wk 6- arthritis, connective tissue disease

Question 1

diagnosing criteria for juvenile rhematoid arthritis

Answer 1

criteria of 4 things:

1. Chronic synovial inflammation of unknown origin
2. Onset in children less than 16 years of age
3. Objective evidence of arthritis in one or more joints for 6 consecutive weeks
4. Exclusion of other diseases

Question 2

3 types of JRA

Answer 2

1. Pauciarticular or monarticular JRA (~40%)
2. Polyarticular JRA(~20%)
3. Systemic JRA (Still’s disease) (~20%)

Question 3

pauciarticular/ monoarticular JRA

Answer 3

• 4 or fewer joint affected Asymmetrical or symmetrical
• Early or late onset discrete joints affected

Question 4

polyarticular JRA

Answer 4

• 5 or more joint involved, typically small joints of hands and feet
• Seronegative (early onset) or seropositive (late onset)

mean onset is around 10 years
more common in girls

Question 5

SYSTEMIC jra

Answer 5

• Often symmetrical onset usually <5 yrs onset with fever & precipitated by
  infection

Question 6

Is there a specific test for JRA, What diagnostic tests could you use in this condition that arent specific

Answer 6

No
rheumatoid factor and antinuclear antigen are screening tests but they can be raised in healthy children with infection or other pathology

Full blood count - anaemia, elevated WBC and platelet count (inflammation)
elevated ESR and CRP

Question 7

what diagnositic test is useful for enthesitis arhritis

Answer 7

human leukocyte antigen (HLA B27)

Question 8

Does JRA show up on x ray imaging

Answer 8

arthritis shows up late in disease on x ray

Question 9

what imaging is gold standard for JRA

Answer 9

MRI
early cartilage and soft tissue changes and synovitis

Question 10

typical foot malformations with JRA

Answer 10

1. pes valgoplanus
2. pes cavus
3. pseudocavus

Question 11

what health profession helps with JRA

Answer 11

peads rehmatologist

Question 12

TREATMENT for JRA

Answer 12

symptom relief, maintaining function

1. Aspirin/NSAIDs
2. intravenous and intra articular corticosteroids
3. methotrexate
4. biologic if MTX doesnt work
   and
5. physical therapy for muscle strength and joint ROM
   6.hydrotherapy under paeds physiotherapy - water tempt for inflammatory relief and exercise medium
6. surgery in chronic cases

Question 13

prognosis of JRA

Answer 13

up to 60% of cases resolve prior to adulthood

Question 14

role of podiatrist for treatment of JRA

Answer 14

-manage ROM
-prevent joint alignment issues
-footwear advice/modification
-padding/strapping
-orthoses

Question 15

what impression do you take of accomodative othroses’

Answer 15

Foam impression box (SWB/WB/as it lies)- no STJ neurtral

capturing in a compensated foot

Question 16

what impression do you take of functional orthoses

Answer 16

suspension cast- holding foot in position that will influence the outcome of orthosis

Question 17

what joints is septic arthritis common in

Answer 17

large joints (hip, knee, elbow, shoulder)

Question 18

clinical features of septic arthritis in infant

Answer 18

fever, irritable, sepsis abnormal posture, joint pain, psudeoparalysis

Question 19

clinical features of septic arthritis in child

Answer 19

fever, severe pain, muscle spasms

Question 20

how can septic arthritis happen

Answer 20

haemostasis spread- bacteria carried by bloodstream from infectious area and introduced into joint

contigunous spread- infection overlaying the joint (osteomyleitis or soft tissue infection)

direct implantation- injected into joint or penetrating through trauma

Question 21

what investigations could you use to diagnose septic arthritis

Answer 21

1. CRP/ESR elevated levels
2. radiography/US for effusion levels
3. joint aspiration

WBC is unreliable

Question 22

septic arthritis treatment

Answer 22

hospital visit
-empirical IV antibiotics
-drainage/ clean out of joint
-immobilisation post drainage
-culture for infection
-directed IV antibiotics
-oral antibiotics for 3 weeks

Question 23

what is the most common cause of limp with hip pain in kids under 10

Answer 23

transient synovitis of hip (inflammatory arthirtis of hip)

Question 24

clinical features of transient synovitis of hip

Answer 24

pain in hip, anteromedial thigh and knee,
reduced range of motion,
limp,
unilateral pain,
psuedo paralysis (kids dont want to move joint)
more common in boys under 10

