Review Article
An Overlapping Syndrome of Allergy and Immune Deficiency in Children
Table 1
Primary immune Deficiencies and their genetic background, associated with T-cell dysfunction and aberrant IgE production.
| Primary immune deficiencies with elevated IgE | T-cell dysfunction | Immune deficiency | Genetic background |
| Treg cell deficiency | Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) | Forkhead box protein 3 (Foxp3) signal transducer and activator of transcription 5b (STAT5b)CD25 | Treg cell dysfunction | Wiskott-Aldrich syndrome (WAS) | Wiskott-Aldrich syndrome protein (WASP) | Treg cell deficiency T-cell oligoclonality | Omenn syndrome (OS) | recombination activation genes (RAG1,RAG2) Artemis IL-7R zeta-associated protein, 70 kD (ZAP-70,) DNA ligase | Reduced NK cell cytotoxicityskewed Th1 phenotype | Comel-Netherton syndrome (CNS) | Serin protease inhibitor Kazal type (SPINK) lymphoepithelial Kazal type inhibitor (LEKTI) | T-cell oligoclonality | DiGeorge syndrome (DGS)—atypical complete form | Microdeletion 22q11 | Reduced Th17 cells Treg cell dysfunctionmultiple cytokine signaling defect | Hyperimmunoglobulin E syndrome (HIES) | Signal transducer and activator of transcription 3 (STAT3) dedicator of cytokinesis 8 (DOCK8) tyrosine kinase 2 (Tyk2) |
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