Liver transplantation for Wilson's disease

Bellary, S.V.; Hassanein, Tarek; Thiel, David H. Van

doi:10.1016/0168-8278(95)80194-4

Cited by 103 publications

(50 citation statements)

References 16 publications

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“…Zinc administration inhibits lipid peroxidation by increasing glutathione availability [16]. Patients with acute hepatic failure or advanced chronic liver disease have been reported to benefit from orthotopic liver transplantation, which corrects the underlying metabolic defect and may, thus, improve neurological and/or psychiatric symptoms [2,14,55]. Recurrence of copper-associated liver damage in a transplanted liver has not been reported in a WD patient thus far.…”

Section: Clinical Background and Diagnosis Of Wdmentioning

confidence: 97%

Wilson disease

Langner

Denk

2004

Virchows Arch

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Wilson disease (WD) is an autosomal recessive disorder of copper metabolism. Since daily copper intake exceeds the body's requirements, effective means of excreting excess copper are essential. These are accomplished by ATP7B, a new member of the cation-transporting p-type ATPase family, which is mainly expressed in the liver and mediates both copper secretion into plasma (coupled with ceruloplasmin synthesis) and its excretion into bile. Thus far, more than 200 mutations of the WD gene have been detected, causing impairment of ATP7B function and, ultimately, copper accumulation. Excess copper, however, induces free-radical reactions and lipid peroxidation. Resultant liver damage leads to steatosis, inflammation, cirrhosis, and, occasionally, fulminant liver failure. The diagnosis of WD is commonly made on the basis of typical clinical and laboratory findings, including low serum ceruloplasmin, increased urinary copper excretion, and increased hepatic copper content. Since liver morphology is non-specific, and copper histochemistry may lead to both false-negative and false-positive results, the pathologist usually only suspects the disease or assists in its confirmation. Although the value of molecular genetic testing is limited due to the high number of possible gene mutations, polymerase chain reaction may be useful for the evaluation of family members of homozygous index patients.

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Section: Clinical Background and Diagnosis Of Wdmentioning

confidence: 97%

Wilson disease

Langner

Denk

2004

Virchows Arch

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“…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%.…”

Section: Metabolic Diseasesmentioning

confidence: 99%

Liver transplantation

Carithers

2000

Liver Transpl

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Liver transplantation has revolutionized the care of patients with end-stage liver disease. Liver transplantation is indicated for acute or chronic liver failure from any cause. Because there are no randomized controlled trials of liver transplantation versus no therapy, the efficacy of this surgery is best assessed by carefully comparing postoperative survival with the known natural history of the disease in question. The best examples of this are in primary biliary cirrhosis and primary sclerosing cholangitis, for which well-validated disease-specific models of natural history are available. There are currently relatively few absolute contraindications to liver transplantation. These include severe cardiopulmonary disease, uncontrolled systemic infection, extrahepatic malignancy, severe psychiatric or neurological disorders, and absence of a viable splanchnic venous inflow system. One of the most frequently encountered contraindications to transplantation is ongoing destructive behavior caused by drug and alcohol addiction. The timing of the surgery can have a profound impact on the mortality and morbidity of patients undergoing liver transplantation. Because of the long waiting lists for donor organs, the need to project far in advance when transplantation might be required has proven to be one of the greatest challenges to those treating patients with end-stage liver disease. Three important questions must be addressed in a patient being considered for liver transplantation: (1) when should the patient be referred for possible transplantation? (2) when should the patient be listed for transplantation? and (3) when is the patient too sick to have a reasonable chance of surviving the perioperative period?

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“…In this patient group, WD is a possible etiology for ALF amongst others like toxic liver damage, acute hepatitis or ischemic liver damage [6] . The mortality of patients is high, and orthotopic liver transplantation (OLT) often remains the only therapeutic option, which has been shown to be an effective treatment for patients with FWD [7] . The purpose of this study is to outline differences in clinical and biochemical findings between FWD and ALF of other etiologies that might identify cases of FWD.…”

Section: Introductionmentioning

confidence: 99%

Diagnostic criteria for acute liver failure due to Wilson disease

Eisenbach

Sieg²,

Stremmel³

et al. 2007

WJG

101

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AIM:To describe the diagnostic criteria for acute liver failure due to Wilson disease (WD), which is an uncommon cause of acute liver failure (ALF). M E T H O D S :We c o m p a r e d f i n d i n g s o f p a t i e n t s presenting with ALF due to WD to those with ALF of other etiologies. RESULTS:Previously described criteria, such as low alkaline phosphatase activity, ratio of low alkaline phosphatase to total bilirubin or ratio of high aspartate aminotransferase (AST) to alanine aminotransferase (ALT), failed to identify patients with ALF due to WD. There were significant differences in low ALT and AST activities (53 ± 43 vs 1982 ± 938, P < 0.0001 and 87 ± 44 vs 2756 ± 2941, P = 0.037, respectively), low choline esterase activity (1.79 ± 1.2 vs 4.30 ± 1.2, P = 0.009), high urine copper concentrations (93.4 ± 144.0 vs 3.5 ± 1.8, P = 0.001) and low hemoglobin (7.0 ± 2.2 vs 12.6 ± 1.8, P < 0.0001) in patients with ALF caused by WD as compared with other etiologies. Interestingly, 4 of 7 patients with ALF due to WD survived without liver transplantation.

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Liver transplantation for Wilson's disease

Cited by 103 publications

References 16 publications

Wilson disease

Wilson disease

Liver transplantation

Diagnostic criteria for acute liver failure due to Wilson disease

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