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peds Flashcards

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1
Q

botox mechanism

A

inhibition of presynaptic release of acetylcholine at the neuromuscular junction

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2
Q

mechanism of baclofen

A

GABA agonist, binds to GABA receptors in the spinal cord to block excitatory neurotransmitter release

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3
Q

best predictor for walking in CP

A

ability to sit independently by 2

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4
Q

inheritance of Duchenne’s

A

x-linked recessive, absent dystrophin

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5
Q

indications for scoliosis surgery in Duchenne’s

A

posterior fusion if >20-30 degrees, before FVC < 35%

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6
Q

inheritance of spinal muscular atrophy

A

autosomal recessive mutation in survival motor neuron 1 (SMN 1)

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7
Q

what determines SMA disease severity?

A

number of SMN 2 genes and amount of SMN protein produced

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8
Q

CMT type 1 inheritance

A

autosomal dominant duplication of PMP 22 on chromosome 17

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9
Q

what foot muscles are weak in CMT?

A

peroneus brevis and tib ant

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10
Q

what foot muscles are strong in CMT?

A

peroneus longus, posterior tib

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11
Q

acceptable alignment for proximal humerus fracture >12 yo

A

<50% displacement, <40 degrees angulation

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12
Q

xray view to best see lateral condyle fracture

A

internal rotation oblique

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13
Q

what metaphyseal diaphyseal angle indicates risk of progression in Blount’s?

A

> 16

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14
Q

inheritance of tibial hemimelia

A

autosomal dominant

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15
Q

spine reasons to keep kids out of contact sports

A

“DOCK EM” downs syndrome, os odontoideum, congenital stenoais, klippel feil, effacement of CSF on MRI, myelomalacia

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16
Q

inheritance of Scheuermann’s kyphosis

A

autosomal dominant

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17
Q

what is the blood volume in kids?

A

75ml/kg

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18
Q

systemic and local inflammatory response in kids compared to adults

A

dampened systemic, robust local

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19
Q

femoral head blood supply before 4 years

A

medial and lateral circumflex

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20
Q

femoral head blood supply after 4 years

A

mostly medial femoral circumflex

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21
Q

what vessel is at risk with piriformis entry nail?

A

deep branch of medial femoral circumflex

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22
Q

order of closure of distal tibial physis

A

central -> posterior -> medial -> lateral

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23
Q

what is tillaux fracture

A

avulsion of AITFL

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24
Q

position of talus and calc on clubfoot xrays

A

parallel on both AP and lateral

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25
treatment for dynamic supination in 3-5 yo w/ ho clubfoot
anterior tibialis tendon transfer to lateral cuneiform
26
which coalition has the anteater sign on xray?
calcaneonavicular
27
which coalition has the c-sign on xray?
talocalcaneal
28
muscle imbalance in CMT cavus foot
weak tib ant, peroneus brevis, and intrinsics. strong peroneus longus (long is strong)
29
how do you address claw toes in CMT?
EHL transfer to 1st MT or IP fusion
30
soft tissue procedure for flexible cavus foot
plantar release, longus to brevis transfer
31
what spine pathology is common in achondroplasia?
decreasing interpediculate distance from L1-L5, foramen magnum stenosis, thoracolumbar kyphosis
32
spine pathology a/w pseudoachondroplasia
C1-2/atlantoaxial instability
33
inheritance of diastrophic dysplasia
autosomal recessive mutation in DTDST gene
34
condition a/w cauliflower ears, hitchhikers thumbs, short stature (rhizomelic), and cleft palate
diastrophic dysplasia
35
mutations in multiple epiphyseal dysplasia
COMP, type IX collagen (COL9A2 and 9A3)
36
what condition should you think of if you see bilateral perthes?
multiple epiphyseal dysplasia
37
Morquio syndrome inheritance
autosomal recessive deficiency in galactosamine-6-sulfatase or beta galactosidase (cause keratin sulfate accumulation)
38
what condition is a/w increased risk of malignant hyperthermia?
OI
39
inheritance of Marfan's syndrome
autosomal dominant mutation in fibrillin-1 (FBN1) which causes increase in TGF-b levels
40
treatment for hypophosphatemic (X-linked dominant) vitamin D resistant rickets
phosphate and vitamin D
41
inheritance of hypophosphatasia
autosomal recessive
42
labs in hypophosphatasia
low alk phos, high urinary phosphoethanolamine
43
inheritance of Larsen syndrome
autosomal dominant FLNB mutation
44
how does Larsen syndrome present?
multiple joint dislocations (hip, knee, shoulder, radial head - reduce knees before hips), cardiac defects, cervical kyphosis
45
hunter syndrome inheritance
x-linked recessive mutation in iduronate-2-sulfatase enzyme
46
what other tests should you get in radial clubhand?
CBC (fanconi's anemia), renal US, echo
47
which type of polydactyly is more common in african americans?
postaxial. if caucasian, get workup
48
what is the main factor that dictates treatment for hypoplastic thumb?
stability of CMC joint
49
treatment for hypoplastic thumb with unstable CMC joint
index finger pollicization
50
what is alpha angle on DDH US and what is normal?
bony acetabulum, normal > 60
51
what is beta angle on DDH US and what is normal?
femoral head cartilaginous coverage, normal < 55
52
primary blood supply to proximal femoral physis before age 4
posterosuperior branch to lateral cervical ascending artery (comes from medial femoral circumflex)
53
inheritance of Aperts
AD mutation in FGFR2
54
blocks to reduction in DDH
TAL, pulvinar, hypertrophied labrum, inferior capsule, psoas tendon
55
gene a/w PFFD
sonic hedgehog
56
kocher criteria
WBC > 12k, ESR > 40, inability to bear weight, T > 101.5/38.6
57
factors predictive of septic arthritis in order of senstivity
fever > CRP > ESR > refusal to bear weight > WBC
58
incidence of septic arthritis with 3/4 Kocher criteria
93%
59
most common sequela of posteromedial tibial bowing
3-4 cm LLD
60
gene a/w fibular hemimelia
sonic hedgehog
61
most frequent location of OCD
posterolateral portion of medial femoral condyle
62
which risser stage is peak height velocity
0
63
most common deformity in congenital scoliosis
unilateral bar
64
most likely to progress deformity in congenital scoliosis
unilateral bar with contralateral hemivertebra
65
most common bacteria for osteo following varicella
group A beta-hemolytic strep

Miller (12 decks)

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