Peds Flashcards

1
Q

when does the NS first appear during gestation?

A

day 21

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2
Q

the neural tube closes at _____ days from ____ to ____

A

23-25
anterior to posterior

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3
Q

what are the 3 primary brain vesicles?

A

prosencephalon
mesencephalon
rhombencephalon

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4
Q

by week ____ of gestation there are 5 vesicles

A

5

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5
Q

which adult structures come from the telencephalon?

A

cerebral hemispheres

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6
Q

which adult structures come from the diencephalon?

A

thalamus
GP
hypothalamus

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7
Q

which adult structures come from the mesencephalon?

A

midbrain

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8
Q

which adult structures come from the metencephalon?

A

pons
cerebellum

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9
Q

which adult structures come from the myencephalon?

A

medulla

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10
Q

which supplement should be taken to prevent neural tube defects?

A

folate

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11
Q

dysraphism is disorders of the

A

neural tube closure

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12
Q

spina bifida occulta

A

vertebral arch defect only
10% of population
usually asymptomatic

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13
Q

spina bifida meningocele

A

dura and arachnoid herniation through vertebral defect

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14
Q

spina bifida myelomeningocele

A

herniation of spinal cord and meninges through defect

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15
Q

where is spina bifida occulta most common at (levels)

A

L5-S1

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16
Q

which spina bifida can present with tethered cord syndrome and persistent neuro defects?

A

myelomeningocele

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17
Q

what is the most common presentation of tethered cord?

A

unilateral LMN dysfunction in 1 leg

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18
Q

what is an Arnold-Chiari Malformation?

A

congenital abnormality of hindbrain downward elongation of brainstem and cerebellum in to cervical portion of SC

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19
Q

type 1 Arnold-Chiari Malformation

A

cerebellar tonsils displaced >6mm

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20
Q

type 2 Arnold-Chiari Malformation

A

associated myelomeningocele

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21
Q

type 3 Arnold-Chiari Malformation

A

associated encephalocele

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22
Q

how does an Arnold-Chiari HA present?

A

HA with increased ICP and CN Sx

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23
Q

what is a Dany-Walker Malformation?

A

cerebellar vermis developmental anomaly with large cyst in posterior fossa

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24
Q

sx of Dany-Walker Malformation

A

mental retardation
hydrocephalus
spastic diplegia

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25
Q

what is craniostenosis?

A

skull sutures fuse early

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26
Q

developmental delay is diagnosed when a developmental domain quotient is <___%

A

70%

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27
Q

global developmental delay is diagnosed when there is delay in ____ developmental domain(s)

A

2 or more

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28
Q

at what age should babies be walking and saying their first word?

A

12 months

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29
Q

what is a vital sign for peds?

A

head circumference

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30
Q

red flags for peds

A

head size crossing 2 percentiles
failure to respond to sounds
fisting beyond 3 months
rolling before 3 months
persistent MORO >6 months
persistent ATNR & not rolling >7 mo
not sitting >9 mo
delayed smiling, laughing, cooing, first words
handedness below 12 months
no words after 2 years
persistent echolalia >3 years
noticeable stutter after 4 years
decline in speech ability or vocabulary

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31
Q

a baby should not have handedness until

A

12 months

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32
Q

T/F: fragile X syndrome only occurs in males

A

T

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33
Q

characteristics of trisomy 21

A

single palmar crease
broad flat face
short nose
hypotonia
umbilical hernia

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34
Q

what is the most common chromosomal cause intellectual disability?

A

fragile X syndrome

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35
Q

what does TORCHS stand for when considering infectious causes of developmental delay?

A

Toxoplasmosis
Other (Zika)
Rubella
Cytomegalovirus
Herpes Simplex, HIV
Syphilis

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36
Q

what is the most important intervention for children with developmental delay?

A

referral to specialist

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37
Q

what is a key characteristic of cerebral palsy?

A

it’s NONPROGRESSIVE

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38
Q

what is the most common form of CP?

A

spastic

39
Q

which type of CP is hypotonic? hypertonic?

A

hypotonic - ataxic
hypertonic - spasticity

40
Q

what is the most common combination for mixed CP?

A

spastic with dyskinesias

41
Q

what is a common cause of spastic hemiparesis?

A

intrauterine stroke affecting the cerebral artery

42
Q

is the arm or leg more affected in spastic hemiparesis?

A

arm

43
Q

if a child develops handedness before 18 mo, what is suspected?

A

spastic hemiparesis

44
Q

spastic diplegia effects the ___ more

A

legs (weakness)

45
Q

scissoring gait is seen in spastic _____

A

diplegia
d/t adductor spasm

46
Q

what blood supply is affected in spastic diplegia?

