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Seltene Lungenerkrankungen pp 297–302Cite as

Primäre ziliäre Dyskinesie

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Zusammenfassung

Motile Zilien sind Ausstülpungen der Zelloberfläche, die in der Lage sind, aktive Bewegungen auszuführen und dadurch Zellen (z.B. Spermien) oder Flüssigkeiten (z. B. in den Atemwegen) in Bewegung zu setzen. Der Begriff Primäre ziliäre Dyskinesie (PCD) beschreibt eine Gruppe genetisch und klinisch heterogener hereditärer Erkrankungen, bei denen die Dysfunktion motiler Zilien zu einer gravierenden Störung der Atemwegs-Clearance führt. Dies hat eine chronische Sekretretention in den oberen und unteren Atemwegen zur Folge, auf deren Basis es zu chronisch-rezidivierenden Infektionen sowie Inflammationsprozessen und schließlich zu einer irreversiblen Gewebsdestruktion kommt.

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Authors and Affiliations

  1. Klinik für Kinder- und Jugendmedizin, Allgemeine Pädiatrie, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, Geb. A1, 48149, Münster, Deutschland

    Dr. med. Claudius Werner & Prof. Dr. med. Heymut Omran

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  1. Dr. med. Claudius Werner
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  2. Prof. Dr. med. Heymut Omran
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Corresponding author

Correspondence to Claudius Werner .

Editor information

Editors and Affiliations

  1. Pneumologie und Beatmungsmediz, Universitätsklinikum Heidelberg, Heidelberg, Baden-Württemberg, Germany

    Michael Kreuter

  2. Ruhrlandklinik, Universitätsklinikum Essen, Essen, Nordrhein-Westfalen, Germany

    Ulrich Costabel

  3. Universitätsklinikum Heidelberg, Heidelberg, Baden-Württemberg, Germany

    Felix Herth

  4. Zentrum für Pneumologie & Thoraxchir., LungenClinic Großhansdorf, Großhansdorf, Schleswig-Holstein, Germany

    Detlef Kirsten

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Werner, C., Omran, H. (2016). Primäre ziliäre Dyskinesie. In: Kreuter, M., Costabel, U., Herth, F., Kirsten, D. (eds) Seltene Lungenerkrankungen. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-48419-7_27

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  • DOI: https://doi.org/10.1007/978-3-662-48419-7_27

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-48418-0

  • Online ISBN: 978-3-662-48419-7

  • eBook Packages: Medicine (German Language)

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