[Visceral manifestations of Wilson-Konovalov disease (analysis of 22 cases)]

Twenty-two patients with Wilson-Konovalov disease (WKD) had not only hepatic but also visceral symptoms: renal affection, cutaneous vasculitis, arthralgias, endocrine disorders, etc. In 8 of these 22 patients extrahepatic symptoms appeared 10 years before hepatic signs. The latter were distinguished by the frequency of edematic-ascitic syndrome, hemolytic episodes, hemorrhagic syndrome; low activity of serum aminotransferase. Initial neurological symptoms were registered in 6 patients only. 12 patients benefited from copper-eliminating therapy. 4 patients had advanced process which caused death. To detect WKD early, special diagnostic tests should be conducted in all subjects under 45 with hepatic symptoms or WKD-specific visceral manifestations. Examination of the patients' sibs is also desirable.