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Cystic Fibrosis (i) - Coggle Diagram
Cystic Fibrosis (i)
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Clinical features
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on exam: hyperinflation due to air trapping, coarse inspiratory creps, expiratory wheeze, clubbing
persistent loose cough, purulent sputum
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Management
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aim = prevent progression of lung disease, maintain adequate nutrition + growth
resp tx
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physio @ least twice a day, clears secretions
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continuous prophylactic antibiotics (e.g. fluclox), with additional rescue antibiotics if resp sx increase or lung function declines
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chronic pseudomonas infection a/w rapid lung function decline, slowed by used of daily neb antipseudomonal antibiotics
neb DNAse/hypertonic saline decreases sputum viscosity, aids clearance
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Genetics
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correlation between genotype + phenotype for lung disease = rare, stronger for GI disease
suggests that additional factors determine lung disease severity - microbes, smoking, social deprivation, other modifier genes
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Dx
CXR shows hyperinflation, peribronchial shadowing, bronchial wall thickening, ring shadows
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