Roche-Sicot J, Benhamou J P
Ann Intern Med. 1977 Mar;86(3):301-3. doi: 10.7326/0003-4819-86-3-301.
In three patients, the first manifestation of Wilson's disease was a syndrome in which acute intravascular hemolysis and acute liver failure were associated. This syndrome developed in three periods; the first, lasting 3 to 14 days, was characterized by fatigue, fever, and jaundice; the second, lasting 1 or 2 days, by severe intravascular hemolysis; and the third, lasting 2 to 6 days, by hepatic encephalopathy. All of the patients died from liver failure 7 to 21 days after the onset of the syndrome. The association of acute intravascular hemolysis and acute live failure is a characteristic manifestation of Wilson's disease; it is rarely associated with other liver diseases. This association 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.
在三名患者中，威尔逊氏病的首发表现为一种伴有急性血管内溶血和急性肝衰竭的综合征。该综合征分为三个阶段发展；第一阶段持续3至14天，特征为疲劳、发热和黄疸；第二阶段持续1或2天，以严重的血管内溶血为特征；第三阶段持续2至6天，以肝性脑病为特征。所有患者均在该综合征发作后7至21天死于肝衰竭。急性血管内溶血与急性肝衰竭的关联是威尔逊氏病的特征性表现；它很少与其他肝脏疾病相关。这种关联可能是由于铜蓄积导致肝细胞坏死所致，其后果是急性肝衰竭以及坏死肝细胞释放到血浆中的大量铜对红细胞的破坏。
