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Das Wiskott-Aldrich Syndrom

  • B. H. Belohradsky
  • C. Griscelli
  • H. H. Fudenberg
  • W. Marget
Conference paper
Part of the Ergebnisse der Inneren Medizin und Kinderheilkunde / Advances in Internal Medicine and Pediatrics book series (KINDERHEILK. NF, volume 41)

Zusammenfassung

Das klinische Bild und die Pathogeneseforschung des Wiskott-Aldrich Syndroms (WAS) sind aufgrund der charakteristischen Symptomentrias (thrombocytopenische Blutungen, gesteigerte Infektionsanfälligkeit, Ekzem) und des recessiv-geschlechtsgebundenen Erbganges nicht nur für den Pädiater von Interesse, sondern in gleichem Maß für den Hämatologen, Immunologen, Dermatologen und Genetiker (89, 106, 107, 113, 149, 152, 166, 191, 207, 222, 248, 260, 264, 265, 276, 286). Dennoch ist es, 40 Jahre nach der Erstbeschreibung, immer noch nicht gelungen, Ätiologie und Pathogenese des Syndroms zu klären. Damit fehlen auch weiterhin die Voraussetzungen für eine kausale Therapie und die eindeutige Abgrenzung von ähnlich verlaufenden Krankheiten.

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Copyright information

© Springer-Verlag Berlin · Heidelberg 1978

Authors and Affiliations

  • B. H. Belohradsky
    • 1
  • C. Griscelli
    • 2
  • H. H. Fudenberg
    • 3
  • W. Marget
    • 1
  1. 1.Abteilung für Antimikrobielle Therapie und Infektionsimmunologie (Vorstand: Prof..Dr. W. Marget)Kinderklinik der Universität München im Dr. von Haunerschen Kinderspital (Direktor: Prof. Dr. K. Betke)München 2W-Germany
  2. 2.Hôpital des Enfants MaladesUnité d’Immuno-HématologieParis Cedex 15France
  3. 3.Department of Basic and Clinical Immunology and MicrobiologyMedical University of of South CarolinaCharlestonUSA

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