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Morbus Wilson

  • U. Von Arnim
  • W. F. Caspary

Zusammenfassung

Der Morbus Wilson ist eine autosomal rezessiv vererbte Kupferspeicherkrankheit (Gendefekt am Chromosom 13). Die defekte biliäre Kupfer-Exkretion führt zur Akkumulation von Kupfer in der Leber, wodurch die Leber progressiv bis zur Zirrhose geschädigt wird. Durch die Kupferablagerungen entstehen zudem Symptome neurologischer, hämatologischer und renaler Krankheiten. Die Prävalenz beträgt 1:30000 Lebendgeburten. Bei früher Diagnose und rechtzeitiger Therapie (Penicillamin) kann die Progression verhindert und die Lebenserwartung erhöht werden.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2001

Authors and Affiliations

  • U. Von Arnim
  • W. F. Caspary

There are no affiliations available

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