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CASE REPORTS  
CMH 2012 December;18:411-415.
Published online 2012 December 24. doi:http://dx.doi.org/10.3350/cmh.2012.18.4.411
Copyright © 2012 The Korean Association for the Study of the Liver
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Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria
Pyoung-Jae Park, Shin Hwang, Young-Il Choi, Young-Dong Yu, Gil-Chun Park, Sung-Won Jung, Sam-Youl Yoon, Gi-Won Song, Tae-Yong Ha, and Sung-Gyu Lee
Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Corresponding Author: Shin Hwang ,Tel: +82-2-3010-3930, Fax: +82-2-474-9027, Email: shwang@amc.seoul.kr
ABSTRACT
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.
Keywords: Liver transplantation; Acute-on-chronic liver failure; Erythropoietic protoporphyria
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