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~Ortho Boards~ > Pediatrics > Flashcards

Pediatrics Flashcards

1
Q

If left untreated, pediatric lateral condyle fractures lead to which deformity?

A

Cubitus valgus; or lateral spurring with pseudo-cubitus varus. Can see a tardy ulnar nerve palsy due to cubitus valgus.

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2
Q

What percentage of the overall humeral length comes from the distal growth plate?

A

The distal humerus growth plate contributes approximately 20% of growth to overall humeral length.

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3
Q

In a fracture of a pediatric medial epicondyle, where does it most commonly displace? And why?

A

Anteromedially; due to flexor-pronator mass

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4
Q

What XR is most accurate to assess degree of displacement in pediatric medial epicondyle fracture?

A

Distal humeral axial radiographs

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5
Q

Refracture following pediatric femur fracture is most common with external fixation and […] deformity

A

varus

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6
Q

the typical malunion deformity following a pediatric femur fractur is […] and […] of the distal fragment.

A

varus; flexion

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7
Q

In salter Harris II fractures, the physis fails on the […] side, and the metaphysis on the […] side

A

tension; compression

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8
Q

The gastrocnemius heads originate just […] to the distal femoral physis

A

proximal

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9
Q

Knee collateral ligaments attach just […] to the physis

A

distal

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10
Q

Irreducible SH type II distal femur fractures are most often due to interposed periosteum on the […] side of fracture

A

tension

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11
Q

ACL insertion is […] posterior to the intermeniscal ligament

A

9 mm

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12
Q

In pediatric tibia eminence fractures, failure occurs through […] bone

A

deep cancellous

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13
Q

If the tibial eminence propagates to the plateau, the […] tibial plateau is most common.

A

medial

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14
Q

Type I tibial eminence fracture is non-displaced or displaced less than […].

A

3 mm

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15
Q

Non-operatively management tibial eminence fractures should be casted for […] weeks

A

3-4

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16
Q

Type II tibial eminence fractures may fail to reduce due to the entrapped […], entrapped […], or the pull of the […] attachment

A

medial meniscus; intermeniscal ligament; lateral meniscus

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17
Q

Arthrofibrosis following a tibial eminence fracture is more common with […] treatment.

A

surgical

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18
Q

A tibial eminence fracture with a concomitant […] injury, is an independent risk factor for RTOR for MUA.

A

ACL

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19
Q

Following a tibial eminence fracture with ACL laxity, functional instability is […].

A

uncommon

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20
Q

Overall prognosis of a tibial eminence fracture is good with […] percent returning to prior level of sport

A

85

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21
Q

The growth plate zone where the cells are increasing in number is the zone of […]

A

proliferation

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22
Q

What muscles are weak in CMT?

A

Peroneal brevis; tibialis anterior, intrinsics in hands and feet

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23
Q

What is the inheritance pattern for CMT?

A

Autosomal dominant

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24
Q

What chromosome is affected by CMT?

A

Duplication of chromosome 17

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25
Q

What protein is affected in CMT genetics?

A

peripheral myelin protein 22 (PMP22)

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26
Q

What foot deformities are seen in CMT?

A

Cavovarus; claw toes

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27
Q

Unopposed pull of the […] muscle creates a cavovarus foot in CMT

A

peroneus longus

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28
Q

NCS in CMT demonstrates […] nerve conduction velocities with […] distal latencies noted in peroneal, ulnar, and median nerves, as well as […] amplitude nerve potentials due to axonal loss

A

low; prolonged; low

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29
Q

What is the initial deformity in a cavovarus foot seen in CMT?

A

Plantarflexed first ray

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30
Q

The cavus foot in CMT is caused by […] overpowering weak […]

A

peroneus longus; tibialis anterior

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31
Q

The varus foot deformity in CMT is caused by […] overpowering weak […]

A

tibialis posterior; peroneus brevis

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32
Q

Dystrophin in expressed in […] and […] muscle.

A

striated; cardiac

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33
Q

What is the inheritance pattern for Duchenne Muscular Dystrophy?

A

X-linked recessive

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34
Q

Are proximal or distal muscles affected first in Duchenne muscular dystrophy?

A

Proximal

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35
Q

Duchenne MD muscle weakness begins in what muscle group?

A

Gluteal muscles

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36
Q

Gower’s sign is observed to compensate for […] and […] muscle weakness.

A

gluteus maximus; quadriceps

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37
Q

Which muscle in the upper extremity is usually spared in Duchenne MD?

