Treatment of Wilson’s disease: What are the relative roles of penicillamine, trientine, and zinc supplementation?

Schilsky, Michael L.

doi:10.1007/s11894-001-0041-4

Cited by 77 publications

(66 citation statements)

References 20 publications

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“…Number 7 July 2016 a clinically relevant copper maintenance therapy in WD (9). All MB-treated animals showed restoration of normal serum AST levels that lasted for at least 2 weeks, after which AST levels rose again ( Figure 5A).…”

Section: Short-term Mb Application Reverses Liver Damage In Vivomentioning

confidence: 99%

“…This lifelong therapy is effective if commenced before the onset of advanced hepatic or neurologic disease (6)(7)(8). The same holds true for zinc salts, which are primarily used in mild cases of WD to decrease copper absorption via the gastrointestinal tract or as copper maintenance therapy in chelator-treated WD patients (1,5,9). However, in circumstances of acute liver failure -caused by delayed diagnosis, treatment failure, or rapidly developing fulminant hepatitis -death is almost certain unless liver transplantation is performed (1, 5, 7, 10, 11).…”

Section: Introductionmentioning

confidence: 99%

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Methanobactin reverses acute liver failure in a rat model of Wilson disease

Lichtmannegger¹,

Leitzinger²,

Wimmer³

et al. 2016

Journal of Clinical Investigation

138

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Section: Short-term Mb Application Reverses Liver Damage In Vivomentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Methanobactin reverses acute liver failure in a rat model of Wilson disease

Lichtmannegger¹,

Leitzinger²,

Wimmer³

et al. 2016

Journal of Clinical Investigation

138

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“…TETA is currently used as the second line treatment, in the form of trientine, for Wilson's disease, mainly for those patients with penicillamine allergy or intolerance (14,88,89). It is more commonly used in children with Wilson's disease (16,66).…”

Section: Current Clinical Applications and Therapeutic Implicationsmentioning

confidence: 99%

Triethylenetetramine Pharmacology and Its Clinical Applications

2010

Molecular Cancer Therapeutics

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Triethylenetetramine (TETA), a Cu II -selective chelator, is commonly used for the treatment of Wilson's disease. Recently, it has been shown that TETA can be used in the treatment of cancer because it possesses telomerase inhibiting and anti-angiogenesis properties. Although TETA has been used in the treatment of Wilson's disease for decades, a comprehensive review on TETA pharmacology does not exist. TETA is poorly absorbed with a bioavailability of 8 to 30%. It is widely distributed in tissues with relatively high concentrations measured in liver, heart, and kidney. It is mainly metabolized via acetylation, and two major acetylated metabolites exist in human serum and urine. It is mainly excreted in urine as the unchanged parent drug and two acetylated metabolites. It has a relatively short half-life (2 to 4 hours) in humans. The most recent discoveries in TETA pharmacology show that the major pharmacokinetic parameters are not associated with the acetylation phenotype of N-acetyltransferase 2, the traditionally regarded drug acetylation enzyme, and the TETA-metabolizing enzyme is actually spermidine/spermine acetyltransferase. This review also covers the current preclinical and clinical application of TETA. A much needed overview and up-to-date information on TETA pharmacology is provided for clinicians or cancer researchers who intend to embark on cancer clinical trials using TETA or its close structural analogs. Mol Cancer Ther; 9(9); 2458-67. ©2010 AACR.

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“…Both methods of treatment lead, though by different means, to reduction of the copper content in the body-decuprination. These therapeutic procedures are accompanied by changes in the concentration of copper and zinc in the serum and their excretion in the urine and/or stool [8][9][10][11][12][13][14].…”

Section: Introductionmentioning

confidence: 99%

Copper and Zinc in the Serum, Urine, and Hair of Patients with Wilson’s Disease Treated with Penicillamine and Zinc

Dastych

Procházková

Pokorný³

et al. 2009

Biol Trace Elem Res

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The purpose of this study was to determine the different levels of copper and zinc in the serum, urine, and scalp hair of patients with Wilson's disease receiving different, currently accepted methods of treatment to reduce the copper load (penicillamine-group 1, n = 8; zinc-group 2, n = 8; penicillamine+zinc-group 3, n = 8). Blood, urine, and hair samples were collected from the patients. All three treatments resulted in a significant decrease of the serum copper levels. Significantly increased levels of zinc in the serum were detected in the patients in groups 2 and 3 (19.1 and 18.8 micromol/l, respectively; p < 0.05). Copper excretion in the urine significantly increased during its administration to groups 1 and 3 (11.5 and 7.94 micromol/24 h respectively; p < 0.001) due to the effect of penicillamine. The administration of zinc as monotherapy (group 2) or in combination with penicillamine (group 3) led to an increase of its excretion (25.3 and 22.4 micromol/24 h, respectively; p < 0.01). Only an insignificant rise of the copper content in the hair was found in all three groups of patients. The content of zinc in the hair did not differ significantly in any of the groups in comparison with the control group.

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Treatment of Wilson’s disease: What are the relative roles of penicillamine, trientine, and zinc supplementation?

Cited by 77 publications

References 20 publications

Methanobactin reverses acute liver failure in a rat model of Wilson disease

Methanobactin reverses acute liver failure in a rat model of Wilson disease

Triethylenetetramine Pharmacology and Its Clinical Applications

Copper and Zinc in the Serum, Urine, and Hair of Patients with Wilson’s Disease Treated with Penicillamine and Zinc

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