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Schmerztherapie bei Morbus Fabry

Pain therapy for Fabry’s disease

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Zusammenfassung

Der Morbus Fabry ist eine X-chromosomal vererbte lysosomale Speicherkrankheit mit Defizienz der α-Galaktosidase A und konsekutiver multipler Organbeteiligung. Ein frühes und auch in späteren Krankheitsstadien häufiges Symptom sind Schmerzen. Diese Schmerzen sind von verschiedenen Provokationsfaktoren abhängig und können die Lebensqualität massiv beeinträchtigen. Sogenannte Fabry-Krisen können eine intensivmedizinische Behandlung erforderlich machen. Die Schmerzen werden überwiegend als neuropathisch eingeordnet und sind therapeutisch schwer zu beeinflussen. Zudem muss die medikamentöse Behandlung an die bei M. Fabry meist bestehende kardiale und renale Beteiligung angepasst werden. In dieser Übersichtsarbeit wird evidenz- und erfahrungsbasiert eine Anleitung für die Schmerztherapie bei M. Fabry gegeben.

Abstract

Fabry’s disease is an X-chromosome linked lysosomal storage disorder with α-galactosidase A deficiency and subsequent multiple organ involvement. An early and common symptom also in later stages of the disease is pain. This pain depends on various precipitating factors and can severely compromise the quality of life. So-called Fabry crises can lead to the necessity for intensive care treatment. The pain can be classified as predominantly neuropathic and is difficult to treat. In addition, medication has to be adjusted to concomitant cardiac and renal involvement in Fabry’s disease. This review gives guidance for pain therapy in Fabry’s disease based on the available evidence and on experience.

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Interessenkonflikt

Die korrespondierende Autorin weist für sich und ihre Koautoren auf folgende Beziehung hin: Kooperationsprojekte mit Fa. Genzyme. Die Erstellung dieses Manuskripts wurde von der Genzyme Corporation unterstützt. Die Autoren haben den Text unabhängig und dem aktuellen Stand der Wissenschaft entsprechend verfasst.

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Authors and Affiliations

  1. Neurologische Klinik, Universitätsklinikum Würzburg, Josef-Schneider-Str. 11, 97080, Würzburg, Deutschland

    C. Sommer & N. Üçeyler

  2. Klinik und Poliklinik für Neurologie, Universitätsklinikum Münster, Münster, Deutschland

    T. Duning

  3. Sektion für Neurologische Schmerzforschung und -therapie, Klinik für Neurologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Haus 41, Kiel, Deutschland

    K. Arning, R. Baron & D. Naleschinski

  4. Innere Medizin D, Universitätsklinikum Münster, Münster, Deutschland

    E. Brand

  5. Medizinische Klinik mit Schwerpunkt Nephrologie und Internistische Intensivmedizin, Charité-Universitätsmedizin, Campus Virchow-Klinikum, Berlin, Deutschland

    S. Canaan-Kühl

  6. Neurologische Klinik, Universität Erlangen-Nürnberg, Erlangen, Deutschland

    M. Hilz

  7. Medizinische Klinik und Poliklinik I, Universitätsklinik Würzburg, Würzburg, Deutschland

    C. Wanner & F. Weidemann

Authors
  1. C. Sommer
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  2. N. Üçeyler
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  3. T. Duning
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  4. K. Arning
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  5. R. Baron
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  6. E. Brand
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  7. S. Canaan-Kühl
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  8. M. Hilz
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  9. D. Naleschinski
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  10. C. Wanner
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  11. F. Weidemann
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Corresponding author

Correspondence to C. Sommer.

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Sommer, C., Üçeyler, N., Duning, T. et al. Schmerztherapie bei Morbus Fabry. Internist 54, 121–130 (2013). https://doi.org/10.1007/s00108-012-3204-5

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  • DOI: https://doi.org/10.1007/s00108-012-3204-5

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