Test 3 Flashcards

1
Q

What is abuse?

A

actual harm, either physical, emotional, or sexual.

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2
Q

What is neglect?

A

withdrawing or not giving adequate care when they have the means.

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3
Q

What are some methods of physical abuse in children?

A

hitting, slapping, kicking, or punching. Whipping with belts, shoes or electrical cords. inflicting burns with a lit cigarette or lighter. immersing child or body part into scalding water. shaking the child violently. tying the child to fence, tree, bed, or other object. Throwing against wall, down stairs, etc. choking or gagging the child. fracturing arms, legs, or skull. Deliberately administering excessive amounts of medications/drugs. Deliberately withholding prescribed medications.

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4
Q

What are some common forms of sexual abuse?

A

oral-genital contact. fondling and caressing the genitals. anal intercourse/sodomy. sexual intercourse. rape. prostitution. forced viewing of or participation of pornography. encouraging nude photos or sexual activity via internet or video.

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5
Q

What are some risk factors for physical abuse?

A

poverty. violence in the family. prematurity or low birth weight. unrelated male primary caretaker. parents who were abused as a children. age less than 3 years. child disability or condition that requires a great deal of care. parental substance abuse or social isolation.

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6
Q

What are some risk factors for sexual abuse?

A

absence of natural father or having a step father. being female. mother’s employment outside of the home. poor relationship with parent. parental relationship characterized by conflict. parental substance abuse or social isolation.

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7
Q

What are some clinical manifestations of physical abuse in children and adolescents?

A

multiple bruises in various stages of healing. scald burns with clear lines of demarcation and in glove or stocking distribution. rope, belt, or cord marks. burn scars in various stages of healing. multiple fractures in various stages of healing. spiral fractures not explained by accident. shortness of breath and distress upon being moved, indicating chest contusions and possible rib fractures. sedation from overmedication. exacerbation of chronic illness. cranial and abdominal injuries. change in behavior or school performance. fear and avoidance of certain people or situations.

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8
Q

What are some clinical manifestations of sexual abuse in children and adolescents?

A

vaginal discharge. blood stained underpants or diapers. genital redness, pain, itching, or bruising. difficulty walking or sitting. UTI. STIs. sleeping problems. bed-wetting. new or excessive sexual curiosity or play. fear of strangers. constant masturbation. curling into fetal position. changes in eating habits. abrupt changes in behavior or school performance. excessively seductive behavior.

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9
Q

What are some clinical manifestations of neglect?

A

hungry or dirty a lot. undernourishment. unclean clothes or body. inappropriate clothing for the season. untreated medical or dental problems. poor physical growth or developmental delay. lack of supervision. being withdrawn or stealing food.

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10
Q

How do you evaluate possible abuse?

A

interview child and parent alone and together. child may hide it and not tell anyone. compare reported incident with degree of injury and child’s developmental level. and consider cultural practices.

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11
Q

What is Munchausen by proxy?

A

a psychiatric disorder where, typically the mother makes the child sick.

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12
Q

What are characteristics of Munchausen by proxy?

A

usually children under 6. symptoms disappear when parent is not present. mother seems cooperative and knowledgeable.

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13
Q

Who gets help for Munchausen by proxy?

A

the mother is proxy 90% of the time and requires psychiatric help.

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14
Q

Are nurses legally required to report suspected abuse?

A

yes

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15
Q

How do you report suspected abuse?

A

call cps or police. be judgmental and objective. no opinions. documentation is very important. use photographs and diagrams. use child’s and parents own words.

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16
Q

How do you manage child abuse?

A

treat physical injuries. provide safe environment. mental health support for child. parental support- may not be abused by parents or abuse is unknown to parents.

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17
Q

How do you prevent abuse and neglect?

A

know risk factors. identify families at risk. provide resources and support. identify and report suspected abuse and neglect.

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18
Q

What are some pediatric considerations with congenital heart defects?

A

immature heart more sensitive to volume or pressure overload = increased risk of heart failure.
unable to increase stroke volume to increased cardiac output = decreased compliance.
must increase heart rate to increase cardiac output.
mixing of blood due to defect results in desaturated blood.
chronic hypoxemia = polycythemia
severe hypoxemia = bradycardia and eventually cardiac arrest

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19
Q

What is stroke volume?

A

the amount of blood ejected from left ventricle with each contraction.

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20
Q

What is cardiac output?

A

amount of blood ejected each minute.

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21
Q

What is an issue with polycythemia?

A

more RBC = more viscous blood = increased risk for blood clots

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22
Q

Different findings in assessment with congenital heart defects?

