Sindromul Hiper-Ige – De La Complexitate Patogenică La Manifestări Clinice
Pediatruro(2022)
Abstract
Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by increased serum IgE levels associated with eczema, recurrent lung and skin infections and with various connective tissue, skeletal and vascular abnormalities. Disease mechanisms involve STAT3 signalling pathway, deficiency of which leads to synthesis of many T helper 1 (Th1) cytokines, such as IFN-γ and TNF-α, and inhibition of proinflammatory and antiinflammatory responses regulated by IL-6 and IL-10. Clinical features of HIES involve the immune system, connective tissue, skeleton, skin and dentition, with variations in severity and timing of onset. The early recognition of HIES can lead to a better treatment of the associated complications.
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