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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Diagnostic criteria for allergic fungal sinusitis have not been established, and clinical information consists primarily of isolated case reports. We proposed five diagnostic criteria for allergic fungal sinusitis including: (1) the demonstration of the characteristic eosinophil-rich allergic mucin visually or histopathologically, (2) a positive fungal stain or culture from the sinus at surgery, and (3) the absence of immunodeficiency or diabetes. With these criteria, seven patients in our metropolitan area with allergic fungal sinusitis were identified in a short period. Initial symptoms in our seven patients reflected those in 99 case reports in that two children were first seen with proptosis, one child and three adults with nasal congestion, and one adult with symptoms of chronic sinusitis. All had pansinusitis as shown on x-ray films. Six patients were atopic, five had nasal polyposis, and five had Curvularia species cultured from the sinuses. Infections with Bipolaris species, asthma, and chronic sinusitis were less common in our patients than in those previously reported. Recurrent symptoms and additional surgery sometimes resulted when the diagnosis was delayed by failure to obtain silver stains for fungus on surgical material sent for histopathologic review. Sinus tomography showed that the fungal material in the sinuses was of high density, which distinguished it from polyps or bacterial exudate. Bony compression, erosion, and rupture of the sinus walls were common. Results of IgE levels, precipitin determinations, and eosinophil counts were variable in both our patients and those in the literature. On the basis of our review, we believe that the simple diagnostic criteria proposed are appropriate for both research and clinical purposes.
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PMID:Diagnostic criteria for allergic fungal sinusitis. 762 60

X chromosome-linked immunodeficiency with hyper-IgM (HIGM1, MIM number 308230) is a rare disorder characterized by recurrent bacterial infections, very low or absent IgG, IgA and IgE, and normal to increased IgM and IgD serum levels. HIGM1 has been suggested to result from ineffective T-cell help for B cells. We and others have identified a novel, TNF-related activation protein (TRAP) that is exclusively expressed on the surface of stimulated T cells. TRAP, a type II transmembrane protein of M(r) 33,000, is the physiological ligand for CD40 (refs 5-8). Crosslinking of CD40 on B cells induces, in the presence of lymphokines, immunoglobulin class switching from IgM to IgG, IgA or IgE. Mapping of the TRAP gene to the X-chromosomal location q26.3-q27.1 (ref. 6) suggested a causal relationship to HIGM1, which had previously been assigned to Xq26 (refs 12-14). Here we present evidence that point mutations in the TRAP gene give rise to nonfunctional or defective expression of TRAP on the surface of T cells in patients with HIGM1. The resultant failure of TRAP to interact with CD40 on functionally intact B cells is responsible for the observed immunoglobulin isotype defect in HIGM1.
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PMID:Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM. 842 98

The ligand for CD40 (CD40L) is a membrane glycoprotein on activated T cells that induces B cell proliferation and immunoglobulin secretion. Abnormalities in the CD40L gene were associated with an X-linked immunodeficiency in humans [hyper-IgM (immunoglobulin M) syndrome]. This disease is characterized by elevated concentrations of serum IgM and decreased amounts of all other isotypes. CD40L complementary DNAs from three of four patients with this syndrome contained distinct point mutations. Recombinant expression of two of the mutant CD40L complementary DNAs resulted in proteins incapable of binding to CD40 and unable to induce proliferation or IgE secretion from normal B cells. Activated T cells from the four affected patients failed to express wild-type CD40L, although their B cells responded normally to wild-type CD40L. Thus, these CD40L defects lead to a T cell abnormality that results in the failure of patient B cells to undergo immunoglobulin class switching.
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PMID:CD40 ligand gene defects responsible for X-linked hyper-IgM syndrome. 843 51

We studied the ability of B lymphocytes from patients with X-linked hyper IgM syndrome (HIGM1) to be activated via the CD40 membrane receptor. HIGM1 is caused by a CD40 ligand gene mutation, leading to defective expression on the membrane of activated T lymphocytes. We found that triggering of B cells by an anti-CD40 monoclonal antibody or the soluble CD40 ligand plus interleukin (IL)-4 or IL-10 led to B cell proliferation and/or differentiation towards IgG, IgA and IgE secretion. This was reflected by transcription of C gamma, alpha and epsilon membrane isotype expression and IgG, IgA and IgE production. These results confirm the integrity of B cells in patients with the HIGM1 immunodeficiency and open up new therapeutic possibilities.
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PMID:Induction by anti-CD40 antibody or soluble CD40 ligand and cytokines of IgG, IgA and IgE production by B cells from patients with X-linked hyper IgM syndrome. 769 Mar 28

