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Pneumologie 2002; 56(7): 448-456
DOI: 10.1055/s-2002-32875
Serie: Seltene Lungenerkrankungen (2)
© Georg Thieme Verlag Stuttgart · New York

Alveolarproteinose

Pulmonary Alveolar ProteinosisH.  Teschler1 , T.  E.  Wessendorf1
  • 1Zentrum für Pneumologie und Thoraxchirurgie, Ruhrlandklinik, Essen
Herrn Prof. Dr. Karl-Heinz Rühle zum 60. Geburtstag gewidmet
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Publication History

Publication Date:
19 July 2002 (online)

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Definition

Die Alveolarproteinose ist ein seltenes alveoläres Füllungssyndrom, das 1958 von Rosen und Mitarbeitern beschrieben wurde [52]. Bei der Alveolarproteinose akkumulieren große Mengen von Phospholipiden und Lipoproteinen in den Azini und angrenzenden peripheren Lufträumen (Abb. [1]).

Abb. 1 Histologisches Bild der Alveolarproteinose (PAS-Färbung).

Das angereicherte Material enthält Bestandteile des pulmonalen Surfactant in pathologischer Zusammensetzung [22]. Von der primären (idiopathischen) wird die sekundäre Alveolarproteinose bei bösartigen Erkrankungen, Infektionen, nach Behandlung mit Zytostatika und nach Inhalation von Chemikalien, Metall- oder Quarzstäuben unterschieden [7] [11] [36] [39] [53]. Die kongenitale Form der Alveolarproteinose ist die häufigste Lungenparenchymerkrankung des Neugeborenen, wird autosomal rezessiv vererbt und ist auf das Fehlen des Surfactant-Protein B (SP-B) oder eine verminderte Expression des βc-Rezeptors für GM-CSF zurückzuführen [15] [35] [50] [54] [61].

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Prof. Dr. med. H. Teschler

Ruhrlandklinik, Zentrum für Pneumologie und Thoraxchirurgie

Tüschener Weg 40

45239 Essen

Email: Helmutt@t-online.de