[Wilson-Konovalov cirrhosis].
The results from some complex investigations were summed up, electron microscopic includ., of 18 patients with Wilson--Konovalov disease. The dynamic follow up of the patients with a predominantly liver symptomatic justifies, in a considerable part of them, the terminology "Wilson--Konovalov" cirrhosis. The mitochondrial changes found suggest disorders in hepatocyte energetic potential. The objectivized manifest or discrete neutral-lipid or phospho-lipid build-ups in the hepatic parenchymal cell suggest multidirectional disorders in lipid metabolism with lysosome participation. The pathogenetic long-term treatment with d-penicillamine substantially improves not only the clinical symptomatics but the remote prognosis of the disease as well.
D000293:Adolescent; D000328:Adult; D001707:Biopsy, Needle; D002467:Cell Nucleus / Q000648:ultrastructure; D002648:Child; D005260:Female; D006527:Hepatolenticular Degeneration / Q000473:pathology*; D006801:Humans; D010535:Laparoscopy; D008099:Liver / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D008930:Mitochondria, Liver / Q000648:ultrastructure
Tsiroza na Wilson-Konovalov.