Pulmonary Cystic Fibrosis

Pulmonary cystic fibrosis is a CFTR chloride-channel defect causing thick, sticky airway mucus that leads to recurrent bacterial infection (Staph aureus, Pseudomonas), chronic neutrophilic inflammation, bronchiectasis, and progressive obstructive lung disease. Clinical: chronic cough, sputum, recurrent exacerbations, wheeze, hemoptysis, and eventual respiratory failure. Treatment: airway clearance (chest physiotherapy), inhaled antibiotics/mucolytics, systemic antibiotics for exacerbations, CFTR modulators (ivacaftor), and lung transplant in end-stage disease.

Common Symptoms

Chronic cough/sputum; recurrent infection; wheeze; bronchiectasis; hemoptysis; progressive dyspnea

Causes and Pathogenesis

CFTR mutation impairs chloride/water transport, dehydrating airway surface liquid and thickening mucus, causing obstruction, infection, and neutrophilic inflammation/bronchiectasis.

Treatment

Airway clearance; inhaled antibiotics/dornase alfa; systemic antibiotics for exacerbations; CFTR modulators; pancreatic enzymes; lung transplant

Preventive Measures

Newborn screening; early aggressive therapy; infection control; nutrition.

Department to Visit

It is recommended to visit the Respiratory department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.