Unit 22 Flashcards

1
Q

MD

A

sk m wasting/degen. progressive

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2
Q

what are 3 things cause MD

A

atrophy
necrosis of m. fiber
pseudohypertrophy

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3
Q

pseudohypertrophy

A

m tries to regen but CT and adipose is deposited instead of m.

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4
Q

which MD is most common

A

duchenne md

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5
Q

prevelance of duchenne md

A

1 in 3500 live male births

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6
Q

Et MD

A

monogenic, rescessive, x linked

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7
Q

how does MD pass

A

mother to son

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8
Q

why does md pass mom to son

A

mom has 2 x chrs, and if one is def and passes that one to son, since son only has 1 x, this is what mnfts

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9
Q

where is the def gene located in MD

A

short arm of x chr

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10
Q

how do we tell apart types of Md 5

A
m group
age of onset
genetics
mode of inheritance
progression/rate of disase
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11
Q

what does the def gene code for

A

dystrophin, a memb protein

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12
Q

where is dystrophin located

A

inner sarcolema of m cell

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13
Q

fx of dystrophin

A

attatches contractile filaments to points on the cell

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14
Q

where does dystrophin attach filaments to

A

eachother
sarcolema
ground matrix

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15
Q

when there is poor contractile protein attachment what happens to m fibers (step 1 patho)

A

necrosis with use

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16
Q

what happens when m fibers being to necros (step 2 patho)

A

attempt to regen and repair

17
Q

what does attempted regen and repair of m cells cause (step 3 patho)

A

more necrosis

18
Q

what does necrosis mean for m memb perm

A

change in permeability

19
Q

what happens with the change in perm that occurs with def dystrophin

A

allows influx of Ca

20
Q

what E is released when ca influxes into cells

A

enzymes (CK

21
Q

what are filaments doing to themselves with contraction

22
Q

why does fibrofatty ct replace M

A

body is trying to restrict, limit and adapt to changes rt cell necrosis by attempting to inc m mass. body cant regen m so psuedohypertrophy

23
Q

when does injury set in

A

with m use

24
Q

what age do we normally see mnfts? why

A

2-3yo because at this age toddlers begin to utilize more m

25
what is the main mnfts of md
progressive m weakness or change in m action
26
what types of m does md affect
all, esp resp and cardaic
27
what does progession of md to to resp and cardiac fx
changes in breathing, CO, cardiomyopathy
28
why is there no contraction or rleaxation by m tissue in progressed md
psuedohypertrophic m cant contract/relax
29
what is important when begining dx for md
seperating this out from a n. issue
30
what do we look at for dx of md
voluntary movement serum CK biopsy
31
what do we analyze with vol movement
how much movement can they do alone
32
what do we keep in mind when looking at serum CK levels
levels inc with age
33
what confirms dx for md
biopsy
34
what do we look for in biopsy for md
defecttive dystrophin on sarcolemma CT, adipose, Ca deposits necrosis
35
tx
no cure supportive and symptomatic comfort
36
what is important to remember about the damage in md
irreversible
37
what is the most life threatening aspect in md
breathing
38
what type of exercises can we do with md pts? why is this problematic
breathing. | inc injury occurs with inc m use, but if we dont use the m at all -> total atrophy
39
what primary prevention measures can we do for md
carrier status assesment on mom prior to conception for def x chr prenatal testing at 12 wks gest