Monitoring Therapy in Wilson%26apos;s Disease by the Oral Radiocopper Test

作者：Guenther P; Kuehn H J; Hermann W^*

来源：Aktuelle Neurologie, 2013, 40(4): 195-199.

DOI：10.1055/s-0033-1334900

• Wilsons%26apos
• s disease
• oral radiocopper test
• zinc
• triene
• penicillamine

Background: Wilson%26apos;s disease is a rare autosomal recessive disorder of hepatic copper transport leading to an inhibition of the biliary excretion of copper. Overloads of the metal mainly in the liver and basal ganglia lead to hepatic but also to extrapyramidal motor as well as psychiatric clinical symptoms. Depending on the stage of the disease, therapy with chelating drugs and zinc is possible but must be given lifelong without longer interruptions. With early diagnosis and consequent treatment, the prognosis of Wilson%26apos;s disease is excellent and usually the need for liver transplantation can be avoided. Monitoring of treatment is essential because therapy with chelating penicillamine is complicated by side effects, triene is not always available and non-responders to zinc therapy are reported. %26lt;br%26gt;Method: For the evaluation of the inhibition of enteral resorption of copper in our study an oral radiocopper test was performed in 58 patients with Wilson%26apos;s disease. Patients were given 10 MBq Cu-64 and radioactivity was measured in serum at one and/or, respectively, 3 h after intake. Altogether 91 tests were evaluated. %26lt;br%26gt;Results: No inhibition of copper resorption was found in 1 patient under penicillamine therapy. A partial inhibition of enteral resorption could be proved for triene in 6 patients. 48 tests showed a sufficient inhibition with zinc therapy. In 36 tests of the patients taking zinc the inhibition was proved to be not sufficient. In 6 of them it could be improved by increasing the dosage. Triene has been shown to inhibit the resorption of copper as well by this test. No inhibition is seen with penicillamine. %26lt;br%26gt;Conclusion: The high prevalence of insufficient effectiveness of zinc therapy requires a continuous control. The radiocopper test allows an estimation of the influence of zinc on copper resorption in Wilson%26apos;s disease. Further studies have to be performed to evaluate the utility of this test.

• 出版日期2013-5