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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
What protein is affected in CMT genetics?
peripheral myelin protein 22 (PMP22)
26
What foot deformities are seen in CMT?
Cavovarus; claw toes
27
Unopposed pull of the [...] muscle creates a cavovarus foot in CMT
peroneus longus
28
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
low; prolonged; low
29
What is the initial deformity in a cavovarus foot seen in CMT?
Plantarflexed first ray
30
The cavus foot in CMT is caused by [...] overpowering weak [...]
peroneus longus; tibialis anterior
31
The varus foot deformity in CMT is caused by [...] overpowering weak [...]
tibialis posterior; peroneus brevis
32
Dystrophin in expressed in [...] and [...] muscle.
striated; cardiac
33
What is the inheritance pattern for Duchenne Muscular Dystrophy?
X-linked recessive
34
Are proximal or distal muscles affected first in Duchenne muscular dystrophy?
Proximal
35
Duchenne MD muscle weakness begins in what muscle group?
Gluteal muscles
36
Gower's sign is observed to compensate for [...] and [...] muscle weakness.
gluteus maximus; quadriceps
37
Which muscle in the upper extremity is usually spared in Duchenne MD?
Deltoid
38
In Becker's muscular dystrophy, the protein [...] is usually decreased instead of absent.
dystrophin
39
How is Guillain-Barre syndrome diagnosed?
CSF analysis
40
In Duchenne MD, [...] decreases rate of scoliosis requiring fusion from 92% to 20%
prednisone/deflazacort
41
What is deflazacort?
oxazoline derivative of prednisone
42
In Duchenne MD, early PSF is indicated in curves of [...] degrees in non-ambulatory patients, or FVC less than [...] percent.
20-30; 35
43
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
cardiac and pulmonary function
44
What foot deformity is commonly seen in Duchenne MD?
Equinovarus
45
Mutation the fibrillin-1 gene causes what genetic syndrome?
Marfan syndrome
46
What is the inheritance pattern for Marfan syndrome?
Autosomal dominant
47
What gene is affected in Marfan syndrome?
Fibrillin-1
48
What chromosome is affected in Marfan syndrome?
chromosome 15 (locus CH 15q21)
49
What acetabular abnormality can be seen in Marfan syndrome?
protrusio acetabuli
50
What spinal canal deformities can be seen in Marfan syndrome?
Dural ectasia (>60%); meningocele
51
Prior to any surgery on a patient with Marfan syndrome [...] consultation is warranted.
cardiology
52
Bracing in scoliosis for Marfan syndrome is [...] effective than in AIS
less
53
What is the most common complication following spinal fusion surgery in Marfan syndrome?
fixation failure
54
In osteogenesis imperfecta, there is insufficient [...] production
osteoid
55
What gene is affected in osteogenesis imperfecta?
COL1A1 or COL1A2
56
In osteogenesis imperfecta, COL 1A1 or COL 1A2 genetic mutation causes abnormal [...] via a glycine substitution in the procollagen molecule
collagen cross-linking
57
In osteogenesis imperfecta, COL 1A1 or COL 1A2 genetic mutation causes abnormal collagen cross-linking via a [...] substitution in the procollagen molecule
glycine
58
What is the inheritance pattern of osteogenesis imperfecta?
can be both autosomal dominant and autosomal recessive
59
What are the severe autosomal dominant types of osteogenesis imperfecta?
Type II and III
60
Saber shin appearance of tibia is associated with what genetic syndrome?
osteogenesis imperfecta
61
Patients with osteogenesis imperfecta have coxa [...]
vara
62
What medication is indicated in most cases of OI fractures to reduce fracture rate, pain, improve ambulation
bisphosphonates
63
Bisphosphonates demonstrate marked improvement in pain at [...] wk after initiation in fracture care for osteogenesis imperfecta
1-6
64
Bisphosphonates inhibit [...]
osteoclasts
65
Chronic use of bisphosphonates in osteogenesis imperfecta causes [...] seen on radiographs
horizontal metaphyseal bands
66
In histology of osteogenesis imperfecta, histology shows increased diameter of [...] and [...]
haversion canals and osteocyte lacunae
67
On histology of osteogenesis imperfecta, there is replicated [...]
cement lines
68
On histology of osteogenesis imperfecta, there is increased number of [...] and [...] cells.
osteoblasts; osteoclasts
69
In ostegenesis imperfecta scoliosis, curves less than [...] degrees can be observed
45
70
Mucopolysaccharidoses is associated with genu [...]
valgum
71
Mucopolysaccharidoses is associated with [...]-shaped phalanges
bullet
72
Mucopolysaccharidoses is associated with [...] dwarfism
proportionate
73
Mucopolysaccharidoses is associated with increased rate of [...] syndrome
carpal tunnel
74
Is Morquio syndrome associated with mental retardation?
