Presentation
[Cystic fibrosis]

Meconium ileus (in neonates)

Respiratory disease

• Bronchiectasis
• Obstructive pulmonary disease
• Persistent endobronchial infection
  Early:
     * Staphylococcus aureus
     * Haemophilus influenzae
     * Gram-negative bacilli
  Later:
     * Pseudomonas aeruginosa
• Pansinusitis
• Nasal polyps
• Allergic bronchopulmonary aspergillosis (in 10% of pt)

Pancreatic insufficiency

• Steatorrhoea
• Diabetes mellitus

Other GIT symptoms

• Constipation
• Intussusception
• Distal intestinal obstruction syndrome
• Rectal prolapse
• Cholelithiasis
• Cholecystitis
• Focal biliary cirrhosis
• Portal hypertension

Skeletal symptoms

• Hypertrophic osteoarthropathy
• Retardation of growth and bone age
• Demineralisation

Male infertility (due to vas deferens absence)

Impaired growth and delayed puberty due to chronic ill health