[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.
Ignatova
T M
TM
Il'ina
N A
NA
Potapova
A V
AV
Iusov
S P
SP
Popova
I V
IV
D000293:Adolescent; D000328:Adult; D002614:Chelating Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use
D002614:Chelating Agents; D010396:Penicillamine
Vistseral'nye proiavleniia bolezni Vil'sona-Konovalova (analiz 22 nabliudeniĭ).