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Aktuelle Neurologie 2013; 40(04): 195-199
DOI: 10.1055/s-0033-1334900
Originalarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Kontrolle der Zinktherapie bei Morbus Wilson mit dem oralen Radiokupfertest

Monitoring Therapy in Wilson’s Disease by the Oral Radiocopper Test
P. Günther
1   Nervenärztliche Gemeinschaftspraxis, Leipzig
,
H.-J. Kühn
2   Medizinische Fakultät, Universität Leipzig
,
W. Hermann
3   Klinik und Poliklinik für Neurologie, Paracelsus Klinik Zwickau
› Author Affiliations
Further Information

Publication History

Publication Date:
22 February 2013 (online)

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Zusammenfassung

Hintergrund:

Der Morbus Wilson ist eine seltene Kupferstoffwechselstörung, die bei früher ­Diagnosestellung und konsequenter Therapie eine sehr gute Prognose hat. Nebenwirkungen oder eine nachlassende Wirksamkeit können Komplikationen einer medikamentösen Langzeitbehandlung sein. Besonders bei der Behandlung mit Zink haben sich Berichte über ein Versagen der Therapie gehäuft.

Material und Methoden:

Bei 58 Patienten mit einem Morbus Wilson unter Langzeittherapie wurde in insgesamt 91 Mal ein oraler Radiokupfertest durchgeführt und damit das Ausmaß der enteralen Resorptionshemmung von Kupfer bestimmt. 84 Messungen erfolgten unter einer Zinkmedikation, eine unter D-Penizillamin und sechs unter einer Behandlung mit Trien.

Ergebnisse:

Die mit Zink behandelten Patienten zeigten in 48 Tests eine ausreichende, in 36 Fällen aber eine ungenügende Hemmung der enteralen Kupferresorption. Für Trien konnte eine partielle enterale Resorptionshemmung von Kupfer gezeigt werden.

Schlussfolgerung:

Die Behandlung des Morbus Wilson erfordert in jedem Fall eine regelmäßige Kontrolle. Der orale Radiokupfertest erlaubt eine quantitative Aussage zum Einfluss von Zink auf die Kupferresorption beim Morbus Wilson. Sein Nutzen muss durch weitere Studien näher definiert werden.

Abstract

Background:

Wilson’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’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.

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’s disease. Patients were given 10 MBq 64Cu and radioactivity was measured in serum at one and/or, respectively, 3 h after intake. Altogether 91 tests were evaluated.

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.

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 Wilsonʼs disease. Further studies have to be performed to evaluate the utility of this test.

Schlüsselwörter

Morbus Wilson - oraler Radiokupfertest - Zink - Trien - D-Penizillamin

Keywords

Wilsons’s disease - oral radiocopper test - zinc - triene - penicillamine
 

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