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These changes in the fat tissue surrounding the heart create a local anti-thrombotic environment
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Clinical Features Respiratory * Chronic cough * Recurrent chest infections * Wheezing * Shortness of breath * Bronchiectasis Gastrointestinal * Failure to thrive * Malabsorption * Steatorrhea (fatty stools) * Abdominal distension * Meconium ileus in neonates Other Features * Salty-tasting skin * Nasal polyps * Digital clubbing * Delayed growth Common Organisms * Pseudomonas aeruginosa * Staphylococcus aureus Investigations Diagnostic Tests * Sweat chloride test (gold standard screening test) * Elevated chloride concentration supports diagnosis * Genetic testing for CFTR mutations Additional Tests * Chest X-ray * Sputum culture * Pulmonary function tests * Fecal elastase (pancreatic insufficiency) Management Respiratory Care * Chest physiotherapy * Airway clearance techniques * Nebulized saline * Antibiotics for infections * Bronchodilators when indicated Nutritional Management * High-calorie diet * Pancreatic enzyme replacement * Fat-soluble vitamin supplementation (A, D, E, K) Disease-Modifying Therapy * CFTR modulators in eligible patients Complications * Bronchiectasis * Recurrent pneumonia * Respiratory failure * Diabetes mellitus * Liver disease * Infertility (especially in males) Think of cystic fibrosis in a child with recurrent chest infections, failure to thrive, steatorrhea, and salty skin
