pediatic Hematology Flashcards

1
Q

New born normal range for Hgb

A

14-24

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2
Q

Infant norm range for Hgb

A

10-17

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3
Q

Child Hbg norm range

A

9.5-15.5

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4
Q

Adult Hgb norm ranges

A

12-18

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5
Q

Diet for infants with IDA (iron deficiency anemia)

A

Iron fortified formula or cereal

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6
Q

Dietary sources for an older child with IDA

A

Fish, liver, whole grains, lagoons, green leafy veggies, other cereals with iron

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7
Q

Oral iron supplement

A

Ferrous sulfate ( fer-in-sol )

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8
Q

Iron admin

A

Give on an empty stomach
Give w citrus juices (vit c)
Use a dropper or straw
Stools will be tarry
Safety
Do no give with milk

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9
Q

What do we want to teach parent about iron supplement ?

A

Can upset stomach
Cause black tarry or dark green stools, cause constipation, so increase fluids and fiber

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10
Q

How long does it take to increase RBC for IDA?

A

3 mo
(Takes 120 days for RBC to mature)

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11
Q

Why Do we give fruit juices (vit c ) with iron admin

A

Vit c increases absorption of iron into blood stream so blood can deliver it

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12
Q

Why Do we use a dropper or straw for iron admin

A

Stains teeth

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13
Q

What can we do to prevent over dose of iron supplement ?

A

Locked safely and out of reach for children

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14
Q

Why shouldn’t you give an iron supplement with milk?

A

Will decrease iron reabsorption

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15
Q

Sickles cell RBC life span

A

< 40 days

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16
Q

Why are we so concerned with anemia in children?

A

Diminished cognitive function
Behavioral changes
Delayed growth & development
Death can be a result

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17
Q

What populations are at highest for sick cell anemia

A

African American
Males > females

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18
Q

Triggers for sickle cell anemia

A

Dehydration
Acidosis
Hypoxia
Temp changes( cold)
Infection

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19
Q

Why don’t you see s/s of sickle cell in new borns

A

Born w 80% fetal Hgb and it does not sickle

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20
Q

When do you start seeing signs and symptoms of sickle cell in babies

A

At around 6 mo

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21
Q

What can a sickle cell crisis be caused by

A

Sickness , dehydration, cold weather w/o gloves or coat

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22
Q

PCA pump opoids for Vasooclusive crisis for severe pain

A

Morphine
Hydromorphone
Hydrocodone
Ketolorac

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23
Q

Ketorolac

A

Visceral pain in middle of body
Works as well as narcotic
Given for VOC pain management

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24
Q

Hydroxyurea

A

Increases fetal hemoglobin production
Given for VOC

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25
Q

Why is meperidine (Demerol) contra indicated for vasooclussive crisis

A

Causes seizures

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26
Q

Mild to moderate painVOC

A

Tylenol ibuprofen

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27
Q

Someone with a Beta thalassemia major RBC lifespan

A

30-60 days

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28
Q

Clinical manifestations of IDA

A

Pallor/ paleness of mucous membranes
Tiredness fatigue

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29
Q

Possible findings with a child with IDA

A

Over weight “milk baby”
Dietary intake low in iron
Milk intake over 32 ox a day
Pica habit

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30
Q

Pica habit

A

Body craving other sources of iron
Eating iron and clay

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31
Q

High risk populations of IDA

A

Preterm infants , multiple births
Mother has iron deficiency
Infants 6-24 mo
Toddlers
Female adolescents

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32
Q

how long does it take for oral iron to increase the RBC count in IDA?

A

3 mo for mature RBC to get out

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33
Q

Why would someone with IDA have a repeat h& h done in a month

A

To observe reticulate count to see generating of RBC

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34
Q

In a pt diagnosed with IDA who has to have a repeat H&H in a month and turns out it hasn’t improved what could that mean?

A

Will further investigate to see if there is an iron absorption issue or chronic bleeding

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35
Q

What do you give to pediatric pt with severe anemia and what also may you add IF tissue hypoxia is severe?

