Rheumatology & Ortho q-bank Flashcards
What is the most common primary malignancy of bones in kids? #2?
Osteosarcoma
Ewing’s sarcoma
M:F 1.5:1
How does osteosarcoma present and where?
Bone pain, limp, swelling. Rarely B-symptoms.
Located at epiphyses / metaphyses of maximum growth sites (distal femur, proximal tibia, humerus)
How to work up bone tumour?
X-ray CBC, chem (usually normal) LDH and AlkP (elevated) MRI mets work-up: CT chest + bone scan Ewing's requires BM bx x2
Osteosarcoma management?
Local resection VERY important + chemotherapy (doxo, MTX, ofosfamide, cisplatinum)
NO radiation.
5 year survival 75%
negative prognostic factors: pelvic tumours, widespread lung mets, bone mets (<20% survival)
Ewing’s presentation?
Systemic symptoms.
Pain, swelling, decreased ROM.
Femur and pelvis most common sites, equal distribution among extremities and central axis.
*primarily white people
Ewing’s treatment?
Radiation therapy
Chemo
Resection
Chemo
75% cure in small, nonmets, local tumours
<30% with mets to bone or bone marrow at dx
Syndromes / conditions predisposing to osteosarcoma?
Retinoblastoma
Li-Fraumeni
Paget disease
Radiotherapy
Presents with gradually increasing bone pain at femur or tibia, worse at night and relieved by aspirin/NSAIDs.
Osteoid osteoma.
25% not on xray, CT required
Treat by resection, some spontaneously resolve.
List 7 causes of intoeing gait
1) metatarsus adductus (types: flexible, bone, heel-cord)
2) talipes equinovarus (serial casting)
3) internal tibial torsion (reassure)
4) femoral anteversion (most common, clinical dx, no W sit)
5) spasticity
6) paralysis
7) maldirected acetabulum
Progression of treatment for JIA?
- NSAIDs (4-6wks)
- intra-articular steroid injection
- Methotrexate
- biologic DMARD ie: TNF-alpa antagonist (etanercept, adalimumab/humera, infliximab/remicaide)
- systemic steroids for severe systemic illness, bridge therapy awaiting DMARD response, uvveitis control *may not prevent joint destruction in addition to many s/e’s
Who is at greatest risk of severe JIA uveitis and how to manage?
ANA + disease, < 6yo, oligoarticular
initial: mydriatics and steroids
then MTX or anti-TNF-alpha
Juvenile dermatomyositis diagnosis?
- primarily clinical diagnosis based on characteristic rash (heliotrope, Gottron’s) with three signs of symmetric, proximal muscle weakness
- supported by elevated muscle enzymes (CK, AST, ALT, LDH) and EMG (denervation & myopathy)
- if in doubt or to grade disease: muscle biopsy (necrosis & inflammation)
*photosensitive, bulbar weakness= VFSS, PFTs
Which auto-antibodies are associated with dermatomyositis?
ANA +ve in 80%
anti-Jo1 and anti-Mi2 may indicate severe disease
Dermatomyositis treatment progression?
Corticosteroids Methotrexate Hydroxychloroquine for rash MMF IVIG cyclophosphamide PT OT sun avoidance
HSP organs involved?
Palpable purpura… 15-60% develop recurrences! milder each time
MSK: arthritis/arthralgias (75%)
GI: pain, intussusception, bleeding
GU: HTN, hematuria, proteinuria (50%) with renal disease as long-term complication in 1-2%, ESRD in 8% of HSP nephritis. Can develop up to 6 months after diagnosis
CNS: ICH, sz, headache
rarely: orchitis, carditis, eye, testes torsion, pulmonary hemorrhage r
Lab evaluation for HSP?
leukocytosis, thrombocytosis, anemia
increased ESR, CRP
high IgA
LOOK CLOSELY AT:
blood pressure
urinanalysis
serum creatinine
Name examples of enthesitis-related arthritis
The spondyloarthritides except psoriasis:
ankylosing spondylitis
IBD-associated arthritis
reactive arthritis
If an older boy develops LE joint pain and back of the heel pain, what is his most likely diagnosis?
Spondyloarthritis
HLA-B27
oligoarthritis +/- enthesitis
What are features of inflammatory back pain?
nighttime pain morning stiffness improved by activity worsened by rest insiduous onset
Presentation of juvenile ankylosing spondylitis?
oligoarthritis + enthesitis (mainly LEs and hips)
rarely axial involvement before adulthood
uncommonly with fever and weight loss –> don’t miss IBD!
SCFE risk factors?
OBESITY
black, polynesian, male
hypothyroidism, hypopituitary, osteodystrophy
if thin patient, <10 presents with SCFE: screen for endocrinopathy (ie GH, cortisol)
SCFE or LCPD physical exam?
limp
shortened & externally rotated lower extremity
limited internal rotation, adduction and flexion
SCFE vs LCPD treatment
SCFE: pin it
LCPD: cast it
When to screen for scoliosis?
