MSK - Ortho and Rheum Flashcards

1
Q

You are seeing an 18 month old baby with a femur fracture. The baby has not had any previous long bone fractures, but there is a history of rib fractures in the past. You are concerned about non-accidental trauma, but feel that you should rule our Osteogenesis Imperfecta.
a. Name 3 physical features that would support a diagnosis of OI.

A
Blue sclera
Early deafness
Easy bruising 
Joint laxity
Short stature (ranges from mild to severe depending on type of OI)
Bowing of long bones
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2
Q

Child presents with abdominal pain and arthritis. Picture of purpura on buttocks.
A) What is the diagnosis?
B) What is the MOST likely cause of long-term morbidity?

A

A) Henoch-Schonlein purpura

B) Renal disease and hypertension (needs to be monitored for 1 year after episode)

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3
Q

Child with monoarticular JRA involving one knee. List 3 long-term orthopedic complications of arthritis.

A
  1. leg length discrepancy
  2. flexion contractures
  3. osteoporosis (from glucocorticoid use)
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4
Q

A 13 year old girl comes to you with a complaint of hip pain for the last month and a slight limp. You do the following hip XR. What is the diagnosis (shows dislocation of femoral head):

a. SCFE
b. AVN
c. JIA

A

a. SCFE

Classic presentation is limp with externally rotated, adducted leg - external rotation increases as hip is flexed

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5
Q

What is true in this child. Hip x-ray shown = SCFE

a) sports-related trauma
b) more frequent in females
c) associated with steroid use
d) subsequent bone necrosis

A
d) subsequent bone necrosis (AVN); other complication is chondrolysis (acute dissolution of articular cartilage in hip)
● Classic: obese, African American boy btwn 11-16 y.o.
● RF/ Trends
o Black
o Obesity
o Male
o Left > Right
o Bilateral in 60% of cases
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6
Q

How do you classify a SCFE as stable vs unstable?

A

If they can walk on it, it is stable - but they should not walk on it because this can cause it to become unstable

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7
Q

28 week baby currently ventilated for severe RDS. Also has associated anomaly (picture of club foot). What to do:

  1. Refer to ortho immediately for serial plastic (did not say plaster) casting
  2. Refer to ortho once off ventilator
  3. Refer to ortho at discharge
  4. Refer to ortho at 3 months of age
A
  1. Refer to ortho at discharge
    - ideally casting should be started within the first month of life, but for prems in the NICU can defer treatment several weeks until foot has grown and infant has stabilized (do not start casting in NICU)
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8
Q

What are the components of a club foot and what is it’s medical name?

A

Talipes equinovarus
Cavus - plantar flexion of first metarsal
Adductus - of forefoot and midfoot
Varus - hindfoot
Equinus - hindfoot
CAVE is also the order it is corrected in

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9
Q

Child with achondroplasia. Which is true:

a. decreased life expectancy
b. despite small foramen magnum, they rarely get cord compression
c. borderline to mild developmental delay
d. spinal stenosis commonly occurs in childhood

A

b. despite small foramen magnum, they rarely get cord compression

  • intelligence is normal unless CNS complications develop (e.g. hydrocephalus)
  • with appropriate intervention and monitoring risk of cord compression at foramen magnum can be minimized
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10
Q

Babe with clubfoot - name ONE part of the treatment.

A

Weekly casting using Ponsetti technique
Most need tenotomy for equinus at end of casting
Then bracing (boots and bars)

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11
Q

Which feature is typical for achondroplasia?

a. proximal limb shortening
b. distal limb shortening
c. short mid-portion of the bone
d. non-specific shortening

A

a. proximal limb shortening aka rhizomelic shortening of the arms and leg

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12
Q

A 12 year old boy comes to you with a complaint of left knee pain and swelling for the past month. He has also had pain over the right heel. Which of the following would you expect to find on family history:

a. Psoriasis
b. Ankylosing spondylitis
c. Rheumatoid arthritis

A

b. Ankylosing spondylitis
- enthesitis (inflammation at tendon insertion point) common
- juvenile ankylosing spondylitis:
- boys > girls
- begins often with oligoarthritis and enthesitis
- arthritis commonly in lower extremities and often hips

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13
Q

In an adolescent with pain below the knee after running, the x-ray shows changes compatible with Osgoode-Schlater’s disease. All are done except :

a. Protective gear
b. Ice may be used to reduce swelling
a. Only stop activities which cause pain
c. Quadriceps strengthening exercises when pain resolves
d. Cast for 3-4 weeks

