Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A new familial immunodeficiency disease characterised by recurrent and persistent pyoderma, folliculitis, and atopic dermatitis is described in a father and son. It is accompanied by abnormalities of lymphocyte function (including defective proliferative responses to phytomitogens, and subnormal response in immunoglobulin production after stimulation of the lymphocytes by pokeweed mitogen) and defective leucocyte chemiluminescence responses, which were associated with defective ability for intracellular killing of microbial organisms. The abnormalities of lymphocyte and leucocyte function, as well as the clinical manifestations, responded dramatically to treatment with the histamine-1 antagonist, chlorpheniramine, suggesting that the underlying defect in this disease may relate to defective histamine metabolism or abnormal expression of histamine receptors on lymphocytes and leucocytes.
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PMID:Pyoderma eczema and folliculitis with defective leucocyte and lymphocyte function: a new familial immunodeficiency disease responsive to a histamine-1 antagonist. 613 27

Atopic eczema in infants and children usually responds to a management programme involving patient education, the avoidance of environmental irritants, the regular use of emollients and the application of topical steroids. If a child fails to respond to this management programme, the dermatologist should initially ensure that the diagnosis is correct and exclude a coexistent disease process (for example, infection or immunodeficiency) that may be hampering response to treatment. The next step is to ensure that environmental irritants have been identified and eliminated from the child's environment and that prescribed medication is being used as instructed. Allergy assessment and ancillary therapy are reserved for those infants and children with severe disease not responding to basic therapy.
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PMID:Atopic eczema in children: what to do when treatment fails to work. 877 63

In immunocompromised patients, warts occur frequently and can be extensive. We describe a 24-year-old patient with severe therapy-resistant warts. In addition to human papillomavirus infection, he had chronic sinusitis, candidiasis, and atopic dermatitis. Anergy to delayed-type hypersensitivity skin test reaction, significant CD4 lymphopenia, and diminished in vitro T-cell proliferative response and interferon-gamma production indicated a deficiency of cellular immunity. Extremely low concentrations of serum IgM and IgG2 and a severe deficiency of in vitro IgM production pointed also to a humoral immunodeficiency syndrome. This case represents a combination of cellular and humoral immunodeficiencies that has not been previously described in association with warts.
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PMID:Verrucosis of hands and feet in a patient with combined immune deficiency. 948 98

Twelve German shepherd dogs, each diagnosed as having a recurrent or refractory deep pyoderma (i.e., German shepherd dog pyoderma [GSP]), were evaluated for several parameters over a six-year period. Results indicated that GSP could be associated with flea allergy dermatitis, atopic dermatitis, food allergy, cell-mediated immunodeficiency, or hypothyroidism, or could be an idiopathic disease. The combination of diseases present for a given dog varied from case to case. Adequate control of the pyoderma was achieved only after each identified underlying disease was treated specifically, along with aggressive concurrent medical therapy using systemic antibiotics and medicated baths.
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PMID:German shepherd dog pyoderma: a prospective study of 12 dogs. 920 74

Type 2 cytokines, such as interleukin-4 (IL-4) and IL-13, are associated with immunoglobulin E (IgE) production. This association has also been observed in CD8+ T cells from patients infected with leprosy and human immunodeficiency virus (HIV). Using intracellular cytokine staining and flow cytometry, the cytokine profile [IL-2, IL-4, IL-10, IL-13, and interferon (IFN)-gamma] of both CD4+ and CD8+ memory/effector T cells circulating in atopic dermatitis (AD) patients was investigated at the single cell level. The levels of type 2 cytokines in CD4+ T cells or CD8+ T cells in AD patients with high levels of serum IgE (AD-H), low levels of serum IgE (AD-L), and healthy controls were compared. Increased production of IL-4 and IL-13 in both CD4+ CD45RO+ T cells and CD8+ CD45RO+ T cells after 4 h in vitro stimulation with phorbol 12-myristate 13-acetate and ionomycin, was more prominent in AD-H patients than in AD-L patients or healthy controls, whereas IFN-gamma-producing CD4+ CD45RO+ T cells and CD8+ CD45RO+ T cells were relatively diminished in AD-H patients. CD4+ T cells and CD8 + T cells from AD-H patients, cultured for 48 h with phorbol 12-myristate 13-acetate and ionomycin, released larger amounts of IL-4 and IL-13 but smaller amounts of IFN-gamma than both types of cells from AD-L patients or healthy controls. In addition, when stimulated with immobilized anti-CD3 monoclonal antibody (MoAb) and anti-CD28 MoAb, CD4+ CD45RO+ T cells and CD8+ CD45RO+ T cells from AD-H patients contained more IL-4-producing cells but fewer IFN-gamma-producing cells compared with healthy controls. Finally, spontaneous mRNA expression of IL-4 in blood CD8+ CD45RO+ T cells isolated from AD-H patients was increased, as determined by reverse transcriptase-polymerase chain reaction. Therefore, in AD patients with high IgE levels, type 2 cytokine (IL-4 and IL-13) expression is associated with IgE production, in both CD4+ CD45RO+ T cell and CD8+ CD45RO+ T cell subsets.
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PMID:Increased type 2 cytokine expression by both CD4+ CD45RO+ T cells and CD8+ CD45RO+ T cells in blood circulation is associated with high serum IgE but not with atopic dermatitis. 985 20

