Erythropoietic protoporphyria: juvenile protoporphyrin hepatopathy cirrhosis and death.
Severe hepatic cirrhosis and failure in erythropoietic protoporphyria is rare. An 11-year-old boy is described who developed protoporphyrin hepatopathy (protoporphyrin 5.75 mg/gm liver wet weight), cirrhosis, and liver failure and died.
D002648:Child; D004920:Erythropoiesis; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008103:Liver Cirrhosis / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008854:Microscopy, Electron; D008856:Microscopy, Fluorescence; D011166:Porphyrins / Q000378:metabolism*; D011524:Protoporphyrins / Q000378:metabolism*; D012867:Skin / Q000473:pathology
D011166:Porphyrins; D011524:Protoporphyrins