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

The extent of suppression of antibody response by bursectomy (Bx) was examined as a measure of the seeding sequence of different clones from the bursa to peripheral lymphoid tissues. Chickens were bursectomized surgically 1, 4 or 7 days after hatching and immunized later with four antigens: sheep red blood cells (SRBC); Bordetella pertussis (Bp); human serum albumin (HSA); influenza virus (IV). The kinetics of the antibody responses were delayed in bursectomized birds when compared with the control groups. The following order in the degree of immunosuppression was established: Bp greater than HSA greater than SRBC greater than IV. This is discussed in relation to the 'sequential maturation' theory of ontogenesis of immunocyte differentiation. The data also stress the limitation of non-specific markers for assessing partial immunodeficiency states.
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PMID:Immunodeficiency in the chicken. I. Disparity in suppression of antibody responses to various antigens following surgical bursectomy. 16 36

Pituitary dwarf mutants of the Snell-Bagg and Ames mouse strains develop severe immunodeficiency of the thymus-dependent system which frequently leads to a fatal wasting syndrome. This immunodeficiency is a consequence of defective pituitary influences which will cause 1) an inadequate production of immunocompetent cells due to a central developmental defect primarily affecting the thymus and 2) the inability of immunocompetent cells to undergo a rapid and efficient antigen-induced proliferation and differentiation into antibody-forming cells. The relevance of the dwarf mouse model to a possible association between human immunodeficiency and endocrine disease is briefly discussed.
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PMID:The pituitary dwarf mouse: a model for study of endocrine immunodeficiency disease. 16 85

A fatal syndrome of certain Arabian foals which begins at about 25 days of age (range 14 to 46 days) and which runs a course of about 23 days (range 13 to 42 days) is described. The syndrome, which affected 17 foals on a single farm is further characterised by pneumonia, in some instances by dermatitis (dermatophilosis) and other infections, together with a progressive decline in health till death at about 49 days of age (range 34 to 77 days), despite intensive therapy. Four of the foals, on histopathological evidence, had adenviral pneumonia, in 2 foals there was histopathological evidence of an immunodeficiency disease and an adenovirus was isolated from 1 foal. It is argued, however, that the 17 foals were affected by a single, specific syndrome (an immunodeficiency disease) and that this disease is inherited as a simple, recessive, autosomal gene.
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PMID:Inheritance of a lethal immunodeficiency disease of Arabian foals. 16 84

The high frequency of hepatitis B antigen (HBsAg) in hepatocellular carcinoma (HCC) patients has led to the hypothesis that immunoresponsiveness to hepatitis B virus (HBV) may be deficient in some patients, and that the immune response deficiency may have a genetic basis. Radioelectrocomplexing (REC), a radioimmunoassay in gel based on the principle of counterimmunoelectrophoresis (CIE), has been used to identify four HBV immune status subgroups: 1) HBsAg +ve/HBsAb +ve; 2) HBsAg +ve/HBsAb -ve; 3) HBsAb -ve/HBsAb +ve; 4) HBsAg -ve/HBsAb -ve/HBsAb -ve. These subgroups comprise 2, 6, 70, and 22 percent, respectively, among blood donors, and 32, 19, 23, and 26 percent, respectively, among HCC patients. Although the HBV exposure rates in the two groups were similar, the immune complexemic rates and HBs antigenemic rates were significantly higher in HBB patients than in the blood donors. It is proposed that the failure of termination of HBV infection revealed by these high rates reflects an immunodeficiency state characterized by an inability to produce high-avidity HBsAb. The immunodeficiency might have a primary genetic basis, or it might be secondary to the immunodepressive effects of concurrent viral or parasitic infections.
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PMID:Immunodeficiency to hepatitis B virus infection and genetic susceptibility to development of hepatocellular carcinoma. 17 28

The case of a 3 month old child with severe combined sex linked immunodeficiency is presented. The diagnosis was well doccumented, during his life. The child presented as a case of mucocutaneous moniliasis resistant to treatment. There was a history of similar cases in the family; diagnosis was made at post-mortem in one cousin and death occurred at early age in all kins so affected. Blood marrow transplant was not feasible in our case because histocompatibility was lacking in the kins studied. Three units of transfer factor were given as well as hyperimmune plasma but the child died in respiratory failure. Autopsy demonstrated pulmonary infection by Pneumocystic carinii and generalized citomegalic inclussion virus infection; almost complete absence of immune tissue was also demonstrated.
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PMID:[Severe mixed immunodeficiency. Report of a case]. 17 1

