Peds Final 5

Front 1

Fragile X- Syndrome

Back 1

Recessive chromosome disorder (one arm of X chromosome is defective)

Front 2

Who does Fragile X Syndrome affect?

Back 2

Males
Females have two XX chromosomes so have a healthy one to make up for the affected one
Females may be carriers

Front 3

Physical Characteristics of Fragile X syndrome?

Back 3

Large head and Long face
Prominent jaw
Large ears
Frequent ear infections
Epicanthal eye folds
Enlarged testicles- fertile- can reproduce
Cardiac disorders

Front 4

What cognitive deficits with Fragile X syndrome?

Back 4

Often mental retardation
]Hyperactivity and Autism
Marked deficits in speech and math

Front 5

What is Down Syndrome?

Back 5

Trisomy 21- disorder of chromosome 21
Extra chromosome- 3 instead of 2

Front 6

Chance of Down Syndrome increases with mom's ___

Back 6

age

Front 7

What are physical characteristics of down syndrome?

Back 7

Small head
Flat forehead
Broad and Flat nose
Small/low set ears
Protruding tongue
Wide/short neck
Epicanthal eye folds
Brushfield spots (white spots on iris)
Simian line on palm

Front 8

What are Brushfield's spots?

Back 8

white spots on iris

Front 9

What is a simian line on palm?

Back 9

Rather than three lines may be one straight across

Front 10

What diseases are kids with down syndrome more prone to?

Back 10

Increased risk for leukeimia (20 times greater chance)
Prone to cardiac defects
May have altered immune functions
alzheimers after age 30

Front 11

How are children with down syndrome, cognitively?

Back 11

Lower IQ usually cognitively challenged

Front 12

What is Spina Bifida?

Back 12

A neural tube defect. A sac at a site somewhere along spinal cord that is encased in a fine membrane and contains meninges, spinal fluid, and nerves

Front 13

Spine Bifida AKA _____________

Back 13

Myelomeningocele

Front 14

What are major problems with Spina Bifida?

Back 14

Sac is prone to tears through which CSF leaks
Flaccid, weakness, areflexic partial paralysis of the lower extremities
Varying degrees of sensory deficit

Front 15

What determines the degree of sensory deficit in Spina Bifida?

Back 15

Depends on where sac is on spinal cord, the higher the defect the more problems occur

Front 16

What cranial problem can occur due to Spina Bifida?

Back 16

Hydrocephalus (increased ICP)

Front 17

What are S/S of Hydrocephalus (increased ICP)?

Back 17

-Sensory disturbances
-Affects nerve supply to bladder- Constant dribbling of urine or overflow incontinence
-Poor anal sphincter tone
-Constipation and urinary retentions due to lack of nerve innervation
-Developmental Problems

Front 18

What care is needed for urinary retention due to hydrocephalus?

Back 18

-Need in and out caths for life
-Metrophenoff- shunt to empty bladder at umbilical site. Instead of straight cathing self. Decreased risk of infection. Much easier.

Front 19

What is the nursing goal when treating child with Spina Bifida?

Back 19

To prevent trauma and infection of site

Front 20

What nursing interventions to protect Myelomeningocele?

Back 20

*Position on abdomen or side, restrain - To prevent pressure and trauma to sac
*Thoroughly clean buttocks, genitals, and back after each void/defecation - To prevent infection/contamination of sac
*Apply protective covering to sac- To prevent rupture or DRYING

Front 21

What nursing assessments for babies with spina bifida?

Back 21

•Assess amount of sensation and movement below defect site
•Monitor for complications (skin breakdown is a big one)
•Avoid latex products (40-50% of SB pts have latex allergies)

Front 22

What is Hydrocephalus?

Back 22

An imbalance between the production of CSF and its accumulation within the intracranial cavity and in infants enlargement of the head. As the excess CSF accumulates in the ventricle system, the ventricles become dilated/enlarged and the brain is compressed against the skull.

