How many children are affected by CF in the UK?
1 in 25,000
1 in 2500
1 in 250
1 in 250,000
How many people are carriers of CF in the UK?
1 in 25
Inheritance of CF is autosomal dominant
∆F508 is the most common CF mutation
The defective protein in CF is called:
CFTR
CTFR
CRTF
In the most common mutation of CFTR, the protein can escape the ER but once in the cell membrane dose not function properly
Positive Guthrie testing for CF is diagnostic of the condition
Low Na concentration in sweat on sweat test suggests CF
Growth of patients with CF is unaffected
Clinical features of CF are similar in all patients
Which of the following medications are not used in treatment of CF?
Bronchodilators (e.g. salmeterol)
Anticholinergics (e.g. ipratropium)
Antibiotics
Steroids
Mucolytics (e.g. DNase)
Beta-blockers
CFTR modulators are currently the mainstay of treatment for CF in the UK
Median survival in CF is currently estimated at:
31 years
41 years
51 years
61 years
