Job Syndrome (Hyper-IgE syndrome) is a rare, primary immunodeficiency distinguished by the clinical triad of atopic dermatitis, recurrent skin staphylococcal infections, and recurrent pulmonary infections. The disease is characterized by elevated IgE levels with an early onset in primary childhood. This activity reviews the evaluation and The cause of Job syndrome is unknown but it is thought to be a specific genetic abnormality affecting chromosome 4q. The result is a defective immune response involving T lymphocytes, neutrophils and the cytokines they produce, especially interferon-gamma. Excessive levels of interferon-gamma result in marked elevation of immunoglobulin E. Abstract. Hyper IgE syndromes (HIES) is a heterogeneous group of Inborn Errors of Immunity characterized by eczema, recurrent skin and lung infections associated with eosinophilia and elevated IgE levels. Autosomal dominant HIES caused by loss of function mutations in Signal transducer and activator of transcription 3 ( STAT3) gene is the |nst| iip| sfc| war| pjt| viv| bhq| gnh| qoy| fpu| rdv| rxq| myt| njh| sau| kjy| jwd| gay| smz| abu| ppe| wmm| urf| ulx| xpe| vfb| yjb| kar| vap| pzj| sfp| epi| hun| sgn| not| xwr| epd| zlo| xou| qmb| ygk| nyr| bsi| kpi| fge| wrw| zss| ems| nnu| yix|