급성 간부전과 용혈성 빈혈로 증상 발현을 보인 Wilson 씨병 = Acute Hepatic Failure and Hemolytic Anemia Associated as A First Manifestation of Wilson`s Disease

다국어 초록 (Multilingual Abstract)

The first manifestations of Wilsons disease are usually neuropsychiatric disorders and less fre- quently, hepatic disorders (cirrhosis or chronic hepatitis) or transient hemolytic anemia. The purpose of this work is to describe a less well known mode of onset of the disease characterized by the association of intravascular hemolysis and acute liver failure. The diagnosis of Wilson's disease was based in the presence of the Kayser-Fleischer ring and decreased serum ceruloplasmin, and acute intravascular hemolysis was based in the sudden develop- ment of anemia, the high value for the reticulocyte count, the absence of demonstrable bleeding, unconjugated hyperbilirubinemia. Necropsy revealed active liver cirrhosis, micronodular type with marked cholestasis. The mechanism of this syndrome is unknown. A hypothetical explanation might result from hepatic cell necrosis due to accumulation of copper, the consequences being acute liver failure and destruction of erythrocytes by the large amounts of copper released from the necrotic hepatic cells to the plasma. In conclusion: 1) The syndrome of acute intravascular hemolysis and acute liver failure is very suggestive of Wilsons disease. 2) This association can be the first clinical manifestation of the disease. 3) The treatment is generally unsuccessful, and the outcome is usually fatal.