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DOI: 10.1055/s-2002-32875
Alveolarproteinose
Pulmonary Alveolar Proteinosis Herrn Prof. Dr. Karl-Heinz Rühle zum 60. Geburtstag gewidmetPublikationsverlauf
Publikationsdatum:
19. Juli 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