Reply: Adjunctive vitamin E treatment in Wilson disease and suggestions for future trials

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Reply: Adjunctive Vitamin E Treatment in Wilson Disease and Suggestions for Future Trials

Reply:

We have read with interest the suggestion by Shen and Ji to include vitamin E in future therapeutic trials of zinc in patients with hepatic manifestations of Wilson disease (WD). We do endorse this suggestion for any patient with acute liver failure due to WD or other diseases due to copper overload.1 In these patients, hepatocyte apoptosis is induced by activation of the Fas pathway through oxidative damage.2 Inhibition of this devastating effect of copper overload through the restoration of the intracellular oxidative balance by vitamin E supplementation would probably be beneficial and outbalance potential side effects.

We have some hesitation, however, about accepting the suggestion of potential benefits of vitamin E in patients with chronic liver damage due to WD. As Shen and Ji rightly point out, the results of vitamin E and other antioxidants in many chronic diseases are disappointing3 and might even be detrimental,4 as we have described for patients with cystic fibrosis, for example.5 Before the introduction of another intervention with a questionable effect, it is necessary to first properly describe, in a substantial number of patients, the results of decoppering medications that are already available, such as trientine, zinc, and D-penicillamine. Up to January 2008, well-described evidence for efficacy in hepatic WD had been published for only 57 patients on D-penicillamine, 9 patients on zinc, and none on trientine.6 Because prospective randomized trials are not available, a retrospective analysis of patients on any of these medications, like the analysis that we performed for exclusive zinc monotherapy,7 will aid physicians in choosing the optimal medication for their patients with hepatic or neurological WD.

Roderick H. J. Houwen*, Francisca H. H. Linn† §, Karel J. van Erpecum‡, * Department of Pediatrics, University Medical Center, Utrecht, The Netherlands, † Department of Neurology, University Medical Center, Utrecht, The Netherlands, ‡ Departments of Gastroenterology and Hepatology, University Medical Center, Utrecht, The Netherlands, § Department of Neurology, Central Military Hospital, Utrecht, The Netherlands.

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