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Flashcards in Unit 22 Deck (39):
1

MD

sk m wasting/degen. progressive

2

what are 3 things cause MD

atrophy
necrosis of m. fiber
pseudohypertrophy

3

pseudohypertrophy

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

4

which MD is most common

duchenne md

5

prevelance of duchenne md

1 in 3500 live male births

6

Et MD

monogenic, rescessive, x linked

7

how does MD pass

mother to son

8

why does md pass mom to son

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

9

where is the def gene located in MD

short arm of x chr

10

how do we tell apart types of Md 5

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

11

what does the def gene code for

dystrophin, a memb protein

12

where is dystrophin located

inner sarcolema of m cell

13

fx of dystrophin

attatches contractile filaments to points on the cell

14

where does dystrophin attach filaments to

eachother
sarcolema
ground matrix

15

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

necrosis with use

16

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

attempt to regen and repair

17

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

more necrosis

18

what does necrosis mean for m memb perm

change in permeability

19

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

allows influx of Ca

20

what E is released when ca influxes into cells

enzymes (CK

21

what are filaments doing to themselves with contraction

injury

22

why does fibrofatty ct replace M

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

when does injury set in

with m use

24

what age do we normally see mnfts? why

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