A

d. Cast for 3-4 weeks
- mgmt:
- rest and gradual reintroduction of activity (maintain activity level for 1-2 weeks before advancing)
- if more severe, can use knee immobilizer or crutches until pain resolves
- stretching quads and hamstrings while resting can help prevent recurrence once exercising again

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14
Q

Child with 6 wk history of severe ankle pain. Unable to weight bear. Unable to do physiotherapy. The foot is swollen, red, and warm. What is the most likely cause?

a) Reflex sympathetic dystrophy (now called CRPS)
b) Osteomyelitis
c) Munchaussen

A

b) Osteomyelitis (no hx of fever, systemic unwell? BUT WARM joint is not CRPS and most likely osteo > CRPS epidemiology wise)

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15
Q

14 year old boy presents with groin discomfort. X-ray shown shows femoral neck and femoral head not aligned in the middle. What is your diagnosis (1)? What are two steps in management ? What are two risk factors
for this disease (2)?

A
  1. SCFE
  2. admit and put on bed rest - no weight bearing
    - consult ortho (who will pin in the OR)
  3. RF: obesity, male, pubertal, endocrine disease (hypothyroid, hypopituitary, renal osteodystrophy)
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16
Q

What child with a SCFE would need further investigation for an underlying cause? What causes would you worry about?

A

Child under age 10 years - r/o endocrinopathy -hypothyroid, hypopituitarism, renal osteodystrophy)

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17
Q

What are risk factors for Legg-Calve-Perthes?

A

AKA AVN - trauma, family history, low birth weight

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18
Q

Description of a 4 yr old girl who refuses to weight bear. On exam, the only finding is there is tenderness over the proximal tibia (on both sides), What are
your two differential diagnoses.

A
  1. leukemia
  2. tibial stress fractures
  3. toddler’s fracture (9months - 3 years, and distal tibia)
  4. enthesitis/arthritis
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19
Q

Description of child with serum sickness day 10 of abx for URTI, joint pain, anemia, rash, hematuria. Management:

a) Pulse pred
b) IVIG
c) high dose NSAIDS
d) plasmapheresis

A

a) Pulse pred - appropriate for severe reaction

- otherwise usually supportive management with NSAIDs (regular dose)

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20
Q

Picture of SCFE, how to treat:

a. trial of NSAIDS
b. “nail the hip”
c. traction
d. casting
e. arthrocentesis

A

b. “nail the hip”

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21
Q

1 year old child presents with a metaphyseal femur fracture. What is the next step?

a) calcium, phosphate and alkaline phosphatase levels
b) full skeletal survey
c) bone scan
d) no further investigations

A

b) full skeletal survey
CML’s= classic metaphyseal lesions= chip or corner #; bucket handle (high specificity for abuse)
o Looks like chip off corner of long one
o Thought to happen when pulled or swung violently and relatively weaker
growing point of bones break
- skeletal survey should be done in any child under 2 years with suspicion for abuse or under 5 years with suspicious fracture

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22
Q

A 6-month-old child has not been moving her left leg. An x-ray demonstrates a fracture at the corner of her distal tibia (metaphyseal bucket-handle fracture). What
should be done:
a) calcium, phosphate, and alkaline phosphatase levels
b) screen for osteogenesis imperfecta
c) skeletal survey
d) bone scan
e) no further investigations

A

c) skeletal survey

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23
Q

A 3 yo child develops left hip pain following URTI. What is the best way to rule out osteomyelitis?

a) Lack of fever
b) Normal WBC
c) Negative blood culture
d) Bone scan

A

d) Bone scan
- WBC may be increased or normal
- ESR and CRP are usually elevated
- 50% of cases have positive blood culture
- sensitivity of bone scan 90%, MRI more specific

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24
Q

A 2 yo boy is brought in by mom not moving left arm. He’s holding it pronated. Mom admits she pulled on Left wrist during horseplay. Pt not cooperative with exam. Next test:

a) sling with xray
b) sling then orthopedics referral
c) orthopedics referral
d) skeletal survey
e) manipulate at elbow until arm function restored

A

e) manipulate at elbow until arm function restored

- rotate arm into supination while maintaining pressure over radial head until click is felt

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25
Q

Xray of a child/infant’s elbow…story goes that it is painful, but there is no swelling and the parents brought the child in (I think he was 10 months old?) no hx of trauma. No fever. What is your diagnosis? What could have caused
this?

A
Pulled elbow (subluxation of radial head with annular ligament slipping between radial head and distal humerus). Caused by longitudinal traction force applied at elbow (e.g. someone pulling on child's arm to stop them from falling)
- full flexion and extension but limited supination
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26
Q

Dad brings toddler to ED for shoulder pain, xray shows spiral fracture. List the next three things you do.