A

respirations = increased respiratory effort. CHF =wet crackles.
Pulses and Blood Pressure = differences in right and left extremities as well differences in upper and lower extremities.
Color = cyanosis
Heart = abnormal heart sounds, murmurs
Fluid Status = CHF - you don’t want fluid retention
Activity = fatigue easily
General = weight gain

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23
Q

What is the leading cause of death in 1st year of life excluding prematurity?

A

congenital heart disease.

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24
Q

What is the most common cause of heart failure in children?

A

congenital heart disease.

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25
Q

What is a major concern with congenital heart disease?

A

altered perfusion

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26
Q

What do you need to treat prophylactically with congenital heart disease?

A

infective endocarditis.

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27
Q

What is infective endocarditis?

A

the endocardium is injured by high velocity blood flow through a stenotic valve, by turbulent blood flow across a septal defect, or by the positioning of a central venous catheter. fibrin and platelets migrate to the site of the endothelial damage, becoming the formation of nonbacterial thrombotic emboli, where the infective organisms settle.

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28
Q

What are some medications used in infective endocarditis prophylaxis?

A

amoxicillin for oral use. ampicillin for IM or IV use.

if allergic to penicillin: cephalexin, clindamycin, azithromycin, and clarithromycin.

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29
Q

What are some general symptoms of CHD?

A

vary by type and size of defect. may be asymptomatic. murmur. poor growth. tire easily. exercise intolerance. chest pain. arrhythmias. syncope.

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30
Q

What defects have increased pulmonary blood flow?

A

patent ductus arteriosus (PSA) and ventricular septal defect (VSD)

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31
Q

What defect has decreased pulmonary blood flow?

A

tetralogy of fallot (TOF)

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32
Q

What defect has obstructed systemic blood flow?

A

coarctation of the aorta (COA)

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33
Q

What defect has mixed defects?

A

transposition of the great arteries.

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34
Q

With congenial heart defects, when do they require infective endocarditis prophylaxis?

A

before and perhaps after surgery.

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35
Q

What are symptoms of increased pulmonary blood flow defects (PDA & VSD)?

A

increased HR, RR, BMR. Murmur. diaphoresis during feeds. poor weight gain. frequent respiratory infections. fluid in lungs. congestive heart failure = if not treated properly.

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36
Q

When is Patent ductus arteriosus more common?

A

pre-term babies

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37
Q

What is patent ductus arteriosus?

A

blood shunts from aorta to pulmonary arteries. L to R shunt.

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38
Q

What additional symptoms occur with patent ductus arteriosus?

A

bounding pulses. widened pulse pressure ( increase in systolic, decrease in diastolic). hypotension when cardiac output is low.

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39
Q

What is the treatment for PDA?

A

treat medically first, IV indomethacin and IV ibuprofen to inhibit prostaglandin action and stimulate the closure in premature infants. if this does not work, surgical ligation is required.

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40
Q

What happens if PDA is left untreated?

A

pulmonary hypertension.

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41
Q

What is the most common congenital heart defect?

A

Ventricular Septal Defect (VSD)

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42
Q

What is ventricular septal defect?

A

septal opening between ventricles. L to R shunt.

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43
Q

What are additional symptoms with VSD?

A

may be asymptomatic if small. can have a loud, harsh systolic murmur.

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44
Q

What is the treatment for VSD?

A

most small ones close on their own. surgery for large or if CHF develops. surgical patching or closure of the hole.

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45
Q

What are symptoms of decreased pulmonary blood flow defects?

A

murmur, hypoxia = cyanosis, clubbing. hyper cyanotic episodes ( tet spells). polycythemia. poor growth. delayed milestones. exercise intolerance. fatigue. doesn’t feed well. oxygen not getting to brain.

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46
Q

What four defects are included in tetralogy of fallot (TOF)?

A

pulmonic stenosis (primary defect).right ventricular hypertrophy. VSD. overriding aorta.

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47
Q

What is the treatment for TOF?

A

Prostaglandin E1 (PGE1) for cyanosis in newborns = keeps ductus arteriosus open allowing oxygenation of the blood. surgical repair at 1-2 years

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48
Q

What is the treatment of tet spells?

A

knee chest position, toddlers will squat. calm infant or child. supplemental oxygen.

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49
Q

What is a more aggressive treatment of tet spells?

A

IV morphine & propranolol - sedation. IV fluid bolus. Dopamine or phenylephrine.

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50
Q

What can tet spells lead to?

A

seizures, CVA, or death.

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51
Q

What symptoms occur with obstructed systemic blood flow defects?

A

murmur. diminished pulses. poor color. delayed cap refill. urine output is decreased. CHF with pulmonary edema.

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52
Q

What is coarctation of the aorta?

A

constriction in the aorta that obstructs systemic blood outflow. often in the descending aorta.

