musculoskeletal disorders Flashcards
Juvenile idiopathic arthritis
- JIA is not a single disease, as the rterm includes all forms of childhood arthritis
three systems used to diagnose and classify childhood arthritis
- American college of Rheumatology criteria for JIA
- European League against rheumatism criteria for JIA
- international league of associations for rheumatology criteria for JIA
diagnosis of JIA
- 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
Systemic JIA
- 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
polyarticular JIA
- 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
Oligoarticular JIA
- 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
Primary clinical manifestation
- joint swelling, pain, and stiffness
-morning stiffness - muscle atrophy, weakness poor muscle endurance
- ## systemic manifestation
secondary clinical manifestation
- limited joint motion; soft tissue contracture
- fatigue
- decreased aerobic capacity
- growth abnormalities
- osteopenia; osteoporosis
- difficulties with ADL
- participation restriction
medical management
- NSAIDS
- 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
medical management
- methotrexate
- most common disease modifying antirheumatic drug
- prescribed to children with poly and systemic JIA
- weekly oral administration
goal of pharmacologic therapy
- control arthritis; preventing joint erosion
- manage extra articular manifestation
PT intervention in acute phase
- maintaining and preserving joint function
- RICE- avoid heat, US and diathermy
- splinting
PT intervention in subacute and chronic
- restoration and compensation of cuntion and activities
- balance rest and exercise
aerobic fitness prescription
- 2x/week for 45-60 mins of moderate to vigorous intensity
resistance training prescription
- 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
athrogryposis multiplex congenital defined
- 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
Forms of AMC
- amyoplasia 43% lack of muscle formation or development
- contrcatures syndrome 35%
- distal asrthogryposis 7%
AMC etiology
- lack of fetal movement
- associated with neurogenic and myopathic diorders
- decr amniotic fluid, especially in 3rd trimester, may inhibit freedom of movement in utero
factor implicated in AMC are
- 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
AMC type 1
- Jacknifed 55%
- flexed with dislocated hips, extended knees,clubfeet, internally rotated shoulder flexed elbows and flexed and ulnarly deviated wrists
AMC type 2
- 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
DEvelopment, strength and mobility infancy Type 1
- 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
DEvel3opment strength and mobility Type 2
- 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
standing for AMC
- 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
stretching and splinting infancy
- 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
knee contractures addressed early by
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
osteogenesis imperfect OI
-brittle bones disease, characterized by lax joints, weak muscles and diffuse osteoporosis
classification of OI
- 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
Type 1 OI
- 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
Type 2 OI
- 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
-
Type 3 OI
- 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
Type 4 OI
- mild to moderate deformity
- short stature after birth
- sclera normal
- dentionogenesis imperfect is common (teeth)
- hearing loss variable
- prohnosis for ambulation is excellent
type 5 OI
- 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
type 6 OI
- autosomal recessive and extremely rare
- moderate to severe deformity but with normal teetch and sclera
type 7 OI
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
type 8 OI
- 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
diagnosis of OI
- 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
medical management o fOI
no cure
- use of biosphonates
- whole body vibration
- internal fixation with intramedullary rods
intervention positions for OI
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
intervention for infancy of OI
- 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
