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