Pediatrics

Question 1

Degree on Adams forward bend that warrants referral

Answer 1

7 degrees

Question 2

AIS degrees and treatment

Answer 2

<25 and skeletally immature = Observation
<40 and skeletally mature = Obserbvation
25- 40 and skeletally immature = Brave
> 50 = surgery

Question 3

Levels in AIS that require CTLSO

Answer 3

Apex The 7 or higher

Question 4

Age for early onset scoliosis

Answer 4

less than ten years old

Question 5

RVAD in early onset with high progression

Answer 5

Greater than 20

Question 6

Scoliosis in NF

Answer 6

Two types:
Nondystrophic
Dystrophic
- Vertebral scalloping
- Enlarged foramina
- Pencilling of ribs

Treat with anterior and posterior fusion due to high risk of pseudoarthrosis

Question 7

Treatment of scheurman’s kyphosis

Answer 7

PSF with curves over 90

Question 8

Trias of Klippel Feil syndrome

Answer 8

Low hairline, webbed neck, limited cervical ROM

Avoid contact sports

Question 9

When to abandon pavlik in DDH

Answer 9

if not reduced in 3 weeks

Question 10

5 obstacles to reduction in DDH

Answer 10

1. Inverted limbus
2. TAL
3. Pulvinar
4. Inferomedial capsule
5. Iliopsoas

Question 11

When to add pelvic osteotomy in DDH

Answer 11

> 4 years old

Question 12

When to leve dislocated hip alone

Answer 12

Bilateral > 6
Unilateral > 8

Question 13

Mutation in cleidocranial dysplasia

Answer 13

CBFA1 mutation
Autosomal dominant

Question 14

Mutation in Aperts

Answer 14

FGFR2

Rose bud limbs

Question 15

Mutation in SED

Answer 15

COL2A1

Question 16

Diatrophic dysplasia

Answer 16

AR
Choromosome 5, DTSDT gene sulfate transporter gene
Cauliflower ears and hitchhikers thumb

Question 17

Hurler
Deficiency?
Accumulation?

Answer 17

Def: alpha L iduronidase
Accumulation: Dermatan sulfate

Question 18

San Filippo accumulation

Answer 18

heparin sulfate

Question 19

Morquio acuumulation

Answer 19

keratan sulfate

Question 20

OI mutation

Answer 20

COL1A2

missense mutation with glycine substitution on procollagen leading to abnormal crosslinking

Question 21

Larsen syndrome

Answer 21

AD or AR
Filamin B mutation
Multiple joint dislocations

Question 22

MOA of Baclofen

Answer 22

activates GABA

Question 23

CP kid, how to predict if able to ambulate?

Answer 23

Sitting at age 2

Question 24

Acetabular deficiency in NM dysplasia vs DDH

Answer 24

NM: posterior superior
DDH: anterolateral

Question 25

Rett syndrome inheritance

Answer 25

XLD

Question 26

SCHFx , predictor for infection?

Answer 26

Young age

Question 27

Osteotomy for residual hip dysplasia with open triradiates?

Answer 27

Pemberton or Salter

Question 28

3 causes of in toeing

Answer 28

Infant- metatarsus adductus
Toddler - Internal tibial torsion
Child - femoral anteversion

Question 29

2 residual complications following tx of clubfoot

Answer 29

Dynamic supination - split ATT to lateral cuboid

Dorsal bunion - FHL lengthening FHB to ext transfer

Question 30

Genetics of CMT

Answer 30

PMP22 on Ch 17

Question 31

Genetics of Fredreichs ataxia

Answer 31

AR
GAA repeats on frataxin leading to spinocerebellar degeneration

Question 32

Campomelic dysplasia

Answer 32

SOX 9 mutation
Bowing of long bones w/ cutaneous dimpling, hypoplastic scapulae

Question 33

Collagen defect in downs

Answer 33

type 6

Question 34

Fractures in myelomeningocele

Answer 34

Present like infection