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Immunologic Deficiency Syndromes --- Immunologic Deficiency Syndromes --- diagnosis --- therapy
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Critically ill --- Immunologic deficiency syndromes --- Immunosuppression --- Critical Care --- Immunologic Deficiency Syndromes --- Immunologic Deficiency Syndromes --- Immunosuppression --- diagnosis --- therapy
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Immunological deficiency syndromes --- Immunologic Deficiency Syndromes --- Immunologic Deficiency Syndromes --- etiology --- therapy
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Phagocytes including neutrophil granulocytes and macrophages are important cells of the innate immune system whose primary function is to ingest and destroy microorganisms. Neutrophils help their host fight infections by phagocytosis, degranulation, and neutrophil extracellular traps. Neutrophils are the most common type of circulating white blood cells and the principal cell type in acute inflammatory reactions. A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency that renders patients vulnerable to recurrent infections by Staphylococcus aureus and Gram-negative bacteria being the most life-threatening. Neutropenia may be classified as mild, moderate or severe in terms of numbers in the peripheral blood, and intermittent, cyclic, or chronic in terms of duration. Besides well-known classic severe congenital neutropenia, chronic neutropenia appears to be associated with an increasing number of primary immunodeficiency diseases (PIDs), including those of myeloid and lymphoid lineage. A comprehensive overview of the diverse clinical presenting symptoms, classification, aetiological and genetic etiologies of chronic isolated and syndromic neutropenia is aimed to be reviewed.
Immunodeficiency. --- Immune deficiency --- Immunologic deficiency --- Immunological deficiency --- Immunity --- Immunosuppression
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La sclérose systémique (SSc) est une maladie auto-immunitaire, d'étiologie inconnue, qui affecte le tissu conjonctif et les vaisseaux de la peau et des organes internes.Une publication récente a suggéré que des anticorps stimulants, dirigés contre les récepteurs du poGF. sont impliqués dans le développement de la maladie. Ces anticorps stimulent les fibroblastes. qui produisent des espèces réactives dérivées de l'oxygène et du collagène. Cette découYerte ouvre de nouvelles perspectives dans le diagnostic et le traitement de la sclérose systémique.L'objectif de ce mémoire était de confirmer l'existence de ces anticorps, et d'évaluer leur intérêt dans le pronostic et le diagnostic de la maladie.Vingt-quatre sera de patients sclérodermiques nous ont été fournis (14 SSc diffuse, 10 SSc limitée). Nous avons purifié les immunoglobulines provenant de ces sera au moyen d'une technique standard de chromatographie, basée sur l'affinité des immunoglobulines envers la protéine A/G. Nous avons vérifié l'intégrité des anticorps purifiés en établissant la correspondance entre le taux d'anticorps antinucléaires, anticentromères et antitopoisomérase 1 (Scl-70), d'une part dans les échantillons d'immunoglobulines obtenus, et d'autre part dans les sera.U n modèle cellulaire (32D) a été construit en vue de tester l'activation des récepteurs du PDGF par ces anticorps. Quatre méthodes différentes ont été utilisées afin de mettre en évidence cette activation. Nous avons ainsi mesuré la prolifération cellulaire, la production d·espèces réactives de l'oxygène, la transduction du signal et la phosphorylation des récepteurs du PDGF. Un laboratoire indépendant a également étudié les sera de sept autres patients sclérodermiques .Nous avons montré au travers de ces tests, qu'aucun des échantillons d'immunoglobulines purifiées de patients sclérodermiques, n'activait les récepteurs du PDGF, en comparaison aux immunoglobulines de patients contrôles, alors qu'une forte activation était observée lors de la stimulation par le PDGF.Nos résultats remettent en question l'existence d'autoanticorps agonistes des récepteurs du PDGF dans la sclérose systémique. Systemic sclerosis (SSc) is a severe connective tissue disease of unknown etiology, characterized by fibrosis of the skin and multiple internal organs. A recent report suggested that the disease is driven by stimulatory autoantibodies to the platelet-derived growth factor (PDGF) receptors, which stimulate fibroblasts proliferation and collagen production. These results opened novel research avenues for the diagnosis and treatment of systemic sclerosis. Objective:The aim of this work was to test the diagnostic and prognostic value of autoantibodies to thePDGF receptors in scleroderma.Patients and methods:Twenty-four systemic sclerosis patients (14 diffuse SSc, IO limited SSc) were included in the study. We purified immunoglobulins from serum by standard protein A/G chromatography . IgG preparations contained the expected level of antinuclear autoantibodies, anticentromere and antitopoisomerase 1 (SCL70), compared to the corresponding sera. PDGF receptor activation was tested using four different sensitive bioassays, namely cell proliferation, reactive oxygen species production , signal transduction and receptor phosphorylation, which was also assayed on a separate set of seven SSc patients in an independent research center.Results:Purified IgG from SSc patients did not activate the PDGF a or 13 receptors in these tests, in comparison to normal IgG, although stimulation with recombinant PDGF consistently produced a strong signal.Conclusions:Our results question the existence of agonistic autoantibodies to the PDGF receptors in systemic sclerosis.
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Thymus Hormones --- Immunologic Deficiency Syndromes --- therapeutic use --- drug therapy
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Immunogenetics. --- Immunologic Deficiency Syndromes. --- Deficiency Syndrome, Immunologic --- Deficiency Syndromes, Antibody --- Deficiency Syndromes, Immunologic --- Immunologic Deficiency Syndrome --- Immunological Deficiency Syndromes --- Antibody Deficiency Syndrome --- Antibody Deficiency Syndromes --- Deficiency Syndrome, Antibody --- Deficiency Syndrome, Immunological --- Deficiency Syndromes, Immunological --- Immunological Deficiency Syndrome --- Syndrome, Antibody Deficiency --- Syndrome, Immunologic Deficiency --- Syndrome, Immunological Deficiency --- Syndromes, Antibody Deficiency --- Syndromes, Immunologic Deficiency --- Syndromes, Immunological Deficiency --- Antibodies --- Immunogenetic --- Genes, Immunoglobulin --- deficiency --- Immunodeficiency. --- Immunogenetics --- IMMUNOLOGIC DEFICIENCY SYNDROMES --- IMMUNOLOGIC DEFICIENCY SYNDROMES. --- Immunologic deficiency syndromes. --- Immunodeficiency --- Immunologic Deficiency Syndromes --- Genetics --- Immunity --- Immunology --- Serology --- Immune deficiency --- Immunologic deficiency --- Immunological deficiency --- Immunosuppression --- Immunological aspects --- Genetic aspects
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This second edition presents discussions of gene identification, mutation detection, and clinical and research applications for over 100 genetic immune disorders. It offers the principles of immunology, genetics, and molecular biology as they pertain to the primary immunodeficiency diseases.
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