Korean J Hepatol > Volume 16(1); 2010 > Article
The Korean Journal of Hepatology 2010;16(1): 83-88.
doi: https://doi.org/10.3350/kjhep.2010.16.1.83
Cholestyramine resin for erythropoietic protoporphyria with severe hepatic disease: a case report
Dong-Jun Yoo, M.D., Han Chu Lee, M.D., Eunsil Yu, M.D.1, Young-Joo Jin, M.D., Ju-Hyun Shim, M.D., Kang Mo Kim, M.D., Young-Suk Lim, M.D., Young-Hwa Chung, M.D., Yung Sang Lee, M.D., Dong-Jin Suh, M.D.
Department of Internal Medicine, 1Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
ABSTRACT
Erythropoietic protoporphyria (EPP) is a rare disorder of heme biosynthesis caused by mutations in the gene encoding the enzyme ferrochelatase. In EPP, deficient ferrochelatase activity leads to the excessive production and biliary excretion of protoporphyrin (PP). The major clinical features of EPP are photosensitivity and hepatobiliary disease that may progress to severe liver disease, that are caused by the toxicity of PP. EPP-related liver disease has been treated medically or surgically including liver transplantation. We described a 20-year-old male with severe liver disease who was diagnosed with EPP based on clinical and laboratory findings. He was treated with cholestyramine resin. Six months after the treatment, he was doing well without any abdominal pain or photosensitivity. (Korean J Hepatol 2010;16:83-88)
KeyWords: Erythropoietic protoporphyria; Cholestyramine; Liver
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