Question 25

difference in ROM, fever, ESR and synovial fluid in transient synovitis and sepctic arthritis

Answer 25

TS: ROM- guarded hip rotation fever- low grade ESR- <15 synovial fluid- clear septic: ROM- pronounced, spasm, guarding, fixed position Fever- high ESR->20 synovial fluid- WBC/bacteria

Question 26

connective tissue do what, made up of what

Answer 26

hold structures of body together, elastin/collagen collagen found in- tnedons, ligaments, skin, cartilage, bone and blood elastin- ligaments and skin

Question 27

until what age are kids expected to be hypermobile (general hypermobility)

Answer 27

3 females, 4 males

Question 28

beighton score for hypermobility

Answer 28

5/9

Question 29

what is hypermobility spectrum disorder

Answer 29

hypermobility that becomes chronically painful and assciated with impaired function (strains, sublax, dislocations)

Question 30

clinical features of hypermobility spectrum disorder

Answer 30

joint/muscle pain fatigue rolling ankles common

Question 31

what is ehlers danlos syndrome

Answer 31

group of genetic connective tissue disorders can be autosomal dominant or recessive inherited it is decrease in tensile strength and integrity of skin, joints andother connective tissues

Question 32

most common type of Ehlers danlos syndrome

Answer 32

hypermobile type 5- only subtype with an unknown genetic basis/protein pathway

Question 33

clinical features of classic/classic like EDS (type 1/2)

Answer 33

skin hypersensitivity wide, atrophic scars spheroid formation joint hypermobility delay in milestones hernias due to organ shift -skin/joints affected

Question 34

clinical features of vascular EDS (type 4)

Answer 34

most severe form mortality reduced to 50years acrogeria thin, pale, transluscent skin fragile blood vessels/ruptures high risk during vagina delivery extensive bruising facial features charaacteristic

Question 35

clinical fetures of hypermobile EDS

Answer 35

type 5, most common skin hyperextensibility dislocations, sublaxations, hyperextension, pes planus muscle atrophy/weakness brusing smooth skin early onset osteopenia/OA

Question 36

tests for EDS

Answer 36

skin elasticity thumb to wrist bruising pectus exavatum - h-EDS

Question 37

types of EDS

Answer 37

1. hypermobile EDS (type 5) 2. classic EDS (type 1 3. classic like EDS (type 2) 4. vasuclar EDS (type 4)

Question 38

management of EDS

Answer 38

activity moderation sport technique footwear mods/devices for stability and offloading strapping/padding

Question 39

marfans syndrome gene and what does it cause

Answer 39

autosomal dominant highest cause of morbitity/mortality from aneurysmal dilation, aortic regurgitation and dissection

Question 40

clinical features of marfans

Answer 40

-excess linear growth of long bones (long thumb for example, wrapping hand around wrist) -hypermobility -pectus defomrity -scoliosis/kyphosis -facial features (sunken eyes, head width/length ratio, under developed cheekbones) -ocular abnormalities- colour of eyes -skin straie -henrias

Question 41

osteogenesis imperfecta clinical features

Answer 41

brittle bone disease -fragile bones/osteopenia -regular factures -small stature -blue sclerae -hearing loss -brittle teeth -muscle weakness -hypermobility

Question 42

what is epidermis bullosa

Answer 42

group of rare genetic diseases of skin causing easy bruising/wounds elastin/collagen deficiences

Question 43

marfans testing

Answer 43

thumb sign, pertrudes past ulnar border wrist wrap with thumb and 5th finger ectopia lentis

Question 44

brighton criteria what do you need

Answer 44

dignoses hypermobility specturm disorders need one of the following: -2 major criterias -1 major and 2 minor -4 minor -2 minor (first degree relative)

Question 45

brighton crtieria

Answer 45

major: -beightons 5/9 or more -arthralgia 5 or more joints for 3 months or more minor: -beightons 1, 2, 3 -back pain or arthralgia longer than 3 months -sublaxation -soft tissue rheumatism -abnormal skin (striae, atrophic healing, excessive extensibility) -ocular signs -marfanoid appearance -exclusion if known marfans or EDS

Question 46

beightons score used to measure

Answer 46

generalised hypermobility

Question 47

LLAS used for

Answer 47

hypermobility of lower limb

Question 48

pGALS used for

Answer 48

detecting abnormal joints

Question 49

PGALS indicated in

Answer 49

-unwell child with fever -limping child -delayed/regression milestones -clumsy child