Changes in protoporphyrin distribution dynamics during liver failure and recovery in a patient with protoporphyria and Epstein-Barr viral hepatitis.
Acute liver failure with cholestasis, histologic and serologic evidence of Epstein-Barr viral infection, and associated autoimmune hemolytic anemia occurred in a patient with lifelong protoporphyria. Changes in previously established baseline protoporphyrin distribution dynamics in erythrocyte, plasma, and fecal excretion compartments were observed during the period of severe hepatic dysfunction and recovery. These changes were consistent with predictions of a previously described conceptual model for human protoporphyria.
D000208:Acute Disease; D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000378:metabolism / Q000473:pathology; D001706:Biopsy; D002779:Cholestasis / Q000378:metabolism / Q000473:pathology; D005260:Female; D006525:Hepatitis, Viral, Human / Q000378:metabolism* / Q000473:pathology; D004854:Herpesvirus 4, Human; D006801:Humans; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000378:metabolism* / Q000473:pathology; D011164:Porphyrias / Q000378:metabolism* / Q000473:pathology; D011166:Porphyrins / Q000378:metabolism*; D011524:Protoporphyrins / Q000378:metabolism*; D014018:Tissue Distribution
D011166:Porphyrins; D011524:Protoporphyrins