Liver and hepatocerebral dystrophy

Wilson-Konovalov disease (WKD) is one of the most difficult to diagnose liver disease due to the long latent course in the early stages and the large polymorphism of symptoms in the advanced stage of the disease. The main signs of WKD are liver diseases, neuropsychiatric disorders, Kayser-Fleischer ring, acute episodes of hemolysis, often in combination with acute liver failure. The diagnosis is especially difficult in children and adults with active forms of liver disease. Timely diagnosis is an important medical and social problem, since early pathogenetic therapy improves the quality of life and social adaptation of patients. The article deals with the pathogenesis of BVK, the clinical and morphological features of the variants of the course of the disease: fulminant hepatic failure, chronic hepatitis and latent variant with the development of liver cirrhosis. An abdominal form with early development of edema and ascitic syndrome on the background of a violation of the synthetic function of the liver was identified. A detailed description of the role of a violation of copper metabolism To overcome the diagnostic problem, several clinical and laboratory signs are evaluated, as well as genetic testing in a point system; this assessment allows you to calculate the likely and very likely diagnosis. The tactics of drug therapy and the possibility of optimizing it are considered, the advantage of liver transplantation is evaluated. Two clinical observations of Wilson-Konovalov’s disease are presented, demonstrating the capabilities of various diagnostic methods (1st observation) and features of the course of the hepatic form of the disease (2nd observation).

гепатоцеребральная дистрофия (Болезнь Вильсона-Коновалова), нарушения обмена меди, церулоплазмин, печеночная форма, абдоминальная форма, неврологические нарушения, диагностика, Д-пеницилламин, цинктерал, трансплантация печени, hepatocerebral dystrophy (Wilson-Konovalov disease), disorders of copper metabolism, ceruloplasmin, hepatic form, abdominal form, neurological disorders, diagnosis, D-penicillamine, zincteral, liver transplantation