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Het Marfan-syndroom

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Aangeboren hartafwijkingen bij volwassenen

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Inleiding

Het Marfan-syndroom is een autosomaal dominant overervende bindweefselziekte met karakteristieke manifestaties in met name het hart, de grote vaten, de ogen en het skelet. Er is een grote variabiliteit in klinische expressie. Progressieve dilatatie van de aorta ascendens is een van de belangrijkste verschijnselen, waardoor patiënten met het Marfan-syndroom reeds op jonge leeftijd het risico lopen op plotse dood door aortadissectie of -ruptuur . De prevalentie wordt geschat op 1:5000, waarvan de meerderheid familiair is, maar ongeveer 15-30% wordt veroorzaakt door nieuwe mutaties.

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Author information

Authors and Affiliations

  1. Hartcentrum, afd. Radiologie, Academisch Medisch Centrum, Amsterdam, Netherlands

    M. Groenink

  2. Hartcentrum, Academisch Medisch Centrum, Amsterdam, Netherlands

    B.J.M. Mulder

Authors
  1. M. Groenink
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  2. B.J.M. Mulder
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Editor information

Editors and Affiliations

  1. Bohn Stafleu Van Loghum B.V., Houten, The Netherlands

    B. J. M. Mulder

  2. MIDDELSTUM, The Netherlands

    P.G. Pieper

  3. Heart Lung Center Utrecht Dept. Cardiology, Utrecht, The Netherlands

    F.J. Meijboom

  4. University Hospital Groningen Department of Cardiology, Groningen, The Netherlands

    J.P.M Hamer

Copyright information

© 2013 Bohn Stafleu van Loghum

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Groenink, M., Mulder, B. (2013). Het Marfan-syndroom. In: Mulder, B., Pieper, P., Meijboom, F., Hamer, J. (eds) Aangeboren hartafwijkingen bij volwassenen. Bohn Stafleu van Loghum, Houten. https://doi.org/10.1007/978-90-368-0307-6_21

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  • DOI: https://doi.org/10.1007/978-90-368-0307-6_21

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  • Publisher Name: Bohn Stafleu van Loghum, Houten

  • Print ISBN: 978-90-368-0306-9

  • Online ISBN: 978-90-368-0307-6

  • eBook Packages: Dutch language eBook collection

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