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Amyloidose

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Seltene Lungenerkrankungen

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Zusammenfassung

Unter dem Begriff Amyloidose wird eine heterogene Gruppe von Erkrankungen zusammengefasst, bei denen unterschiedliche Proteine in unlöslicher Form deponiert werden und dadurch die umgebenden Strukturen und Organe schädigen. Jedes Vorläuferprotein, das Amyloid bilden kann, induziert ein charakteristisches Muster des Organbefalls und typische Krankheitsbilder mit variabler Ausprägung. Bei Autopsien finden sich oft Amyloidablagerungen in verschiedenen Kompartimenten der Lunge, doch sind diese nicht immer mit Krankheitssymptomen verbunden. Der Nachweis einer Amyloidose geschieht histologisch mithilfe der Kongorotfärbung, die Identifizierung des zugrunde liegenden Proteins mit immunologischen Methoden in spezialisierten Zentren. Kontaktdaten finden sich im Internet und sind in diesem Beitrag tabellarisch zusammengefasst. Für die weitere Abklärung und die Erstellung eines Behandlungsplans existieren klinische Pfade und Empfehlungen, die dieser Beitrag in Form von Abbildungen, Tabellen und Literaturangaben zur Verfügung stellt. Alle Patienten mit Amyloidose sollten von einem dieser Zentren (mit)betreut werden, da die Expertise von prognostischer Bedeutung ist bei einer seltenen Erkrankung zentralisierte Forschung unser Handeln zum Wohle der aktuell und zukünftig Erkrankten bestimmen muss.

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Authors and Affiliations

  1. Abteilung Pneumologie & Schlafmedizin, Ruhrlandklinik, Westdeutsches Lungenzentrum, Universitätsklinikum Essen, Tüschener Weg 40, 45239, Essen, Deutschland

    Prof. Dr. med. Dipl. Ing. Helmut Teschler

  2. Abteilung Pneumologie & Schlafmedizin, Ruhrlandklinik,Westdeutsches Lungenzentrum, Universitätsklinikum Essen, Tüschener Weg 40, 45239, Essen, Deutschland

    Dr. med. Björn Kleibrink

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  1. Prof. Dr. med. Dipl. Ing. Helmut Teschler
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Correspondence to Helmut Teschler .

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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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Teschler, H., Kleibrink, B. (2016). Amyloidose. 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_21

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