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" Inborn Errors of Immunity and Phagocytosis : "
F Güttler
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BL
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| Record Number
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775955
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b595951
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| Main Entry
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F Güttler
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| Title & Author
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Inborn Errors of Immunity and Phagocytosis : : Monograph based upon Proceedings of the Fifteenth Symposium of The Society for the Study of Inborn Errors of Metabolism.\ F Güttler
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| Publication Statement
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Dordrecht : Springer Netherlands, 2012
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(393 pages)
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| ISBN
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9401161976
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: 9789401161978
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| Contents
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Front ; Contents; Contents; List of Contributors; Preface; SECTION ONE; Defects of Cell-mediated Immunity; Immunodeficiency diseases --; a review*; Genetics of the immune system; SECTION TWO; Enzyme Defects and Immunodeficiency; 3 I nborn errors of specific immunity: adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency; Adenosine deaminase deficiency: enzyme replacement therapy and investigations of the biochemical basis of immunodeficiency; Prenatal diagnosis and heterozygote detection in adenosine deaminase deficiency. Pathogenic mechanisms in deficiencies of adenosine deaminase and purine nucleoside phosphorylasePurine nucleoside phosphorylase deficiency associated with cellular immunodeficiency: immunological studies during treatment; Purine nucleoside phosphorylase deficiency associated with cellular immunodeficiency: metabolic studies during treatment; Severe combined immunodeficiency with B lymphocytes: a selective defect of precursor T cells; Purine metabolism and the control of lymphocyte proliferation. Effects of exogenous adenosine on normal human lymphocytes. Activity of adenosine deaminase and purine nucleoside phosphorylase in lymphocytes of man, horse and cattleAgeing and activities of purine metabolizing enzymes in leukocytes; I mmune function in Down's syndrome; Disorders of NonƯspecific Immunity; Morphological and biochemical alterations of polymorphonuclear neutrophil (PMN) leukocytes from patients with inborn errors of phagocytic function: a comprehensive review; Experimental approaches to the role of mononuclear phagocytes in non-specific immunity; Molecular bases of the metabolic excitability of phagocytes. Chronic granulomatous disease --; biochemistry with special reference to oxygen metabolismThe protective role of glutathione; Defective initiation of the metabolic stimulation in phagocytizing granulocytes; Modification of genetic expression in phagocytes; Neutrophil granulocyte chemotaxis in a reversible Boyden chamber; Simplified tests of leukocytic function; Antigen-induced neutrophil dysfunction in a patient with chronic eczema, recurrent Icold' staphylococcal infections and hyperimmunoƯglobulinaemia E. Lack of myeloperoxidaseƯmediated iodination in granulocytes from a patient with generalized pustular psoriasisFunctional characteristics of neutrophil granulocytes from children with recurrent respiratory infections; infection in a girl with chronic granulomatous disease treated with transfusions of granulocytes; Discussion; Screening for Immunodeficiency; Round Table Discussion; Index.
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| Subject
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Internal medicine.
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| LC Classification
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RC606.F488 2012
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F Güttler
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J W T Seakins
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R A Harkness
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