Liver transplantation for Wilson’s disease: Indications and outcome

Abstract

The objective of this study was to determine the indications for and results of liver transplantation in patients with Wilson's disease on the basis of results of a survey with retrospective review of data obtained on 55 transplants performed at centers in the United States and Europe. The study group comprised 32 females and 23 males, aged 8.5 to 51 yr, with features diagnostic of Wilson's disease. Indication for orthotopic liver transplantation included hepatic insufficiency (n = 32), wilsonian fulminant hepatitis (n = 21), intractable neurological Wilson's disease (n = 1) and gastrointestinal hemorrhage (n = 1). Forty-three patients have survived, at this writing, from 3 mo to 20 yr. Mean and median survival after orthotopic liver transplantation were 2.7 and 2.5 yr, respectively. Survival at 1 yr was 79%. Nonfatal complications occurred in five patients. Of the seven patients given transplants for hepatic insufficiency who manifested neurological and/or psychiatric manifestations at the time of orthotopic liver transplantation, four showed improvement of these symptoms. One patient given a transplant for intractable neurological disease improved but died of a vascular complication. Our data demonstrate that liver transplantation is life-saving but not without risk for patients with wilsonian fulminant hepatitis or chronic severe hepatic insufficiency unresponsive to medical therapy. Furthermore, neurological or psychiatric symptoms due to Wilson's disease may improve after liver transplantation; however, the role of this procedure in the management of patients with neurological Wilson's disease in the absence of hepatic insufficiency is still uncertain.

Serum Copper Is Decreased in Premature Newborns and Increased in Newborns with Hemolytic Jaundice

“…Because the liver has a marked capacity to excrete copper, chronic copper toxicity is rare. In individuals with liver disease, however, a moderate, sudden increase in plasma copper may lead to accumulation of the metal, but the precise role of the latter in subsequent hepatic injury is unclear (20,21 ). Additionally, the eventual hepatocyte dysfunction from copper overload may lead to cell death with release of copper into the blood (9,20 ).…”

Liver transplantation

“…Liver transplantation is the only treatment for patients with FHF, those with chronic liver disease who fail to respond to chelation therapy, or for recurrent disease in patients who discontinue treatment. [80][81][82] Liver transplantation usually reverses the metabolic abnormalities associated with Wilson's disease; however, long-standing neurological dysfunction may not improve in some patients. [83][84][85] Survival rates 1 year after transplantation have ranged from 70% to 90%.…”

Association of Dopamine Receptor Gene Polymorphisms with the Clinical Course of Wilson Disease

“…WD is associated with a wide spectrum of symptoms (hepatic, neurological, psychiatric, and others) as well as great variability in clinical presentation and outcome (Roberts and Schilsky 2008;Ala et al 2007;Ferenci et al 2003Ferenci et al , 2007Schilsky et al 1994). Although these differences remain largely unexplained, several factors are known to impact clinical presentation of WD, including gender (Schilsky et al 1994;Litwin et al 2012a) and genotype (Stapelbroek et al 2004;Gromadzka et al 2005Gromadzka et al , 2006. It is also suspected that WD presentation may be influenced by polymorphisms in the genes encoding prion-related protein, methylenetetrahydrofolate reductase, interleukin-1 receptor antagonist, and apolipoprotein-E Gromadzka et al 2011a, b;Schiefermeier et al 2000;Litwin et al 2012b).…”