IX

Question 1

most common supratentorial and infratentorial CNS tumor

Answer 1

astrocytoma

Question 2

parinauds syndrome

Answer 2

paralysis upward gaze

seen with pineal gland tumors

Question 3

where do creaniopharyngiomas arise

Answer 3

sella turcica

Question 4

bilateral retinal hemorrhages in infant

Answer 4

shakey baby syndrome- shearing of subdural veins

Question 5

galactosemia deficient enzyme

Answer 5

galactose-1-phosphate-uridyl transferase deficiency

Question 6

Sx galactosemia

Answer 6

newborn with failure to thrive, bilateral cataracts, jaundice, hypoglycemia

Question 7

galactosemia patients are at inc risk for what type sepsis

Answer 7

e coli neonatal sepsis

Question 8

aura tells you what about seizure

Answer 8

partial seizure

Question 9

complex partial seizure

Answer 9

have automatisms during LOC

Question 10

partial seizure

Answer 10

no LOC

Question 11

craniopahryngiomas are from what

Answer 11

rathkes pouch epithelial remnants

Question 12

associated Sx with cranipharyngioma

Answer 12

DI
growth failure
hypothyroidism
GH deficiency

Question 13

Dx cranipharygioma is made

Answer 13

CT or MRI

Question 14

what is repeat sequence in fragile X

Answer 14

CGG

Question 15

Fridereich ataxia

Answer 15

unsteady gait, weakness
decreased vibratory and position in lower extremities
ankle jerks absent bilaterally
T wave inversions in inferior and lateral chest leads

Question 16

inheritance friderich ataxia

Answer 16

auto recessive

Question 17

auto dominan muscular dystrophy

Answer 17

myotonic dystrophy

CTG repeats in DMPK gene chromosome 19q13.3

Question 18

myotonic dystrophy presentation

Answer 18

facial weakness, hand grip myotonia, dysphagia
can get arrhythmias
cataracts, balding, testicualr atrophy and infertility

Question 19

When does duchene muscular dystrophy being

Answer 19

2-3y.o

Question 20

NF1 manifeastations

Answer 20

cafe au lait
inguinal freckles
iris hamartomas
neurofibroms
increase risk of optic glioma

Question 21

which NF has bilateral acoustic neuromas

Answer 21

type 2

Question 22

Glascow coma scale that warrants CT

Question 23

features NF1

Answer 23

cafe au lait, macrocephaly, feeding problems, short stature, learning disabilities
fibromas, neurofibromas and different tumore

Question 24

Tx botulinum toxin

Answer 24

respiratory support
NG tube feeding
IB human botulism Ig

Question 25

Todd paralysis

Answer 25

after a seizure goes away hemiparesis with little sensory abnormalities

Question 26

what is mutated in osteogenesis imperfecta

Answer 26

auto dominant | COL1A1

Question 27

Kawasaki disease pathogenesis

Answer 27

acute vasculitis of small and medium aa

Question 28

symptoms kawasaki

Answer 28

``` high fever, irritability, systemic inflammation conjunctivitis oral mucosal- strawberry tongue rash erythea, edema,desquamation cervical lymphadenopathy ```

Question 29

lab findings in kawasaki

Answer 29

elevated CRP leukocytosis with neurophilia reactive thrombocytosis sterile pyuria on UA

Question 30

Tx kawasaki

Answer 30

aspirin and IV Ig

Question 31

atlantoaxial instability

Answer 31

downs

Question 32

symptoms atlantoaxial instability

Answer 32

``` behavioral changes toticollis urinary incontinence vertebrobasillar symptoms UMN damage ```

Question 33

legg calce perthes

Answer 33

idiopathic avascular necrosis of femoral capital epiphysis

Question 34

codmans triangle in metaphysis

Answer 34

osteosarcoma

Question 35

ewings sarcoma locations

Answer 35

diaphyses of long bones

Question 36

mets of ewings

Answer 36

lungs and lymph nodes

Question 37

onion skinning of lesion in diaphysis/metaphysis | or moth eaten

Answer 37

ewings

Question 38

costochondral jion hypertrophy or rachititc rosary

Answer 38

rickets | vit D deficiency

Question 39

supplementation Vit D amount in infants

Answer 39

400 IU

Question 40

bucket handle fractures and rib fractures in a kid

Answer 40

abuse

Question 41

what type of injury in osgood schlatter

Answer 41

traction apophysitis

Question 42

what antibiotics cause serum sickness

Answer 42

beta lactams and sulfa drugs most commonly

Question 43

signs of serum sickness

Answer 43

fever, urticaria, polyarthralgia | headache, edema, lymphadenopathy, splenomegaly

Question 44

what type of HS reaction is serum sickness

Answer 44

III

Question 45

sandpaper like rash

Answer 45

scarlet fever

Question 46

steven johnson syndrome

Answer 46

bullous lesions | painful hemorrhagic oral erosions

Question 47

hypodense lesion on xr

Answer 47

osteoid osteoma

Question 48

Tx osteoid osteoma

Answer 48

NSAIDs and close watch

Question 49

if patient has trendelenburg gait next step is

Answer 49

US hips

Question 50

features acute rheumatic fever

Answer 50

joint, carditis, nodules, erythema marginatum, syndenham chorea, fever, arthalrgias, inc ESR and cRP prolonged PR interval

Question 51

how do you prevent scarlet fever

Answer 51

treating GAS with penicillin