Hematology and Immunity of Children Flashcards

1
Q

reduced number of red blood cells
decreased availability of hemoglobin
reduced volume of packed red blood cells

A

anemia

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2
Q

how is anemia caused

A

blood loss or destruction of RBCs

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3
Q

inadequate suply or loss of iron

A

iron deficiency anemia

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4
Q

who are at higher risk for iron deficiency anemia

A

infancy to 36mos

adolescence

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5
Q

what are general signs of iron deficiency anemia

A
irritability
anorexia
lethargy
tachycardia
pallor
fatigue
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6
Q

in iron deficiency anemia why would an infant be over weight?

A

called “milk babies” because they are getting excessive milk intake and not enough iron is in the milk

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7
Q

what 3 interventions are used for iron deficiency

A

diet
iron supplements
blood transfusion is severe

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8
Q

when giving iron supplementation what should be given with it

A

vitamin c because it helps absorption

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9
Q

when giving iron what should you NOT give with it

A

vit d (milk) it decreases absorption

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10
Q

how should oral iron supplements be given

A

at side of mouth towards back of teeth

and brush teeth after administration!! iron causes tarry green stools too

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11
Q

boomerang looking RBCs blocking blood flow

A

sickle cell anemia

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12
Q

what are the 4 triggers to vaso-occlusion in sickle cells anemia

A

hypoxemia
dehydration
change in body temp
infection

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13
Q

can sickled RBCs reverse itself over and over

A

yes! until the point in time it stays sickeled

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14
Q

in sickle cell anemia why do we not see any symptoms until middle to late infancy

A

because the fetal hemoglobin cells do not sickle and are with them until 6mos of age

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15
Q

what are the general signs of sickle cell anemia

A

smaller in height/weight than peers
delay in sexual maturation
chronic anemia

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16
Q

how is sickle cell tested after birth

A

finger or heel stick that gets results in 6 min

sickledex

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17
Q

what are the 4 “crises” of sickle cell anemia (we will only be tested over the first one)

A

vaso-occlusive
sequestration
aplastic
hyperhemolytic

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18
Q

this crisis of sickle cell anemia is known as the “pain event or episode”

A

vaso-occlusive crisis

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19
Q

in the vaso-occlusive sickle crisis very painful and symptoms of pain are a result of

A

the obstruction at THAT SITE (lasting 4-6 days)

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20
Q

what are sickle cell crisis management

A
rest
hydrate
analgesics
lyte replacements
blood replacement (temporary)
antiobiotics
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21
Q

what medication is used for sickle cell crisis

A

hydroxyurea (increases fetal Hgb)
and short term oxygen therapy (* remember it does NOT reverse sickling but will help them breath and relax) *prolonged oxygen use can depress been marrow

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22
Q

why do vaso-occlusive crisis pts not become addicted to meds

A

because their crisis only lasts about 4-6 days

*since we are not worried about them becoming addicted, their pain meds should be admin on a schedule!

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23
Q

what is important teaching of sickle cell pts

A
seek early tx for fever 101.3 or greater
prophylactic med (penicillin and immunizations)
adequate hydration
avoid COLD compresses
avoid low O2 environments
avoid contact sports
rest
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24
Q

body makes less HGB than normal because RBCs are damaged, no beta protein, so the RBC is destroyed prematurely

A

B thalassemia aka
Cooley’s anemia aka
mediterranean anemia

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25
Q

B thalassemia kids rely on frequent ______ ______ and because of this and hemolyzed RBC we get _____ overload. ____ ______ tries to make more RBCs and there is a build up in the _____ ______ making the _____ bigger

A

blood transfusions; iron; bone marrow; bone marrow; bones

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26
Q

what does a child with B thalassemia look like

A

large head, enlarged maxilla, protrusion of the lip and upper teeth, shorter in stature
skin takes on bronze appearance (excess iron)

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27
Q

excess iron storage in tissues of the body

A

hemosiderosis

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28
Q

excess iron storage in tissues that results in cellular death

A

hemochromotosis

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29
Q

what is the goal for B thalassemia pt

A

maintain adequate HGB levels by transfusion program

*lifelong and are usually every 3-5wks

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30
Q

in B thalassemia pt what is used to remove iron from the body

A

chelating agents (deferoxamine)

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31
Q

what teaching is done for deferoxamine (removes iron from body in B thalassemia pt)

A

admin at night while sleeping for 8-10 hrs
can be IV or Subq for 5-7 days
admin with vit C
avoid iron wish foods
turns urine reddish
*deferasirox is the same thing but is PO and used for children older than 2

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32
Q

if a child with B thalassemia has a splenectomy what is important to teach the family/pt

