Abstract
Copper toxicity In liver, brain and kidney due to a congenital failure of the copper excretory mechanism in the liver is the main problem in patients with M. Wilson. Oral zinc sulphate as only treatment for this disease has successfully been used for several years as alternative therapy for D-Penicillamin which has several serious side effects. Quantitative balance studies comparing both forms of treatment were done in 2 sets of children. During the balance study a standardized hospital diet was used and identical food portions frozen for analysis of each patient. All fecal and urinary excretions were sampled each day for a sevenday period.
Additionally normal liverfunction tests and absence of clinical symptoms were found In both groups. Oral challenge with a high dose of D-Penicillamin in one day showed a significant rise in copper excretion in both groups, indicating still incomplete decoppering. Liver biopsy copper content confirmed this finding.
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Bouquet, J., Cossack, Z., Sinaasappel, M. et al. 5 THE EVALUATION OF TREATMENT IN WILSON'S DISEASE. Pediatr Res 20, 1034 (1986). https://doi.org/10.1203/00006450-198610000-00059
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DOI: https://doi.org/10.1203/00006450-198610000-00059