CPS Cystic Fibrosis Flashcards
1
Q
What is CF?
A
A disease affecting epithelial cells
Changes iob transport across epithelial cell membrane
Affects movement of chlorine
Changes viscosity and tenacity of mucus
2
Q
What does CF mainly affect?
A
Lungs, Pancreas, Sweat Glands, Ductus defferents
3
Q
Genetic
A
Autosomal recessive
1:25 people carriers, 1:22 caucasians, 1:2500
Triple base deletion on chromosome 7
CFTR gene rendundant, responsible for transmembrane Cl- transport
4
Q
Symptoms
A
Thich mucus in lungs hard to clear, causing infection
More infections
Pancreas
Poor pancreatic secretion
Less pancreatic lipase, fats not absorbed
Steattorhea
Sweat - high sodium, salty
Ductus defferens - Not formed in 90% of CF cases
5
Q
How is CF managed?
A
Limit lung infections
Improve ion transport
Deliver normal CFTR gene
