[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.