[The liver in a case of protoporphyria (author's transl)].
A 18 year old man with protoporphyria had discrete, sunlight-induced skin changes at the nose and the back of his hands, and liver disease. The transaminases ranged from 30 to 200 U/1, uro- and coproporphyrins in the urine were in the normal range. There was no evidence of a progression of the liver disease during 4 years. At peritoneoscopy the liver was yellow-brown with a granular surface. Microscopically fibrosis was present and globular pigment was distributed mostly in hepatocytes. These findings had been misinterpreted as "chronic active hepatitis with choletasis and fibrosis". This observation shows that liver disease in protoporphyria may be present with a normal urinary excretion of uro- and coproporphyrins and suggests that in cases of chronic liver disease of unknown origin the gastroenterologist ought to pay attention to sunlight intolerance and the pathologist to the yellow-brownish pigment of protoporphyrin.
D000293:Adolescent; D006801:Humans; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000209:etiology* / Q000473:pathology; D008297:Male; D011164:Porphyrias / Q000150:complications* / Q000201:enzymology / Q000378:metabolism; D011166:Porphyrins / Q000378:metabolism; D011524:Protoporphyrins / Q000378:metabolism; D000637:Transaminases / Q000378:metabolism
D011166:Porphyrins; D011524:Protoporphyrins; D000637:Transaminases
Die Leber bei einem Fall von Protoporphyrie.