A

Deltoid

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38
Q

In Becker’s muscular dystrophy, the protein […] is usually decreased instead of absent.

A

dystrophin

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39
Q

How is Guillain-Barre syndrome diagnosed?

A

CSF analysis

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40
Q

In Duchenne MD, […] decreases rate of scoliosis requiring fusion from 92% to 20%

A

prednisone/deflazacort

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41
Q

What is deflazacort?

A

oxazoline derivative of prednisone

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42
Q

In Duchenne MD, early PSF is indicated in curves of […] degrees in non-ambulatory patients, or FVC less than […] percent.

A

20-30; 35

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43
Q

Prior to PSF in Duchenne MD, […] studies should be obtained as significant declines in function of both organ systems may make spinal fusion too high-risk

A

cardiac and pulmonary function

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44
Q

What foot deformity is commonly seen in Duchenne MD?

A

Equinovarus

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45
Q

Mutation thefibrillin-1 gene causes what genetic syndrome?

A

Marfan syndrome

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46
Q

What is the inheritance pattern for Marfan syndrome?

A

Autosomal dominant

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47
Q

What gene is affected in Marfan syndrome?

A

Fibrillin-1

48
Q

What chromosome is affected in Marfan syndrome?

A

chromosome 15 (locus CH 15q21)

49
Q

What acetabular abnormality can be seen in Marfan syndrome?

A

protrusio acetabuli

50
Q

What spinal canal deformities can be seen in Marfan syndrome?

A

Dural ectasia (>60%); meningocele

51
Q

Prior to any surgery on a patient with Marfan syndrome […] consultation is warranted.

A

cardiology

52
Q

Bracing in scoliosis for Marfan syndrome is […] effective than in AIS

53
Q

What is the most common complication following spinal fusion surgery in Marfan syndrome?

A

fixation failure

54
Q

In osteogenesis imperfecta, there is insufficient […] production

55
Q

What gene is affected in osteogenesis imperfecta?

A

COL1A1 or COL1A2

56
Q

In osteogenesis imperfecta, COL 1A1 or COL 1A2 genetic mutation causes abnormal […] via a glycine substitution in the procollagen molecule

A

collagen cross-linking

57
Q

In osteogenesis imperfecta, COL 1A1 or COL 1A2 genetic mutation causes abnormal collagen cross-linking via a […] substitution in the procollagen molecule

58
Q

What is the inheritance pattern of osteogenesis imperfecta?

A

can be both autosomal dominant and autosomal recessive

59
Q

What are the severe autosomal dominant types of osteogenesis imperfecta?

A

Type II and III

60
Q

Saber shin appearance of tibia is associated with what genetic syndrome?

A

osteogenesis imperfecta

61
Q

Patients with osteogenesis imperfecta have coxa […]

62
Q

What medication is indicated in most cases of OI fractures to reduce fracture rate, pain, improve ambulation

A

bisphosphonates

63
Q

Bisphosphonates demonstrate marked improvement in pain at […] wk after initiation in fracture care for osteogenesis imperfecta

64
Q

Bisphosphonates inhibit […]

A

osteoclasts

65
Q

Chronic use of bisphosphonates in osteogenesis imperfecta causes […] seen on radiographs

A

horizontal metaphyseal bands

66
Q

In histology of osteogenesis imperfecta, histology shows increased diameter of […] and […]

A

haversion canals and osteocyte lacunae

67
Q

On histology of osteogenesis imperfecta, there is replicated […]

A

cement lines

68
Q

On histology of osteogenesis imperfecta, there is increased number of […] and […] cells.

A

osteoblasts; osteoclasts

69
Q

In ostegenesis imperfecta scoliosis, curves less than […] degrees can be observed

70
Q

Mucopolysaccharidoses is associated with genu […]

71
Q

Mucopolysaccharidoses is associated with […]-shaped phalanges

72
Q

Mucopolysaccharidoses is associated with […] dwarfism

A

proportionate

73
Q

Mucopolysaccharidoses is associated with increased rate of […] syndrome

A

carpal tunnel

74
Q

Is Morquio syndrome associated with mental retardation?

A

No (other mucopolysaccharidoses are though)

75
Q

toluidine blue-spot test is used to analyze excess […] in the urine

A

mucopolysaccharides

76
Q

[…] is used to analyze excess mucopolysaccharides in the urine

A

toluidine blue-spot test

77
Q

Morquio syndrome is characterized by accumulation of […]

A

keratin sulfate

78
Q

beta-galactosidase deficiency (type A) or beta-galactosidase deficiency (type B) deficiency is seen in what mucopolysaccharidosis syndrome?

