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Flashcards in peds33 Deck (49):
1

prognosis for SMA

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

2

infantile botulism

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

3

food sources of botulinum toxin

honey

4

what does the botulinm toxin do?

prevents the presynaptic release release of Ach

5

when is onset of symptoms for infantile botulism

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

6

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

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

7

management of botulism

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

8

prognosis of botulism

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

9

myotonia

inability to relax contracted muscles

10

congenital myotonic dystrophy

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

11

genetics of myotonic dystorpy

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

12

clinica lfeatures of myotonic dystrophy

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

13

arthrogryposis

multiple joit contractures, seen in myotonic dystrophy

14

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

not at birth; develops later, by 5 yo

15

management of myotonic dystrophy

supprotive; infant may require assisted ventilation and G tube feedings

16

prognosis for myotnic dystrophy

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

17

hydrocephalus

increased CSF under pressure in the ventricle in the brain

18

causes of hydrocephalis

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

19

non-communicating hydrocephalus

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

20

communicating hydroceph

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

21

hydrocephalus ex vacuo

not true hydrocephalus; ventricular enlargement due to brain atrophy

22

chiari type II malformation

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

23

chiari type II malform is associated with what other thing?

lumbosacral myelomeningocele

24

dandy-walker malformation

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

25

congenital aqueductal stenosis

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

26

acquired causes of hydrocephalus

intraventricular hemorrhage, bacterial meningitis, brain tumors

27

sunset sign

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

28

brisk DTRs but with usually downward plantar response

sign of incr ICP

29

evaluation of hydrocephalus

urgent head CT

30

management of hydrocephalu

ventriculoperitoneal shunt; complications include shunt infection and shunt obstruction

31

prognosis of hydroceph

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

spina bifida

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

33

myelomeningocele

herniation of the spinal cord and meninges through a bony cleft

34

meningocele

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

35

which is more common- myelomeningocele or meningocele?

myelomeningocele

36

SB occulta

hairy patch or dimple; no herniation and no neuro deficit

37

how does a meningocele present?

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

38

melomeningocele

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

39

spina bifida associated anomaies

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

40

association between hydroceph and spina bifida

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

41

cervical hydrosyringomyelia

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

42

management of meningocele

surgical repair

43

management of meningomyelocele

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

44

most common causes of coma in kids less than 5

abuse or near-drowning

45

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

drug overdsose and accidental head injury

46

CSF or blood draining from the nose indicates what?

basilar skull fracture

47

decerebrate posturing

extension of arms and legs; indicates subcortical injury

48

decorticate posturing

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

49

asymm motor response

suggests hemispheric brain injury