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Cystic Fibrosis
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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
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