Cf week
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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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Cf week

CF week


Anatomy of the lrt

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

Respiratory epithelium


Bronchus

Bronchus


Bronchiole

Bronchiole


Terminal bronchiole

Terminal bronchiole


Respiratory bronchiole

Respiratory bronchiole


Alveoli

Alveoli


Physiology mechanics

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

Physiology

  • Ventilation

    • Pulmonary ventilation vs alveolar

    • What causes dead space?


Physiology1

Physiology

  • Diffusion

    • What are the layers of the blood-air-barrier?

    • What effects the rate of diffusion?


Physiology2

Physiology

  • Perfusion

    • Normal V/Q ratio

    • Hypoxic pulmonary vasoconstriction


Pathology of obstruction

Pathology of obstruction

  • Tubes – muscular vs non-muscular

  • Non-mechanical

  • Mechanical - extramural, intramural and intraluminal

  • Examples

  • Consequences


Cf week

CF

  • Type of inheritance?

  • Incidence


Sequelae

Sequelae

Lung

  • Abnormal mucous, less, thicker

  • Infection/ inflammation

  • Bronchiectasis

    Pancreas

  • Pancreatic obstruction leads to…

    Sweat gland

  • Decreased reabsorption of NaCl.


Pancreatic pathology

Pancreatic pathology


Sweat gland

Sweat gland


Other abnormalities

Other abnormalities

  • Bilateral absence of ductus deferens

  • Meconium ileus

  • Liver pathology

  • Males generally infertile, woman are not.


Diagnosis

Diagnosis

  • Sweat test and DNA test for CFTR mutation

  • 6 classes of mutation


Management

Management

Can you list 5 management options?


Management1

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

Psychosocial/burden of CF

  • Prognosis

  • Time consuming, life altering illness.

  • Costly

  • Good opportunity to learn about support and respite groups


Surface anatomy

Surface anatomy

6,8 and 10 for lung.8, 10 and 12 for pleurae.


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