Abstract:
:A 60-year-old white male presenting with a clinical picture of obstructive jaundice was subsequently found to have erythropoietic protoporphyria. The diagnosis was suspected because of a history of life-long photosensitivity and was confirmed by finding high levels of erythrocyte protoporphyrin. Liver biopsy revealed birefringent deposits of protoporphyrin by polarization microscopy accompanied by severe hepatic injury and fibrosis. The patient died rapidly from liver failure, and at autopsy the biliary tree was patent. Despite the autosomal dominant transmission of erythropoietic protoporphyria, we failed to detect any family members with the disease. This report is concluded with a brief discussion of the liver involvement in erythropoietic protoporphyria.
journal_name
Gastroenterologyjournal_title
Gastroenterologyauthors
Singer JA,Plaut AG,Kaplan MMsubject
Has Abstractpub_date
1978-03-01 00:00:00pages
588-91issue
3eissn
0016-5085issn
1528-0012pii
S0016508578000657journal_volume
74pub_type
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