Unit 12 Flashcards
where is CF restricted
to any exocrine gland
Et of CF
genetic
which gene is affected in CT
cystic fibrosis transmemb regulator gene
what chr is the CFTR gene on
7
what does cftr gene code for
cftr protein
characteristics of cftr
autosomal rescessive, monogenic
first step of patho in CF
cftr forms cl channel on epith wall -> cell memb is impermeable to cl
why is cl able to move from capiliarry into epithcell ok
cl moves from cap to cell via diffusion
where is the cl problem in cg
in the goblet cell, related to excretion of Cl out of cell
what happens when cl accumlates inside of the cell
inc concentration of cl inside cell
what happens to cells with inc cl concentrations
h20 enters cell to dec [cl]
what happens to Na in CF
it follows water back into the cl saturated cell
why is mucous in CF thick and sticky
dec h20 in mucous makes it thick and sticky - accum of mucociliary blanket
what happens to mucociliary clearance in CF
decreases
what type of environment devs in CF
environment that increases amount of microbial agents
what does inc microbes in RT lead to
chornic aw obstr and infc rt inc mucous
what happens when aw is inflamed/infected
influx of neutrophils
what does an influx of neutrophils in CF lead to
rls of elastase/mediators
what happens with inc rls of elastase /mediators
dev of chronic bronchitis, resp failure
what do 90% of CF pts die from
sev pulm disease
dx for cf
basic work up
sweat test
other gi/gu/rt mnfts
newborn screen for trypsinogen
sweat tests
measures Nacl in sweat. pts with inc CF have inc CL - 2-5x norm amount)
newborn screen for trypsingoen
inc levels may indicate obstr in pancreatic ducts as secs move into blood
why are pancreatic ducts blocked
inc in thick secretions dt cf causes blockage
why is trypsinogen measures not trypsin
because it has not been activated by bile
tx for cf
no cure
supportive tx
diet mod, pancreatic e supplementation
gammaglobulin to tx change in \IR
what is a comp of cf
infc
what drug may be used to tx cf
DNAse
fx of dnase
drug that breaks down dna to dec mucous stickiness (inc dna -> inc viscosity
what does dnase not do
destroy intact cells
what does dnase destroy
dna in lysed cells
why may a pt with cf need pancreatic e supplementation
because secs are not able to be activated dt duct blockage, so we need to supplement enzymes
