Chronic bronchitis and emphysema, restrictive lung disorders, diffuse interstitial lung disease, idiopathic pulmonary fibrosis, pleural space disorders, neuromuscular disorders, chest wall deformities, pulmonary hypertension, pulmonary embolism, kidneys

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Biomedical Science
BMS 460
D.Rao Veeramachaneni

13 November Bronchitis Emphysema Age (yr) 40 – 45 50 – 75 Dyspnea Mild; late Severe; early Cough Early; abundant sputum Late; scant sputum Infections Common Occasional Respiratory Insufficiency Repeated Terminal Cor Pulmonale Common Rare; terminal Airway Resistance Increased Normal/slight increase Elastic Recoil Normal Low Chest Radiography Prominent vessels, large Hyperinflation, small heart heart Appearance Blue bloater Pink puffer Close association of chronic bronchitis and emphysema Bronchiolar and bronchial injury + bronchospasm, infection, hypersection of mucus → reversible obstruction in bronchioles and small bronchi Continued and repeated injury (smoking), continued and repeated infection → chronic bronchitis Destruction of alveolar walls + occasional respiratory infection → emphysema Chronic bronchitis with destruction of alveolar walls + emphysema with continued and repeated injury (smoking), continued and repeated infection → chronic bronchitis and emphysema Restrictive Lung Disorders Show Poor lung/chest compliance (ΔV/ΔP) Reduced total lung capacity, vital capacity and functional residual volume Normal or elevated expiratory flow rates (air spaces are open due to lung rigidity) Elevated P (A-a)2difference (↓ PaO 2 – reduced diffusing capacity Hypoxemia even at rest in later stages (V:P mismatch and impaired O d2ffusion) Normal or ↓PaCO → 2espiratory alkalosis Signs and Symptoms Dyspnea – effort induced at first but eventually present even at rest Cyanosis and finger clubbing Ground glass appearance on x-ray (infiltrates) Cor pulmonale General examples of restrictive lung diseases Parenchyma disorders (Fibrotic Interstitial Disease): Diffuse interstitial disease, sarcoidosis, hypersensitivity pneumonitis, occupational lung diseases Atelectatic defects: ARDS (adult/infant) Pleural space disorders: pneumothorax and pleural effusion Neuromuscular, chest wall and obesity disorders Infections: pneumonia, severe acute respiratory syndrome, tuberculosis Decreased lung volumes Diffuse Interstitial Lung Disease Infiltration of the alveolar walls with cells, fluid and connective tissue which may progress to irreversible fibrosis Precipitated by injury to type I alveolar cells or capillary endothelium Increased presence of collagen in interstitium Obliteration of capillary endothelium Dilated bronchioles and large air-filled sacs Immune response: inflammation, fibrosis and destruction of alveoli Idiopathic Pulmonary Fibrosis (IPF) Caused by ‘repeated cycles’ of epithelial activation/injury by some unidentified agent Epithelial activation/injury → TGF-β1 Transforming Growth Factor-β1, the driver of the process Fibroblasts and myofibroblasts → deposition of collagen and extracellular matrix → pulmonary fibrosis Inhibits caveolin in fibroblasts which inhibits collagen/extracellular matrix deposition Pleural Space Disorders Pneumothorax Accumulation of air in the pleural space Primary pneumothorax – spontaneous and more often in tall, thin men between 20 – 40 years of age (cigarette smokers) Secondary pneumothorax – complications from pre-existing diseases (asthma, infectious diseases [pneumonia, TB] or interstitial lung disease) Tension pneumothorax – penetrating or nonpenetrating injury. Air enters during inspiration but cannot exit during expiration. Venous return and cardiac output decline as mediastinum shifts. Pleural effusion Accumulation of more than 15 or so ml of fluid (pus?) in pleural cavity which is normally taken up by parietal pleural lymphatics Makeup of pleural effusion: low or high protein fluids, blood, infection, or lymph (chylothorax) Pathogenesis: changes in pleural capillary hydrostatic, colloid osmotic pressures or intrapleural pressure as well as inflammation of pleurae and blockage of lymphatic drainage will impact fluid build-up. Normally less than 15 ml in pleural space – up to 300 ml before major symptoms Neuromuscular Disorders Amyotrophic lateral sclerosis (ALS): neuromuscular degenerative disease affecting lung function due to muscle weakness and wasting. Irreversible and progressive Poliomyelitis: paralysis of diaphragm and intercostal muscles Muscular dystrophies: hereditary; progressive muscle weakness and eventually hypoxemia, hypercapnia and respiratory infections Guillain-Barre Syndrome: immunological basis; demyelination of peripheral nerves; recovery in most cases Myasthenia Gravis: neuromuscular junction defect with worsening of symptoms with exercise Chest Wall Deformities Kyphoscoliosis: compressed lungs lea
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