A

Assuming there is no clear mechanism of injury…

  1. skeletal survey
  2. contact child protective services
  3. immobilize fracture, consider advice from ortho if needed
  4. pain control
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27
Q

9 month old boy who has come in with a story of an accident. Nurse mentions he has been in her 5 times previously with other injuries. He is a well child

otherwise. . Picture of Xray of bones with bony chip off the distal humerus.
(1) What does the xray show
(2) What could have caused this

A
  1. classic metaphysical lesion of distal humerus
  2. physical abuse
    - accidental trauma
    - this type of fracture is caused by accumulation of multiple micro fractures across the metaphysics perpendicular to the axis of the long bone (from shaking/shearing forces)
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28
Q

What is the most common childhood wrist fracture

a. Colles fracture
b. Greenstick
c. Buckle fracture
d. Salter Harris

A

b. Greenstick
c. Buckle fracture - unclear which is correct
● 80% of forearm fractures involve the distal radius and ulna
● majority of forearm fractures are buckle or greenstick fractures
- buckle (or torus): compression fracture
- greenstick: bone bends, but only breaks on the convex side, not all the way through

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29
Q

You are seeing an 8 month old female with a femur fracture and are concerned about non-accidental trauma:
a. What are the next 3 investigations that you will do to support your diagnosis?

A
  1. skeletal survey
  2. liver enzymes (screen for abdominal trauma)
  3. 25-OH-vitamin D, serum calcium, phosphorus, alkaline phosphatase (may be elevated with healing fractures), parathyroid hormone
  4. U/A (for hematuria)
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30
Q

Young girl who has flat feet. Mum is concerned. When she stands the feet are flat and you note that they are valgus and that her forefoot is abducted. When she sits down with her feet in the air, her arches look normal. What is her diagnosis (1) ? What kind of intervention is needed for her (1)?

A
  1. Dx: hypermobile flexible pes planus
  2. generally no treatment needed - common in young children and they generally outgrow it
    - if pain, abnormal wearing down of shoes, or fatigue after walking can consider treatment with orthotics (will help symptoms but not correct pes planus)
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31
Q

Child presents to ER with fever, sore hip what do you do?

a. aspirate joint
b. Xray hip
c. order CBC

A

a. aspirate joint (concern for septic arthritis)
dx: blood culture
- aspiration of joint for gram stain and culture
- WBC, CRP and ESR generally elevated but non specific and normal tests do not rule out septic arthritis
- imaging - X-ray at least
- surgical emergency

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32
Q

14 year old being treated for osteomyelitis. Severe pain despite naproxen and tylenol. Best analgesic:

a. narcotic
b. tricyclic
c. higher dose of NSAID

A

a. narcotic

- follow pain ladder - if giving regular tylenol and NSAID and not achieving good control then escalate to narcotic

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33
Q

A 14-year-old girl with spina bifida at the T12 level presents with a one-day history of a swollen leg and foot with erythema over the anterior tibia. Temp 37.8. WBC 16. ESR 22. Most likely:**

a) fracture
b) cellulitis
c) osteomyelitis
d) deep venous thrombosis
e) erythema nodosum

A

b) cellulitis
- sounds most like cellulitis - may have decreased sensation so less aware of developing infection

  • other thought was DVT as may be immobilized from spina bifida
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34
Q

18 mo M high fever, irritable, not moving hip. Next management issue?

a. IV ABx and blood culture
b. Needle aspiration
c. X-ray

A

b. Needle aspiration

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35
Q

What is the position of the septic hip in a patient with septic arthritis?

a) Abducted and internally rotated
b) Abducted and externally rotated
c) Adducted and internally rotated
d) Adducted and externally rotated

A

b) Abducted and externally rotated

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36
Q

You are seeing a 19 month old boy who has a swollen right knee. A joint aspirate is positive on C&S for S. Aureus. He also has a history of a pneumococcal meningitis
at 9 months of age. You order a CBCd, blood C&S and CT scan of the knee, and start him on IV antibiotics.
a. What 4 further tests will you order?

A

Concern for immunodeficiency:

  • recurrent bacterial infections - think B cell, phagocytic and complement defects
    1. immunoglobulins (IgG, IgM, IgA, IgE)
    2. C3, C4, CH50
    3. vaccine titres for antibody responsiveness
    4. next line - lymphocyte phenotyping, antineutrophil antibody, sweat chloride, bone marrow biopsy
    5. MRI to rule out adjacent osteomyelitis
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37
Q

Child with hip pain for 3 days. Today fever to 39, significant increase in pain. He is sitting with leg flexed and ext rotated. What are the 2 most important diseases on your differential diagnosis. List 3 investigations you would do to differentiate.

