Pathophysiology Two Flashcards

1
Q

what is muscular dystrophy

A

a frequently fatal disease of muscle deterioration

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2
Q

what do you need to know about Duchenne’s

A
  • most common type
  • boys!
  • begins between 2-3 years old
  • affects lower limbs first
  • impacts heart and respiratory muscles later on
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3
Q

what do you need to know about becker muscular dystrophy?

A
  • ages 5-15 appear
  • slower progression than DMD
  • affects hips and pelvic area first
  • causes muscle weakness in the heart for most
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4
Q

T or F: DMD is an X linked recessive gene so it mostly affects females

A

F, is x linked recessive but mostly affects boys

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5
Q

explain the science behind why DMD is bad

A
  • dystrophin is a protein that attaches actin to the extra-cellular matrix
  • in DMD it’s too short and rips the myocyte membrane every time the muscle contracts and allows Ca+ to flow freely into the cell
  • excess Ca+ activates too many proteases and breaks down the proteins
  • this leads to elevated blood creatinekinase so less energy in the cell
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6
Q

what happens to the muscle fibers in myofibrillar myopathy

A

varied sizes

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7
Q

what happens to the internal architecture in myofibrillar myopathy

A
  • irregular
  • aggregate proteins (similar to tau in alzheimers)
  • vacuoles
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8
Q

what happens to endomysium in myofibrillar myopathy

A
  • increased connective tissue
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9
Q

why is desminopathy bad

A

it can lead to dysfunctional cell adherence (think desmosomes in cardiac muscle cells) and muscle cell death

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10
Q

what is desmin?

A

a protein that integrates the sarcolemma, Z disc, and nuclear membrane in sarcomeres and regulates sarcomere architecture

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11
Q

what is the primary function for titin

A
  • stabilize thick filament
  • center thick filament between thin filaments
  • prevent overstretching of the sarcomeres
  • recoil carcomere like a spring after it is stretched
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12
Q

______________ is the name for a defect in titin

A

titinopathy

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13
Q

are titinopathies dominant or recessive?

A

both\

*can be inherited or a random mutation too

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14
Q

a defect in actin or nebulin is called

A

nemaline myopathies

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15
Q

purpose of nebulin

A
  • wraps around actin and connects to Z-disc
  • thin filament molecular reler
  • regulates contractile force generation
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16
Q

is nemaline myopathies dominant or recessive

A

can be either

17
Q

Adult onset ______- is progressive autoimmune based

A

nemaline myopathy

18
Q

strains

A

injury to muscle or tendons

19
Q

sprains

A

injury to ligaments

20
Q

two types of strains

A

acute or chronic

21
Q

how do you treat strains

A

PRICE

22
Q

what are the different grades of strains?

A

1, 2 3,

23
Q

characteristics of grade 1 muscle strain

A
  • minor damage
  • soreness and loss of function
  • self-repair with rest and decreased usage
24
Q

characteristics of grade 2 muscle strain

A
  • “tearing”, heavy damage
  • intense pain, loss of motion, swelling, bruising
  • rest, potential need for brace or casting
  • seek medical assistanc eif symptoms are severe
25
Q

characteristics of grade 3 muscle strain

A
  • total rupture
  • compromised stability
  • extreme pain, bruising and swelling, total loss of motion
  • surgical intervention required to reattach tissue
26
Q

what are risk factors for muscle strains

A
  • sports (especially contact)
  • prior injury
27
Q

how do you prevent muscle strains

A
  • stretching
  • warm ups
  • be in shape for the sport, not use sport to get in shape
  • regular conditioning can prevent workplace injuries in physically demanding occupations
28
Q

what are symptoms of a muscle strain

A
  • pain/tenderness
  • redness/bruising
  • limited motion
  • muscle spasms
  • swelling
  • muscle weakness
  • clicking or popping
29
Q

_______ is a subset of muscle strains

A

DOMS

30
Q

acute strains are due to _________ while chronic muscle strains are due to __________

A

one event
daily wear and tear

31
Q

strains often occur at the __________

A

myotendinous junction

32
Q

strains occur when the energy the muscle is forced to absorb ________– the strength of the tissue

A

exceeds

33
Q

what can you use to help diagnose a strain

A

diagnostic ultrasound

34
Q

how to treat muscle strains

A
  • price
  • PT
  • immobiliation
  • surgical interventions
  • NSAIDS
35
Q

recovery of muscle strains depend upon ___________ and ___________

A

initial severity
complexity