van Eijk Rachel L A, Metselaar Herold J, van der Steen Marijke S, Uil Jan J
Ziekenhuis Gelderse Vallei, Ede, The Netherlands.
Ned Tijdschr Geneeskd. 2011;155(41):A3688.
Acute liver failure is a rare but mostly severe disorder in previously healthy patients. Viral infections and drugs are the most common causes in the Western world. A small percentage of acute liver failure is caused by Wilson's disease. We describe a previously healthy 23-year-old female with acute haemolytic anemia and liver failure as the first manifestations of Wilson's disease. There was rapid deterioration to multi-organ failure and the patient died less than 24 hours after initial presentation. Relatively simple laboratory tests can be used for initial screening of acute liver failure due to Wilson's disease. Liver transplantation is the only way to ensure survival of the patient. Rapid transfer to a specialised centre is, therefore, of the utmost concern.
急性肝衰竭在既往健康的患者中是一种罕见但大多较为严重的病症。在西方世界,病毒感染和药物是最常见的病因。一小部分急性肝衰竭由威尔逊病引起。我们描述了一名既往健康的23岁女性,其以急性溶血性贫血和肝衰竭作为威尔逊病的首发表现。病情迅速恶化为多器官功能衰竭,患者在首次就诊后不到24小时死亡。相对简单的实验室检查可用于对威尔逊病所致急性肝衰竭的初步筛查。肝移植是确保患者存活的唯一方法。因此,迅速转诊至专科中心至关重要。