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)

Spontaneous histamine release and basophil response to IgE-dependent (anti-IgE) and IgE-independent (formyl-methionine peptide, calcium ionophore A23187) stimuli were evaluated in 15 patients with acquired immunodeficiency syndrome (AIDS), 8 with AIDS related complex (ARC), 7 with lymphadenopathy syndrome (LAS), 11 seropositive asymptomatic subjects, 10 human immunodeficiency virus (HIV)-seronegative drug addicts, and 20 normal subjects. Both spontaneous histamine release and anti-IgE-induced histamine release were significantly increased in HIV-infected subjects, in comparison with seronegative drug addicts and normal controls. Basophil response to anti-IgE was higher in AIDS/ARC patients than in seropositive asymptomatic subjects and LAS patients, although the difference was not statistically significant. When basophils were challenged with 0.1 microM formyl-methionine peptide, a significantly increased histamine secretion was found in HIV-infected subjects; conversely, at the higher formyl-methionine peptide concentration (10 microM), as well as at all calcium ionophore A23187 concentrations, histamine release was similar in all the studied groups. No correlation was found among anti-IgE-induced histamine release, total lymphocyte counts, CD4+ and CD8+ T cell counts, and total serum IgE levels. These findings indicate that infection with HIV is associated with an increased basophil releasability. This could be of some relevance in the increased incidence of allergic manifestations and adverse drug reactions observed in AIDS patients.
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PMID:Enhanced basophil releasability in subjects infected with human immunodeficiency virus. 168 23

Basophil leukocytes from 39 HIV-infected patients with various degrees of immunodeficiency and disease progression were stimulated with an HIV antigen preparation. Cells from 19 of 22 patients with AIDS and all of six patients with milder degrees of HIV-related disease showed significant histamine release. In contrast, cells from 11 asymptomatic HIV-infected patients and 11 healthy control persons released no histamine. The histamine release induced by HIV antigen was found to be inversely correlated to the number of CD4 positive T lymphocytes. These results indicate that the histamine release was related to both the clinical stage of disease and the degree of immunodeficiency. Passive sensitization experiments showed that IgE, but not IgG, was responsible for the induction of histamine release, indicating the reaction to be type 1 allergic. The histamine release caused by HIV might be involved in the development of disease because of the immunomodulating properties of this mediator.
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PMID:HIV antigen-induced release of histamine from basophils from HIV infected patients. Mechanism and relation to disease progression and immunodeficiency. 171 97

The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS.
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PMID:Early bone marrow transplantation in an infant with Wiskott-Aldrich syndrome. 179 57

The autopsy material of 15 children aged from 2 months to 3 years from the zonal group of increased risk of the ecologic pathology, acquired immunodeficiency and viral infections was assessed morphologically and clinically. Decreased number of T-cells (T4, T8), an increase of the level of serum IgA, IgE and immune complexes, HIV-antibodies (4 cases) were found in the patients. The method of the molecular hybridization by means of virus-specific 32P-DNA probes was used. Bronchopneumonia was the cause of death. Severe deficiency of the organs and cells of the immune system, alternative-proliferative lung inflammation, mainly in the form of pneumonitis and alveolitis, were found. The latter differed either individually or as a result of the predominant infectious agent (RNA- or DNA-viruses, pneumocysts, bacterial flora, fungi). Considerable immunity dysfunctions enhanced the intensity of the specific features in pneumonia morphology.
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PMID:[Intrauterine and postnatal pneumonia in acquired immunodeficiency of infants]. 180 64

