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This comprehensive overview delves into the anatomy and physiology of the respiratory system, focusing on the airway branchings from the trachea to the alveoli, pleural cavity, and lung structures. It examines normal ventilation processes, the mechanisms of diffusion, and the normal V/Q ratio, as well as the pathology of cystic fibrosis (CF). The course covers CF's inheritance, incidence, and various lung and pancreas complications, providing effective management options including physiotherapy and medications. Additionally, it highlights the psychosocial impact of living with CF.
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Anatomy of the LRT • Airway branchings & LMB vs RMB • Name levels from trachea to alveoli • Changes as we descend resp tract? • Pleural cavity • Lungs – lobes and fissures • Circulation • Innervation
Physiology - mechanics • Pleural pressure • Muscles and movements in inspiration • Muscles and movements in expiration • Passive vs active expiration • Forces keeping alveoli open vs closed
Physiology • Ventilation • Pulmonary ventilation vs alveolar • What causes dead space?
Physiology • Diffusion • What are the layers of the blood-air-barrier? • What effects the rate of diffusion?
Physiology • Perfusion • Normal V/Q ratio • Hypoxic pulmonary vasoconstriction
Pathology of obstruction • Tubes – muscular vs non-muscular • Non-mechanical • Mechanical - extramural, intramural and intraluminal • Examples • Consequences
CF • Type of inheritance? • Incidence
Sequelae Lung • Abnormal mucous, less, thicker • Infection/ inflammation • Bronchiectasis Pancreas • Pancreatic obstruction leads to… Sweat gland • Decreased reabsorption of NaCl.
Other abnormalities • Bilateral absence of ductus deferens • Meconium ileus • Liver pathology • Males generally infertile, woman are not.
Diagnosis • Sweat test and DNA test for CFTR mutation • 6 classes of mutation
Management Can you list 5 management options?
Management • Chest physiotherapy Postural drainage and manual techniques Breathing & airway clearance +/- PEP devices Exercise therapy • Medications Bronchodilators (preventers and relievers) Anti-inflammatory therapy Mucolytics & pulmozyme(rhDNase) • IV antibiotics (infective exacerbations) • Oxygen therapy (infective exacerbations, or end stage) • Nutritional support (+/- enzymes) • Transplantation (end stage) • Potential gene therapy - aim to restore CTFR function
Psychosocial/burden of CF • Prognosis • Time consuming, life altering illness. • Costly • Good opportunity to learn about support and respite groups
Surface anatomy 6,8 and 10 for lung. 8, 10 and 12 for pleurae.