Most patients with common variable immunodeficiency (CVI) have normal numbers of circulating B cells but low concentrations of serum Ig. To determine if the hypogammaglobulinemia is caused by an intrinsic B cell defect, we studied B cell function of 22 CVI patients. Cultured B cells from all CVI patients underwent normal proliferation and synthesized normal quantities of IgE in the presence of anti-CD40 and IL-4. If cultured with anti-CD40 and IL-10, four patterns of Ig isotype synthesis were observed. Six CVI patients produced normal amounts of IgM, IgG, and IgA. Four patients produced normal quantities of IgM and IgG. Of the remaining 12 patients who failed to synthesize IgG and IgA, 8 produced normal and 4 synthesized decreased amounts of IgM. Analysis of the IgG subclasses produced by 10 patients with IgG-secreting B cells revealed that IgG4 was the most affected subclass, followed by IgG2; synthesis of IgG3 and IgG1 remained normal. Similarly, in the six IgA producing patients, IgA2 was more often affected than IgA1. The hierarchy of Ig isotype and subclass synthesis corresponds to Ig heavy chain constant region gene location on chromosome 14. Thus, circulating B cells of CVI patients are committed to synthesize one or more Ig isotypes or subclasses, and under proper conditions can proliferate, mature into Ig-secreting cells, and undergo class switch to IgE.
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PMID:Activated B cells from patients with common variable immunodeficiency proliferate and synthesize immunoglobulin. 769 Jul 75

Antibody- and cell-mediated responses to sulfamethoxazole (SMX) were analyzed in AIDS patients with or without a history of hypersensitivity and in negative controls. In 20 of 20 (P < 0.01) human immunodeficiency virus (HIV)-seropositive patients with skin reactions to cotrimoxazole, we found SMX-specific antibodies, while only 9 of 20 and 17 of 20 HIV-seropositive patients without a history of hypersensitivity to cotrimoxazole had SMX-specific immunoglobulin M (IgM) and IgG, respectively. The levels of specific IgM and IgG were higher in patients with skin reactions than in patients without reactions (IgM, 1.0 +/- 0.19 versus 0.47 +/- 0.23 [P < 0.001]; IgG, 0.68 +/- 0.15 versus 0.47 +/- 0.14 [P < 0.001] [mean optical density values +/- standard deviations]). Seronegative controls with no history of exposure to sulfa compounds did not have SMX-specific IgG or IgM antibodies, and controls with a history of intake of SMX with or without reactions had low levels of IgG and IgM. The SMX-specific IgG subclasses were exclusively IgG1 and IgG3. None of the patients had detectable SMX-specific IgE or IgA antibodies nor did they exhibit a cell-mediated response as measured by a lymphocyte proliferation assay. Antibodies to SMX recognized N-acetyl-sulfonamide, N-(2-thiazolyl)-sulfanilamide, sulfadiazine, and sulfisoxazole but did not recognize sulfanilamide or 3-amino-5-methyl isoxazole in an inhibition assay. It is not known whether the SMX-specific antibodies associated with hypersensitivity reactions to SMX in HIV-seropositive patients have a pathogenic role in these reactions. Sulfanilamide or 3-amino-5-methyl isoxazole, on the other hand, could be potential alternative therapies in HIV-seropositive patients with a history of skin reactions to SMX.
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PMID:Immune response to sulfamethoxazole in patients with AIDS. 769 29

Human immunodeficiency virus (HIV) infection-associated B-cell hyperstimulation, in particular, the chronic stimulation of B cells to undergo isotype switching, may play an important role in the pathogenesis of acquired immunodeficiency syndrome-associated lymphoma (AIDS lymphoma). Isotype switching can be induced by various immune system factors, including cytokines, cell-surface stimulatory molecule interactions, and CD23. CD23 is a B-cell differentiation and activation marker expressed on mature B cells that is lost after isotype switching; soluble CD23 (sCD23) also is a B-cell-stimulatory factor. Because sCD23 is associated with Ig isotype switching, and because an enhancement of isotype switching may contribute to the genesis of AIDS lymphoma, we examined serum sCD23 levels in a retrospective study of HIV-seropositive subjects who had gone on to develop lymphomas. Subjects were participants in the Multicenter AIDS Cohort Study at UCLA, a study of the natural history of AIDS. Greatly elevated sCD23 serum levels were seen in subjects who developed AIDS lymphoma, when compared with others with AIDS (without lymphoma), or to HIV-seronegative or HIV-seropositive subjects who did not have AIDS. Because the induction of IgE has been tied to the activity of CD23, serum IgE levels were also examined in this study, and found to be significantly elevated in those who developed AIDS lymphoma. These findings suggests that serum sCD23 levels potentially may serve as a clinical tool for early detection of lymphomas in people who have HIV infection. Also, these observations provide clues on possible pathogenetic mechanisms that result in lymphomagenesis in the context of HIV infection and AIDS.
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PMID:Elevated serum levels of soluble CD23 (sCD23) precede the appearance ofacquired immunodeficiency syndrome--associated non-Hodgkin's lymphoma. 770 91