Front 23

How can hydrocephalus be caused by a myelomeningocele?

Back 23

The hindbrain (cerebellum) tends to slip down the back of skull and into the spinal cord area causing the CSF to not be able to circulate past that area. Result is buildup of CSF in the ventricles.

Front 24

Triad for Increased Intracranial pressure (ICP)?

Back 24

Blood (hemorrhage)
Brain (tissue/tumor)
CSF (accumulation)

Front 25

What are physical S/S of hydrocephalus?

Back 25

Bulging fontanel
Irritability/lethargy
Macewen’s (cracked pot) sign
Frontal enlargement of skull
Setting sun sign

Front 26

Macewen’s (cracked pot) sign

Back 26

sound produced because sutures separate and when percussed produce this sound

Front 27

Setting sun sign

Back 27

iris of the eye is lower than the bottom eyelid, like a setting sun

Front 28

What is a LATE sign of ICP/hydrocephalus?

Back 28

Cushing’s Triad

Front 29

Cushing’s Triad

Back 29

Bradycardia
Irregular respirations
Increased SBP and widened pulse pressure

Front 30

Treatments for Hydrocephalus:

Back 30

•Treat underlying cause if possible
•Relief of excess CSF in the ventricles (shunting)

Front 31

What type if shunt is used to relieve excess CHF in the ventricles?

Back 31

Ventriculoperitoneal Shunt

Front 32

Ventriculoperitoneal Shunt

Back 32

Tubing that diverts the CSF from the ventricles into the peritoneal cavity where it can be reabsorbed

Front 33

What are problems that can happen with Ventriculoperitoneal Shunt?

Back 33

Obstruction
Infection

Front 34

What are S/S of obstruction of verntroperitoneal shunt?

Back 34

early morning HA upon waking, vomiting upon waking

Front 35

What is the most common early sign of increased ICP?

Back 35

early morning HA upon waking, vomiting upon waking

Front 36

What are S/S of Infection of verntroperitoneal shunt?

Back 36

lethargy/irritability, confusion

Front 37

What are causes of neural tube defects?

Back 37

Folic Acid deficiency in utero
Spinal cord tube does not close or fuse properly inutero

Front 38

What is a cleft lip?

Back 38

A failure of the upper lip to close, leaving a space just under the nose.

Front 39

How soon is surgery for a cleft lip performed?

Back 39

Within 3 months

Front 40

IS a cleft lip easy to detect?

Back 40

Yes, easily detected at birth

Front 41

What is a cleft palate?

Back 41

A failure of the soft, hard, or both palates to close. It may be open, leaving a passage between the oral and nasal cavities, or may be closed, in which case the missing palate will not be noticeable to visual observation.

Front 42

How easy is a cleft palate to detect?

Back 42

Sometimes not obvious because it is not outwardly visible.

Front 43

When are surgeries performed to repair cleft palate?

Back 43

usually done before 18 months.

Front 44

What causes cleft lip and palate?

Back 44

folic acid deficiency, genetic, and environmental factors.

Front 45

How is the severity of cleft lip/palate determined?

Back 45

depends on the amount of tissue involved and whether it has an impact on feeding.

Front 46

Why may a child with cleft lip/palate have trouble feeding?

Back 46

The child may not be able to generate negative pressure to allow an adequate suck.

Front 47

What problems may come with cleft lip/palate?

Back 47

dentition
speech
hearing (lots of otitis media)
respiratory
feeding
and self-concept may be effected.

Front 48

Can cleft palate be corrected with one surgery?

Back 48

It can require one or multiple surgeries to repair.

Front 49

What are post op nursing interventions after cleft lip/palate repair?

Back 49

Restraints (elbow immobilizers) for the child to keep him from putting his hands in his mouth and tearing the sutures.
Keep him supine or side-lying.
Assess vitals frequently, I/Os

Front 50

How to give oral liquids to child post-op cleft lip/palate repair?

