Cystic fibrosis Flashcards
(10 cards)
What type of genetic disorder is cystic fibrosis?
An autosomal recessive genetic disorder.
Which organs are primarily affected by cystic fibrosis?
Lungs, pancreas, liver, intestine, and reproductive organs.
What are the main clinical signs of cystic fibrosis?
Pulmonary disease with recurrent infections and viscous sputum, and malabsorption due to pancreatic insufficiency.
What is Ivacaftor?
A CFTR protein potentiator that improves chloride transport.
What is the first-line mucolytic for cystic fibrosis?
Dornase alfa.
Which bacterial infection in CF is a major driver of lung function decline?
Pseudomonas aeruginosa.
What is the first-line inhaled antibiotic for chronic Pseudomonas aeruginosa?
Nebulised colistimethate sodium.
When should long-term azithromycin be used in CF?
In patients with deteriorating lung function or frequent exacerbations.
Oral corticosteroids are used if azithromycin is ineffective.
What enzyme supplement is given for exocrine pancreatic insufficiency?
Pancreatin.
What is used for cystic fibrosis-related liver disease with abnormal LFTs?
Ursodeoxycholic acid (unlicensed).
