22nd February 2024

Clinical Features

Respiratory symptoms

Frequent respiratory tract infections are a hallmark of cystic fibrosis patients. CF is the most common cause of bronchopulmonary infection in childhood and a major cause in early adult life. Sinusitis and nasal polyps are common manifestations. Shortness of breath, reduced exercise tolerance, fatigue, chronic productive cough and haemoptysis occur later once airway flow limitation and bronchiectasis develop. Spontaneous and secondary pneumothorax may also occur. Respiratory failure, cor-pulmonale and use of long-term oxygen eventually develop.

Gastrointestinal symptoms

Multiple gastrointestinal organs contain CFTR epithelial channels.

Common gastrointestinal associations with CF:

• Steatorrhea
• Development of diabetes mellitus
• Meconium ileus
• Pancreatitis 
• Fatty liver which can lead to liver cirrhosis
• Chronic malabsorption and maldigestion
• Meconium ileus and bowel obstruction