Congenital hepatic fibrosis associated with Mallory bodies and copper retention.
New observations are described in a case of congenital hepatic fibrosis. Histochemical stains of liver tissue for copper (rhodanine) and copper associated protein (orcein) were positive and hepatic copper concentration, measured by atomic absorption spectroscopy, was markedly elevated being 550 micrograms/g dry weight (NR less than 30 micrograms/g dry weight). Mallory bodies were observed in several areas in liver sections. These observations have not been previously recorded in congenital hepatic fibrosis. Increased hepatic copper concentration in this case may have contributed to the production of liver damage. Hepatic copper concentration should be specifically measured in other patients with congenital hepatic fibrosis.
D000328:Adult; D003300:Copper / Q000378:metabolism*; D004721:Endoplasmic Reticulum / Q000648:ultrastructure; D006801:Humans; D008099:Liver / Q000648:ultrastructure*; D008107:Liver Diseases / Q000151:congenital* / Q000378:metabolism / Q000473:pathology; D008297:Male
D003300:Copper