Ignatova T M, Il'ina N A, Potapova A V, Iusov S P, Popova I V
Klin Med (Mosk). 1995;73(6):23-6.
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.
22例威尔逊-科诺瓦洛夫病（WKD）患者不仅有肝脏症状，还有内脏症状：肾脏病变、皮肤血管炎、关节痛、内分泌紊乱等。在这22例患者中，有8例肝外症状出现在肝脏体征出现前10年。后者的特点是水肿-腹水综合征、溶血发作、出血综合征的发生率较高；血清转氨酶活性较低。仅6例患者出现初始神经症状。12例患者从排铜治疗中获益。4例患者病情进展导致死亡。为了早期发现WKD，应对所有45岁以下有肝脏症状或WKD特异性内脏表现的受试者进行特殊诊断检查。对患者的同胞进行检查也是可取的。