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53
Q

What are additional symptoms of coarctation of the aorta?

A

may be asymptomatic.
mild = feet cooler than hands, leg cramps.
BP lower in legs than arms. UE pulses bounding, LE weak or absent

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54
Q

What percentage of babies with COA develop CHF?

A

30% develop CHF by 3 months

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55
Q

What is the treatment of COA?

A

in a symptomatic newborn: PEG1. inotropic medications - digoxin, dopamine, milrinone. Diuretics to keep fluid levels low. oxygen. surgery includes balloon dilation or surgical resection.

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56
Q

Can coarctation of the aorta return after a surgical fix?

A

yes

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57
Q

What is the goal in the treatment of COA?

A

prevent congestive heart failure.

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58
Q

What are symptoms of congestive heart failure?

A

tires easily. poor weight gain. diaphoresis. irritability. frequent infections. respiratory symptoms - distress, productive cough, fine or coarse crackles. periorbital or facial edema. JVD in older children. cardiomegaly. weak peripheral pulses. hypotension.

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59
Q

What is the goal in treatment of CHF?

A

make heart pump more efficiently and remove excess fluid. correction of defect. supportive therapy.

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60
Q

What are some medications for treatment of CHF?

A

digoxin (lanoxin), furosemide (Lasix), thiazides (diuril). Spironolactone (Aldactone). ACE inhbitors, and Carvedilol (Coreg).

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61
Q

What is the action of Digoxin?

A

slows the heart rate, increases cardiac filling time, and increases cardiac output. .

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62
Q

When is digoxin used?

A

with increased pulmonary blood flow

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63
Q

What is the action of furosemide?

A

rapid diuresis; blocks reabsorption of sodium and water in renal tubules.

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64
Q

What is the action of thiazides?

A

maintains diuresis, decreases absorption of sodium, water, potassium, chloride, and bicarbonate in renal tubules.

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65
Q

What is the action of spironolactone?

A

maintains diuresis (potassium sparing)

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66
Q

What is the action of ACE inhibitors?

A

promotes vascular relaxation and reduced peripheral vascular resistance, reduces afterload.

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67
Q

What is the action of carvedilol?

A

improves left ventricular function, promotes vasodilation of systemic circulation.

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68
Q

When is carvedilol used?

A

for chronic heart failure and dilated cardiomyopathy.

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69
Q

What is some preoperative nursing care done for heart surgery?

A

assess and manage symptoms of CHF. monitor PGE1 therapy. manage tet spells. monitor for thromboembolism. prevent infection. reduce fatigue. optimize nutrition. optimize cognitive and physical development. administer medications. teach surgical and postop expectations. provide psychological support. teach home care.

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70
Q

What are some nursing diagnoses with heart defects?

A
risk for infection. 
decreased nutrition : less than bodily requirements.
delayed growth and development
activity intolerance/fatigue
caregiver role strain.
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71
Q

What is some postoperative nursing care done for heart surgery?

A

Provide pain control. promote respiratory function. assess for complications. manage fluids. careful I/Os. gradually increase activity levels.

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72
Q

What is some home care teaching post-op from heart surgery?

A

place infants and children in car safety seats with a small blanket over the incision to protect from straps. sponge bathe children. avoid soaking incision until sutures are out and incision is healed. do not use oils creams, lotions, or ointments on the incision. cover incision with clean shirt, keep it dry and clean. do not lift child by arms. encourage a nutritious diet. gradually increase activity as tolerated. no rough play for 6 weeks. can return to school in 3 weeks but no backpack for several weeks.give meds for prophylaxis and pain control as prescribed. report any signs of infection or complication. live virus vaccinations should be delayed.

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73
Q

Do infants have a concept of death?

A

no, but can see changes in parents

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74
Q

How do toddlers react to death?

A

cannot separate temporary abandonment and death. may react to changes in parents behavior and daily routines.

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75
Q

How do preschoolers react to death?

A

aware of body changes and deterioration. death is temporary. magical thinking, feelings of guilt if sibling dies. think they can bring someone back.

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76
Q

How do school age children react to death?

A

have increased awareness sand preoccupation with death = death anxiety.

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77
Q

How do adolescents react to death?

A

death is difficult. doesn’t want to be dependent or different. hard for this age group to accept own death.

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78
Q

What are fontanels?

A

fibrous membranes in the skull that allow for growth of the brain and skull.

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79
Q

When do fontanels close?

A
anterior = 18 months
posterior = 2-3 months
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80
Q

When does the fontanels stop fusing?

A

2 years of age

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81
Q

When is the skull full size?

A

16 years of age

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82
Q

What are some pediatric differences in the musculoskeletal system?

