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.
- Inheritance follows autosomal recessive pattern.
- Glandular ducts become obstructed by abnormal secretory
- Mucociliary transport is impeded.
- Recurrent lower respiratory tract infections. Recurrent infections from colonized
- Sinusitis, nasal polyps, Clubbing ,prolonged expiration use of accessory muscles
are common physical findings
- CXR shows upper lobe bronchiectasis, peribronchial thickening,
- Obstructive defect in PFT.Widened A-a gradient with
hypoxemia is common.
- Appropriate therapy for respiratory infections.
- Supportive therapy with bronchodilators, Oxygen as needed.
- Lung transplant in advanced cases.
- Better antibiotics, respiratory toilette and lung transplant programs have
considerably extended the life span of patients with CF.