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.
- Eventuates to diffuse
bronchiectasis.
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
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.