A

fontanels
long bones are porous and less dense = high fracture rates
rapid bone growth facilitates healing.
ends of long bones are cartilaginous, growth occurs and epiphyses.
ligaments and tendons are stronger than bone until puberty

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83
Q

What do you assess in the musculoskeletal system?

A

muscles, joints, bones, tendons, ligaments, mobility, range of motion, limitations, symmetry, and length.

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84
Q

What is club foot (talipes equinovarus)?

A

congenital abnormality of foot position, etiology unknown. can be unilateral or bilateral

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85
Q

What are the three deformities of club foot?

A

forefoot - equinus (toward the heel)
midfoot - supination (downward)
hindfoot - varus (inward)

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86
Q

How is club foot diagnosed?

A

ultrasound at 16-20 weeks gestation
appearance at birth
xray to determine severity.

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87
Q

What is the treatment of club foot?

A

in utero - nothing
tx occurs after birth, the sooner the better, before ossification of the bones.
serial casting is the treatment of choice - long leg casts changed weekly.
surgery if needed.

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88
Q

What is developmental dysplasia of the hip (DDH)?

A

femoral head & acetabulum are improperly aligned. not always a true dislocation. etiology unknown. can be unilateral or bilateral. formally called congenial dislocated hip.

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89
Q

What are come characteristics of DDH?

A

limited abduction of affected hip. positive ortolani-barlow maneuver. “hip click.” asymmetry of thigh and gluteal fat folds. telescoping of the thigh (allis sign), and limp in older untreated child.

90
Q

What is ortolani-barlow maneuver

A

done by Doctors. “hip click” heard when legs are rotated out and pulled and/or rotated in and pushed.

91
Q

What is allis sign/

A

child laid on back, knees bent & together, affected leg lays shorter than the other.

92
Q

How is DDH diagnosed?

A

physical exam, ultrasound, xray (not always reliable at birth), and/or ortolani-barlow exam

93
Q

What is the treatment for DDH?

A

pavlik harness. skin traction, usually Bryant traction. surgery. spica casts. braces.

94
Q

When is skin traction (Bryant traction) used?

A

<3 years old, <30 lbs. butt off of bed. only temporarily effective. child can not be left alone. done prior to surgery.

95
Q

What is the pavlik harness?

A

most common treatment <6 months old. holds joint in place. worn 23 hours a day.

96
Q

What is scoliosis?

A

a lateral s or c shaped curvature of the spine. may have a rotational deformity. is progressive. can be idiopathic or compensatory.

97
Q

Who is more likely to experience scoliosis?

A

girls during a growth spurt

98
Q

At what age due vertebrae reach maturity?

A
females = 15 years old
males = 17 years old
99
Q

What are some characteristics of scoliosis?

A

asymmetry of trunk. uneven hip and shoulders. rib hump. prominent scapula. narrowed disk spaces. no pain initially.

100
Q

How is scoliosis diagnosed?

A

physical exam and xrays. MRI, CT, and/or bone scan.

101
Q

What is the goal of treatment of scoliosis?

A

limit or stop progression of the curvature.

102
Q

What is the treatment of mild scoliosis?

A

physical rehabilitation with exercises

103
Q

What is the treatment for moderate scoliosis?

A

TLSO brace 23 hours/day (boston, Milwaukee, spinecor)

104
Q

What is the treatment for severe scoliosis?

A

spinal fusion with rods/wires. halo traction for unstable spine.

105
Q

What are some nursing diagnoses with musculoskeletal disorders?

A
impaired physical mobility
risk for impaired skin integrity
risk for delayed growth and development
risk for altered elimination
risk for disturbed body image
risk for injury
106
Q

What are some teaching points with brace care?

A
comfort, mobility
increase wearing time slowly
skin and circulation assessments.
thin liner under brace, no wrinkles.
no powders or lotions.
monitor for wear and tear
107
Q

What are some teaching points with cast care?

A

24-48 hour drying time. handle using hands, not fingers. position at level of heart or higher. monitor drainage or bleeding. frequent NV and skin assessment. petaling of rough edges. keep clean and dry - sponge baths or wrap in plastic. do not put anything in cast. call doctor if loss of feeling, sudden pain that doesn’t subside with pain meds, bleeding, s/s infection, negative NV check, foul smelling.

108
Q

What is some post op care for musculoskeletal disorders?

A

same as routine cast care plus: more frequent NV checks. ice packs. pain management. activity restrictions.

109
Q

What are some considerations with musculoskeletal disorders?

A

respiratory assessments. NV assessments. immobility concerns. position changes. hydration/nutrition. safety/adaptions, and growth and development.

110
Q

What is the role of the endocrine system?

A
fetal sexual differentiation - 7-8 weeks gestation.
growth and development
reproductive processes
metabolism
behavior
111
Q

What are some disorders of the pituitary function?

