Article
Hyper-IgE in the allergy clinic--when is it primary immunodeficiency?
Allergy - 1 Nov 2018
Ponsford Mark J, Klocperk Adam, Pulvirenti Federica, Dalm Virgil A S H, Milota Tomas, Cinetto Francesco, Chovancova Zita, Rial Manuel J, Sediva Anna, Litzman Jiri, Agostini Carlo, van Hagen Martin, Quinti Isabella, Jolles Stephen
Abstract excerpt
The 2017 International Union of Immunological Societies (IUIS) classification recognizes 3 hyper-IgE syndromes (HIES), including the prototypic Job's syndrome (autosomal dominant STAT3-loss of function) and autosomal recessive PGM3 and SPINK5 syndromes. Early diagnosis of PID can direct life-saving or transformational interventions; however, it remains challenging owing to the rarity of these conditions. This can...
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