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.