musculoskeletal disorders

Question 1

Juvenile idiopathic arthritis

Answer 1

• JIA is not a single disease, as the rterm includes all forms of childhood arthritis

Question 2

three systems used to diagnose and classify childhood arthritis

Answer 2

• American college of Rheumatology criteria for JIA
• European League against rheumatism criteria for JIA
• international league of associations for rheumatology criteria for JIA

Question 3

diagnosis of JIA

Answer 3

• defined as persistent arthritis lasting at least 6 weeks in one or more joints in a child younger than 16 years of age when all other cuases are eliminated
• etiology- genetic predisposition with external trigger

Question 4

Systemic JIA

Answer 4

• 4-17% cases
• M=F onset throughout childhood
• spiking fever 1-2 times/day for at least 2 weeks. fever typically accompanied by an evanescent rash on trunk or limbs
• systemic signs: pleuritic, pericarditid, myocarditis, hepatosplenomegaly, lymphadenopathy

Question 5

polyarticular JIA

Answer 5

• RF positive: onset 2-4 and 6 to 12
  RF neg: onset late childhood or adolescence
• arthritis in 5 or more joints: symmetrical. affecting both large and small joints. joints are swollen and warm but rarely red
  -rheumatoid nodules on elbows, tibial crests and fingers. less common in RF negative

Question 6

Oligoarticular JIA

Answer 6

• 27-56% of children with JIA
• onset in early childhood peak at 2-4 years
• female> male
• low grade inflammation in 4 or fewer joints
• joints are swollen and may be warm
• systemic signs unusual

Question 7

Primary clinical manifestation

Answer 7

• joint swelling, pain, and stiffness
  -morning stiffness
• muscle atrophy, weakness poor muscle endurance
• ## systemic manifestation

Question 8

secondary clinical manifestation

Answer 8

• limited joint motion; soft tissue contracture
• fatigue
• decreased aerobic capacity
• growth abnormalities
• osteopenia; osteoporosis
• difficulties with ADL
• participation restriction

Question 9

medical management

- NSAIDS

Answer 9

• most widely used first line therapy
• -naproxen, tolmetin and ibuprofen at emost common
• reduce fever, pain and inflammation, but do not alter the course of the disease

Question 10

medical management

- methotrexate

Answer 10

• most common disease modifying antirheumatic drug
• prescribed to children with poly and systemic JIA
• weekly oral administration

Question 11

goal of pharmacologic therapy

Answer 11

• control arthritis; preventing joint erosion

- manage extra articular manifestation

Question 12

PT intervention in acute phase

Answer 12

• maintaining and preserving joint function
• RICE- avoid heat, US and diathermy
• splinting

Question 13

PT intervention in subacute and chronic

Answer 13

• restoration and compensation of cuntion and activities

- balance rest and exercise

Question 14

aerobic fitness prescription

Answer 14

• 2x/week for 45-60 mins of moderate to vigorous intensity

Question 15

resistance training prescription

Answer 15

• 2x week. light weights with 2-3 sets to 10-15 reps
• use isometrics with acute joint inflammation
• progress to concentric and eccentric as inflammation subsides

Question 16

athrogryposis multiplex congenital defined

Answer 16

• a non-progressive neuromuscular syndrome present at birth that is characterized by severe joint contractures, muscle weakness and fibrosis
• prescence of contractures in 2 or more body areas

-children bright and motivated

Question 17

Forms of AMC

Answer 17

• amyoplasia 43% lack of muscle formation or development
• contrcatures syndrome 35%
• distal asrthogryposis 7%

Question 18

AMC etiology

Answer 18

• lack of fetal movement
• associated with neurogenic and myopathic diorders
• decr amniotic fluid, especially in 3rd trimester, may inhibit freedom of movement in utero

Question 19

factor implicated in AMC are

Answer 19

• hyperthermia of the fetus
• prenatal virus
• fetal vascular compromise
• septum of the uteru
• decr amniotic fluid
• gene deformity
• muscle and/or connective tissue development abnormalities
• CNS or SC malformation

Question 20

AMC type 1

Answer 20

• Jacknifed 55%
• flexed with dislocated hips, extended knees,clubfeet, internally rotated shoulder flexed elbows and flexed and ulnarly deviated wrists

Question 21

AMC type 2

Answer 21

• frog legs 45%
• the second pattern present with abducted, internally rotated hips , flexed knees, clubfeet, internally rotated shoulder, extended elbows flexed and ulnarly deviated writs

