Wilson – Konovalov Disease: Clinical Cases with Different Manifestations and Outcomes

А im: to describe clinical cases of Wilson – Konovalov disease in pediatric patients. Key points. The first case demonstrates the manifestation with unexplained mild elevation aminotransferases at age 6 years. Despite persistent hyperenzymemia, no additional laboratory tests were prescribed. At 10, patient showed signs liver failure and neurological symptoms. Laboratory instrumental examination enabled diagnose stage cirrhosis. diagnosis was confirmed Federal medical center. delayed made 4 years after hyperenzymemia revealed. relief observed orthotopic transplantation right lobe from a living related donor. under surveillance for 10 transplantation. second shows another variant course manifested as an acute hepatitis. To clarify etiology disease, hospitalized. In 2 weeks, developed symptoms failure, progressive hemorrhagic syndrome, hepatic encephalopathy. Death fulminant hepatitis occurred 3 weeks onset onset. Conclusion. Healthcare workers should be aware disease. Screening is recommended children increase transaminases, chronic