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

Häftad, Engelska, 2012

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Beskrivning

In view of this and the steady advance of clinical and biochemical investigation of the polymorphonuclear neutrophil phago cyte, it appeared timely to review the inborn errors of immunity and phagocytosis at the fifteenth annual symposium of the Society for the Study of Inborn Errors of Metabolism at Elsinore, Denmark on September II-14th, I~77.

Produktinformation

Utgivningsdatum:2012-02-13
Mått:155 x 235 x 23 mm
Vikt:614 g
Format:Häftad
Språk:Engelska
Antal sidor:391
Förlag:Springer
ISBN:9789401161992

Utforska kategorier

Klinisk medicin och internmedicin inom Medicin
Pediatrik inom Medicin

Innehållsförteckning

Section One Defects of Cell-mediated Immunity.- 1 Immunodeficiency diseases — a review.- 2 Genetics of the immune system.- Section Two Enzyme Defects and Immunodeficiency.- 3 Inborn errors of specific immunity: adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency — The Milner Lecture.- 4 Adenosine deaminase deficiency: enzyme replacement therapy and investigations of the biochemical basis of immunodeficiency.- 5 Prenatal diagnosis and heterozygote detection in adenosine deaminase deficiency.- 6 Pathogenic mechanisms in deficiencies of adenosine deaminase and purine nucleoside phosphorylase.- 7 Purine nucleoside phosphorylase deficiency associated with cellular immunodeficiency: immunological studies during treatment.- 8 Purine nucleoside phosphorylase deficiency associated with cellular immunodeficiency: metabolic studies during treatment.- 9 Severe combined immunodeficiency with B lymphocytes: a selective defect of precursor T cells.- 10 Purine metabolism and the control of lymphocyte proliferation. Effects of exogenous adenosine on normal human lymphocytes.- 11 Activity of adenosine deaminase and purine nucleoside phosphorylase in lymphocytes of man, horse and cattle.- 12 Ageing and activities of purine metabolizing enzymes in leukocytes.- 13 Immune function in Down’s syndrome.- Section Three Disorders of Non-specific Immunity.- 14 Morphological and biochemical alterations of polymorphonuclear neutrophil (PMN) leukocytes from patients with inborn errors of phagocytic function: a comprehensive review.- 15 Experimental approaches to the role of mononuclear phagocytes in non-specific immunity.- 16 Molecular bases of the metabolic excitability of phagocytes.- 17 Chronic granulomatous disease — biochemistry with special reference to oxygenmetabolism.- 18 The protective role of glutathione.- 19 Defective initiation of the metabolic stimulation in phagocytizing granulocytes.- 20 Modification of genetic expression in phagocytes.- 21 Neutrophil granulocyte chemotaxis in a reversible Boyden chamber.- 22 Simplified tests of leukocytic function.- 23 Antigen-induced neutrophil dysfunction in a patient with chronic eczema, recurrent ‘cold’ staphylococcal infections and hyperimmunoglobulinaemia E.- 24 Lack of myeloperoxidase-mediated iodination in granulocytes from a patient with generalized pustular psoriasis.- 25 Functional characteristics of neutrophil granulocytes from children with recurrent respiratory infections.- 26 Pneumocystis carinii infection in a girl with chronic granulomatous disease treated with transfusions of granulocytes.- Discussion.- Section Four Screening for Immunodeficiency.- Round Table Discussion.