Examine AP & lateral, Adam’s forward bend test, superficial abdominal reflex
girls: at 10 and 12 years of age
boys: once at 13 or 14
What are atypical findings on radiograph for scoliosis?
left thoracic curve double thoracic curve high thoracic curves widening of the spinal canal erosive or dysplastic changes
What features of scoliosis allow you to just observe?
Curve <20 and skeletally immature
Curves 20-45 in skeletally mature
What features of scoliosis mandate bracing?
Curve >30 in skeletally immature patient
Curve 20-25 that has progressed >5 degrees in a skeletally immature patient
Ineffective in curves >45
What features of scoliosis mandate surgery?
Curves >45 in skeletally immature patients
Curves >50 in skeletally mature patients
Curves >35 if significant shift of the trunk relative to the pelvis and LEs
Mnemonic for features of clubfoot?
CAVE cavus (midfoot) adductus (forefoot) varus (hindfoot) equinus (hindfoot) order of correction also follows this order
Name 4 ways that maternal SLE can affect the neonate
1) congenital heart block *** only permanent effect, <3% in Ro & La +ve mothers
2) photosensitive or discoid rash (3-4 mo, within first 6 weeks)
3) hematologic anemia, thrombocytopenia or neutropenia
4) hepatomegaly +/- splenomegaly, hepatitis
What are long-term orthopedic complications of JIA?
leg-length discrepancy flexion contractures popliteal cysts short stature osteoporosis
What is the classic triad of osteogenesis imperfecta?
fragile bones
blue sclera
early deafness
Autosomal dominant!
Type 1 collagen, other connective tissue laxity, easy bruising, mild short stature.
FULL LIFESPAN. Easy fracturing decreases after puberty.
How to confirm osteogenesis imperfecta?
collagen biochemical studies usig dermal fibroblasts
What do kids with OI die from?
Cardiopulmonary complications!
- recurrent pneumonias, decreasing fxn, cor pulmonale
Neurological complications also common.
What are the classification criteria for JIA?
age <16 hrs arthritis >/= 6 weeks -polyarthritis: >/= 5 joints -oligoarthritis: = 4 joints -systemic disease: arthritis with rash and characteristic quotidien fever (***rarely ANA+ or RF+)
What do you think of a patient with SoJIA and low white blood cell or platelet count?
Do they have MAS?
Ferritin values can peak in MAS too
What do you think of a patient with SoJIA and super high ESR/CRP/WBC/Plts?
That’s totally normal.
Also, hemoglobin 70-100 is normal.
What kind of cell counts make you suspicious for septic arthritis?
> 50-100 thousand cells / mm^3
What are features of systemic onset JIA?
1) fever: >/=2 weeks, quotidien, >/=39C, documented daily for at least 3 days
2) arthritis in >/=1 joint for >/=6 weeks
3) at least one of the following:
evanescent erythematous rash
generalized LN enlargement
HSM
serositis (ie pericarditis)
How to make SLE diagnosis?
4 of 11 criteria simultaneously
1) malar rash
2) photosensitivity
3) discoid rash
4) oral ulcers
5) arthritis
6) serositis
7) renal disorder
8) neurologic disorder
9) hematologic disorder
10) ANA
11) Anti-DNA/Sm/phospholipid Ab/VDRL
side note: women»_space;> men (90%!)
How often is ANA + in SLE patients?
In the healthy population?
95-99% + ANA in SLE patients
20% in general population
What is the most specific Ab in SLE?
DsDNA
What Ab correlates with disease activity in SLE?
Anti-Smith
What Ab is present most often in drug-induced lupus?
Anti-histone antibodies
What Ab is related to other connective tissue disorders and Raynauds?
Anti-ribonucleoprotein antibody
How long do you treat rheumatic fever without carditis?
5 years or 21 years of age, whichever is longer
How long do you treat rheumatic fever with carditis but without residual heart disease?
10 years or until 21 years of age, whichever is longer
How long do you treat rheumatic fever with residual heart disease?
10 years or until 40 years of age- lifelong if they’ll take it!
How long can it take for Osgood-Schlatter to heal?
12-24 months
-rest, ice, NSAIDs, limit exacerbating activity, consider protection, strengthen quads
What are Jones criteria Major manifestations?
Polyarthritis Carditis Nodules Erythema marginatum Chorea
What are Jones criteria Minor manifestations?
Clinical:
arthralgia
fever
Laboratory: elevated acute phase reactants ESR CRP prolonged PR interval
Name three causes of bilateral toe walking
Idiopathic (habit) Tight achilles tendon Cerebral palsy Muscular dystrophy Duchennes Autism spectrum disorder Spinal dysraphism
What position do patient’s hold with a septic hip?
flexed, abducted and externally rotated
What is Reiter syndrome?
Reactive arthritis after an enteric infection
“can’t see, can’t pee, can’t climb a tree”
salmonella, shigella, yersinia, campy, CHLAMYDIA
usually HLAB27 positive
