Cystic Fibrosis Flashcards
What chromosome is affected in cystic fibrosis
Chromosome 7
Which classes of cystic fibrosis are most severe
Class/type 1-3
Which classes of cystic fibrosis are more milder
Classes/type 4-6
Which type of cystic fibrosis mutation is most common
F508 which is type 2
Ways of screening prenatal for CF
Pre-implantation genetic diagnosis
Chronic villous sampling
Amniocentesis
What ion is being measured in sweat test
Chloride ion which will be elevated
> 60mmol/l is abnormal
Describe the stool of someone affected by cystic fibrosis
White in color
Floats
Foul smell
What pathophysiological reason cause recurrent chest infections in people with cystic fibrosis
Water level in lungs is reduced
Causes cilla to be unable to move mucus
Shearing of moving mucus damages cillia and cells
Pathogens stay in lungs as they are not being removed by cillia and mucus
CT signs of CF
Tramlines
Signet rings
Mucus plugging
Consolidation(infection)
Treatment of pancreatic insufficiency
Replace enzymes via tablets
Make diet high energy plus high calorie supplement drinks (no low fat)
Fat soluble vitamins and minerals supplements
Treatment of respiratory features of CF
Airway clearance via physiotherapy
Mucolytics (make mucus thinne)
Bronchorilators
Treatment of chronic infections due to CF
Antibiotics (azithromycin)
Treatment of fibrosis/scarring/bronchiectasis due to CF
Supportive treatment as much of this is irreversible
Main complications of CF
Diabetes
Osteoporosis
Pneumothorax
Haemostasis
Osteoporosis in CF
What is it and treatment
Bone density falls in patients which causes an increased risk of fracture
May exclude transplant
Treatment- bine protection drugs and weight bearing exercises
