Other obstructive diseases

• bronchiectasis: permanent dilation of bronchioles and bronchi due to destruction of supporting tissue

  • obstruction: tumors, foreign bodies, impaction of viscous mucus

  • infection and necrotizing pneumonia with virulent organisms

  • cough is copious and purulent

• cystic fibrosis: most common lethal genetic disease affecting the white population (1 in 2300 births)

  • defects involve exocrine glands

    • lung: bronchiectasis and pneumonia caused by thick sputum

    • pancreas: exocrine deficiency

    • sweat glands: high Na, Cl, K in sweat

  • onset is often in childhood

  • life expectancy has increased with improved care—people used to die before they turned 20, but now they are living longer

  • histology
    

  • clubbing, cyanosis
    

  • damage to exocrine components in pancreas
    

• Kartagener’s syndrome

  • congenital structural or functional defect of cilia due to microtubular dysorganization or dysfunction

  • impaired clearance of airway: stasis, infection, bronchiectasis

Category: Pathology Notes