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)

Neonatal host defense simulates a clinical state of immunodeficiency that predisposes the preterm and term newborn to overwhelming bacterial sepsis. There are various immunologic components that are deficient in the newborn and new methods to enhance their function. Defects in both the quantitative and qualitative aspects of the neonatal phagocyte contribute substantially to the immaturity of neonates' immune systems. The neonate lacks an adequate number of granulocyte bone marrow progenitor cells, and has a decreased neutrophil storage pool and an increased tendency to peripheral neutropenia during neonatal sepsis. Additionally, the neonatal granulocyte demonstrates altered physiologic function compared with that found in the adult with respect to chemotaxis, phagocytosis, oxidative metabolism, and bacterial killing. Some recent clinical studies have suggested the benefit of using adult neutrophil transfusions as adjuvant treatment during neonatal bacterial sepsis, yet other studies have found the use of polymorphonuclear neutrophil leukocyte transfusions to be inconclusive. Reduced circulating immunoglobulins and impaired production of specific antibody have also led to recent trials in the use of prophylactic intravenous immunoglobulin in preterm infants predisposed to sepsis. Recently, hematopoietic colony-stimulating factors have been demonstrated to improve in vitro neonatal neutrophil physiologic activity. Future therapy of neonatal sepsis will depend on new nontoxic methods for enhancing neonatal host defense.
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PMID:Neonatal neutrophil host defense. Prospects for immunologic enhancement during neonatal sepsis. 264 45

A boy with combined immunodeficiency having low natural killer (NK)-cell activity received thymopoietin pentapeptide (TP-5) treatment, transplanted with T cell-depleted HLA-haploidentical bone marrow (BMT) cells from his father and with thymus tissue from an infant at different times during the first year of life. He showed a marked increase in large granular lymphocytes (LGL) both during the treatment with TP-5 and after BMT. The LGL generated following TP-5 injection had a T3+Leu11- surface phenotype and low NK activity. In contrast, the LGL appearing after BMT showed T3-, Leu7+, and/or Leu11+ surface phenotypes, had high NK- and K-cell activities, and were lymphokine-activated killer (LAK)-cell precursors. These killer activities were assigned to the Leu7-Leu11+ subset and proved to be of recipient origin. LGL proliferation following BMT was accompanied by neutropenia, which was improved in association with a reduction in the number of LGL and the appearance of T cells of BMT donor origin following thymus transplantation. This suggested the inhibition of granulopoiesis by the LGL and an in vitro study revealed that the Leu7+Leu11- subset of LGL suppressed the growth of granulocyte/macrophage colony-forming units. These results indicated that phenotypically different LGL could be generated by different treatments and that the LGL showing NK activity were distinct from those regulating granulopoiesis. It was also suggested that the generation of LGL was controlled by T cells.
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PMID:Phenotypical and functional heterogeneity of the large granular lymphocytes increased after various treatments in a patient with combined immunodeficiency. 264 8

Neonatal host defense simulates a clinical state of immunodeficiency that predisposes the preterm and term newborn to overwhelming bacterial sepsis. Defects in both the quantitative and the qualitative aspects of the neonatal phagocyte contribute significantly to the immaturity of their immune system. The neonate lacks adequate numbers of granulocyte bone marrow progenitor cells and has a decreased neutrophil storage pool and an increased tendency to peripheral neutropenia during neonatal sepsis. Additionally, the neonatal granulocyte demonstrates altered physiological function compared with that found in the adult with respect to chemotaxis, phagocytosis, oxidative metabolism, and bacterial killing. Reduced circulating immunoglobulins and impaired production of specific antibody are additional hallmarks of altered neonatal immunity. The use of adult neutrophil transfusions for the treatment of neonatal sepsis has shown promise in some clinical studies and no difference in others. Recent investigations have examined the use of intravenous gamma-globulin for prophylaxis and treatment of neonatal sepsis. The following review summarizes the state of immunodeficiency in the newborn and the potential role of polymorphonuclear leukocyte transfusions in the treatment of overwhelming neonatal bacterial sepsis.
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PMID:Neutrophil transfusions in the treatment of neonatal sepsis. 266 51

