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Miller > peds > Flashcards

peds Flashcards

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
Q

treatment for dynamic supination in 3-5 yo w/ ho clubfoot

A

anterior tibialis tendon transfer to lateral cuneiform

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26
Q

which coalition has the anteater sign on xray?

A

calcaneonavicular

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27
Q

which coalition has the c-sign on xray?

A

talocalcaneal

28
Q

muscle imbalance in CMT cavus foot

A

weak tib ant, peroneus brevis, and intrinsics. strong peroneus longus (long is strong)

29
Q

how do you address claw toes in CMT?

A

EHL transfer to 1st MT or IP fusion

30
Q

soft tissue procedure for flexible cavus foot

A

plantar release, longus to brevis transfer

31
Q

what spine pathology is common in achondroplasia?

A

decreasing interpediculate distance from L1-L5, foramen magnum stenosis, thoracolumbar kyphosis

32
Q

spine pathology a/w pseudoachondroplasia

A

C1-2/atlantoaxial instability

33
Q

inheritance of diastrophic dysplasia

A

autosomal recessive mutation in DTDST gene

34
Q

condition a/w cauliflower ears, hitchhikers thumbs, short stature (rhizomelic), and cleft palate

A

diastrophic dysplasia

35
Q

mutations in multiple epiphyseal dysplasia

A

COMP, type IX collagen (COL9A2 and 9A3)

36
Q

what condition should you think of if you see bilateral perthes?

A

multiple epiphyseal dysplasia

37
Q

Morquio syndrome inheritance

A

autosomal recessive deficiency in galactosamine-6-sulfatase or beta galactosidase (cause keratin sulfate accumulation)

38
Q

what condition is a/w increased risk of malignant hyperthermia?

A

OI

39
Q

inheritance of Marfan’s syndrome

A

autosomal dominant mutation in fibrillin-1 (FBN1) which causes increase in TGF-b levels

40
Q

treatment for hypophosphatemic (X-linked dominant) vitamin D resistant rickets

A

phosphate and vitamin D

41
Q

inheritance of hypophosphatasia

A

autosomal recessive

42
Q

labs in hypophosphatasia

A

low alk phos, high urinary phosphoethanolamine

43
Q

inheritance of Larsen syndrome

A

autosomal dominant FLNB mutation

44
Q

how does Larsen syndrome present?

A

multiple joint dislocations (hip, knee, shoulder, radial head - reduce knees before hips), cardiac defects, cervical kyphosis

45
Q

hunter syndrome inheritance

A

x-linked recessive mutation in iduronate-2-sulfatase enzyme

46
Q

what other tests should you get in radial clubhand?

A

CBC (fanconi’s anemia), renal US, echo

47
Q

which type of polydactyly is more common in african americans?

A

postaxial. if caucasian, get workup

48
Q

what is the main factor that dictates treatment for hypoplastic thumb?

A

stability of CMC joint

49
Q

treatment for hypoplastic thumb with unstable CMC joint

A

index finger pollicization

50
Q

what is alpha angle on DDH US and what is normal?

A

bony acetabulum, normal > 60

51
Q

what is beta angle on DDH US and what is normal?

A

femoral head cartilaginous coverage, normal < 55

52
Q

primary blood supply to proximal femoral physis before age 4

A

posterosuperior branch to lateral cervical ascending artery (comes from medial femoral circumflex)

53
Q

inheritance of Aperts

A

AD mutation in FGFR2

54
Q

blocks to reduction in DDH

A

TAL, pulvinar, hypertrophied labrum, inferior capsule, psoas tendon

55
Q

gene a/w PFFD

A

sonic hedgehog

56
Q

kocher criteria

A

WBC > 12k, ESR > 40, inability to bear weight, T > 101.5/38.6

57
Q

factors predictive of septic arthritis in order of senstivity

A

fever > CRP > ESR > refusal to bear weight > WBC

58
Q

incidence of septic arthritis with 3/4 Kocher criteria

A

93%

59
Q

most common sequela of posteromedial tibial bowing

A

3-4 cm LLD

60
Q

gene a/w fibular hemimelia

A

sonic hedgehog

61
Q

most frequent location of OCD

A

posterolateral portion of medial femoral condyle

62
Q

which risser stage is peak height velocity

A

0

63
Q

most common deformity in congenital scoliosis

A

unilateral bar

64
Q

most likely to progress deformity in congenital scoliosis

A

unilateral bar with contralateral hemivertebra

65
Q

most common bacteria for osteo following varicella

A

group A beta-hemolytic strep

Miller (12 decks)

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