A

growth hormone deficiency - short statured kid
growth hormone excess - rare in children
constitutional delay of puberty - late bloomer
precocious puberty - early than 8 or 9 y/o

112
Q

What is growth hormone deficiency (or hypopituitarism)?

A

decreased activity of pituitary gland. diagnosed earlier in boys than girls because boys have a greater growth rate.

113
Q

What are some causes of hypopituitarism?

A

infarction of gland, CNS infection, or tumors.

114
Q

What does hypopituitarism lead to?

A

delayed development and short stature.

115
Q

What are some initial characteristics of hypopituitarism?

A

birth weight & length normal. by 1 year, below the 3rd percentile. grow < 5cm/year

116
Q

What are some characteristics of hypopituitarism in infants ?

A

hypoglycemic seizures. jaundice. undescended testicles. hyponatremia. micropenis.

117
Q

What are some characteristics of hypopituitarism in children?

A

short stature. youthful facial features. delayed tooth development. delayed skeletal & sexual maturation. overweight with abdominal fat. higher pitched voice. decreased muscle mass.

118
Q

How is hypopituitarism diagnosed?

A

physical assessment and evaluation. low levels of IGF-1 (insulin growth factor 1). decreased bone age on radiologic exam. provocative growth hormone testing.

119
Q

What is the treatment of hypopituitarism?

A

treat cause of problem.
growth hormone replacement- somatropin, nutropin. SQ injections daily, or every other day until acceptable height is reached or growth slows less than 1 inch/year or advanced bone age (around puberty).
meds to delay onset of puberty if goal isn’t met.
monitor hormone levels.

120
Q

What causes growth hormone excess ( or hyperpituitarism)?

A

may be pituitary adenoma, but its rare in children.

121
Q

What are some characteristics of hyperpituitarism?

A

accelerated linear growth - may reach 7 or 8 feet if it occurs before puberty. acromegaly may occur if increased growth occurs after closure of epiphyseal places.

122
Q

How is hyperpituitarism diagnosed?

A

diagnosis may be delayed because culturally we value tall stature. increased IGF-1 levels. increased bone age on radiologic exam. xrays to detect tumor.

123
Q

What is the treatment for hyperpituitarism?

A

dependent upon cause. possible removal of pituitary gland - may need life long replacement afterwards. radiation therapy. high doses of sex steroids to close epiphyseal plates.

124
Q

When is the normal puberty age for girls?

A

8-13

125
Q

When is the normal puberty age for boys?

A

9.5-14

126
Q

What is constitutional delay of puberty?

A

known as “late bloomers” not a medical issue. often hereditary. causes low self esteem.

127
Q

What are characteristics of constitutional delay of puberty in girls?

A

no breast development by age 13; no pubic hair by age 14; or no menarche within 4 years of breast development.

128
Q

What are characteristics of constitutional delay of puberty in boys?

A

no testicular enlargement or scrotal changes by 14; no pubic hair by 15; or lack of genital growth within 4 years of testicular enlargement.

129
Q

What is the cause of constitutional delay of puberty?

A

most often hereditary or runs in families. check family history. sometimes caused by hypogonadism.

130
Q

What is the complications of constitutional delay of puberty?

A

short stature and emotional stress.

131
Q

How is constitutional delay of puberty diagnosed?

A
family history is important. 
bone age radiologic exam.
physical assessment.
FSH &amp; LH blood levels - decreased menarche in females
CBC &amp; thyroid function tests
132
Q

How is constitutional delay of puberty treated?

A

usually doesn’t require treatment. emotional support important.
extreme delay: boys - testosterone injections
girls- oral ethinyl estradiol & medroxprogesterone

133
Q

What is precocious puberty?

A

pituitary hormones stimulate the gonads prematurely. the appearance of any secondary sexual characteristics : before age 8 in girls, before age 9 in boys.

134
Q

What are characteristics of precocious puberty?

A

advanced bone and sexual maturity. appear tall for age initially, eventual short stature (hormones close epiphyseal plates). mood swings. emotional labiality.

135
Q

How is precocious puberty diagnosed?

A

physical examination. increased hormone levels. increased bone age. GnRH stimulation test. brain x-ray to rule out tumor.

136
Q

What is the treatment for precocious puberty?

A

monitor growth patterns if close to normal puberty age.
GnRH analog - to balance where they should be.
Lupron (leuprolide acetate) injections monthly
Synarel (nafarelin acetate) intranasal 2x/day
treatment of tumor if present

137
Q

What is turner syndrome?