Resort therapy-induced immunological changes were studied in 67 children from the unfavourable environmental areas, out of them 38 children (an experimental group) on Vetoron and 29 untreated children (a control group). The examinees were found to have secondary (acquired) immunodeficiency appeared as lower levels of immunoglobulin (Ig) A and higher levels of IgM, IgG and IgE. The health resort therapy improved the body's overall responsiveness--elevated the levels of IgA and IgM-reduced these of IgE, enhanced the activity of alkaline phosphatase and increased monocyte counts. The use Vetoron enhanced the efficiency of spa therapy in the examined children versus the matched ones, significantly elevated the relative levels of T lymphocytes and IgG and increased the activity of phagocytes.
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PMID:[The effect of water-soluble beta-carotene (Vetoron) on the immune status of children from ecologically unfavorable regions]. 770 13

C57BL/6 mice infected with a murine leukemia virus (MuLV) mixture designated LP-BM5 develop an immunodeficiency syndrome termed MAIDS, characterized by a variety of T and B cell abnormalities, including elevated levels of IgE, suggesting that IL-4 expression is increased in these animals. It has been suggested that the immunodeficiency associated with MAIDS is caused by a conversion of immune responses normally characterized by Th1 development towards a Th2-dominated response. Mice of the same strain, infected with Leishmania major, mount a protective Th1 response with the induction of high levels of IFN-gamma and undetectable IL-4. We therefore infected mice with L. major at differing time points before and after virus infection and assessed the effects on T cell responsiveness, cytokine production and survival to L. major, as well as the effect on MAIDS-associated pathology. We have also immunized C57BL/6 mice with trinitrophenol-keyhole limpet haemocyanin (TNP-KLH), which leads to a predominantly Th2 response, and compared the effects of MAIDS on the response to TNP-KLH with the effect of MAIDS on L. major infection. Our results show that significant immunodeficiency with regard to infection by L. major is only apparent after 8 weeks of LP-BM5 MuLV infection, by which time T and B cell defects are well advanced. Further, we have found that the strongly polarized Th1 response stimulated by L. major infection can modulate the effect of MAIDS on T cells, leading to the survival of antigen-specific T cells. Our results suggest that the impairment of immune responses to either TNP-KLH or L. major is due not to an alteration of the balance of Th1/Th2 subsets but to a general loss of reactivity in antigen-specific CD4+ cells. However, prior activation of Th1 but not Th2 cells can inhibit the development of lymphoproliferation and immunodeficiency caused by MAIDS.
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PMID:Modulation of specific T cell responses by concurrent infection with Leishmania major and LP-BM5 murine leukemia viruses. 771 9

Elevation of IgE has been associated with T-cell dysregulation and with the occurrence of opportunistic infections in patients with acquired immunodeficiency syndrome. The precise cause of IgE overproduction during the early stages of human immunodeficiency virus (HIV)-1 disease, however, has not been established. In light of reports demonstrating that IgE production may be affected by vitamin E levels in an animal model, we evaluated nutritional status in relationship to plasma IgE levels and immune parameters in 100 asymptomatic HIV-1-seropositive and 42 HIV-1-seronegative homosexual men. Approximately 18% of the HIV-1-seropositive population demonstrated biochemical evidence of plasma vitamin E deficiency (< 5 micrograms/ml). Subsequent analysis of available samples indicated a dramatic elevation of IgE levels (308 +/- 112 IU/ml) in vitamin E-deficient seropositive subjects (n = 9) as compared with age and CD4-matched HIV-1-seropositive persons with adequate vitamin E levels (n = 16, 118.1 +/- 41.1 IU/ml) and significantly lower levels (59.5 +/- 15.7 IU/ml) in HIV-1-seronegative men (n = 20, p = 0.01). This effect, which was independent of CD4 cell count, did not appear to be influenced by atopic or gastrointestinal parasitic disease. The low plasma vitamin E levels were related at least in part to dietary intake (r = 0.552, p = 0.01), suggesting that supplementation may be warranted in HIV-1-infected persons in whom vitamin E deficiency develops. Analysis of covariance revealed a strong relationship between IgE levels and CD8 cell counts (p < 0.006), and between IgE level and vitamin E deficiency (p < 0.039).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Elevated IgE level in relationship to nutritional status and immune parameters in early human immunodeficiency virus-1 disease. 772 70


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