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Autosomal recessive
Increased viscosity of secretions (lungs and pancreas)
defect in CF transmembrane conductance regulator gene - cAMP regulated chloride channel
In UK 80% due to deltaF508 in long arm ch7
affects 1 in 2500 births with carrier at 1 in 25

Organisms which may colonise
Staph aureus
Pseudomonas aruginosa
Burkholderia ceppacia
Aspergillus

''Management''
regular (bd) chest physiotherapy and postural drainage (by parents) Deep breathing also useful
high calorie diet, including high fat intake (was low fat but now changed)
vitamin supplementation
pancreatic enzyme supplements taken with meals
heart and lung transplant