Plasmacytoma-bearing mice (PC-mice) develop a polyclonal B cell immunodeficiency syndrome characterized by marked impairment of: a) primary antibody responses and b) proliferative responses to B cell mitogens. The present investigations used two-color flow cytometry to examine B lymphocytes from the spleens and lymph nodes of PC-mice and found decreased surface membrane expression of surface IgM (sIgM), transferrin receptors (TfR) and IgE FcR (CD23), increased expression of class II MHC, but normal expression of B220, Mel-14, Fc gamma RII, and Fc mu R. These changes were not related to the H chain class or the amount of Ig produced by the plasmacytoma. When cultured with IL-4, B lymphocytes from PC-mice increased their expression of sIgM and class II MHC, but not of CD23. Several findings implicate transforming growth factor-beta 1 (TGF-beta 1) in the mechanism that modulates receptor expression on B lymphocytes in PC-mice: a) ascites fluid from PC-mice contains large quantities of TGF-beta 1; b) supernatants of cultured spleen cells from PC mice contain up to eightfold more TGF-beta than is found with normal spleen cells; c) cloned plasmacytoma cells produce TGF-beta in vitro; and d) the abnormal phenotype of B cells from PC-mice, i.e., decreased CD23, sIgM, and TfR, and increased class II MHC, is induced on normal B cells cultured in the presence of TGF-beta 1. Because sIgM, TfR, class II MHC, and CD23 are molecules that play fundamental roles in the activation of normal B cells, their modulation by TGF-beta 1: a) identifies molecular mechanisms that could account for some of the known immunosuppressive properties of TGF-beta 1 and b) implicates TGF-beta in the pathogenesis of the polyclonal B cell immunodeficiency that is characteristic of plasma cell tumors.
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PMID:Immune dysfunction in mice with plasmacytomas. I. Evidence that transforming growth factor-beta contributes to the altered expression of activation receptors on host B lymphocytes. 182 99

CD23 is expressed on mature B cells and is identical to a low-affinity IgE Fc epsilon receptor type II (Fc epsilon R II). The C terminal portion of CD23 is released to the serum as soluble Fc epsilon R II (sFc epsilon R II), which may be involved in regulation of IgE synthesis. We studied sFc epsilon R II levels in normal children and in patients with immunodeficiencies, including common variable immunodeficiency (CVI), partial DiGeorge syndrome, and immunodeficiency associated with ectodermal dysplasia to examine the relationship of sFc epsilon R II levels to B cell numbers and other immunoparameters. Serum Fc epsilon R II levels are higher in younger children (younger than 3 years) and decline gradually with age. In 11 patients with CVI with normal numbers of B cells (greater than 6%), sFc epsilon R II levels were comparable to that of control subjects. Five patients with CVI with deficiencies of peripheral B cells had levels of sFc epsilon R II similar to levels of control subjects. In all but one patient with partial DiGeorge syndrome, sFc epsilon R II levels were not significantly elevated, despite the presence of elevated peripheral B cell numbers. Of six patients with ectodermal dysplasia, four demonstrated increased Fc epsilon R II levels, a finding not correlated with serum IgE levels or with peripheral eosinophil or B cell numbers.
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PMID:Soluble Fc epsilon R II levels in normal children and patients with immunodeficiency diseases. 182 80

The occurrence of cancer, immunodeficiency, and diseases with possible autoimmune aetiology were studied in 355 blood relatives of 12 patients with common variable immunodeficiency (CVID). The family members were identified through the patients and interviewed after completing a questionnaire, their diseases were medically confirmed by local general practitioners. In two families consanguineous marriages were identified with the coefficients of inbreeding of 0.03125 and 0.01563, respectively: one patient, a dizygotic twin of an unaffected sister, was a granddaughter of first cousins, the second patient was the third daughter of second cousins. These cases of CVID strongly support the autosomal recessivity of the underlying genes. One male patient with CVID was shown to be related to a patient with X-linked hypogammaglobulinaemia, both sharing a common carrier. The different clinical courses of their diseases suggest two genetically determined immunodeficiencies and genetic heterogeneity. No family had an unusual clustering of cancer. The occurrence of tumours in the blood relatives of CVID patients was not significantly higher than in the relatives of spouse controls. Immunological examination of 30 first degree relatives of the CVID patients revealed three children (2 males and 1 female) with selective IgA deficiency, in one boy combined with elevated serum IgE level. Four relatives with rheumatoid heart disease, 12 cases of gastric or duodenal ulcer, and 14 relatives with thyroid disease represented the most often encountered diagnoses with a possible autoimmune component in their aetiology.
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PMID:Family studies in common variable immunodeficiency. 188 Apr 4

