Abstract
After 27 months of daily penicillamine administration (250 mg 3 or 4 times daily) a portal cirrhosis in a 10-year-old girl was found to have healed clinically and histopathologically. This case shows that even an advanced portal liver cirrhosis in Wilson's disease can be healed by adequate penicillamine therapy. Hence, it is important that all patients with liver cirrhosis who are younger than 30 years of age are examined for any signs of Wilson's disease. Copper metabolism studies with Cu64-labeled copper citrate revealed characteristic differences between the homozygote patient and her heterozygote parents and healthy subjects.
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