Cystic Fibrosis (CF) or mucoviscidosis

 

 An inherited condition in which the exocrine glands produce abnormally viscous mucus, causing chronic respiratory and digestive problems

  • mutation in chloride channel transport proteins
  • results in thick, viscous mucus formation
  • effects:
  1. pancreas
  2. lungs
  3. liver
  4. intestine
  5. reproductive tract
  6. increase sweat salt content

 

Manifestations

  1. productive cough
  2. wheezing
  3. hyperinflation of lung fields on chest x ray
  4. obstructed airway pulmonary function tests
  5. colonization of airways with pathological bacterial early in life
  6. sinusitis and nasal polyps
  7. GI difficulties
  8. pancreatic insufficiency
  9. recurrent abdominal pain
  10. biliary cirrhosis
  11. vitamin deficiency
  12. recurrent pancreatitis
  13. weight loss
  14. GU difficulties – infertility
  15. clubbing at extremities

 

Dx

  1. elevated Cl sweat concentration
  2. Hx
  3. genetic testing

 

Management

  1. chronic bacterial infections
  2. bronchodilators
  3. PT chest therapy
  4. mucolytic agents
  5. anti-inflammatories
  6. corticosteroids
  7. O2
  8. gene therapy
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