A
  1. septic arthritis of hip
    - osteomyelitis
  2. MRI of hip
    - joint aspirate
    - U/S (for effusion and fluid collection)
38
Q

Child presents with 6 week history of pain after mild ankle injury – now won’t bear weight, hurt to touch, hot, no objective signs of arthritis. What is the diagnosis? Treatment?

A

Dx: osteomyelitis
Tx: IV antibiotics - depends on age but cefazolin a general good choice
- tx duration 3-4 weeks - depends on clinical response

39
Q

5 year old fever 38.5 degrees, had a recent URTI. Hip was externally rotated and child not weight bearing. ESR was 40. Diagnosis?

a) Transient synovitis
b) JIA
c) Septic arthritis

A
c) Septic arthritis
Kocher Criteria For septic arthritis
● T >38.5
● WBC >12
● ESR >40
● Inability to weight bear
● CRP >23
40
Q

A 2 y/o child with in-toeing. Father reports having had the same problem as a child for which he was fitted with immobilization boots. On exam, child has a searching toe, metatarsus adductus, and internal tibial torsion. What to do:

  1. Observe
  2. Discourage W sitting
  3. Cast
  4. Refer to ortho
A
  1. Observe
    *note: internal tibial torsion doesn’t need anything done
    but the metatarsus adductus should at least be treated with stretching (though most resolve spontaneously), and potentially casting depending on if it’s flexible or not. W sitting is related to internal femoral torsion, but you could still discourage it to prevent that from happening later
    - internal tib torsion: starts to correct as child begins walking - may correct as early as 4 years or up to
    10 years
41
Q

Parents bring 3 year old for intoeing gait. You find tibial torsion and flexible metatarsus adductus. His father had a similar condition. Neurologic examination is

normal. You advise:
a. stop W sitting
b. ortho referral for in shoe prosthesis
c. no treatment necessary as this invariably resolves
d. Dennis splint
e. x-ray of lower limbs

A

c. no treatment necessary as this invariably resolves

Make sure metatarsus adductus is flexible and recommend stretching exercises

42
Q

2-year-old child with flexible flat feet. Father and aunt had the same abnormality. You suggest:

a) no intervention is necessary
b) must wear shoes all the time
c) needs orthotics
d) foot exercises
e) orthopedics referral

A

a) no intervention is necessary

43
Q

An 18 month old infant of African immigrant parents presents to you because he is not cruising or walking. He was exclusively breastfed until the age of 6 months, and now eats mainly purees, fruits and vegetables. On examination there is prominence at his distal radius and ulna and medially at his proximal tibia. Which of the
following lab abnormalities will support your diagnosis:
a. Low PTH
b. High ionized Ca
c. Low 25-OH vit D
d. Low ALP

A

c. Low 25-OH vit D
Expecting Low Ca and Vitamin D in diet
● PTH high
● High Alk Phos (compensation)

Rickets

44
Q

An 8 year old boy with an unremarkable past medical history comes to you because his mother notices that he walks on his toes.
a. Name 4 causes of bilateral toe walking.

A
  • should walk heel-toe by age 4 - refer to ortho if still toe walking by 4
  • Causes
    o Idiopathic (no abnormalities on exam, just when walking), +/- Heel cord contracture
    o Cerebral Palsy
    o Duschenne Muscular Dystrophy
    o Tethered spinal cord
    o Autism spectrum disorder
45
Q

Best determinant of scoliosis on Physical Exam.

  1. leg length discrepancy
  2. asymmetric shoulder height
  3. asymmetric ribcage on bending over
  4. asymmetry on lateral flexion
A
  1. asymmetric ribcage on bending over
46
Q

14 yo girl with idiopathic scoliosis. Give 3 indications for referral to ortho.