Back 50

give them with a dropper

Front 51

What psychosocial problems may arise from cleft lip/palate?

Back 51

Parents may have anxiety and failure to attach.

Front 52

What can cause increased ICP?

Back 52

increase in brain tissue (tumor), blood (hemorrhage), or CSF. It can be caused by infection, trauma, hypoxia, poisoning, seizures, substance abuse, stroke, electrolyte, acid-base imbalance, or tumor.

Front 53

Increased ICP leads to decreased...,which means that decreased amounts of ..... are delivered to the brain

Back 53

decreased cerebral perfusion pressure
decreased amounts of oxygen and nutrients are delivered to the brain

Front 54

_________ is most important indicator of neurological dysfunction.

Back 54

LOC

Front 55

Decreased LOC can be categorized as (5 things)

Back 55

Confusion
Delirium
Lethargy
Stupor
Coma

Front 56

Confusion

Back 56

disoriented to place, time, person. Loss of clear thinking. May be able to answer simple questions

Front 57

Delirium

Back 57

Disoriented, fear, irritability. Mental/motor excitement

Front 58

Lethargy

Back 58

Profound slumber in which speech and movement are limited. Falls asleep without stimulation.

Front 59

Stupor

Back 59

Deep sleep. Only aroused with vigorous repeated stimulation usually pain, but returns to sleep when stimulus removed.

Front 60

Coma

Back 60

Unconscious, can’t be aroused even with pain.

Front 61

Early signs of increased ICP include

Back 61

HA, visual distrubances, N/V, dizziness, pupils not reactive or equal, sunsetting eyes, slight change in LOC, restlessness, irritability, bulging fontanelle, wide sutures, increased head circumference, dilated scalp veins, high pitched – catlike cry.

Front 62

Late signs of increased ICP include

Back 62

Cushings triad, significant decreased LOC, seizures, fixed and dilated pupils

Front 63

it is important to assess increased ICP ______

Back 63

early!

Front 64

How to assess increased ICP?

Back 64

Use Glasgow coma scale, lumbar puncture to detect abnormalities in CSF, CBC, urinalysis, toxicology, blood culture, EEG to assess damaged areas of the brain, CT to detect lesions, abnormalities

Front 65

How to treat increased ICP?

Back 65

Treat with O2 if gas exchange is inadequate
Correct electrolyte imbalances
Provide antibiotics for suspected infection.
If ICP is increased by obstruction of CSF, then a ventricular catheter may be inserted to relieve pressure.

Front 66

Nursing assessments for increased ICP?

Back 66

Assess cranial nerves, pupils, vitals, gag reflex, motor, eye movements to set baseline

Front 67

What will eyes look like in cases of Intracranial mass (tumor)?

Back 67

unilateral dilated and reactive pupil

Front 68

What will eyes look like in cases of Impending Brainstem Herniation?

Back 68

A fixed and dilated pupil

Front 69

What will eyes look like in cases of Brainstem Herniation from increased ICP?

Back 69

Bilateral fixed and dilated pupils

Front 70

Nursing care for increased ICP focuses on maintaining ______, monitoring ______ status, routine care, _______ stimulation, and emotional support.

Back 70

airway
neuro
sensory

Front 71

We want to Keep ICP at < __ mm Hg.

Back 71

20

Front 72

_________ or __________ solution may be used to lower ICP.

Back 72

Mannitol or
hypertonic solution

Front 73

How to position pt with increased ICP?

Back 73

If no cervical spine injury, raise the head of the bed no more than 30 degrees.

Hip flexion is avoided.

Front 74

How can pt void if experiencing increased ICP?

Back 74

Must use a urinary catheter.

Front 75

How high to keep O2 sats for pts with increased ICP?

Back 75

above 95%.

Front 76

What kind of environment for increased ICP?

Back 76

Keep environment quiet.
Pain management and temperature control are important.
Provide skin care, ophthalmic ointment, provide comforting touch, perform passive ROM exercises.