Full Description
The rapid growth of immunology has greatly increased our understanding of disease; this growth has also generated a subject which at times appears separated from some of the basic medical sciences. Recent studies in the areas of purine metabolism and of polymorphonuclear neutrophil phago cyte function have, however, linked immunology and clinical medicine with biochemistry. The precise defects of the inborn errors of metabolism have now provided good evidence for the importance of purine metabolism specifically the enzymes adenosine deaminase and nucleoside phosphorylase in lymphocyte function. 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. The papers presented at that meeting form the basis of this volume which brings together contributions from immunologists, biochemists and clinicians. This interdisciplinary communication should be helpful to those concerned with immune function in their patients or in the laboratory. The book is divided into four sections, One: defects of cell-mediated immunity, Two: enzyme defects and immunodeficiency, Three: disorders of non-specific immunity and Four: screening for immunodeficiency. Section One contains two reviews, one on immunodeficiency from Robert Good's group in New York and another on the genetics of the immune system from Arne Svejgaard of Copenhagen.
Contents
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.
