IX Flashcards

1
Q

most common supratentorial and infratentorial CNS tumor

A

astrocytoma

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2
Q

parinauds syndrome

A

paralysis upward gaze

seen with pineal gland tumors

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3
Q

where do creaniopharyngiomas arise

A

sella turcica

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4
Q

bilateral retinal hemorrhages in infant

A

shakey baby syndrome- shearing of subdural veins

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5
Q

galactosemia deficient enzyme

A

galactose-1-phosphate-uridyl transferase deficiency

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6
Q

Sx galactosemia

A

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

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7
Q

galactosemia patients are at inc risk for what type sepsis

A

e coli neonatal sepsis

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8
Q

aura tells you what about seizure

A

partial seizure

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9
Q

complex partial seizure

A

have automatisms during LOC

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10
Q

partial seizure

A

no LOC

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11
Q

craniopahryngiomas are from what

A

rathkes pouch epithelial remnants

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12
Q

associated Sx with cranipharyngioma

A

DI
growth failure
hypothyroidism
GH deficiency

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13
Q

Dx cranipharygioma is made

A

CT or MRI

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14
Q

what is repeat sequence in fragile X

A

CGG

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15
Q

Fridereich ataxia

A

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

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16
Q

inheritance friderich ataxia

A

auto recessive

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17
Q

auto dominan muscular dystrophy

A

myotonic dystrophy

CTG repeats in DMPK gene chromosome 19q13.3

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18
Q

myotonic dystrophy presentation

A

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

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19
Q

When does duchene muscular dystrophy being

A

2-3y.o

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20
Q

NF1 manifeastations

A
cafe au lait
inguinal freckles
iris hamartomas
neurofibroms
increase risk of optic glioma
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21
Q

which NF has bilateral acoustic neuromas

A

type 2

22
Q

Glascow coma scale that warrants CT

A
23
Q

features NF1

A

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

24
Q

Tx botulinum toxin

A

respiratory support
NG tube feeding
IB human botulism Ig

25
Q

Todd paralysis

A

after a seizure
goes away
hemiparesis with little sensory abnormalities

26
Q

what is mutated in osteogenesis imperfecta

A

auto dominant

COL1A1

27
Q

Kawasaki disease pathogenesis

A

acute vasculitis of small and medium aa

28
Q

symptoms kawasaki

A
high fever, irritability, systemic inflammation
conjunctivitis
oral mucosal- strawberry tongue
rash
erythea, edema,desquamation
cervical lymphadenopathy
29
Q

lab findings in kawasaki

A

elevated CRP
leukocytosis with neurophilia
reactive thrombocytosis
sterile pyuria on UA

30
Q

Tx kawasaki

A

aspirin and IV Ig

31
Q

atlantoaxial instability

A

downs

32
Q

symptoms atlantoaxial instability

A
behavioral changes
toticollis
urinary incontinence
vertebrobasillar symptoms
UMN damage
33
Q

legg calce perthes

A

idiopathic avascular necrosis of femoral capital epiphysis

34
Q

codmans triangle in metaphysis

A

osteosarcoma

35
Q

ewings sarcoma locations

A

diaphyses of long bones

36
Q

mets of ewings

A

lungs and lymph nodes

37
Q

onion skinning of lesion in diaphysis/metaphysis

or moth eaten

A

ewings

38
Q

costochondral jion hypertrophy or rachititc rosary

A

rickets

vit D deficiency

39
Q

supplementation Vit D amount in infants

A

400 IU

40
Q

bucket handle fractures and rib fractures in a kid

A

abuse

41
Q

what type of injury in osgood schlatter

A

traction apophysitis

42
Q

what antibiotics cause serum sickness

A

beta lactams and sulfa drugs most commonly

43
Q

signs of serum sickness

A

fever, urticaria, polyarthralgia

headache, edema, lymphadenopathy, splenomegaly

44
Q

what type of HS reaction is serum sickness

A

III

45
Q

sandpaper like rash

A

scarlet fever

46
Q

steven johnson syndrome

A

bullous lesions

painful hemorrhagic oral erosions

47
Q

hypodense lesion on xr

A

osteoid osteoma

48
Q

Tx osteoid osteoma

A

NSAIDs and close watch

49
Q

if patient has trendelenburg gait next step is

A

US hips

50
Q

features acute rheumatic fever

A

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

51
Q

how do you prevent scarlet fever

A

treating GAS with penicillin