A

Ix: standing PA and lateral of entire spine and calculate Cobb angle
Referral:
- progression of curve by 5-10 degrees
- curve >20 degrees (prepubertal) or >30 degrees (pubertal)
- left curve
- pain
- abnormal PFTs

47
Q

14 yr old who hasn’t been to see you in 2 yrs. She has scoliosis with Cobb angle of 50 degrees. What is the most appropriate management?

a. observe
b. refer for brace/ orthosis
c. refer for posterior spinal fusion
d. electrical muscle stimulation

A

c. refer for posterior spinal fusion

- likely postmenarchal and has high degree curve - bracing unlikely to be effective

48
Q

Teen post op scoliosis repair. Day 6. Billious vomiting. Weight loss. ABdo pain. What is most likely complication?

a) Superior mesenteric artery syndrome
b) Adhesions
c) Volvulus

A

a) Superior mesenteric artery syndrome

49
Q

3 yo girl generally well but has had 3 weeks of right knee swelling. Best initial treatment?

a. Steroids
b. Methotrexate
c. NSAIDS

A

c. NSAIDS
- tx for transient synovitis: NSAIDs and limitation of activity - can take up to 2 weeks for resolution of symptoms
- ddx to consider:
- septic arthritis
- reactive arthritis (following enteropathic or urogenital infection)
- post-infectious arthritis (following some other infection like viral URTI)
- acute rheumatic fever
- lyme arthritis
- JIA

50
Q

3 y/o with mono arthritis for a few weeks. What is the most likely test result:

  1. Positive RF
  2. Positive ANA
  3. Increased WBC
  4. Increased ESR
  5. Decreased Hgb
A
  1. Increased ESR

- most likely transient synovitis which can have mild increased in ESR and CRP

51
Q

12 yo girl with arthritis, some blood in the urine. Three things on differential?

A

SLE
HSP
Post strep GN
Systemic vasculitis

52
Q

Child recovered from recent reactive arthritis. Has residual 30 degree painless flexion contracture of knee. Name 3 non-pharmacologic treatment modalities.

A
  • stretching/physio
  • bracing
  • surgery
  • botox
53
Q

Child with monoarthritis. Name 3 medications from different classes of medications that are used for treatment.

A
  1. NSAIDs: ibuprofen
  2. Corticosteroids: prednisone
  3. DMARDs: methotrexate
  4. Biologics: infliximab
  5. Immunosuppressants: cyclophosphamide
54
Q

Boy had recent illness - fever, diarrhea, conjunctivitis, urethritis now with sacroilitis. You diagnose him with Reiter syndrome. List 4 organisms that could cause Reiter syndrome.

A

Reiter syndrome: reactive arthritis with uveitis and urethritis - following GI or GU infection
- “can’t see, can’t pee, can’t climb a tree”
- salmonella
- shigella
- yersinia
- campylobacter
- chlamydia
Post infectious arthritis - not GU/GI specific
- strep, Hep B, rubella, parvo B19, varicella, CMV

55
Q

Which of the following is the best confirmatory test for juvenile dermatomyositis?

a. EMG
b. CK
c. ANA

A

a. EMG

- key features are proximal muscle weakness and heliotrope rash and Gottron’s papules

56
Q

What are the diagnostic criteria for juvenile dermatomyositis?

A

heliotrope rash/Gottron’s papules + 3 of:

  • symmetric proximal muscle weakness
  • elevated of 1+ muscle enzymes (CK, AST, LDH)
  • EMG changes
  • muscle biopsy showing inflammation (only indicated if diagnosis is uncertain or to grade severity)
57
Q

12 year female with a 2 week history of fever, polyarthralgia. Looks well, but pale. T=37.6. No joint swelling. Trace protein and blood in urine. Normal creatinine. Most likely diagnosis is:

a. systemic JIA
b. SLE
c. rheumatic fever
d. Goodpasture’s syndrome

A

b. SLE
- most common complaints in kids are fever, fatigue, hematologic abnormalities, arthralgias and arthritis (symmetric polyarthritis)
- renal involvement usually asymptomatic so need to look for it (HTN, proteinuria, hematuria, edema, nephrotic syndrome, renal failure)

Note: *arthritis must be present to diagnose ANY type of JIA
- Goodpasture: pulmonary hemorrhage and glomerulonephritis

58
Q

What is the diagnostic criteria for JIA? How do you classify it?

A
  • arthritis (dx of arthritis = swelling OR 2 of decreased ROM, pain with motion, warmth)
  • duration 6 weeks minimum
  • classify based poly (5+ joints involved in first 6 months after onset), oligo (1-4 joints), or systemic onset (arthritis with fever and rash)
59
Q

10-year-old girl with a 1 week history of fever (not greater than 38.5) and migratory arthritis. On examination there is evidence of arthritis in the right wrist, but the rest of the exam is normal. WBC is 25 with a left shift. ESR 45. Most likely diagnosis:

a) acute rheumatic fever
b) juvenile rheumatoid arthritis
c) systemic lupus

A

a) acute rheumatic fever

Jones criteria - 2 major OR 1 major and 2 minor
Minor: elevated ESR, CRP, temp 38.5, arthralgia