The purpose of this study was to determine the prevalence of granulocyte-associated immunoglobulins (GA-IgG) during the follow-up of asymptomatic human immunodeficiency virus (HIV)-infected subjects. An increase in GA-IgG was detected in 32 asymptomatic HIV seropositive subjects by a granulocyte immunofluorescence test (GIFT). At study onset, 7 (21.8%) had a positive GIFT. The prevalence of positive GIFT increased with time in the study subjects. No neutropenia was found. No significant difference was observed in subjects with positive or negative GIFT with respect to mean CD4 lymphocyte count and mean neutrophil count. The presence of GA-IgG was not associated to a positive direct antiglobulin test or of elevated platelet associated IgG.
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PMID:Granulocyte-associated immunoglobulins in asymptomatic HIV-infected subjects. 269 92

Conditioning therapy with aggressive chemotherapy and irradiation induces a state of transient combined immunodeficiency in bone-marrow transplant recipients. This promotes the occurrence of severe cytomegalovirus (CMV) infections, the most frequent lethal complication after bone-marrow transplantation (BMT) at present. Forty-four BMT recipients received CMV-IgG-hyperimmunoglobulin for CMV prophylaxis intravenously. The efficacy of this prophylaxis and possible risk factors for the occurrence of CMV-induced interstitial pneumonia (IP) were analyzed. Risk factors for the promotion of a CMV-IP were: additional immunosuppressive therapy after BMT, CMV-positive serostatus of the recipient, CMV-seropositive granulocyte transfusion, CMV infection immediately prior to BMT, and HLA-haploidentical BMT. In this study the incidence of graft-versus-host disease was low and was not associated with the incidence of CMV infections. The use of T-cell-depleted grafts did not result in increased CMV infections or IP and may possibly have improved the immunological reconstitution.
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PMID:Cytomegalovirus (CMV) infections in patients receiving CMV-IgG-hyperimmunoglobulin prophylaxis after bone-marrow transplantation. 282 58

Soluble interleukin 2 receptors (IL 2R) from human retrovirus-infected cells were analyzed. All the T cell lines integrated with human T cell lymphotropic virus (HTLV)-I and -II, or transfected with HTLV-I gag-pX gene, were found to release high levels of IL 2R constitutively. In contrast, this was not found in T cell lines in which HTLV-I was integrated but not expressed, human immunodeficiency virus (HIV)-infected T cell lines, or T cell, B cell, granulocyte and macrophage cell lines which were HTLV-I negative. These results raise the possibility that the pX-gene product might stimulate the generation of soluble IL 2R. In the sera from patients with adult T cell leukemia, large amounts of IL 2R were released, in contrast to sera from healthy carriers of HTLV-I. The molecular weight of IL 2R was determined to be about 50 KD by size-exclusion HPLC.
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PMID:High levels of soluble interleukin 2 receptors released from human retrovirus-infected cells. 288 14

Cells of the humoral immune system are particularly affected by a mutation at the X chromosome linked immunodeficiency disease (xid)locus. Although B cells are made in normal numbers, they fail to become phenotypically and functionally diverse. Consequently, poor antibody responses are mounted to certain types of Ag. There have been some indications that other types of hemopoietic cells may be influenced by the mutation and development of the humoral immune system is unusually dependent on the presence of T lymphocytes. We now describe an analysis of the lympho-hemopoietic environment studied with long term bone marrow cultures. Contrary to expectations, cultures initiated with cells from homozygous female or hemizygous male mice with the mutant allele established more quickly than normal. The accelerated initial growth pattern was clearly linked to the xid mutation. Artificial mixtures of marrow exhibited intermediate growth kinetics. Experiments with H-2 congenic and T6 chromosome marked cells did not reveal an intrinsic dominance of growth in nonadherent xid cells. Similar results were obtained with culture conditions which favored production of myeloid or lymphoid cells. These findings would be consistent with subtle changes in the bone marrow microenvironment resulting from the xid mutation. The pedigree of the mouse strains had a significant influence on lymphopoiesis in long term bone marrow cultures. Lymphocytes of BALB/c origin dominated over CBA/H background cells in cultures established from mixtures of the two, but this did not correlate with any functional deficiency in CBA/H stromal cells. In fact, establishment of an adherent layer was a rate-limiting step in initiating long term cultures and this could be achieved with a low dose inoculum of CBA/H marrow. Even more dramatic effects were found in hemopoietic cells from doubly defective C3H.nu/nu-xid mice. The bone marrow of these athymic animals contained normal numbers of granulocyte/macrophage progenitors. However, lymphoid cultures could not be reproducibly established with their cells and myelopoiesis was never observed in vitro. The relatively simple conditions which pertain in culture make it possible to appreciate effects of mutations and pedigree on hemopoiesis which are unremarkable in intact animals.
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PMID:The xid mutation affects hemopoiesis in long term cultures of murine bone marrow. 291 Oct 9

