Disease relevance of Job's Syndrome

• Sera from nonatopic healthy donors and patients with hyper-IgE syndrome, allergic respiratory disease, i.e., allergic rhinitis and asthma, and atopic dermatitis were assayed for the presence of IgG and IgM antibodies to IgE [1].
• Clinically defined immunodeficiencies, such as hyper-IgE syndrome and idiopathic CD4 lymphocytopenia, are also discussed [2].
• Recurrent colonic histoplasmosis after standard therapy with amphotericin B in a patient with Job's syndrome [3].
• Our data suggest that patients with hyper-IgE syndrome may be benefited by TF therapy and they lend further support to the notion that T lymphocyte deficiency may be the basis of the eczema in this syndrome [4].
• Pentoxifylline was also shown in vitro to restore the normal chemotactic capacity of neutrophils from patients with known functional defects, i.e. myelodysplastic syndromes, lazy leucocyte syndrome, juvenile parodontitis, hyper-IgE-syndrome and liver cirrhosis [5].

High impact information on Job's Syndrome

• The interleukin-4 receptor variant Q576R in hyper-IgE syndrome [6].
• Levamisole in Job's syndrome [7].
• An IL-5-producing T cell clone derived from a patient with the hyper-IgE syndrome was used as a positive control [8].
• The hyper-IgE syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent skin abscesses, pneumonia, and highly elevated levels of serum IgE [9].
• These results suggest that monocytes from patients with hyper-IgE syndrome are activated to resorb bone via products of the prostaglandin synthase (cyclooxygenase) pathway [10].

Chemical compound and disease context of Job's Syndrome

• Antibody responses to protein, polysaccharide, and phi X174 antigens in the hyperimmunoglobulinemia E (hyper-IgE) syndrome [11].
• We determined the histamine content in the skin of 22 adults with atopic dermatitis, one patient with hyper-IgE-syndrome, and 20 controls by the enzymatic double isotope assay [12].
• Demonstration of a cold abscess by gallium-67 imaging in a patient with Job syndrome [13].
• Lithium: a potential treatment for hyper-IgE syndrome [14].

Biological context of Job's Syndrome

• Although decreased interferon-gamma (IFN-gamma) production in patients with hyper-IgE syndrome is pointed out and known as a cause of reduced neutrophil chemotaxis, precise mechanism of their inadequate production of IFN-gamma remains unknown [15].
• In contrast, when blood from patients and controls was stimulated with heat-killed S. aureus or C. albicans, a severe imbalance towards a Th2 phenotype was found, with 10- to 30-fold reduction in the IFNgamma/IL-10 ratios in the hyper-IgE syndrome patients [16].

Anatomical context of Job's Syndrome

• We have previously shown that Fc epsilon receptor-positive (Fc epsilon R+) T cell lines from patients with the hyper IgE syndrome secrete IgE-binding factors which selectively enhance IgE but not IgG synthesis in cultures of B cells obtained from patients with allergic rhinitis but not from nonatopic subject [17].
• CONCLUSION: The lymphocytes of patients with hyper-IgE syndrome have an impaired response to IL-12, resulting in decreased IFN-gamma production, which may be of key importance in the pathogenesis of the immune abnormalities of hyper-IgE syndrome [18].
• Mononuclear leukocytes of patients with the hyperimmunoglobulinemia E syndrome (Job syndrome) produce low or undetectable levels of this lymphokine [19].
• Peyer's patches (PP) in germ-free rats (GF) and in the hyper-IgE syndrome patient (HIES) differ from their conventional rat (C) and healthy human (HH) counterparts in that GF rats contained fewer (two-fold) PP and none was detected in HIES [20].

Gene context of Job's Syndrome

• In this study, we demonstrated that IL-4 also can enhance spontaneous IgE synthesis by PBMNCs of allergic/atopic patients and patients with Buckley's or hyper-IgE syndrome [21].
• Increased expression of interleukin-13 but not interleukin-4 in CD4+ cells from patients with the hyper-IgE syndrome [22].
• Abnormal expression of CD54 in mixed reactions of mononuclear cells from hyper-IgE syndrome patients [23].
• We measured the in vitro production of interferon-gamma (IFN-gamma) in five cases of hyper-IgE syndrome (HIgE), induced by mitogens, calcium ionophores and phorbol ester [24].
• Cytokine and chemokine dysregulation in hyper-IgE syndrome [25].

Analytical, diagnostic and therapeutic context of Job's Syndrome

• However, selective insufficiency in the secretion of IFN-gamma molecules was found in patients with hyper-IgE syndrome.Confocal laser scanning microscopy clearly demonstrated the accumulation of IFN-gamma in patients with hyper-IgE syndrome [15].

References