60
Q

Child presents with fever, polyarthritis, increased ESR and ASOT=625. Echo is normal. Best management:

A

Mgmt of acute rheumatic fever without heart disease:

  1. ASA for fixed period (dosing progressively lowered over 6 weeks, then stopped)
  2. Pen V for 10 days to eradicate strep infection
  3. Strep prophylaxis: Pen G benzathine IM q4 weeks, or BID Pen V or daily sulfasalazine until age 21
61
Q

Child with a 2 week history of migratory arthritis, fever 38.5, a normal echo, WBC 18, ESR 75, throat swab negative, and an ASOT > 625. What should you do:

a. ASA 100mg/kg until arthritis resolves
b. ASA prn pain, treat 10 days with penicillin
c. ASA 100mg/kg until arthritis resolves, treat 10 days with penicillin
d. ASA 100 mg/kg until arthritis resolves, treat 10 days with penicillin followed by q monthly IM penicillin
e. ASA 100mg/kg until arthritis resolves, treat 10 days with penicillin, oral penicillin for dental procedures

A

d. ASA 100 mg/kg until arthritis resolves, treat 10 days with penicillin followed by q monthly IM penicillin

62
Q

8y female with subtle choreoathetotic movements. Handwriting has worsened and she is emotionally labile . Evaluations including a throat culture are negative. What is the most likely diagnosis?

a. Huntington’s chorea
b. Syndenham’s chorea
c. SLE

A

b. Syndenham’s chorea
- chorea, emotional lability and hypotonia/incoordination and pronation of the hand causing difficulties writing are all classic features
- culture and ASOT often negative as can occur months after strep infection
- treat with long term antibiotic prophylaxis
- chorea resolves in 6-9 months

63
Q

Rheumatic fever – which most likely causes long-lasting problems?

a) carditis
b) chorea
c) nodules
d) Polyarthritis

A

a) carditis

- cardiac is long-term sequelae of ARF

64
Q

Child has a history of recurrent fevers, evanescent pink rash, lymphadenopathy, hepatomegaly. His musculoskeletal exam is normal. What’s the diagnosis?

a) Leukemia
b) Scarlet fever
c) Lyme disease
d) Systemic JIA

A

d) Systemic JIA
- fevers once-twice daily for at least 2 weeks, and unwell during this time

Dx: arthritis in min. 1 joint x2 wks with, or preceded by, fever documented daily (“quotidian”) x 2 weeks and accompanied by min 1 of
o Evanescent (nonfixed) erythematous rash (macular, non pruritic, transient +/- crops over trunk + limbs)
o Generalized lymph node enlargement
o Hepatomegaly or splenomegaly or both
o Serositis

65
Q

What are some prognostic factors for systemic onset JIA?

A

Systemic JIA worse prognosis in general

  • worse prognosis if:
  • polyarticular
  • fever >3 months
  • increased inflammatory markers on BW x>6 months
66
Q

Features of Kawasaki disease include all EXCEPT:

a) elevated WBC (yes)
b) ANA negative (yes)
c) platelet count 75
d) urine with 10-15 WBC/hpf (yes)

A
c) platelet count 75
● Leukocytosis, Anemia, Thrombocytosis after week 1
● Elevated CRP and ESR
● Hypoalbuminemia
● Hyponatremia
● Abnormal plasma lipids
● Sterile pyuria
● Elevated serum transaminases and GGT
● Pleocytosis of CSF
● Leukocytosis in synovial fluid
67
Q
Kawasaki dz, parents refuse IVIG. What is an acceptable
alternative?
a) ASA
b) ASA + steroids
c) ASA + cyclophosmide
d) steroids alone
A

b) ASA + steroids
- ASA always anyway
- steroids second line

68
Q

3 year old with a history of fevers. Occur every 4 to 12 weeks for 1-4 days. Growing well. Treated for numerous otitis and pharyngitis.
A) multiple viral infections
B) familial med fever
C) CVID

A

A) multiple viral infections

69
Q

List 5 warning signs of primary immunodeficiency

A
  1. 4 new ear infections in a year
  2. 2 serious sinus infections in a year
  3. 2 pneumonias within a year
  4. 2 deep seeded infections in life
  5. 2 months on antibiotics with little effect
  6. needing IV abx to clear infection
  7. recurrent abscesses
  8. persistent thrush or fungal infection
  9. FTT
  10. family hx of primary immunodeficiency
70
Q
A 12-year old female presents with a two-week history of fevers, arthralgias, and myalgias. She is pale but otherwise looks well. She has blood and protein in her urine.
WBC 18. Platelets normal.
a) acute rheumatic fever
b) Wegener’s granulomatosis
c) systemic lupus erythematosus
d) juvenile rheumatoid arthritis
A

c) systemic lupus erythematosus
- arthritis, anemia, arthralgia, renal disease in a 12 yo female - fits with SLE
- classic presentation SLE: fever, rash, renal, MSK, heme (anemia, leukopenia, TCP)
- Wegener’s: granulomatosis with polyangiitis: respiratory symptoms (SOB, cough), fatigue, malaise, fever and renal involvement so is possible BUT less common

71
Q

What is the diagnostic criteria for SLE?

