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Metabolic Disorders
 
 

Cystic Fibrosis

 
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Manifestations

    • Complications of Cystic Fibrosis

    • Respiratory

    • Bronchiectasis
    • Respiratory infections
    • Sinusitis
    • Pancreatic

    • Exocrine - fat malabsorption, malnutrition, vitamin A/D/E/K deficiency
    • Endocrine - diabetes mellitus
    • Pancreatitis
    • Gastrointestinal

    • Meconium ileus
    • Distal intestinal obstruction syndrome (DIOS)
    • Gastroesophageal reflux
    • Biliary cirrhosis
    • Malnutrition
    • Musculoskeletal

    • Arthropathy
    • Osteoporosis
    • Reproductive

    • Infertility - congenital absence of vas deferens, thick cervical mucus
    • Risk of transmission to children
    • Other

    • Significant functional impact
    • Depression

Management

    • Management Strategy

    • Pharmacologic Options

    • CFTR potentiator (G511D mutation) - ivacaftor
    • CFTR potentiator / corrector combination (delta F508 mutation) - ivacaftor / lumacaftor
    • Respiratory

    • Sputum clearance - bronchodilators, mucolytics, hypertonic saline, physiotherapy, postural drainage
    • Manage infection - monitor for colonisation, manage exacerbatons
    • Reduce inflammation - using macrolides (azithromycin)
    • Vaccinations - influenza, pneumococcus
    • Lung transplant
    • Pancreas

    • Exocrine - pancreatic enzyme replacement (creon), vitamin supplementation (A, D, E, K)
    • Endocrine - insulin if required
    • Gastrointestinal

    • Nutritional support
    • Constipation - aperients
    • Reflux - proton pump inhibitor
    • Biliary disease - ursodeoxycholic acid
    • Musculoskeletal

    • Arthropathy - NSAIDs
    • Osteoarthritis - calcium, vitamin D, bisphosphonates
    • Reproductive

    • Infertility - assisted pregnancy if required
    • Genetic counselling and testing of partner
    • Other

    • Maximise social supports
    • Treat depression
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