EPP-associated liver disease require liver transplantation. We describe here a 31-year-old male patient with EPP who experienced acute-on-chronic liver failure and underwent deceased-donor liver transplantation. Surgical and postoperative care included specific shielding from exposure to ultraviolet radiation to prevent photosensitivity-associated adverse effects. The patient recovered uneventfully and was doing well 24 months after transplantation. Future prevention and treatment of liver disease are discussed in detail.
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KEYWORD
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Liver transplantation, Acute-on-chronic liver failure, Erythropoietic protoporphyria
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