A

4/11 of SOAP BRAIN MD - at least 1 clinical and 1 immunologic

  • serositis (pleuritis, pericarditis)
  • oral or nasal ulcers
  • arthritis (oligo or poly)
  • photosensitivity
  • blood cells (all lines low)
  • renal (nephrotic)
  • ANA+
  • immune markers + (anti-Smith, anti-dsDNA, anti-phospholipid)
  • neuropsych (seizures, psychosis)
  • malar rash
  • discoid rash
72
Q

(Picture of a child with an erythematous rash over the face, concentrated over the upper eyelids, and over the MCPs and PIPs) A 6 year old boy comes to you with a history of progressive fatigue over the past month. You note the following rash on physical examination.

a. What is your diagnosis?
b. Name 3 tests that will help to confirm your diagnosis.
c. What one medication would you consider starting at this point?

A

a. juvenile dermatomyositis
b. muscle enzymes (CK, AST, LDH)
- EMG
- muscle biopsy
- proximal muscle weakness - positive Gower’s
c. steroids (mainstay of treatment)

73
Q

Boy with Kawasaki’s disease. Give 5 criteria for Kawasaki’s

A

5 days of fever

  • cervical LAD >1.5cm
  • bilateral non-purulent conjunctivitis
  • swelling/redness/peeling of hands and feet
  • rash (diffuse, maculopapular)
  • oral membrane involvement (cracked, red lips, strawberry tongue, pharyngitis)
74
Q

14 year old boy with low back pain. You suspect ankylosing spondylitis. What are 2 findings on physical exam? What are two tests to help you in this diagnosis? How is this treated?

A
  1. SI tenderness on FABER test
    - Schober test (15cm span marked starting at level of iliac crest while standing) - distance measured after forward bend - positive if distance increases by <5cm
  2. ESR and RF normal
    - HLA B27 positive
    - XR: late findings identified like squaring of vertebral bodies
    - MRI: bone marrow deem adjacent to joint
  3. NSAIDs, intra-articular steroids, DMARDs, physio
75
Q

Neonate with thrombocytopenia, elevated liver enzymes, target lesion rash. What is the diagnosis?

A

Neonatal lupus

76
Q

Kid with JIA, high fever, hypotensive, high ferritin and high trigs, pancytopenic. what is the ddx:

A

macrophage activation syndrome
- occurs most often in first weeks to months after onset of JIA symptoms, though can happen any time in course of disease
Diagnostic criteria - 3/5 of:
- fever >38.5
- splenomegaly
- cytopenia (at least 2/3 cell lines)
- increased triglycerides OR low fibrinogen
- elevated ferritin (often >1000, must be >500)

77
Q

(Picture of a kid with a malar rash). Has fever, joint pain, diffuse rash. Only meds are fluticasone and phenytoin for a seizure disorder. What is the most likely?

a. drug hypersensitivity reaction
b. Juvenile dermatomyositis
c. Systemic lupus erythematosus
d. Juvenile idiopathic arthritis

A

This could be drug induced lupus from phenytoin

  • drug induced lupus can be true SLE in a predisposed individual that is triggered by drug (this will not resolve)
  • or it can be a lupus-like syndrome that is caused by the drug and resolves after the drug is discontinued (though can take months to years for full recovery)
78
Q

Rash over backs of hands, muscle pain, being teased at school for weakness.

a. DMD
b. JDM
c. SLE

A

b. JDM

79
Q

Evanescent rash, lymphadenopathy, hepatomegaly. What does this suggest?

a. lupus
b. systemic onset JIA
c. kawasaki
d. rheumatic fever

A

b. systemic onset JIA

80
Q

A 7 year old boy has a history of recurrent fevers, evanescent pink rash, lymphadenopathy, and hepatomegaly. His MSK exam is normal. What’s the diagnosis?

