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Biochemistry, Pathology and Genetics of Pulmonary Emphysema documents the proceedings of an international symposium held in Sassari, Italy, 27-30 April 1980. Research on the origins of emphysema has acquired more importance than functional diagnostic studies. There are various hypotheses concerning the development of emphysema. Some cases of emphysema are linked to defects in metabolic functions of the vessels while others are linked to a disturbance in repair processes. The papers in this volume are organized into four sections. Section 1 contains studies on the pathology and biochemistry of lung connective tissue. Section 2 deals with animal models. Section 3 on proteases and antiproteases includes studies on the characteristics and identification of biological specimens, and alpha1-proteinase inhibitor. Section 4 takes up the risk factors and therapeutic approaches for lung disease. Other papers in the volume were presented during two roundtable discussions on the biochemistry of connective tissue components in emphysema and therapeutic approaches.
ForewordSection 1: Pathology and Biochemistry of Lung Connective Tissue Introductory Review: Pathological and Pathogenetic Aspects of Chronic Obstructive Lung Disease Evaluation of the Protease-Antiprotease Theory of Human Destructive Lung Disease Lung Elastin Content in Normal and Emphysematous LungsRound Table Biochemistry of Connective Tissue Components in Emphysema The Measurement of Elastin Turnover by the Radioimmunoassay of Urinary Desmosine Excretion Antigenicity of Human Lung Elastin Peptides Measurement of Elastin Degradation In Vivo by Desmosine Radioimmunoassay Biochemical Measurement of Lung Connective Tissue Modifications of 14C-Glucosamine Incorporation into Hamster Lung Glycoconjugates in Elastase Induced Emphysema Glycosaminoglycans in Elastase Induced Emphysema Glycosaminoglycans (GAG) in the pathogenesis of the Flaccid Lung Syndrome Lung Scleroproteins in Young and Adult Rat and in Rat with Spontaneous Emphysema: Comparative Study by Biochemical and Histochemical ApproachSection 2: Animal Models The Tissue Response to Exogenous Elastase Electron and Light Microscopic Studies of the Lungs of Chloramine-T Treated Dogs The Tight-Skin Mouse: an Animal Model of Inherited Emphysema Ultrastructural Evidence of Mucociliary Function Impairment Induced by Elastase General Discussion Chairmen SummariesSection 3: Proteases and Antiproteases A. Basic Aspects and Identification in Biological Specimens Pathophysiological Interpretation of Kinetic Constants of Protease Inhibitors Characterization of Different Elastases. Their Possible Role in the Genesis of Emphysema Interactions between Granulocyte Proteases and Protease Inhibitors in the Lung The Acid Stable Proteinase Inhibitors of the Respiratory Tract. Chemistry and Function Localization of a Low Molecular Weight Protease Inhibitor in the Respiratory Tract Characterization of a Bronchial Inhibitor in the Sputum of Chronic Bronchitics Proteases and Antiproteases in Bronchoalveolar Lavage Serum Derived Protease Inhibitors and Leucocyte Elastase in Sputum and the Effect of Infection A radioimmunoassay for a Granulocyte Protein as a Marker in Studies on the Turnover of Such Cells B. Alpha1-Proteinase Inhibitor Genetic Variants of the Pi System in Normans: Comparison with Some European Populations Respiratory Function Disturbances in Alpha1-Antitrypsin Deficiency Alpha1-Antitrypsin Phenotypes and Pi M Subtypes in Italy Alpha1-Antitrypsin Serum Concentration and Respiratory Symptoms in 1294 Healthy Men A Survey of Alpha1-Antitrypsin Deficiency by the British Thoracic Association Inactivation of Alpha1-Proteinase Inhibitor and Bronchial Mucous Proteinase Inhibitor by Cigarette Smoke In Vitro and In Vivo Oxidation of Alpha1-Proteinase Inhibitor as a Major, Contributing Factor in the Development of Pulmonary Emphysema Chairman SummarySection 4: Risk Factors and Therapeutic Approaches Genetic Factors in Chronic Obstructive Lung Disease Lung Toxicity of Some Atmospheric Pollutants Sex Hormones and the Flaccid Lung Syndrome Intrathoracic Airway Mechanics in Patients with EmphysemaRound Table: Therapeutic Approaches Opportunities for the Specific Therapy of Obstructive Lung Disease Prevention of Elastase-Induced Experimental Emphysema by a Synthetic Elastase Inhibitor Administered Orally Comparative Effects of Reversible and Irreversible Specific Elastase Inhibitors on Elastase-Induced Emphysema Elastase and Lysozyme Secretion by Peritoneal Macrophages; Effects of Dexamethasone and RifampinConclusions Emphysema: Past, Present and Future