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Das Buckley-Syndrom: Rezidivierende, schwere Staphylokokken-infektionen, Ekzem und Hyperimmunglobulinämie E

The buckley syndrome: Recurring, severe staphylococcal infections, eczema and hyperimmunoglobulinemia E

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Zusammenfassung

Fünfzehn Patienten im Alter von drei bis 27 Jahren wurden klinisch und infektionsimmunologisch untersucht. Allen gemeinsam waren schwere rezidivierende, kutane und pulmonale Staphylokokken-infektionen, ein chronisches Ekzem, Bluteosinophilie und eine extreme Erhöhung des Serum-IgE-Spiegels. Acht der Patienten wiesen zudem eine faziale Dysmorphie auf, die durch grobe Gesichtszüge, Prognathie und schlecht modellierte Ohrmuscheln charakterisiert ist. Eine deutliche Osteoporose, vor allem der Wirbelkörper, wurde bei acht Patienten beobachtet. Ein konstanter Granulozyten-Chemotaxis-Defekt fand sich nur in drei Fällen, variable oder konstant normale Chemotaxis-Befunde traten in sechs Fällen auf. Eine polyklonale Hypergammaglobulinämie (14 Patienten), erhöhtes IgD (zehn Patienten) wurden nachgewiesen, ebenso ein partieller T-Zell-Defekt (zwei Patienten) und eine fehlende anamnestische Antikörperantwort bei einem Patienten. Therapieversuche mit Immunglobulinen, Transfer-Faktor, Levamisol und anti-H2-Rezeptor-Antihistaminika blieben bei der pathogenetisch ungeklärten Erkrankung ohne anhaltende oder befriedigende klinische und immunologische Ergebnisse.

Summary

Fifteen patients aged between three and 27 years were examined clinically and immunologically. Common to all patients were severe recurring cutaneous and pulmonary staphylococcal infections, chronic eczema, eosinophilia and an extremely elevated serum IgE level. Eight of the patients had in addition facial dysplasia characterised by coarse features, prognathism and poorly formed external ears. Marked osteoporosis, particularly of the vertebral bodies, was observed in eight patients. A constant defect of granulocyte chemotaxis was found in only three patients; fluctuating or constantly normal chemotaxis occurred in six patients. Polyclonal hypergammaglobulinemia was detected in 14 patients, elevated IgD in two patients, a partial T-cell defect in two patients and a history of lack of antibody response in one patient. Therapeutic trials with immunoglobulins, transfer factor, levamisole and anti-H2 receptor-antihistamines did not produce lasting or satisfactory clinical or immunological results in the pathogenetically unidentified disease.

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  1. Abteilung für Antimikrobielle Therapie und Infektionsimmunologie, Universitäts-Kinderklinik, Lindwurmstr. 4, D-8000, München 2

    S. Däumling, M. C. Lalama &  B. H. Belohradsky

  2. Unité d'Immunologie et d'Hématologie, Département de Pédiatrie, Centre Hospitalier Universitaire Necker-Enfants Malades, 149, Rue de Sèvres, D-75730, Paris Cedex 15, France

    D. Buriot,  P. Huu Trung &  C. Griscelli

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  1. S. Däumling
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Däumling, S., Lalama, M.C., Belohradsky, B.H. et al. Das Buckley-Syndrom: Rezidivierende, schwere Staphylokokken-infektionen, Ekzem und Hyperimmunglobulinämie E. Infection 8 (Suppl 3), S248–S254 (1980). https://doi.org/10.1007/BF01639590

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