Cholestyramine resin for erythropoietic protoporphyria with severe hepatic disease: a case report / 대한간학회지
The Korean Journal of Hepatology
;
: 83-88, 2010.
Article
in English
| WPRIM
| ID: wpr-98606
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Protoporphyrins
/
Bilirubin
/
Cholestyramine Resin
/
Protoporphyria, Erythropoietic
/
Edema
/
Erythema
/
Ferrochelatase
/
Liver Diseases
Limits:
Humans
/
Male
Language:
English
Journal:
The Korean Journal of Hepatology
Year:
2010
Type:
Article
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