Effects of anticopper therapy on hepatocellular mitochondria in patients with Wilson's disease: an ultrastructural and stereological study.
Liver biopsy specimens from 7 patients with Wilson's disease (hepatolenticular degeneration), obtained before and after 3 to 5 years of D-penicillamine therapy, were studied by electron microscopy and stereology. The characteristic mitochondrial abnormalities encountered in the hepatocytes of untreated patients were less pronounced or disappeared after treatment in 5 of the 7 patients. Simultaneously, relative mitochondrial volume, surface density of the external mitochondrial membranes, and the number of these profiles per unit area increased, whereas abnormal elevations SGOT and SGPT returned to normal levels.
D000293:Adolescent; D002648:Child; D003300:Copper / Q000032:analysis / Q000037:antagonists & inhibitors*; D006527:Hepatolenticular Degeneration / Q000178:diet therapy / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000032:analysis / Q000473:pathology; D008111:Liver Function Tests; D008930:Mitochondria, Liver / Q000187:drug effects* / Q000648:ultrastructure; D010396:Penicillamine / Q000627:therapeutic use; D010780:Photogrammetry
D003300:Copper; D010396:Penicillamine