Question 22

DEvelopment, strength and mobility infancy Type 1

Answer 22

• stretching of hip flexors and prone positioning
• roll or scooting on bottom as primary means of floor mobility
• delays in ability to attain independent sitting
• typically able to stand when placed well before they can pull to stand
• usually begin to walk with assistance, and assistive device and LE orthotics around 18 months

Question 23

DEvel3opment strength and mobility Type 2

Answer 23

• more positioning options due to hip and knee flexion
• -may have difficulty with prone to elbow extension contrcatures making it difficult to prop
• slower in attaining rolling but faster in sitting and scooting compared to type 1
• depending on strength and amount of bracing required to stand, these children may be unable to independently transition from floor to stand

Question 24

standing for AMC

Answer 24

• standing is very important during years 1 and 2
• families encouraged to start standing ~6 months
• initiate in a standing frame
• floor to stand activities do not usually emerge until the child is securely ambulating

Question 25

stretching and splinting infancy

Answer 25

• stetching programs are imperative in addressing primary imairments of AMC
• -3-5 sets/ day with 3-5 reps each set, hold 20-30 sec
• thermodynamic splints adjusted every 4-6 wks
• AFOs for clubfeet mus have calcaneus aligned in neutral worn 22hr/day

Question 26

knee contractures addressed early by

Answer 26

using splinting and stretching
-first 3-4 months, splint worn up to 20 hr/day

extension contrcatures- anterior thermoplastic knee flexion splints

flexion contractures- posterior extension splints

older infants use splints for appropriate activities

Question 27

osteogenesis imperfect OI

Answer 27

-brittle bones disease, characterized by lax joints, weak muscles and diffuse osteoporosis

Question 28

classification of OI

Answer 28

• silence and danks classified 4 genetic types of OI

-has been expanded to 8 types
first 4 usually autosomal dominant

-defect in Type 1 collage. types 5-8 do not have type 1 collagen defect

Question 29

Type 1 OI

Answer 29

• mild to moderate bone fragility with a few to several fractures
• little or no bone malformation
• most frcatures occur before puberty
• normal birth height/weight
• muscle weakness
• joint laxity
• flat feet
• dislocations and sprains
• avg life expectancy
• associated with blue sclera, triangle face, and hearing loss beginning in 0s or 30s

Question 30

Type 2 OI

Answer 30

• perinatal lethal
  -extreme bone fragility and delay of ossification of skull and facial bones
  -long bones are crumbled
  -infants are small with short, curved , deformed extremities
  -

Question 31

Type 3 OI

Answer 31

• usually autosomal dominant, but rare recessive cases
• severe with progressive deformity of the long bones, skull and psine
• -results in short stature
• severe bone fragility
• several dozen to hundreds of fractures
• blue sclera at birth, lessens with age

Question 32

Type 4 OI

Answer 32

• mild to moderate deformity
• short stature after birth
• sclera normal
• dentionogenesis imperfect is common (teeth)
• hearing loss variable
• prohnosis for ambulation is excellent

Question 33

type 5 OI

Answer 33

• autosomal dominant
• hypertrophic calcification of fractures and surgical osteotomies
• may have calcification of interosseous membranes of radius and ulna leading to limitation of supination/pronation
• 5% of the moderate to severe cases of OI

Question 34

type 6 OI

Answer 34

• autosomal recessive and extremely rare

- moderate to severe deformity but with normal teetch and sclera

Question 35

type 7 OI

Answer 35

autosomal recessive and associated with gene defect that affects translation of collagen

• normal sclera and dentin
• moderate to severe bone fragility and shortening of humerus and femur

Question 36

type 8 OI

Answer 36

• autosomal recessive and caused by mutations of genes that affect translation of collagen with mutation in cartilage protein
• normal sclera , flattened long bones, slender ribs, small to normal head size
• growth deficiency for those that survive to teen years

Question 37

diagnosis of OI

Answer 37

• based on clinical manifestation and skin biopsy that looks at collagen
• the collagen defect determined what type of OI
• x-rays and bone scans show evidence of multiple old fractures and skeletal deformities

Question 38

medical management o fOI

Answer 38

no cure

• use of biosphonates
• whole body vibration
• internal fixation with intramedullary rods

Question 39

intervention positions for OI

Answer 39

sidelying- supported by towel rolls

prone- start with child on caregiver. adv to towel roll ow soft wedge

supine- provide support for arms. hips should be in neutral position with knees over a roll

position should not be changed frequently and should not restrict spontaneous movement

Question 40

intervention for infancy of OI

Answer 40

• often scoot on bottom befor ecrawling
• difficulty with transitions in and out of sitting due to short extremities
• pull to sit contraindicated
• pt should support at trunk/pelvis, never with hands on the legs
• never use baby walker or jumping seats