Orthotopic Liver Transplantation for Wilson???s Disease

Emre, Sükrü; Atillasoy, Evren; Ozdemir, Sebati; Schilsky, Michael L.; Varma, Chelikani; Thung, Swan N.; Sternlieb, Irmin; Guy, Stephen R.; Sheiner, Patricia A.; Schwartz, Myron; Miller, Charles M.

doi:10.1097/00007890-200110150-00008

Cited by 118 publications

(83 citation statements)

References 27 publications

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“…In our series, these two ratios showed no significant differences between the two groups. This is in concordance with other reports that doubt the validity of the two scores for diagnosis of FWD [16,17] . In our series, INR, grade of HE, and MELD score failed to discriminate FWD from ALF due to other causes.…”

Section: Discussionsupporting

confidence: 93%

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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Section: Discussionsupporting

confidence: 93%

Diagnostic criteria for acute liver failure due to Wilson disease

Eisenbach

Sieg²,

Stremmel³

et al. 2007

WJG

101

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“…47 Liver transplantation has been effective for patients with FHF, decompensated cirrhosis, or progressive neurologic disease, although the latter indication remains controversial. 48,49 A1AT is the most common genetic liver disease in infants and children. A1AT deficiency is present in 1:1,600 to 1:2,800 babies born in the United States and Northern Europe.…”

Section: Kris V Kowdley Md University Of Washington Seattle Wamentioning

confidence: 99%

Burden of liver disease in the United States: Summary of a workshop

et al. 2002

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“…8 Whether improvement with MR abnormalities as severe as in our patient is possible at all is not known, although similar clinical deficits in Wilson disease have almost resolved after transplantation. 9,10 Because of its infrequent occurrence, the interpretation of MR imaging abnormalities-reversible or irreversible-has been far from precise. Other postoperative concerns include poor mobilization because of ataxia, risk of aspiration caused by hypokinesis, and possibly delirium.…”

Section: Descriptionmentioning

confidence: 99%

Acquired (non-Wilsonian) hepatocerebral degeneration: Complex management decisions

Wijdicks

Wiesner

2003

Liver Transplantation

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Portal systemic encephalopathy, in its many guises, can be reversible after medical management or liver transplantation. It is much less certain whether patients with a longstanding neurodegenerative syndrome (known in the medical vernacular as acquired hepatocerebral degeneration) can improve. Impressive neuroimaging abnormalities have been reported in this entity. 1 However, the combination of a severe disabling neurologic deficit and widespread magnetic resonance abnormalities tempers the enthusiasm of transplant surgeons to proceed with liver transplantation. In our liver transplantation program, we were recently confronted with such a case, and present herein not only the characteristic magnetic resonance imaging findings but also some of the dilemmas of management. (Liver Transpl 2003;9:993-994.)

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Orthotopic Liver Transplantation for Wilson???s Disease

Cited by 118 publications

References 27 publications

Diagnostic criteria for acute liver failure due to Wilson disease

Diagnostic criteria for acute liver failure due to Wilson disease

Burden of liver disease in the United States: Summary of a workshop

Acquired (non-Wilsonian) hepatocerebral degeneration: Complex management decisions

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