An unusual case of human immunodeficiency virus type 1 (HIV-1) infection in a child was studied. The child, identified as HIV-1 infected at 5 years of age, lived with his parents and a 3-year-old sister. HIV-1 infection was excluded in the mother and sister, but confirmed in the father, who was unaware of his infection and was in good health, apart from an atopic dermatitis on the face and limbs. A portion of the HIV-1 proviral envelope gene was amplified from the father's and child's peripheral blood cells, and the amplified products were cloned and sequenced. Phylogenetic analysis disclosed that the father's and child's viral sequences clustered together, and were clearly distinct from the sequence sets obtained from six epidemiologically unlinked mother-child HIV-1-infected pairs included in the analysis. HIV-1 variability was lower in the child's sequence set than in the father's, and the variability between father's and child's sequences was significantly lower than that found between epidemiologically unlinked cases (p < 0.001). An uncommon APGR motif on the tip of the V3 domain was found in both the father's and child's viral clones. These data, together with the epidemiological investigations, strongly suggest that the child acquired the infection from his father, possibly by exposure to bleeding skin lesions.
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PMID:Horizontal transmission of human immunodeficiency virus type 1 from father to child. 987 Mar 22

The association between the atopic dermatitis, eczema and T-cell immunodeficiency disorders are well known, thus suggesting that bone marrow T-precursors could use the micro-environment of the skin as an extrathymic site for compensatory ontogenesis. In keeping with this hypothesis, we analyzed the atopic dermatitis skin lymphocytic infiltrate phenotypes to establish their ontogenetic stage of development. Cryostatic sections (4 microns) obtained from acute lesional skin biopsies of six patients with extrinsic atopic dermatitis were processed with indirect immunoperoxidase, using a panel of first-step monoclonal antibodies (mAb) specific to CD104 (integrin beta 4 chain), CD90w (Thy 1 antigen), CD44 (phagocytic glycoprotein-1; Pgp-1), CD1a and the DNA polymerase terminal deoxynucleotidyl transferase (TdT). Within the lymphocytic dermal infiltrate different levels of immunoreactivity were observed with respect to CD104, CD90w and CD1a. A strong, spread staining was also detected for mAb specific to Pgp-1 and TdT. Together, the reported features indicate that the atopic dermatitis skin-homing lymphocytes express immunophenotypes which are distinctive of the early T-ontogeny.
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PMID:Expression of T-lineage early developmental markers by cells establishing atopic dermatitis skin infiltrates. 1002 83

The incidence of positive circulating specific immunoglobulin E (IgE) antibodies to latex and evidence of clinical latex sensitivity appears to be increasing since its first description in 1979. Although heightened medical awareness may be a factor, exposure to latex products, particularly rubber gloves, has increased since the discovery of the human immunodeficiency virus (HIV). Atopic individuals are at greater risk of developing latex sensitivity. We identified seven children with atopic eczema who were known to have clinically significant latex allergy and examined the relationship of prior exposure to latex gloves. All children had significant serum levels of specific IgE to latex. Before developing clinical symptoms of latex allergy, all had been exposed to latex in the form of gloves during either inpatient or outpatient treatments of their skin. Exposure of atopic individuals to latex gloves could be a major risk factor for sensitization and could increase the incidence of serious reactions.
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PMID:Latex allergy in atopic children. 1035 33

Interleukin 16 (IL-16) is a chemotactic cytokine which binds to CD4 and affects T cell activation. Here we report a novel single nucleotide polymorphism, T to C, in the promoter region of the IL-16 gene in two distinct Asian populations, Japanese and Thai. This mutation occurs at an allele frequency of approximately 22% and 18%, respectively. Although IL-16 potently suppresses replication of human immunodeficiency virus type 1 (HIV-1), we observed no significant difference in the allele frequency of this polymorphism between HIV-1-infected and non-HIV-1-infected individuals in both Asian populations. Since differential IL-16 levels have been reported to be associated with inflammatory diseases such as systemic lupus erythematosus, atopic dermatitis and allergic asthma, it would be of interest to analyze the allele frequency of this mutation in patients with these autoimmune and allergic diseases.
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PMID:A new polymorphism in the promoter region of the human interleukin-16 (IL-16) gene. 1119 8

Molluscum contagiosum virus (MCV) lesions from Spanish human immunodeficiency virus (HIV)-negative patients were clinically examined and analyzed for virus detection and typing. In a study of 147 patients, 97 (66%) were children under 10 years, of whom 49% had atopic dermatitis. MCV lesions were morphologically indistinguishable among the different age groups, but atopic patients presented larger lesions compared with patients without the disorder. In adults, lesions were observed mainly on the genitals. MCVI was the predominant subtype. The deduced MCVI/MCVII ratio (146:1) was much higher than that found in other geographical areas. Protein preparations of the virus-induced lesions were immunoblotted with sera from 25 MCVI patients. The host-serum antibody response was weak and variable, although no significant differences were found between atopic and nonatopic patients. Three immunoreactive proteins of 74/80, 60, and 35 kDa were detected in almost all the analyzed sera. The 35 and 74/80-kDa proteins were virus specific, whereas the 60-kDa protein band was composed of a mix of human keratins. Immunoblotting of MCV lesions and vaccinia virus-infected cell extracts with either MCV patient serum or a rabbit antiserum against vaccinia virus showed no cross-reactivity of these two human poxviruses at the antigenic level.
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PMID:Molecular epidemiology of molluscum contagiosum virus and analysis of the host-serum antibody response in Spanish HIV-negative patients. 1178 22


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