급성 간부전과 용혈성 빈혈로 증상 발현을 보인 Wilson 씨병

• Published in: The Korean journal of gastroenterology Vol. 21; no. 1; pp. 191 - 195
• Main Authors: 권상옥, Sang Ok Kwon, 이광훈, Kwang Hoon Lee, 심영학, Young Hak Shim, 박진현, Jin Hyun Park, 장우익, Woo Ik Jang, 전근제, Geun Jae Jeon, 최승옥, Seoung Ok Choi
• Format: Journal Article
• Language: Korean
• Published: 대한소화기학회 01-01-1989
• ISSN: 1598-9992

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.