|
KMID : 1103920100160010083
|
|
Korean Journal of Hepatology
2010 Volume.16 No. 1 p.83 ~ p.88
|
|
|
Cholestyramine resin for erythropoietic protoporphyria with severe hepatic disease: a case report
|
|
Yoo Dong-Jun
Lee Han-Chu
Yoo Eun-Sil
Jin Young-Joo
Shim Ju-Hyun
Kim Kang-Mo
Lim Young-Suk
Chung Young-Hwa
Lee Yung-Sang
Suh Dong-Jin
|
|
|
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.
|
|
|
KEYWORD
|
|
|
Erythropoietic protoporphyria, Cholestyramine, Liver
|
|
|
FullTexts / Linksout information
|
|
 
|
|
|
Listed journal information
|
|
   
|