A

most common sex chromosome abnormality in females. missing, abnormal, or partial X chromosome. ( 45, XO)

138
Q

What are the characteristics of turner syndrome in infants? (may not exhibit all)

A

lymphedema of the hands and feet. webbed neck. low hair line. low set ears. high-arched palate.

139
Q

What are the characteristics of turner syndrome in children? (may not exhibit all)

A

short stature. lack of breast development. puberty delay. amenorrhea. typically sterile, all eggs gone by age 2. scoliosis, broad chest. pigmented nevi. typically do well growing, will have a full life.

140
Q

How is turner syndrome diagnosed?

A

prenatal testing. karyotype. testing for other abnormalities.

141
Q

How is turner syndrome treated?

A

monitoring growth - special chart for disorder
growth hormone therapy at 1-2 years until bone age of 14.
anabolic steroids to promote growth
estrogen started in preteen years

142
Q

What is klinefelter syndrome?

A

an extra X in boys. ( 47, XXY)

143
Q

Klinefelter syndrome is the most common cause of what issues?

A

hypogonadism, androgen deficiency, and male infertility.

144
Q

When is klinefelters often diagnosed?

A

when puberty is delayed or with more school performance unless found earlier in genetic testing.

145
Q

What are characteristics of klinefelter syndrome?

A

tall and thin with long arms and legs. delayed or abnormal progression of puberty. decreased testicular size, decreased facial an body hair. gynecomastia. speech & reading delays, memory problems. other anomalies possible

146
Q

How is klinefelter syndrome diagnosed?

A

chromosomal analysis

147
Q

What is the treatment for klinefelter syndrome?

A

testosterone IM injections. start at 11-12 years old. every 3-4 weeks. no stopping injections.

148
Q

What is the goal in the treatment of klinefelter syndrome?

A

stimulate masculinization

149
Q

What is phenylketonuria?

A

disorder of amino acid metabolism. deficient phenylalanine hydroxylase to convert phenylalanine into tyrosine.

150
Q

What occurs if phenylketonuria is left untreated?

A

brain damage, intellectual disability, microcephaly, and growth retardation.

151
Q

What are characteristics of phenylketonuria?

A

musty body and urine odor. vomiting. seizures. hyperactivity. irritability. hypertonia. eczema-like rash

152
Q

How is phenylketonuria diagnosed?

A

Newborn PKU screening required in all states

153
Q

What is Guthrie blood test?

A

newborn metabolic screen

154
Q

When is the Guthrie blood test performed?

A

at least 48 hours of age or repeat at 1-2 weeks, baby must have been fed.

155
Q

What is the treatment for phenylketonuria?

A

specialty formulas. elemental medical foods with modified proteins. low phenylalanine diet for life. avoid high protein foods and aspartame - meat, fish, poultry, nuts, beans, noodles, eggs, etc.

156
Q

What is galactosemia?

A

disorder of carbohydrate metabolism. body cant convert galactose to glucose. metabolites build up and cause damage.

157
Q

What are some early signs of galactosemia?

A

poor suck. failure to gain weight related to vomiting and diarrhea. hypoglycemia. enlarged liver.

158
Q

What are later signs of galactosemia?

A

intellectual disability. jaundice. ascites. sepsis. lethargy. seizures. hypotonia. cataracts. coma.

159
Q

How is galactosemia diagnosed?

A

neonatal screening. labs = elevated galactose, AST, ALT.

160
Q

What is the treatment of galactosemia?

A

without treatment they may die within one month.
lactose or galactose free formula.
lifelong galactose-free diet. no milk or cheese products.

161
Q

What is maple syrup urine disease?

A

disorder of amino acid metabolism. alpha-ketoacid dehydrogenase deficient. 3 essential amino acids cannot be metabolized. higher rates in Pennsylvania Mennonites. build up can cause brain damage, neurologic impairment, & death.

162
Q

What are the characteristics of maple syrup urine disease?

A

poor sucking. poor appetite. irregular respirations. variable muscle tone. ketoacidosis: maple syrup odor of body fluids. seizures. apnea. death.

163
Q

How is maple syrup urine disease diagnosed?

A

neonatal screening. labs - urine and blood

164
Q

What is the treatment for maple syrup urine disease?

A

removal of metabolites from body - may go on dialysis.
lifelong - specially designed medical formulas and food rich in other amino acids.
daily urine ketones. any stressful situation can cause ketoacidosis.

165
Q

What is diaper dermatitis?

A

mainly in babies. irritant contact dermatitis. inflammatory reaction to urine, feces, moisture, and friction. decreased in breast fed babies related to lower pH of stool.

166
Q

What are the characteristics of diaper dermatitis?

A

glazed (shiny) red plaques on skin in contact with diaper. severe - fiery red, raised rash. may have pustules and be very tender. skin folds are not affected.

