CLINICAL CASE OF WILSON-KONOVALOV DISEASE


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Abstract

The paper presents the current data on the nature, characteristics of clinical symptoms, diagnosis and treatment of Witson-Konovatov disease (WKD), as well as clinical observation of the author. Patient A., born in 1992, was admitted to the hospital 16.06.2010, complaining of weakness, fatigue, abdominal distension, and feeling of fullness in the epigastric region, nosebleeds, and menstrual disorders. During a thorough examination has been diagnosed: cirrhosis in the outcome of abdominal form of WKD, fulminant (hemolytic crisis). Complications were: hepatocellular failure, jaundice, portal hypertension (ascites, esophageal varices, splenomegaly), hepatic encephalopathy 2th grade, hemolytic anemia, thrombocytopenia, coagulopathy, hepatorenal syndrome type 1. 09.07.2010 the surgery was performed - orthotopic liver transplantation. Currently, the patient's condition is satisfactory; she has graduated from high school in the capital city, she works. It is emphasized that WKD - one of the few hereditary diseases in which the possible favorable prognosis in the case of early diagnosis and early treatment, and the life expectancy of patients does not differ from that in the general population.

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About the authors

Elena Yurevna Eremina

N.P. Ogarev Mordovia State University

Email: eeu61@mail.ru
MD, Professor, Head of the Department of Internal Medicine Propaedeutics 430005, Republic of Mordovia, Saransk, 68 Bolshevistskaya St

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