A

contact health professional of all fevers of 101 or greater * risk for sepsis in a child with asplenia

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33
Q

what are the main differences in sickle cell anemia and B thalassemia

A

sickle cell anemia the RBCs sickle
and are characterized by crisis
B thalassemia anemia the RBCs are fragile, chronic hypoxia, and iron overload due to blood transfusion

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34
Q

what children are at higher risk for lead poisoning

A
less than 6y/o
poverty
living in older rent prop
urban setting
children with anemia
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35
Q

what are general signs of anemia

A

pallor
tachycardia
fatigue
weakness

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36
Q

how is lead poisoning assessed

A

universal screening at 1 and 2 years of age

target screening

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37
Q

why is lead poisoning so bad

A

crosses the BBB and causes severe neurological effects

*serum lead tests should be taken and if suspected then X-rays can be taken

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38
Q

if a child has <10mcg of lead in their blood what should be done

A
reassess/rescreen in 3 months
environmental assessment (ask)
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39
Q

if a child has 10-44mcg of lead in their blood what should be done

A
reassess/rescreen <3months
environmental investigation (go look)
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40
Q

if a child has >45mcg of lead in their blood what should be done

A
chelation therapy (removes lead)
consider hospitalization
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41
Q

what should nurse teach when it comes to prevention of lead poisoning

A

HH
damp mop floors and window seals
frequent washing of pacifiers and toys

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42
Q

why is chelating therapy given more than one dose and for a period of time

A

because lead moves between blood, soft tissues, organs, bones, and teeth. make sure it gets it all

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43
Q

during chelation therapy in lead poisoning how should you protect kidneys

A

adequate hydration

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44
Q

what is important to know about lead poisoning tx succimer

A

oral for 19 days and it comes in capsule or sprinkle

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45
Q

what is important to know about lead poisoning tx calcium disodium edetate

A

IM or IV but its very painful IM so you should apply EMLA an hour before injection

46
Q

prolonged bleeding anywhere from the body

A

hemophilia

47
Q

what is the hallmark sign of hemophilia

A

hemarthrosis= bleeding into a joint capsule, “target joints”

48
Q

what are symptoms of hemarthrosis (in hemophilia)

A

swelling
redness
pain
loss of movement

49
Q

what is important for you to NOT do when a child has a swollen area at a joint (due to hemophilia)

A

NEVER aspirate it will just cause more bleeding

50
Q

how can we prevent bleeding (hemophilia)

A

exercise and PT (no contact activity tho)
water pick for teeth
if IM needed hold pressure for 15 min
venapuncture instead of heel/finger sticks

51
Q

what does a child usually say they feel if they are experiencing hemophilia in certain are

A

feels pressure and “bubbling”

52
Q

for bleeding (hemophilia) what is the first thing done

A
factor replacement but while we wait RICE should be done
rest
ice
compress
elevate
53
Q

what is important to know about admin of factor replacement

A

admin 2-3x per week as prophylactic but DO NOT have it premixed because it has a short lifespan

54
Q

in a hemophilia pt what med is contraindicated

A

aspirin because it will cause them to bleed more *acetaminophen can be used

55
Q

excessive destruction of platelets and usually follows a viral illness

A

Immune thrombocytopenia

56
Q

what are symptoms of immune thrombocytopenia

A

easy bruising
petechiae
bleeding from MMM
prolonged bleeding from abrasions

57
Q

when should you restrict activities in pt who have IT

A

when their platelets are <50,000

58
Q

what meds are given for IT pt

A

steroids
immune globulin
anti D antibody
*goal of all 3 is to prolong survival rate of the existing platelets

59
Q

why is a splenectomy put off until about 5 years of age in a pt who has IT

A

to decrease chance of infection

60
Q

decreases and eventually eliminates a persons immune system

A

HIV

61
Q

for children under 13 the transmission of HIV is ______ spread

A

vertical (late in pregnancy
L and D
breastfeeding)

62
Q

why are infants born to HIV mothers test positive for HIV

A

due to antibodies from the mom (maternal antibodies persist up to 18 months)

63
Q

what are CM of HIV in children

A
lymphadenopathy
hepatosplenomegaly
oral candidiasis
chronic reoccurent diarrhea
FTT
developmental delay
parotitis
64
Q

How will HIV be managed with meds

A

ART and treating opportunistic infections (due to low immune system) *tx will be LIFELONG

