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Peds > peds33 > Flashcards

peds33 Flashcards

1
Q

prognosis for SMA

A

type 1- die by 1 yo; types 2 and 3- survival until adult years is common

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2
Q

infantile botulism

A

bulbar weakness and paralysis secondary to inget og c. botulinim spores and absorption of the toxin

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3
Q

food sources of botulinum toxin

A

honey

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4
Q

what does the botulinm toxin do?

A

prevents the presynaptic release release of Ach

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5
Q

when is onset of symptoms for infantile botulism

A

12-48 hours after ingestion of spores; constipation is classic first sx; neuro sx follow then paralysis

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6
Q

if you do EMG on botulism patient, what do you see?

A

brief, small amp muscle potentials, with an incremental response during high freq stimulation

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7
Q

management of botulism

A

treatment is supportive with NG feeding and assisted vent as needed; botulism immune globulin improves clinical course; antibiotics are CONTRAindicated

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8
Q

prognosis of botulism

A

outlook is excellent, and complete recovery is expected but may take weeks or months

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9
Q

myotonia

A

inability to relax contracted muscles

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10
Q

congenital myotonic dystrophy

A

aut dom muscle disorder that presents in the newborn period with weakness and hypotonia

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11
Q

genetics of myotonic dystorpy

A

trinucleotide repeat disorder w aut dom inheritance and variable penetration; gene on chrom 19; transmission through mother in 90% of cases

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12
Q

clinica lfeatures of myotonic dystrophy

A

polyhydram due to poor swallowing; decr fetal movements; hypotonia, feeding and respir problems; areflexia, arthrogryposis

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13
Q

arthrogryposis

A

multiple joit contractures, seen in myotonic dystrophy

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14
Q

when dos myotonia develop in a baby with congen myotonic dystrophy?

A

not at birth; develops later, by 5 yo

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15
Q

management of myotonic dystrophy

A

supprotive; infant may require assisted ventilation and G tube feedings

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16
Q

prognosis for myotnic dystrophy

A

mortality 40%; all survivors have mental retardation; feeding problems subside w time

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17
Q

hydrocephalus

A

increased CSF under pressure in the ventricle in the brain

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18
Q

causes of hydrocephalis

A

blockage of CSF flow, decr CSF absorption, or incr CSF production

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19
Q

non-communicating hydrocephalus

A

enlarged ventricles caused by obstruction of CSF flow through the ventricular system

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20
Q

communicating hydroceph

A

enlarged ventricles as a result of incr production of CSF or decr absorption

21
Q

hydrocephalus ex vacuo

A

not true hydrocephalus; ventricular enlargement due to brain atrophy

22
Q

chiari type II malformation

A

downward displacement of the cerebellum and medulla through the foramen magnum, blocking CSF flow

23
Q

chiari type II malform is associated with what other thing?

A

lumbosacral myelomeningocele

24
Q

dandy-walker malformation

A

absent or hypoplastic cerebellar vermis and cystic enlargement of the fourth ventricle, which blocks CSF flow

25
Q

congenital aqueductal stenosis

A

X-linked trait and patient have thumb abnormalities and other CNS anomalies like spina bifida

26
Q

acquired causes of hydrocephalus

A

intraventricular hemorrhage, bacterial meningitis, brain tumors

27
Q

sunset sign

A

tonic downward dviation of both eyes caused by pressure from enlarged third ventricles on the upward gaze center in the midbrain

28
Q

brisk DTRs but with usually downward plantar response

A

sign of incr ICP

29
Q

evaluation of hydrocephalus

A

urgent head CT

30
Q

management of hydrocephalu

A

ventriculoperitoneal shunt; complications include shunt infection and shunt obstruction

31
Q

prognosis of hydroceph

A

patients with aqueductal stenosis have the best cognitive outcome; chiari type 2 have low-normal intelligence; patients w x-linked hydroceph may have severe mental retardation

32
Q

spina bifida

A

any failuere of bone fusion in the posterior midline of the vertebral column

33
Q

myelomeningocele

A

herniation of the spinal cord and meninges through a bony cleft

34
Q

meningocele

A

herniation of the meninges only throug a bony cleft; not usually associated w any neural deficits

35
Q

which is more common- myelomeningocele or meningocele?

A

myelomeningocele

36
Q

SB occulta

A

hairy patch or dimple; no herniation and no neuro deficit

37
Q

how does a meningocele present?

A

fluctuant midline mass overlying the spine; mass is filled with CSF and can be transilluminated; neuro deficits not present

38
Q

melomeningocele

A

fluctuant midline mass anywhere along spine but most likeley in lumbosacral region; neuro deficits are varibale

39
Q

spina bifida associated anomaies

A

hydrocephalus, cervical hydrosyringomyelia, defects in neuronal migration, orthopedic problems, GU defects,

40
Q

association between hydroceph and spina bifida

A

90% of lumbosacral myelomeningoceles are associated with chiari type 2 malformation and hydrocephalus; cervical and thoracic myelomenin are not associated w hydroceph

41
Q

cervical hydrosyringomyelia

A

accum of fluid within the central spinal cord and within the cord itself

42
Q

management of meningocele

A

surgical repair

43
Q

management of meningomyelocele

A

urgent surgical repair within 24 hrs of birth to prevent infection and further trauma to exposed tissue

44
Q

most common causes of coma in kids less than 5

A

abuse or near-drowning

45
Q

in older kids, what is the most common cause of coma?

A

drug overdsose and accidental head injury

46
Q

CSF or blood draining from the nose indicates what?

A

basilar skull fracture

47
Q

decerebrate posturing

A

extension of arms and legs; indicates subcortical injury

48
Q

decorticate posturing

A

flexion of arms and extension of legs; suggest bilateral cortical injury

49
Q

asymm motor response

A

suggests hemispheric brain injury

Peds (99 decks)

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