JRA, Arthrogryosis, and OI Flashcards
(32 cards)
True or false: Osteoarthritis is the most common childhood arthritic condition
False: its Juvenile RA… obviously
What is the incidence of JRA? Prevalence?
Incidence: 13.9/100,000
Prevalence: 113.4/100,000
JRA subtypes
• pauciarticular affects 1 to 4 joints (50 %)
• polyarticular affects 5 or more joints (40 %)
• systemic (10 %)
—can be organs
Etiology and pathophysiology of JRA
Etiology and pathophysiology • unknown, but autoimmune in nature • inflammatory response • proliferation of synovium • adhesions and osteophytes form • subluxation is not uncommon
What medications are used to treat JRA?
Variety of meds including NSAIDS, corticosteroids, gold salt & immunosuppressive drugs
What disabilities will a child with JRA face?
• 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
How do you strengthen children with JRA?
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
True or false: Use PROM to stretch children with JRA to conserve their energy
FALSE
AROM is less aggressive and safer
True or false: Scoliosis can occur with JRA
True
True or false: you must be aggressive when treating JRA
False
True or false: more than half of kids with JRA go on to adulthood without arthritis
True
Usually lost during puberty
What is Arthrogryposis Multiplex Congenita?
Nonprogressive neuromuscular syndrome that is present at birth
What is the incidence of Arthrogryposis Multiplex Congenita?
1/3,000-4,000 births
What is the etiology of Arthrogryposis Multiplex Congenita
- 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
What are the causes of motor weakness in Arthrogryposis Multiplex Congenita in utero?
Neurogenic and myopathic disorder
What is the cause of contracutres in utero in Arthrogryposis Multiplex Congenita?
Immobilization
What will a post natal biopsy of a child with Arthrogryposis Multiplex Congenita reveal?
A post-natal biopsy of the child will show virtually
normal muscle tissue in strong muscles and
fibro-fatty deposits in affected muscles
How will a baby with Arthrogryposis Multiplex Congenita present?
- Joint contractures
- Amyoplasia (lack of muscle development)
- Body areas most affected in descending order of prevalence:
- foot • hip • wrist • knee • elbow and shoulder
What are the 2 general presentations of AMC?
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
True or false: You must be aggressive with babies with AMC
True. Aggressively stretch 3-5x daily
True or False: be aggressive in children with OI
HELLA FALSE
What is osteogenesis imperfecta ?
Inherited disorder of connective tissue
What is the incidence of OI?
1/20,000 live births
What is the prevalence of OI?
16/ 1,000,000
