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Syndrome of Hyperimmunoglobulinemia E - Case Study and a Review of Literature


Authors: R. Špíšek 1;  I. Pončáková 2;  O. Petrů 3;  A. Šedivá 1;  P. Pohunek 2;  J. Vavřinec 2
Authors‘ workplace: Ústav imunologie 2. LF UK a Fakultní nemocnice Motol, Praha Klinika dětské chirurggie 2. LF UK a Fakultní nemocnice Motol, Praha přpřednosta prof. MUDr. J. Vavřinec, DrSc. Klinika dětské chirurggie 2. LF UK a Faki přednosta prof. MUDr. J. Šnajdauf, DrSc.
Published in: Čes-slov Pediat 2004; (3): 147-151.
Category:

Overview

The syndrome of hyperimmunoglobulinemia E (hyper IgE) is characterized by relapsing staphylococcus abscesses and extréme values of sérum IgE. In the čase history of these indiv iduals there are recurrent severe staphylococcus infections of soft tissues, affecting mainly skin, lungs and joints. In the period after staphylococcus pneumonia was cured up, the patients frequently develop pneumatocele. There are also frequent mucocutaneous infections caused by Candidas, skeleton deformities and fractures. Chronic itching dermatitis is typical. The páper summarizes the čase of a girl with long-term history indicating the syndrome of hyper IgE. In špite of the typical anamnesis, the diagnosis was established latě and severe infectious complications developed, while some of them could be prevented by timely onset of adequate therapy. The authors discuss picture of the disease and summarize presently known immunological mechanisms and therapeutic possibilities.

Key words:
immunodeficiency, hyper IgE, eosinophilia, eczema, pneumonia

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Labels
Neonatology Paediatrics General practitioner for children and adolescents
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