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CYSTIC FIBROSIS (abnormal Na2+ and Cl- transport (impaired MCC (reduced…
CYSTIC FIBROSIS
abnormal Na2+ and Cl- transport
decreased Cl secretion from epithelial cells (RESP)
increase in intracellular [Cl]
net movement of H20 + sodium into cell
dehydrated mucous
increased mucous viscocity
obstructs pancreatic duct (exocrine gland dysfunction)
enzymes not carried to small intestine
cant digest fats
impaired MCC
cilia sticks to mucous
good enviro for bacteria
good for
get caught on cilia + thrive
reduced HCO3
mucous more tightly bound
HC03 usually repulses electrostatic bonding of mucous
reduced airway pH
SKIN: sodium + Cl cant enter back into cell
sweat out a lot of salt
decreased/ impaired Cl secretion from epithelial cells of respiratory mucosa
biology
inherited genetic
autosomal recessive
obstructive & restrictive lung disease
CFTR dysfunction
CYCLE of infection & inflamm
bronchiectasis changes in lung
pulmonary fibrosis
abnormally thick & dry mucous
bronchial airway obstruction
infection
inflammation
release of proteases & DNA
thickenned mucous
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