A

Morquio syndrome

79
Q

What is the inheritance pattern of Morquio syndrome?

A

autosomal recessive

80
Q

coxa vara with unossified femoral head is seen in what mucopolysaccharidoses?

A

Morquio syndrome

81
Q

Mucopolysaccharidoses can have odontoid […] leading to cervical instability

A

hypoplasia

82
Q

What is the most severe form of mucopolysaccharidosis?

A

Hurler syndrome

83
Q

Hurler syndrome is characterized by […] deficiency which leads to accumulation of dermatan sulfate.

A

alpha-L iduronidase

84
Q

Hurler syndrome is characterized by alpha-L iduronidase deficiency which leads to accumulation of […].

A

dermatan sulfate

85
Q

What is the inheritance pattern for Hurler syndrome?

A

Autosomal recessive

86
Q

Which mucopolysaccharidosis can present with carpal tunnel syndrome and finger triggering?

A

Hurler syndrome

87
Q

In Hurler syndrome, urine studies show increased levels of […]

A

dermatan sulfate

88
Q

San Filippo syndrome is characterized by accumulation of […]

A

heparan sulfate

89
Q

What is the inheritance pattern of San Filippo syndrome?

A

autosomal recessive

90
Q

Hunter syndrome is characterized by sulpho-iduronate-sulphatase deficiency which leads to accumulation of […]

A

dermatan/heparan sulfate

91
Q

Which FGFR3 domain is mutated in achondroplasia?

A

Transmembrane domain

92
Q

Persistent activation of FGFR3 transmembrane domain leads to continuous inhibition of […] proliferation

A

chondrocyte

93
Q

Activated FGFR3 targeted by […] to be degraded to terminate signal shortly after activation

94
Q

Risk of sporadic mutation in the FGFR3 gene is increased with advanced […] age

95
Q

What chromosome is affected in achondroplasia?

A

chromosome 4P

96
Q

In achondroplasia there is a point mutation in FGFR3 of Gly1138to […] substitution

97
Q

The gain of function mutation on FGFR3 seen in achondroplasia increases inhibition of […] proliferation

A

chondrocyte

98
Q

In rhizomelic dwarfism, the […] portion of the limb is shorter than the […] limb

A

proximal; distal

99
Q

In achondroplasia, there is […] interpedicular distance from L1-S1

100
Q

What craniocervical junction issue is seen in achondroplasia?

A

foramen magnum stenosis

101
Q

Osteopetrosis is a congenital metabolic bone disease caused by defective […] resorption of immature bone

A

osteoclastic

102
Q

Osteopetrosis is caused by osteoclast inability to acidify […]

A

Howship’s lacuna

103
Q

In osteopetrosis, osteoclast dysfunction leads to inability to acidify Howship’s lacuna due to defective […] enzyme or […] channel dysfunction.

A

carbonic anhydrase II; chloride

104
Q

Which cranial nerve is most affected in osteopetrosis?

105
Q

Lack ruffled border and clear zone, islands of calcified cartilage within mature trabeculae is seen in what congenital bone metabolic disease?

A

osteopetrosis

106
Q

What gene is affected in Marfan’s syndrome?

107
Q

Marfan syndrome is caused by […] of fibrillin-1 glycoprotein

A

misfolding

108
Q

In Marfan syndrome, misfiling of fibrillar-1 glycoprotein binds and sequesters […] poorly, leading to accumulation in multiple sites.

109
Q

Morquio syndrome presents with […] intelligence

109
Q

Residual gapping of greater than […] in a pediatric distal tibia fracture has been associated with increased rates of premature physeal closure (PPC).

110
Q

Morquio syndrome presents with atlantoaxial instability due to […]

A

odontoid hypoplasia (hallmark finding)

111
Q

SCFE is associated with […] femoral anteversion and […] femoral neck-shaft angle

A

decreased; decreased

112
Q

In infantile tibia vara, performing an osteotomy by age […] decreases the risk of recurrence.

113
Q

Ortolani maneuver is […] the hip

A

relocating

114
Q

Multiorgan failure (MOF) in pediatric trauma patients usually occurs […] after admission and affects almost all organ systems simultaneously.

A

early (within the first 24 hours)

~Ortho Boards~ (13 decks)

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