A

borderzone of ACA and PCA

47
Q

what is the most severe form of spastic CP?

A

spastic quadriplegia

48
Q

dyskinetic CP occurs due to lesion at the

A

basal ganglia

49
Q

what is the main causes of dyskinetic CP?

A

neonatal hyperbilirubinemia
severe anoxia

50
Q

what is the rarest form of CP?

A

ataxic

51
Q

ataxic CP occurs due to abnormal development of the

A

cerebellum
*trunkal and gait ataxia

52
Q

meds for CP

A

baclofen
diazepma
tizamidine
botulinum A

53
Q

neurocutaneous disease are also called

A

phakomatoses

54
Q

why aren’t neurofibromas removed?

A

they are entangled within the nerve itself

55
Q

sx of CNS neurofibromatosis

A

B acoustic neuromas
meningioma
gliomas

56
Q

sx of PNS neurofibromatosis

A

cafe-au-lait spots
neurofibromas on skin, peripheral nerves, and/or roots
optic pathway glioma

57
Q

presentation of tuberous sclerosis

A

white, hard nodules on surface of brain and jutting into ventricles, calcifications

58
Q

where are lesions in tuberous sclerosis?

A

NS
skin
bones
retina
kidney

59
Q

what is a common sx of tuberous sclerosis?

A

seizures

60
Q

leukodystrophies are a _____ progressive genetic disorder affecting _____

A

demyelinating
myelin metabolism

61
Q

adrenoleukodystrophy has issues with the ____ matter

A

white

62
Q

when do changes appear with adrenoleukodystrophy?

A

around 8 years old
(normal early development)

63
Q

sx of adrenoleukodystrophy

A

only in boys
behavioral changes
visual loss
progressive dementia
seizures
progressively spastic gait

64
Q

metachromatic leukodystrophy is degeneration of myelin where?

A

central AND peripheral

65
Q

T/F: children with tone abnormalities ALWAYS have low tone early on then develop high tone if they are going to present with high tone

A

T

66
Q

what is the most common MD is children?

A

Duchenne MD

67
Q

define muscular dystrophy

A

group of progressive hereditary disorders of muscle

68
Q

define myotonias

A

involuntary persistent muscle activity occurring in response to percussion or activity of the muscle

69
Q

2 categories of myopathies

A

metabolic
congenital

70
Q

psudeohypertrophy of the ____ is seen in Duchenne and Becker MD

A

calf

71
Q

hallmark of Duchenne MD

A

boys enter a WC by 9-12 yo
no dystrophin present

72
Q

Does Becker MD have an early or later onset than Duchenne?

A

later

73
Q

Pts with Becker MD are ambulatory until

A

30 yo

74
Q

Becker MD has isolated ___ weakness

A

quad

75
Q

high ____ is seen in Becker and Duchenne MD

A

CK

76
Q

what med is used to maintain ambulation in dystrophinopathies?

A

corticosteroids

77
Q

what can be a life changing treatment for dystrophinopathies?

A

gene therapy

78
Q

is spinal muscular atrophy UMN, LMN, or both?

A

LMN only

79
Q

spinal muscular atrophy results in progressive loss of

A

motor neurons in SC

80
Q

type 1 spinal muscular atrophy never

A

sit

81
Q

type 2 spinal muscular atrophy never

A

stand

82
Q

type 3 spinal muscular atrophy never

A

run

83
Q

how does type 4 spinal muscular atrophy present?

A

proximal leg weakness as an adult

84
Q

which spinal muscular atrophy types have a normal lifespan?

A

3 and 4

85
Q

what is a common cause of infantile botulism?

A

eating honey
(also corn syrup)

86
Q

infantile botulism prevents the release of

A

ACh

87
Q

sx of Infantile botulism

A

acute generalized weakness
loss of reflexes
hypotonia
ptosis
poorly reactive pupils**
respiratory difficulty

88
Q

which part of the brachial plexus is more commonly injured?

A

upper
C5-C6

89
Q

Erb’s palsy is due to injury of _____ (levels)

A

C5-C6

90
Q

MOI for Erb’s palsy

A

head pushed down away from shoulder

91
Q

how does arm rest with Erb’s palsy?

A

shoulder adduction
elbow extension
pronation

92
Q

Klumpke Palsy is due to injury of ____ levels

A

C8-T1

93
Q

what hand deformity does Klumpke Palsy cause?

A

claw hand

94
Q

MOI for Klumpke Palsy

A

arm is pulled upwards