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CF week

CF week. 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. Respiratory epithelium. Bronchus. Bronchiole. Terminal bronchiole.

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CF week

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  1. CF week

  2. 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

  3. Respiratory epithelium

  4. Bronchus

  5. Bronchiole

  6. Terminal bronchiole

  7. Respiratory bronchiole

  8. Alveoli

  9. Physiology - mechanics • Pleural pressure • Muscles and movements in inspiration • Muscles and movements in expiration • Passive vs active expiration • Forces keeping alveoli open vs closed

  10. Physiology • Ventilation • Pulmonary ventilation vs alveolar • What causes dead space?

  11. Physiology • Diffusion • What are the layers of the blood-air-barrier? • What effects the rate of diffusion?

  12. Physiology • Perfusion • Normal V/Q ratio • Hypoxic pulmonary vasoconstriction

  13. Pathology of obstruction • Tubes – muscular vs non-muscular • Non-mechanical • Mechanical - extramural, intramural and intraluminal • Examples • Consequences

  14. CF • Type of inheritance? • Incidence

  15. Sequelae Lung • Abnormal mucous, less, thicker • Infection/ inflammation • Bronchiectasis Pancreas • Pancreatic obstruction leads to… Sweat gland • Decreased reabsorption of NaCl.

  16. Pancreatic pathology

  17. Sweat gland

  18. Other abnormalities • Bilateral absence of ductus deferens • Meconium ileus • Liver pathology • Males generally infertile, woman are not.

  19. Diagnosis • Sweat test and DNA test for CFTR mutation • 6 classes of mutation

  20. Management Can you list 5 management options?

  21. 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

  22. Psychosocial/burden of CF • Prognosis • Time consuming, life altering illness. • Costly • Good opportunity to learn about support and respite groups

  23. Surface anatomy 6,8 and 10 for lung. 8, 10 and 12 for pleurae.

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