167
Q

What is a complication of diaper dermatitis?

A

secondary infection of candida albicans. bright red, scaly patches with sharp margins. present in skin folds and has satellite lesions. papules and pustules may be present. oral thrush may occur.

168
Q

What is the treatment for diaper dermatitis?

A

Do:
frequent diaper changes, using mild soap & water with BM. super absorbent diapers. barrier products- zinc oxide & mineral oil. expose to air as much as possible.
Don’t:
use baby wipes. use soap with every diaper change. use baby powder.

169
Q

What is atopic dermatitis (eczema)?

A

chronic, superficial inflammatory skin disorder characterized by intense itching.

170
Q

What is the cause of eczema?

A

genetic predisposition. altered immune response. xerosis. environmental triggers.

171
Q

What are characteristics of eczema?

A

pruritis. erythematous patches. papules, vesicles. exudate, and crusts. dry, cracking skin.
over time, chronic: scaling, excoriation. lichenification. pigment changes.

172
Q

What behavior problems can pruritis lead to?

A

sleep interruptions. irritability. appearance of hyperactivity. self-image disturbance.

173
Q

What is the treatment for atopic dermatitis?

A

hydration and lubrication is key.
warm water baths 2x day during flair ups. avoid harsh soaps.
apply occlusive emollients after bath to seal in moisture.
moisturizers 3-4 x day. no perfumes.
avoid environmental triggers.
wet wraps during acute flares.
hypoallergenic formula or breast milk.

174
Q

What are the medications used to treat atopic dermatitis?

A

topical corticosteroids. PO antibiotics as needed for infection. antihistamines for sleep. immunomodulators.

175
Q

What is acne?

A

most common skin disorder in adolescents related to increased androgen production and overproduction of sebum.

176
Q

What is the cause of acne?

A

interaction between increased sebum production, abnormal follicular shedding, and P. Acnes bacteria.
certain medications, oil-based cosmetics, and friction.

177
Q

What are characteristics of acne?

A

comedones- blackheads and white heads.

papules & pustules. nodules & cysts.

178
Q

What is the goal of treating acne?

A

decrease lesions, scarring, and infection.

decrease emotional stress.

179
Q

How do you treat acne?

A

use gentle cleanser twice a day. leave lesions alone. medications.

180
Q

What are the medications for acne?

A

keratolytics (benzoyle peroxide, azelaic acid, sulfur, and salicylic acid)- breaks down comedones.
antibiotics- oral or topical x 6-8 weeks
retinoids ( tretinoin, adapalene, and tazarotene)- slows desquamation and decreased adherence of shedding cells which decreases follicular plugging.
Accutane - severe cases
oral contraceptives
spironolactone - decreases sebum production, works well with OCP

181
Q

What is impetigo?

A

highly contagious bacterial infection. usually on the face, around mouth, hands, neck, and extremities.

182
Q

What is the cause of impetigo?

A

occurs on “damaged skin” self inoculation

183
Q

What are characteristics of impetigo?

A

papules progress to vesicles. vesicles rupture forming erosion. serous drainage result in crusting. pruritius.

184
Q

How do you diagnosis impetigo?

A

physical exam - honey colored crusts.

bacterial culture

185
Q

What is bullous impetigo?

A

caused by staph aureus. vesicles coalesce to form bullae. usually in moist skin folds.

186
Q

What is the treatment for impetigo?

A

soak lesions & gently scrub with antiseptic soap to remove crusts. topical antibiotic x 5-7 days. severe cases need oral antibiotics. if no improvement in 24 hours, notify MD. may return to school 24 hours after treatment. linens - wash in hot water, do not share. sanitize toys. keep fingernails short and clean.

187
Q

What is dermatphytoses (ringworm)?

A

a fungal infection that can be spread by person, animal, or contaminated objects.

188
Q

What is ringworm on the scalp called?

A

tinea capitis

189
Q

What is ringworm on the skin ( trunk, face extremities) called?

A

tinea corporis

190
Q

What is ringworm on the nails called?

A

tinea unguis

191
Q

What is ringworm on the scrotum called?

A

tinea cruris or jock itch

192
Q

What is ringworm on the feet called?

A

tinea pedis or athletes foot

193
Q

What is scabies?

A

a mite burrows into the epidermis and leaves eggs and body wasted. causes intense itching. threadlike linear lesions may be evident.

194
Q

How is scabies treated?

A

permethrin lotion on the entire body, keep lotion on for 8-10 hours. mites are then killed, but itching continues for several weeks. retreatment is necessary in one week to kill newly hatched mites. treat entire family even if not symptomatic, wash bed, clothes, and other shared items.

195
Q

What is pediculosis capitus?

