CYSTIC FIBROSIS

CYSTIC FIBROSIS
Cystic fibrosis is a common inheritable disease characterized by exocrine gland dysfunction. It is the most common cause for chronic obstructive lung disease in young adult.
Pathophysiology
  • Inheritance follows autosomal recessive pattern.
  • Glandular ducts become obstructed by abnormal secretory products.
  • Mucociliary transport is impeded.
Clinical picture
  • Recurrent lower respiratory tract infections. Recurrent infections from colonized Pseudomonas.
  • Sinusitis, nasal polyps, Clubbing ,prolonged expiration use of accessory muscles are common physical findings
  • CXR shows upper lobe bronchiectasis, peribronchial thickening, segmental infiltrates.
  • Obstructive defect in PFT.Widened A-a gradient with hypoxemia is common.
Diagnosis
  • Abnormal sweat test.
Treatment
  • Appropriate therapy for respiratory infections.
  • Supportive therapy with bronchodilators, Oxygen as needed.
  • Lung transplant in advanced cases.
Prognosis

  • Better antibiotics, respiratory toilette and lung transplant programs have considerably extended the life span of patients with CF.

No comments:

Post a Comment

Please leave your comments