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Flashcards in Unit 12 Deck (32):
1

where is CF restricted

to any exocrine gland

2

Et of CF

genetic

3

which gene is affected in CT

cystic fibrosis transmemb regulator gene

4

what chr is the CFTR gene on

7

5

what does cftr gene code for

cftr protein

6

characteristics of cftr

autosomal rescessive, monogenic

7

first step of patho in CF

cftr forms cl channel on epith wall -> cell memb is impermeable to cl

8

why is cl able to move from capiliarry into epithcell ok

cl moves from cap to cell via diffusion

9

where is the cl problem in cg

in the goblet cell, related to excretion of Cl out of cell

10

what happens when cl accumlates inside of the cell

inc concentration of cl inside cell

11

what happens to cells with inc cl concentrations

h20 enters cell to dec [cl]

12

what happens to Na in CF

it follows water back into the cl saturated cell

13

why is mucous in CF thick and sticky

dec h20 in mucous makes it thick and sticky - accum of mucociliary blanket

14

what happens to mucociliary clearance in CF

decreases

15

what type of environment devs in CF

environment that increases amount of microbial agents

16

what does inc microbes in RT lead to

chornic aw obstr and infc rt inc mucous

17

what happens when aw is inflamed/infected

influx of neutrophils

18

what does an influx of neutrophils in CF lead to

rls of elastase/mediators

19

what happens with inc rls of elastase /mediators

dev of chronic bronchitis, resp failure

20

what do 90% of CF pts die from

sev pulm disease

21

dx for cf

basic work up
sweat test
other gi/gu/rt mnfts
newborn screen for trypsinogen

22

sweat tests

measures Nacl in sweat. pts with inc CF have inc CL - 2-5x norm amount)

23

newborn screen for trypsingoen

inc levels may indicate obstr in pancreatic ducts as secs move into blood

24

why are pancreatic ducts blocked

inc in thick secretions dt cf causes blockage

25

why is trypsinogen measures not trypsin

because it has not been activated by bile

26

tx for cf

no cure
supportive tx
diet mod, pancreatic e supplementation
gammaglobulin to tx change in \IR

27

what is a comp of cf

infc

28

what drug may be used to tx cf

DNAse

29

fx of dnase

drug that breaks down dna to dec mucous stickiness (inc dna -> inc viscosity

30

what does dnase not do

destroy intact cells

31

what does dnase destroy

dna in lysed cells

32

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