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Pediatrics PT III > JRA, Arthrogryosis, and OI > Flashcards

JRA, Arthrogryosis, and OI Flashcards

1
Q

True or false: Osteoarthritis is the most common childhood arthritic condition

A

False: its Juvenile RA… obviously

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2
Q

What is the incidence of JRA? Prevalence?

A

Incidence: 13.9/100,000
Prevalence: 113.4/100,000

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3
Q

JRA subtypes

A

• pauciarticular affects 1 to 4 joints (50 %)
• polyarticular affects 5 or more joints (40 %)
• systemic (10 %)
—can be organs

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4
Q

Etiology and pathophysiology of JRA

A 
Etiology and pathophysiology
• unknown, but autoimmune in nature
• inflammatory response
• proliferation of synovium
• adhesions and osteophytes form
• subluxation is not uncommon
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5
Q

What medications are used to treat JRA?

A

Variety of meds including NSAIDS, corticosteroids, gold salt & immunosuppressive drugs

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6
Q

What disabilities will a child with JRA face?

A

• Dependent upon phase, child’s abilities will vary
• Morning usually a painful time, may
start day with a warm bath
• Pain will often vary day to day
• disability will range from mild to severe, dependent upon the child and often dependent upon the day

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7
Q

How do you strengthen children with JRA?

A

Isometric-safer and less painful
PRE- good depending on if they’re mid flair up or not
Functional- safe! Just what they do in a day

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8
Q

True or false: Use PROM to stretch children with JRA to conserve their energy

A

FALSE

AROM is less aggressive and safer

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9
Q

True or false: Scoliosis can occur with JRA

A

True

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10
Q

True or false: you must be aggressive when treating JRA

A

False

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11
Q

True or false: more than half of kids with JRA go on to adulthood without arthritis

A

True

Usually lost during puberty

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12
Q

What is Arthrogryposis Multiplex Congenita?

A

Nonprogressive neuromuscular syndrome that is present at birth

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13
Q

What is the incidence of Arthrogryposis Multiplex Congenita?

A

1/3,000-4,000 births

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14
Q

What is the etiology of Arthrogryposis Multiplex Congenita

A
  • unknown etiology for all but distal arthrogryposis which has a genetic basis
  • one possible etiology is high fever/virus during pregnancy
  • insult probably occurs in the 1st trimester
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15
Q

What are the causes of motor weakness in Arthrogryposis Multiplex Congenita in utero?

A

Neurogenic and myopathic disorder

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16
Q

What is the cause of contracutres in utero in Arthrogryposis Multiplex Congenita?

A

Immobilization

17
Q

What will a post natal biopsy of a child with Arthrogryposis Multiplex Congenita reveal?

A

A post-natal biopsy of the child will show virtually
normal muscle tissue in strong muscles and
fibro-fatty deposits in affected muscles

18
Q

How will a baby with Arthrogryposis Multiplex Congenita present?

A
  • Joint contractures
  • Amyoplasia (lack of muscle development)
  • Body areas most affected in descending order of prevalence:
  • foot • hip • wrist • knee • elbow and shoulder
19
Q

What are the 2 general presentations of AMC?

A

1.
• flexed and dislocated hips
• extended knees
• flexed elbows

2.
• Abd and ER hips
• flexed knees
• extended elbows

Both types exhibit equinovarus, flexed and ulnarly
deviated wrists, internally rotated shoulders

20
Q

True or false: You must be aggressive with babies with AMC

A

True. Aggressively stretch 3-5x daily

21
Q

True or False: be aggressive in children with OI

A

HELLA FALSE

22
Q

What is osteogenesis imperfecta ?

A

Inherited disorder of connective tissue

23
Q

What is the incidence of OI?

A

1/20,000 live births

24
Q

What is the prevalence of OI?

A

16/ 1,000,000

25
Q

What is the presentation of OI?

A

General presentation is lax joints, weakness and

osteoporosis with multiple recurrent fractures

26
Q

What is the most severe and disabling form of OI?

A

Osteogenesis imperfecta congenita

27
Q

Osteogenesis imperfecta congenita

A

• Most severe and
disabling form
• numerous fractures at birth
• dwarfism
• bowing or deformities of the long bones
• poor prognosis with a high mortality rate secondary to ICH or respiratory tract infections

28
Q

Osteogenesis imperfecta tarda

A
  • Fractures occur after birth
  • more mild
  • severity measured as to how much bowing of long bones is present
29
Q

What are the 2 types of osteogenesis imperfecta tarda

A

Type I
• most can ambulate with bracing
• short stature
• bowing of long bones

Type II
• least disabling form
• no bowing of long bones
• average height

30
Q

Which disease is associated with blue sclera?

A

OI

31
Q

Spinal deformities occur in _____% of patients with

OI

A

50%

32
Q

Kyphoscoliosis occurs in ______% cases of OI and may be disabling

A

20-40%

Pediatrics PT III (18 decks)

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