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