The biochemical mechanisms by which a genetically determined deficiency of adenosine deaminase leads to immunodeficiency are still poorly understood and prompted this study. We have examined the effects of the adenosine deaminase inhibitor erythro-9-(2-hydroxy-3-nonyl) adenine hydrochloride (EHNA) upon the response of human peripheral blood mononuclear cells to the mitogen concanavalin A (Con A). Cells isolated from normal volunteers were incubated in microtiter plates in the presence of various inhibitors, and the incorporation of tritrated thymidine or leucine into macromolecular material was measured after 64 h. EHNA at a concentration of 0.3 muM, which inhibited 90% of the adenosine deaminase (ADA) activity in a mononuclear preparation, impaired the incorporation of tritrated leucine into protein; 100 muM EHNA was the minimal concentration that inhibited thymidine uptake. The addition of 15 muM adenosine or 10 muM cyclic AMP to Con A-stimulated lymphocytes inhibited leucine uptake, while millimolar concentrations were required to inhibit thymidine uptake. Lower doses of adenosine and cyclic AMP stimulated thymidine incorporation. The inhibition of thymidine uptake observed with millimolar concentrations of adenosine was independent of the type of mitogen (pokeweed or Con A), the concentration of mitogen, or the medium used, but could be increased if the cells were cultured in a serum with reduced levels of adenosine deaminase. Washout experiments failed to demonstrate a critical period early in immune induction during which adenosine exerted its inhibitory effects. Noninhibitory doses of EHNA potentiated the effects of adenosine and cyclic AMP on leucine and thymidine uptake. EHNA at a concentration of 50 muM also potentiated the inhibitory effects on thymidine uptake of dibutyryl cyclic AMP, butyric acid, norepinephrine, and isoproterenol, but not theophylline. When mitogenesis was assayed by leucine incorporations, no synergy between EHNA and these compounds was apparent. Uridine relieved to some extent the inhibition of blastogenesis produced by adenosine and cyclic AMP, but not by dibutyryl cyclic AMP, norepinephreine, isoproterenol, or theophylline. Neither uridine alone nor uridine plus adenosine protected lymphocytes from the inhibitory effects of EHNA.
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PMID:Effect of adenosine deaminase inhibition upon human lymphocyte blastogenesis. 17 77

1. In Hodgkin's disease patient's immunological in vitro and in vivo parameters are of prognostic importance. 2. Skin test reactivity correlates to peripheral T-lymphocyte counts and Con A induced lymphoblastogenesis. 3. Con A is the most sensitive in vitro indicator for detecting latent immunodeficiency. 4. Hodgkin patients in long term remission after tumor reductive therapy exhibit a qualitative and quantitative lymphocyte defect. 5. In Hodgkin patients Herpes virus related antibody titers are elevated against Epstein Barr virus (EBV). The elevation coincides with a decreased T-cell number and function. Antibodies against other Herpes viruses (HSV, CMV, VZV) are in the normal range, when tested by the complement fixation method.
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PMID:[Immunologic status in Hodgkin patients: correlation with Epstein-Barr virus titers]. 17 35

Chickens inoculated orally with infectious bursal disease virus (IBDV) 1 day after hatching subsequently showed a 50% incidence of immunodeficiency but little mortality. Antibody responses against IBDV and to immunization with sheep red blood cells (SRBC) or human serum albumin (HSA) were suppressed. Serum IgG concentration was decreased while IgM occurred exclusively in its 7S monomeric form (mIgM). An allotypic marker of chicken IgM (Mla) was lacking in mIgM derived from IBDV-infected birds. The loss of Mla occurred gradually in several birds between 3 and 12 weeks after perinatal infection. Inoculation of IBDV into chickens 3 weeks after hatching resulted in 50% mortality level but little immunodeficiency. Paradoxically, the serum IgG concentration was elevated, in comparison with normal birds. Histology of the bursa showed permanent hypo- or aplasia of follicles irrespective of the age of infection. The results suggest that bursal but not peripheral B cells are targets for IBDV, and immunodeficiency results from impaired peripheral seeding of B cells in infected juvenile chickens.
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PMID:Immunodeficiency in the chicken. IV. An immunological study of infectious bursal disease. 17 36

A boy 13 year-old suffered an extremely severe and prolonged attack of hemorrhagic chickenpox with visceral involvement, the diagnosis being confirmed by isolation of varicella-zoster-virus (VZV). There was no other compromising disease. All preceding vaccinations including two against smallpox had been uneventful. The severity of the attack could not be ascribed to any persistent cellular or humoral immunodeficiency. The patient developed a good antibody response. The course of serological reactions to VZV infection was studied extensively using the different techniques of complement fixation and immunofluorescence for IgG, IgM, and IgA antibodies. Therapy was conducted cautiously using cytosine arabinoside (Ara-C) between the 10th and 17th day of disease; the temperature fell and VZV multiplication ceased, strongly suggesting a beneficial influence on the patient, who recovered completely.
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PMID:Severe hemorrhagic varicella with visceral involvement: virological and serological studies during treatment with cytosine arabinoside. 18 29

The absence of erythrocytic adenosine deaminase (ADA) or purine nucleoside phosphorylase (PNP) has been associated with severe immunodeficiency disease in children. We have developed a cell culture model to study the possible relationships between purine salvage enzymes and immunologic function using an established T cell lymphosarcoma (S49) and a potent inhibitor of ADA, erythro-9(2-hydroxy-3-nonyl) adenine (EHNA). Wild-type S49 cells are killed by dexamethasone or dbc AMP, and adenosine (5 muM) in the presence of an ADA inhibitor (6 muM EHNA) also prevents the growth of and kills these S49 cells. It has been proposed that adenosine is toxic to lymphoid cells by virtue of its ability to increase the intracellular concentrations of cyclic AMP. We examined the sensitivity of three mutants of S49 cells, with distinctive defects in some component of cyclic AMP metabolism or action, to killing by adenosine and EHNA. All three mutants are resistant to killing by isoproterenol or cholera toxin and two are resistant to dbc AMP itself, but all are sensitive to killing by adenosine and EHNA. Similarly, two dexamethasone-resistant S49 mutants are as sensitive to adenosine and EHNA as are the wildtype cells. We have also simulated the purine nucleoside phosphorylase deficiency in S49 cells by adding inosine and adenosine to the growth medium. In the presence of EHNA or inosine, the toxic effects of adenosine can be partially reversed by addition of (10-20 muM) uridine, an observation suggesting that adenosine is toxic as the result of its inducing pyrimidine starvation.
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PMID:Characterization of a cell culture model for the study of adenosine deaminase- and purine nucleoside phosphorylase-deficient immunologic disease. 18 61


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