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Zusammenfassung

Das Hyper-IgE-Syndrom (HIES) ist wegen der Beteiligung zahlreicherOrgansysteme, des Krankheitsbeginns im frühen Kindesalter und der wahrscheinlichen Erblichkeit eine Erkrankung, die nicht nur bei Pädiatern, sondern auch bei Dermatologen, HNO-Ärzten, Immunologen und Genetikern Aufmerksamkeit erweckt. Kamen bis zum Jahre 1975 Veröffentlichungen über dieses Krankheitsbild ausschließlich aus den USA, so ist seit 1976 eine Zunahme der Publikationen aus dem europäischen Raum zu verzeichnen. Trotz zunehmendem Interesse an dieser Erkrankung ist es bis heute — 20 Jahre nach der Erstbeschreibung — nicht gelungen, die Pathogenese des Syndroms aufzuklären. Somit fehlen zum einen die Voraussetzungen für eine exakte Abgrenzung des HIES gegenüber anderen Krankheitsbildern ähnlicher Symptomatik, die in der Regel ebenfalls auf Defekten des Immunsystems beruhen, zum anderen fehlt ohne exakte pathogenetische Kenntnisse die Möglichkeit einer gezielten, ggf. sogar kausalen Therapie.

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P. Frick G.-A. von Harnack K. Kochsiek G. A. Martini A. Prader

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Professor Dr. Walter H. Hitzig in Verehrung gewidmet

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Belohradsky, B.H., Däumling, S., Kiess, W., Griscelli, C. (1987). Das Hyper-IgE-Syndrom (Buckley- oder Hiob-Syndrom). In: Frick, P., von Harnack, GA., Kochsiek, K., Martini, G.A., Prader, A. (eds) Ergebnisse der Inneren Medizin und Kinderheilkunde/Advances in Internal Medicine and Pediatrics. Ergebnisse der Inneren Medizin und Kinderheilkunde/Advances in Internal Medicine and Pediatrics, vol 55. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-71052-0_1

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