a) Leukemia
b) Scarlet fever
c) Lyme disease
d) Systemic JIA

A

d) Systemic JIA

* fits best with JIA, but very abnormal that the MSK exam is normal

81
Q

4 yr old boy with fever of 40 degrees despite amoxcil. Maculopapular rash, bilateral conjunctivitis,dry cracked lips. What is the most likely lab finding?

a. hypoalbuminemia
b. neutropenia
c. EBV monospot +
d. + ASOT

A

a. hypoalbuminemia

82
Q

Kid with >=6 day of fever, non purulent conjuctiviits, MP rash. Clinical picture c/w Kawaski’s dx. Most likely lab abnormality?

a. Low albumin
b. Neutropenia
c. Monospot +
d. Positive ASOT

A

a. Low albumin

83
Q

Child with pulmonary findings, eosinophilia, slightly elevated calcium (2.8)

a. miliary TB
b. sarcoidosis
c. cryptococcus
d. blastomycosis

A

b. sarcoidosis
- multisystem granulomatous disease of unknown etiology
- older kids are like adults (mild pulmonary involvement - dry persistent cough, LAD)
- early onset sarcoid (<4 years): rash, uveitis and arthritis
- also nonspecific sx like fever, wt loss, malaise
- CXR: bilateral hilar adenopathy
- small percentage have hypercalcemia/hypercalciuria
- leukopenia, TCP and eosinophilia can be seen

84
Q

14 yr old with fevers, polyarthralgia, wt loss. Appears pale. No arthritis on exam. Urine + for protein and blood. Decreased WBC and lymphs. Normal platelets. What is
the most likely diagnosis.
a. SLE
b. Systemic JIA

A

a. SLE

85
Q

Child with wt loss anemia, normal plts, low WBC, protein/blood in urine. Dx?

a. SLE
b. RF
c. PSGN

A

a. SLE
● most common presenting complaints of children with SLE include fever, fatigue, hematologic abnormalities, arthralgia, and arthritis → arthritis does not have to be
present in a dx of SLE but definitely needs to be present in all forms of JIA

86
Q

4 year girl with arthritis of two months duration. On exam she has a swollen elbow and knee. Her ANA is positive. What is her diagnosis?. What is one complication that she is at risk for. What would your initial treatment be (1) ?

A
  1. oligoarticular JIA
  2. complication: chronic uveitis (girls with oligoarthritis who are ANA positive are highest risk of this) - can lead to cataracts and blindness
  3. NSAIDs
87
Q

Three ways that maternal SLE can affect neonate.

A
  • heart block
  • thrombocytopenia
  • rash
  • hepatitis
88
Q

14 yo girl with leg pain, which is worse after activity and at night. On examination there is tenderness and swelling above the knee joint but no effusion. A radiograph shows a lesion in the distal femur with periosteal elevation. What is the likely cause?

a. osteoid osteoma
b. osteogenic sarcoma
c. subperiosteal hematoma
d. osteomyelitis

A

b. osteogenic sarcoma
- the description of periosteal elevation (aka onion skinning) is more typical for Ewing sarcoma, but these tumours are more common in kids under age 10
- osteomyelitis also possible, but pain worse at night suspicious for cancer

89
Q

Pt with HSP. What would you do to monitor:

a. urinalysis
b. IgA
c. Stool for o/b
d. AXR

A

a. urinalysis
Renal: weekly UA + BP while active clinical disease then monthly x 6 month (to R/O late onset renal disease)
o If all (-) in this FU period then unlikely to develop nephritis after 6 months
o If (+), HTN, renal failure, hematuria -> talk to nephro

90
Q

Child with a head that looked like this - picture of infant with dolichocephaly. What is the diagnosis? What finding on physical examination would support this?

A
  1. craniosynostosis

2. closure of sagittal suture with no mobility, prominent boney ridge

91
Q
Teen (gymnast) insidious onset lower back pain. Pain on extension, hamstring tightness. Paraspinal muscle spasm. Normal neuro exam. Positive TC99 scan. What is
the dx.
a) Spondyloarthropathy
b) Spondylolysis
c) Posterior element overuse
d) Vertebral avulsion fracture
A

b) Spondylolysis
spondylolysis
● anatomic defect (radiolucent gap) in pars interarticularis with adjacent bone sclerosis - develop over time, not present at birth
● mechanism → usually activity related and occurs from repetitive hyperextension - prevalence as high as 47% in certain athletes (gymnasts, weightlifters, football
linemen)
- can be seen on bone scan

92
Q

Emotional lability and choreiform movements

a. Tourettes
b. SLE
c. Huntingtons
d. Sydenhams

A

d. Sydenhams