Cystic Fibrosis

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Last updated 5:56 PM on 4/24/26
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7 Terms

1
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What is cystic fibrosis? How does it present?

Genetic disorder affecting the lungs, pancreas, liver, intestine and reproductive organs.

  • Pulmonary disease with recurrent chest infections

  • Production of copious viscous sputum

  • Malabsorption due to pancreatic insufficiency

2
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Which mucolytic is used in patients with CF?

Dornase alfa is the first choice mucolytic.

3
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Which CFTR modulators are used in CF?

Ivacaftor-tezacaftor-elexacaftor combination

4
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How is staph aureus pulmonary infection treated?

Flucloxacillin

5
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Which immunomodulatory agent is used in CF?

Long-term azithromycin at an immunomodulatory dose

6
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What is used to treat exocrine pancreatic insufficiency?

Pancreatic enzyme replacement therapy

7
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What should be monitored in patients with CF? 4 things

  • Intestinal obstruction - oral or intravenous fluids should be offered to ensure adequate hydration if patients have distal intestinal obstruction syndrome

  • Liver disease - if abnormal, ursodeoxycholic acid can be given until liver function is restored.

  • Diabetes - cystic fibrosis-related diabetes can occur

  • Bone mineral density - cystic fibrosis-related low bone mineral density can occur