Trimethoprim-sulfamethoxazole (TMP-SMX) is frequently used in human immunodeficiency virus (HIV)-infected patients (HIV+) for treatment or prophylaxis of Pneumocystis carinii pneumonia (PCP). Up to 80% of those patients report adverse reactions to that drug combination. To test the hypothesis that these reactions are immunologically mediated, we quantitated specific IgG and IgE SMX-human serum albumin (HSA) antibodies and immune complexes (IC) in HIV+ patients and in HIV controls. Patients with mild HIV disease had elevated specific SMX-HSA IgG and IC levels compared with those having severe disease or with controls. Conversely, patients with severe HIV disease had statistically elevated levels of specific IgE when compared with patients having milder disease or with controls. There were no differences in either specific antibody or IC levels between patients reporting adverse reactions and those who did not. Results suggest that there are increased levels of SMX-HSA-specific antibodies in some HIV+ patients. The presence of these antibodies appears to be related to severity of disease, rather than clinically significant drug sensitivity.
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PMID:Evaluation of immune parameters in HIV+ subjects reporting adverse reactions to sulfamethoxazole. 193 83

The ability to engraft human PBMC or fetal tissue immune cells in the severe combined immunodeficient (SCID) mouse has created a need for characterization of these systems and their application to disease models. We demonstrate that SCID mice reconstituted with PBMC support the growth and differentiation of a restricted set of B cells. Human IgG levels of 1-2 mg/ml (10-20% of normal human serum levels) were routinely achieved in spite of a serum half life of only 12 d. Ig levels peaked around 50 d and Ig production was maintained for greater than 100 d. The Ig was greater than 85% IgG though some IgM, IgA, IgD, and even IgE could be detected. However, the human IgG produced in hu-PBL-SCID mice was pauci-clonal when analyzed by isoelectric focusing and by kappa/lambda light chain usage. Using a new polymerase chain reaction based analysis capable of monitoring individual VH family utilization, we found that the engrafted B cells showed skewed and restricted human VH subfamily utilization. These parameters were markedly variable among hu-PBL-SCID mice reconstituted from the same donor cell population at both early (21-50 d) and late stages (greater than 100 d). Hu-PBL/CVI-SCID mice constructed with cells from patients with common variable immunodeficiency with an in vitro block in terminal B cell differentiation produced human Ig responses that were quantitatively the same as those produced by hu-PBL-SCID mice from normal donors. The hu-PBL-SCID system using PBMC appears to lead to growth and Ig production by a small number of B cells and results in a restricted B cell repertoire.
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PMID:Limited B cell repertoire in severe combined immunodeficient mice engrafted with peripheral blood mononuclear cells derived from immunodeficient or normal humans. 199 50

Under normal condition, serum IgE concentrations are low and controlled by complex regulating mechanisms. Increased IgE synthesis may be associated with a response of IgE antibodies to allergens or parasites. It may also betray a wider disorder in the regulation of IgE production, resulting in a rise of total IgEs of unknown specificity. Broadly speaking, high IgE levels are observed in two main circumstances: allergic ans parasitic diseases. In allergic diseases the diagnostic value of high IhE concentrations is modest, since a search for specific IgEs by cutaneous tests ot laboratory techniques (RAST) is more rewarding than total IgE determination. However, total IgE assays are useful in the detection of atopy in neonates at risk and the identification and therapeutic follow-up of allergic pulmonary aspergillosis. High IgE levels are frequently associated with parasitic diseases, especially helminthiasis. They also reflect a disorder of IgE homeostasis in some types of immunodeficiency, including the classical IgE hyperproduction syndrome. Except for this syndrome, high IgE concentrations merely constitute a secondary diagnostic factor in a wide pathology, but they often bear witness to a subjacent dysregulation of T-cell function.
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PMID:[Significance of an increase of total IgE]. 204 13


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