After bone marrow transplantation (BMT) transient combined immunodeficiency occurs which promotes the occurrence of severe cytomegalovirus (CMV) infections, the most frequent lethal complication at present. 28 patients received a CMV-IgG-hyperimmunoglobulin (CMV-IG) intravenously as CMV-prophylaxis. The efficacy of this treatment and the risk factors for the occurrence of interstitial pneumonia (IP) caused by CMV were analyzed. Risk factors promoting a CMV-IP were: immunosuppression after BMT, CMV-seropositivity of recipient and donor, granulocyte transfusions and HLA-mismatched BMT. In this study graft versus host disease did not influence the occurrence of CMV-IP.
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PMID:[Risk factors for the occurrence of pneumonia caused by cytomegaloviruses in patients with bone marrow transplants during prevention with cytomegalovirus hyperimmune globulin]. 300 Sep 24

A model system for cytokine-induced up-regulation of human immunodeficiency virus type 1 (HIV-1) expression in chronically infected promonocyte clones was established. The parent promonocyte cell line U937 was chronically infected with HIV-1 and from this line a clone, U1, was derived. U1 showed minimal constitutive expression of HIV-1, but virus expression was markedly up-regulated by a phytohemagglutinin-induced supernatant containing multiple cytokines and by recombinant granulocyte/macrophage colony-stimulating factor alone. Recombinant interleukin-1 (IL-1), IL-2, interferon-gamma, and tumor necrosis factor-alpha did not up-regulate virus expression. Concomitant with the cytokine-induced up-regulation of HIV-1, expression of membrane-bound IL-1 beta was selectively induced in U1 in the absence of induction of other surface membrane proteins. This cytokine up-regulation of IL-1 beta was not seen in the uninfected parent U937 cell line. These studies have implications for the understanding of the mechanism of progression from a latent or low-level HIV-1 infection to a productive infection with resulting immunosuppression. In addition, this model can be used to delineate the potential mechanisms whereby HIV-1 infection regulates cellular gene expression.
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PMID:Cytokine-induced expression of HIV-1 in a chronically infected promonocyte cell line. 331 29

Between February 1983 and April 1986 we studied peripheral blood and bone marrow samples from 20 patients with human immunodeficiency virus (HIV) related disease. 14 patients had AIDS, three had ARC, two had PGL and one had ITP as a sole manifestation of HIV related disease. Peripheral blood abnormalities included marked anisocytosis and poikilocytosis, rouleaux formation, neutropenia, lymphopenia, monocytopenia, a left shift in the granulocyte series and, in the patients with AIDS, vacuolated monocytes. The most frequent bone marrow abnormalities were reticuloendothelial iron block, dyserythropoiesis, megaloblastic change and erythroid hypoplasia. Excess histiocytes were noted in four marrows, one exhibiting haemophagocytosis. None of the bone marrows showed lymphopenia. Eight of the 20 marrows were difficult or impossible to aspirate. None of the trephine biopsies showed increased reticulin. The causes of these abnormalities are probably multiple and include opportunistic infections, drug therapy, immune mechanisms and possibly direct insult by the HIV virus.
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PMID:Peripheral blood and bone marrow abnormalities in patients with HIV related disease. 356 82


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