Mukopolysaccharidosen

  • Biörn Ivemark

Zusammenfassung

Mukopolysaccharidosen sind Krankheiten mit abnormem Stoffwechsel der Glukosaminoglukane, was sich in gestörtem Aufbau der Stützgewebe, in extrazellulärer Ablagerung von Glukosaminoglukanen, in einer erhöhten Ausscheidung dieser Substanzen im Urin und oft in einer abnormen Vakuolisierung der zirkulierenden Lymphozyten manifestiert. Bei einigen dieser Krankheitstypen sind Enzymdefekte nachgewiesen worden (z.B. β-Galaktosidase-Störung).

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Literatur

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Copyright information

© Biörn Ivemark und Almqvist & Wiksell Förlag AB, Stockholm 1974

Authors and Affiliations

  • Biörn Ivemark
    • 1
  1. 1.Institutet för pediatrisk patologiKarolinska sjukhusetStrockholm 60Schweden

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