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Cystic Fibrosis (Aetiology (Defect leads to defective chloride secretion…
Cystic Fibrosis
Aetiology
Defect leads to defective chloride secretion and increased sodium absorption across airway epithelium
This channel is found in epithelial cell membranes in lungs, pancreas, GI and reproductive tract
1500 mutations have been identified
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Changes in the composition of airway surface liquid predisposes the lung to chronic pulmonary infections and bronchiectasis
Investigations
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Chest x-ray: hyperinflation, bronchiectasis
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Abdominal ultrasound: fatty liver, cirrhosis, chronic pancreatitis
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Signs
GI
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Pancreatic insufficiency: DM, steatorrhoea
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Treatment
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Other
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Treat arthritis, sinusitis and vasculitis
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Chest
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Physiotherapy: postural drainage, airway clearance techniques
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