Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease

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• 作者：Nishant Verma (1)
  Gautham Pai (1)
  Pankaj Hari (1)
  Rakesh Lodha (1)
  
• 关键词：Wilson disease ; Acute liver failure ; Copper ; Hemolysis ; Plasma exchange
• 刊名：The Indian Journal of Pediatrics
• 出版年：2014
• 出版时间：May 2014
• 年：2014
• 卷：81
• 期：5
• 页码：498-500
• 全文大小：
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• 作者单位：Nishant Verma (1)
  Gautham Pai (1)
  Pankaj Hari (1)
  Rakesh Lodha (1)
  
  1. Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India
  
• ISSN：0973-7693

文摘

Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism which primarily involves the liver and the central nervous system. Rarely, WD can present as acute liver failure (ALF) and this disease is universally fatal in the absence of liver transplantation. The authors report a young girl with WD ALF, who showed signs of recovery after prompt initiation of plasma exchange (PE) and chelation therapy. Though liver transplantation could not be done in this child and the child died 8?d after stopping PE, this case highlights that PE can be a successful medical treatment in WD ALF and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.