Wilson disease as a cause of liver injury in cystic fibrosis

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• 作者：Radana Kotalová ; Milan Jirsa ; Vě ; ra Vá ; vrová ; Slá ; vka Vrá ; belová ; -Pouchlá ; Milan Macek Jr.
• 关键词：Cystic fibrosis ; Wilson disease ; Liver disease ; Cholestasis ; Jaundice
• 刊名：Journal of Cystic Fibrosis
• 出版年：2009
• 出版时间：January 2009
• 年：2009
• 卷：8
• 期：1
• 页码：63-65
• 全文大小：260 K

文摘

Cystic fibrosis-related liver disease affects approximately one third of all patients with cystic fibrosis. Initial signs of other liver diseases including the genetically determined disorders of the liver co-inherited with cystic fibrosis may be obscured by or ascribed to cystic fibrosis-related liver disease. We report a patient shown to suffer simultaneously from cystic fibrosis and hepatic Wilson disease. Our case documents that in patients with cystic fibrosis presenting with liver disease, when unusual clinical and/or laboratory abnormalities appear and fail to respond to standard therapy, a second disease, including rare inherited metabolic disorders such as the hepatic form of Wilson disease or α₁-antitrypsin deficiency, should be suspected.