65
Q

in pt with HIV what type of vaccines will they be able to receive

A

they are very immunocompromised so live vaccines should NOT be given

66
Q

what are cardinal signs of cancer in children

A
unusual mass or swelling
unexplained paleness and loss of energy
sudden tendency to bruise
persistent localized pain or limping
prolonged fever or illness
frequent HA with vomiting
sudden eye or vision changes
excessive rapid weight loss
67
Q

cancer arising from the sympathetic nervous system *more common in abdomen

A

neuroblastoma

68
Q

firm, nontender, irregularly shaped mass in the abd that CROSSES the midline

A

neuroblastoma

69
Q

why is a neuroblastoma called the silent tumor

A

because the dx has been made after it has already been metastasized to other location

70
Q

what is the preferred method to see neuroblastoma tumor

A

CT

71
Q

immature WBCs crowd out the other components of blood and depress the bone marrow production of RBCs

A

path of leukemia

72
Q

what are the symptoms of leukemia

A
pallor
listlessness
irritability
fever
anorexia
weight loss
petechiae
bruising
bone/joint pain
73
Q

what is the definitive dx of leukemia

A

bone marrow aspiration *after dx a lumbar puncture is done to see if there is brain involvment

74
Q

for pt with leukemia, the _______ the leukocyte count at dx the _______ the prognosis

A

higher; worse

75
Q

cancerous tumor arising out of the retina

A

retinoblastoma

76
Q

cat’s eye reflex is hallmark of (white pupil)

A

retinoblastoma

77
Q

what is the tx for retinoblastoma

A

radiation
removal of the eye
chemotherapy

78
Q

for pt with retinoblastoma what should you prepare the parents of

A

expect to see an empty socket after sx

79
Q

fast growing, asymptomatic, firm, lobulated mass located to ONE side of abd

A

wilm’s tumor

80
Q

why should you not palpate the abd of a child who has wilm’s tumor

A

can dislodge cells and spread to adjacent and distant tissue

81
Q

most frequent malignant bone tumor type in children

A

osteosarcoma

82
Q

what are the primary tumor sites of osteosarcoma

A

long bones, especially the legs and distal femur

83
Q

what is the definitive dx tests for osteosarcoma

A

radiologic studies and elevated alkaline phosphatase with some bone tumors

84
Q

2nd most common malignant bone tumor in children and adolescents
arises in marrow frequently in femur, tibia, ulna, humerus etc

A

ewing sarcoma

85
Q

in ewings sarcoma prognosis is best if no _______ has occurred at the time of dx

A

metastasis

86
Q

what lesions of ewing sarcoma have the best potential for cure

A

distal lesions

87
Q

what is the preferred site for bone marrow aspiration

A

posterior superior iliac spine

88
Q

what are the phases of chemo

A

induction therapy
central nervous system prophylaxis
intensification/consolidation therapy
maintenance therapy

89
Q

what is the goal for induction therapy

A

remission

90
Q

what is the goal for central nervous system prophylaxis

A

prevent leukemia cells in CSF

91
Q

what is the goal for intensification/consolidation therapy

A

destroy any residual leukemia

92
Q

what is the goal for maintenance therapy

A

remain in remission

93
Q

at the first sign of infiltration why should you immediately stop chemotherapy infusion

A

because its a sclerosing agent and can literally eat through the skin

94
Q

how can you prevent anaphylaxis to chemo

A

thorough hx of allergies
have emergency meds and mask/bag ready
recognizing hypotension, wheezing and urticaria

95
Q

what are side effects of radiation therapy

A
anorexia
nausea
vomiting
extreme fatigue
skin reactions
96
Q

changes DNA so that it cannot reproduce and is scheduled over 1-6 weeks

A

radiation therapy

97
Q

what is important to teach about radiation

A

do not try to wash mark off of site
do not expose radiated area to sun or heat
no cream or lotion on site (can misdirect radiation)

98
Q

what is point of nadir

A

bone marrow suppression at its greatest, neutrophil count at its lowest

99
Q

what do neutrophils do

A

fight bacterial infections

100
Q

an ANC below _____ warrants evaluation

A

1500

101
Q

ANC below _____ places child at greatest risk for infection

A

500

102
Q

what is the ANC formula

A

(segs+bands)xWBCx10

103
Q

prophylaxis against bacteria

A

trimethoprim-sulfamethoxazole

104
Q

prophylaxis against fungi

A

nystatin

105
Q

prophylaxis against virus

A

no live immunizations

106
Q

infection increases the tendency toward bleeding

A

thrombocytonpenia

107
Q

most likely have no conception of death

A

infants and toddlers

108
Q

death as temporary, a departure, a kind of sleep

A

preschoolers

109
Q

death is irreversible but not necessarily inevitable and may fear mutilation

A

schoolage

110
Q

death is inevitable, irreversible

A

adolescents