A

head lice. they bite which cause itching. nits (eggs) laid along hair shaft.

196
Q

How do you treat pediculosis capitus?

A

eradication of head lice is most dependent on the removal of nits. shampoo with permethrin 1% crème rinse or Nix. comb through hair to remove nits. needs to be repeated in 1-2 weeks. wash all bed clothes.

197
Q

How is pediculosis capitus spread?

A

through sharing of hair accessories, hats, sport helmets, etc.

198
Q

What are pediatric specific hematologic findings?

A

newborn has increased hct and hgb. (hct 45-65)
RBC, hgb fall until 2-3 months of age
fetal hgb slowly decrease after birth
blood volume = 85 mL/kg
clotting factors low in newborn, need vitamin K

199
Q

What is anemia?

A

blood lacking healthy RBCs or hgb.

200
Q

What causes anemia?

A

decreased red blood cell production
loss of red cells by hemorrhage
early death (destruction) of red blood cells

201
Q

What is sickle cell anemia?

A

a disorder of the blood that causes the RBCs to sickle under stress, they clump together during a crisis

202
Q

What are the clinical manifestations of sickle cell anemia?

A

affects all body systems - symptoms related to obstruction of blood flow & destruction of RBCs.
asymptomatic until 4-6 months old.
anemia and jaundice.
pain - acute and chronic

203
Q

What are some sickle cell anemia complications?

A

infection - spleen often nonfunctional due to infarct as early as age 5.
stroke - or TIA
acute chest syndrome - sudden SOB & chest pain
renal failure
cardiomegaly

204
Q

What are the types of sickle cell anemia crises?

A

vaso-occlusive (most common)
splenic sequestration (less common but life threatening)
aplastic crisis

205
Q

What is vaso-occlusive crisis and its cause?

A

a pain crisis, caused by tissue hypoxia and necrosis

206
Q

What is splenic sequestration?

A

blood pools in spleen. causes profound anemia, hypovolemia, and shock.

207
Q

What is aplastic crisis and its cause?

A

caused by infection, exhibits profound anemia, pallor, an fatigue.

208
Q

What causes sickle cell crises?

A
fever and infection.
hypoxia
dehydration
cold environment
strenuous physical activity/exercise
fatigue or emotional stress
environment low in oxygen content
209
Q

What is the management of Sickle Cell Crisis?

A
pain management. 
adequate hydration. 
oxygen, if hypoxic only.
prevent/treat infection 
blood transfusions
prevent/treat complications
210
Q

What are some things you should teach the family of a child with sickle cell anemia?

A
known precipitating factors and avoid.
managing pain at home.
avoid infections, treat immediately.
check ups, immunizations.
medications.
inform all HCP; schools; daycare; etc.
211
Q

Who is most affected by hemophilia?

A

x-link recessive. occurs in 1:5000 male births. females can carry, but males affected. passed from mother to son. carriers may have mild symptoms.

212
Q

What are clinical manifestations of hemophilia?

A

spontaneous bleeding. hemarthrosis (bleeding into joints). deep tissue hemorrhage. nose bleeds. easy bruising. hematuria. prolonged bleeding after procedures.

213
Q

When is hemophilia often noticed?

A

with circumcision or around 6 months when infant is more active and prone to injury.

214
Q

What are some complications with hemophilia?

A
joint deformities &amp; contractures
internal hemorrhaging
transfusion reaction
hypovolemic shock
death.
215
Q

How is hemophilia medically manage?

A

factor VIII - given with any bleeding episode.
DDAVP (desmopressin acetate) - for mild to moderate bleeds
Recombinant factor concentrate - from animal cells, free of viruses and unwanted proteins

216
Q

What is some nursing care done with hemophilia patients?

A

prevent & control bleeding: no rectal temps. avoid IM/SC. heel stick instead of venipuncture. no heparin flush. no contact sports. protective gear. electric razor. get replacement regularly. treat superficial bleeding with pressure x 15 minutes, elevate, ice packs, immobilize.
limit joint involvement and limit pain, no aspirin or NSAIDS.
emotional support

217
Q

What is leukemia?

A

overproduction of immature WBCs, they crowd out good blood components

218
Q

What are causes of leukemia?

A
not well understood.
exposure to infectious agents -virus
genetic factors - common in children with downs
ionizing radiation
chemotherapy agents
219
Q

What are symptoms of leukemia?

A

pallor, fatigue, nose bleeds, bleeding gums, enlarged liver and spleen, bruising, petechiae, fever, joint or bone pain.

220
Q

What is the objective of chemotherapy?

A

to complete remission

221
Q

What are the 4 phases of chemotherapy?

A
  1. induction
  2. consolidation
  3. delayed intensification
  4. maintenance of remission