Unit 12 Flashcards

1
Q

where is CF restricted

A

to any exocrine gland

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2
Q

Et of CF

A

genetic

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3
Q

which gene is affected in CT

A

cystic fibrosis transmemb regulator gene

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4
Q

what chr is the CFTR gene on

A

7

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5
Q

what does cftr gene code for

A

cftr protein

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6
Q

characteristics of cftr

A

autosomal rescessive, monogenic

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7
Q

first step of patho in CF

A

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

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8
Q

why is cl able to move from capiliarry into epithcell ok

A

cl moves from cap to cell via diffusion

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9
Q

where is the cl problem in cg

A

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

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10
Q

what happens when cl accumlates inside of the cell

A

inc concentration of cl inside cell

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11
Q

what happens to cells with inc cl concentrations

A

h20 enters cell to dec [cl]

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12
Q

what happens to Na in CF

A

it follows water back into the cl saturated cell

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13
Q

why is mucous in CF thick and sticky

A

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

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14
Q

what happens to mucociliary clearance in CF

A

decreases

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15
Q

what type of environment devs in CF

A

environment that increases amount of microbial agents

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16
Q

what does inc microbes in RT lead to

A

chornic aw obstr and infc rt inc mucous

17
Q

what happens when aw is inflamed/infected

A

influx of neutrophils

18
Q

what does an influx of neutrophils in CF lead to

A

rls of elastase/mediators

19
Q

what happens with inc rls of elastase /mediators

A

dev of chronic bronchitis, resp failure

20
Q

what do 90% of CF pts die from

A

sev pulm disease

21
Q

dx for cf

A

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

22
Q

sweat tests

A

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

23
Q

newborn screen for trypsingoen

A

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

24
Q

why are pancreatic ducts blocked

A

inc in thick secretions dt cf causes blockage

25
Q

why is trypsinogen measures not trypsin

A

because it has not been activated by bile

26
Q

tx for cf

A

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

27
Q

what is a comp of cf

A

infc

28
Q

what drug may be used to tx cf

A

DNAse

29
Q

fx of dnase

A

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

30
Q

what does dnase not do

A

destroy intact cells

31
Q

what does dnase destroy

A

dna in lysed cells

32
Q

why may a pt with cf need pancreatic e supplementation

A

because secs are not able to be activated dt duct blockage, so we need to supplement enzymes