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Lymphangioleiomyomatose

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Seltene Lungenerkrankungen

Zusammenfassung

Die Lymphangioleiomyomatose (LAM) ist eine der seltenen Lungenerkrankungen. Die Lunge weist dabei

beidseits relativ gleich große Zysten auf, in variabler Anzahl bis hin zu einem fast kompletten zystischen Umbau. Der Erkrankung liegt eine Mosaik Mutation in einem Tumorsuppressorgen (TSC2-Gen) zugrunde. Die LAM Zellen finden am ehesten den Weg von außen in die Lunge. Veränderungen können in der Niere, im Uterus oder in den abdominellen Lymphknoten existieren. Die Erkrankung der Lunge kann Teil der tuberösen Sklerose sein, bei welcher das Gen schon in der Keimbahn mutiert ist, und muss klinisch unterschieden werden. Die proliferative und Lungengewebe zerstörende Aktivität der LAM-Zellen kann durch den mTOR-Inhibitor Sirolimus inhibiert, aber die Krankheit damit alleine nicht geheilt werden. FEV1, die Diffusionskapazität sowie das CT charakterisieren den

Schweregrad ebenso wie die klinischen Einschränkungen. VEGF-D ist ein wertvoller Biomarker.

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Author information

Authors and Affiliations

  1. Medizinische Klinik und Poliklinik I, Universitätsklinikum Leipzig, Liebigstr. 18, 04103, Leipzig, Deutschland

    Prof. Dr. med. Hubert Wirtz

  2. vormals LungenClinic, Großhansdorf, Deutschland

    Prof. Dr. med. Detlef Kirsten

  3. Pneumologie und Pneumologisches Forschungsinstitut am Krankenhaus Großhansdorf, LungenClinic Grosshansdorf, Wöhrendamm 80, 22927, Hamburg, Deutschland

    PD Dr. med. Henrik Watz

Authors
  1. Prof. Dr. med. Hubert Wirtz
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  2. Prof. Dr. med. Detlef Kirsten
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  3. PD Dr. med. Henrik Watz
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Corresponding author

Correspondence to Hubert Wirtz .

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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Wirtz, H., Kirsten, D., Watz, H. (2016). Lymphangioleiomyomatose. 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_17

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  • DOI: https://doi.org/10.1007/978-3-662-48419-7_17

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-48418-0

  • Online ISBN: 978-3-662-48419-7

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