No (other mucopolysaccharidoses are though)
75
toluidine blue-spot test is used to analyze excess [...] in the urine
mucopolysaccharides
76
[...] is used to analyze excess mucopolysaccharides in the urine
toluidine blue-spot test
77
Morquio syndrome is characterized by accumulation of [...]
keratin sulfate
78
beta-galactosidase deficiency (type A) or beta-galactosidase deficiency (type B) deficiency is seen in what mucopolysaccharidosis syndrome?
Morquio syndrome
79
What is the inheritance pattern of Morquio syndrome?
autosomal recessive
80
coxa vara with unossified femoral head is seen in what mucopolysaccharidoses?
Morquio syndrome
81
Mucopolysaccharidoses can have odontoid [...] leading to cervical instability
hypoplasia
82
What is the most severe form of mucopolysaccharidosis?
Hurler syndrome
83
Hurler syndrome is characterized by [...] deficiency which leads to accumulation of dermatan sulfate.
alpha-L iduronidase
84
Hurler syndrome is characterized by alpha-L iduronidase deficiency which leads to accumulation of [...].
dermatan sulfate
85
What is the inheritance pattern for Hurler syndrome?
Autosomal recessive
86
Which mucopolysaccharidosis can present with carpal tunnel syndrome and finger triggering?
Hurler syndrome
87
In Hurler syndrome, urine studies show increased levels of [...]
dermatan sulfate
88
San Filippo syndrome is characterized by accumulation of [...]
heparan sulfate
89
What is the inheritance pattern of San Filippo syndrome?
autosomal recessive
90
Hunter syndrome is characterized by sulpho-iduronate-sulphatase deficiency which leads to accumulation of [...]
dermatan/heparan sulfate
91
Which FGFR3 domain is mutated in achondroplasia?
Transmembrane domain
92
Persistent activation of FGFR3 transmembrane domain leads to continuous inhibition of [...] proliferation
chondrocyte
93
Activated FGFR3 targeted by [...] to be degraded to terminate signal shortly after activation
ubiquitin
94
Risk of sporadic mutation in the FGFR3 gene is increased with advanced [...] age
paternal
95
What chromosome is affected in achondroplasia?
chromosome 4P
96
In achondroplasia there is a point mutation in FGFR3 of Gly1138 to [...] substitution
Arg
97
The gain of function mutation on FGFR3 seen in achondroplasia increases inhibition of [...] proliferation
chondrocyte
98
In rhizomelic dwarfism, the [...] portion of the limb is shorter than the [...] limb
proximal; distal
99
In achondroplasia, there is [...] interpedicular distance from L1-S1
decreased
100
What craniocervical junction issue is seen in achondroplasia?
foramen magnum stenosis
101
Osteopetrosis is a congenital metabolic bone disease caused by defective [...] resorption of immature bone
osteoclastic
102
Osteopetrosis is caused by osteoclast inability to acidify [...]
Howship's lacuna
103
In osteopetrosis, osteoclast dysfunction leads to inability to acidify Howship's lacuna due to defective [...] enzyme or [...] channel dysfunction.
carbonic anhydrase II; chloride
104
Which cranial nerve is most affected in osteopetrosis?
optic
105
Lack ruffled border and clear zone, islands of calcified cartilage within mature trabeculae is seen in what congenital bone metabolic disease?
osteopetrosis
106
What gene is affected in Marfan's syndrome?
FBN1
107
Marfan syndrome is caused by [...] of fibrillin-1 glycoprotein
misfolding
108
In Marfan syndrome, misfiling of fibrillar-1 glycoprotein binds and sequesters [...] poorly, leading to accumulation in multiple sites.
TGF-ß
109
Morquio syndrome presents with [...] intelligence
normal
109
Residual gapping of greater than [...] in a pediatric distal tibia fracture has been associated with increased rates of premature physeal closure (PPC).
2 mm
110
Morquio syndrome presents with atlantoaxial instability due to [...]
odontoid hypoplasia (hallmark finding)
111
SCFE is associated with [...] femoral anteversion and [...] femoral neck-shaft angle
decreased; decreased
112
In infantile tibia vara, performing an osteotomy by age [...] decreases the risk of recurrence.
4
113
Ortolani maneuver is [...] the hip
relocating
114
Multiorgan failure (MOF) in pediatric trauma patients usually occurs [...] after admission and affects almost all organ systems simultaneously.
early (within the first 24 hours)

~Ortho Boards~ (16 decks)

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