A

Blood transfusion of packed RBC to minimize chance of circulatory overload

Supplemental oxygen

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36
Q

Percentages of autosomal recessive emetic order sickle cel anemia

A

25% chance of not being a carrier or having disease
25% chance they will have disease
50% chance they will be a carrier

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37
Q

How to test prenatal for sickle cell anemia

A

Chorionic villas sampling from prenatal tissue
Amniocentesis

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38
Q

After birth diagnostic testing for sickle cell anemia

A

New born screening
Sickledex (sickle cell turbidity test) screening)

And if positive electrophoresis ( confirmative results)

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39
Q

General signs of chronic hemolytic anemia (sickle cell)

A

Frequent infections
Fatigue
Delayed physical growth

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40
Q

VOC

A

Vasoocculsive crisis
Peripheral
Pain crisis ischemia causing pain
(Cells crying for o2)
Most observed sickle cell crisis
Last 4-6 days

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41
Q

Splenic sequestration crisis

A

Will cause drastic decrease in blood volume
Can cause Hypovolemia
If not treated- within hours death can occur
Commonly seen @ ages 6 mo - 5 years

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42
Q

Aplastic crisis

A

Can be triggered by a viral illness = decrease production of RBC

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43
Q

Hyperhemolytic crisis

A

Something has made the body accelerate the rate of RBC destruction

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44
Q

Acute exacerbations of sickle cell crisis

A

Vasoocculsive
Aplastic crisis
Hyperhemolytic crissi
Splenic sequestration crisis

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45
Q

VOC classic or acute signs

A

Acute pain (15 out of 10)
Fever
Severe abdominal pain
Hand foot syndrome in infants
Arthralgia (painful joints)
Leg ulcers (adolescents)
Cerebrovascular accident

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46
Q

Why would someone with VOC be having severe abdominal pain

A

If vassoclusive happens in the abdomen and it may be masked as appendicitis

47
Q

Hand foot syndrome in infants

A

If VOC happens in hands or feet - painful edema

48
Q

Why would leg ulcers happen in an adolescent with VOC

A

Impaired circulation

49
Q

Why would CVA happen in VOC

A

Due to blockage

50
Q

Chronic signs of VOC

A

Splenomegaly
Hepatomegaly
Kidney abnormalities
Bone changes
Retinal detachment

51
Q

When someone has constant VOC and splenomegaly occurs what can that eventually lead to

A

Autosplenectomy

When spleen enlarges =repeated insults of spleen= eventually body kills off spleen

52
Q

When an autosplenectomy occurs after several VOC what does this put a child at risk for?

A

Increase risk infection

53
Q

Hepatomegaly can occur with VOC what can this put a child at risk for

A

L risk for liver damage leading to liver failure

54
Q

What kind of kidney abnormalities can occur with chronic VOC if damage to the kidneys happens

A

Hematuria
Inability to concentrate urine , enuresis, possible renal failure =

55
Q

What kind of bone changes associated with chronic signs of VOC can happen

A

Osteoporosis
Skeletal deformities
Weakened bones

56
Q

If retinal detachment happens due to chronic VOC what can it lead to

A

Blindness

57
Q

Acute chest syndrome is a complication that can happen when occlusion happens within the chest (VOC). What are some s/s associated with acute heat syndrome ?

A

Fever >101.3
Cough (due to blockage flow2lungs)
Chest pain
Tachypnea (increase RR)
Dyspnea
Wheezing
Decrease O2 sat

58
Q

What are the treatments for acute chest syndrome?

A

Antibiotics
Administration of o2
Breathing excercises
Blood transfusion

59
Q

What is the goal for VOC management

A

Manage symptoms and reverse excess sickling

60
Q

VOC crisis management

A

Hydration (prevent dehydration)
Electrolyte replacement
Strict I&O
Analgesics (for severe pain)
Warm compresses
Blood transfusions
Antibiotics
Hydroxyurea

61
Q

Why do we want strict I&O during VOC management

A

Watching for kidney impairment

62
Q

What does warm compresses benefit during VOC management and why dont we want to do cold compress

A

To vasodialate blood vessels and that will make sickle cells flow more easily

If we use cold it will vasoconstriction blood vessels and contraindicates because cold is a trigger of VOC

63
Q

When teaching parents how to avoid triggers for VOC what are some teachings we can include

A

Avoid strenuous exercise (leads2dehydration)
Avoid high altitudes (cold places)
Avoid being around sick folks
Seek care at first sign of infection
Use prophylactic penicillin if prescribed
Keep child wheel hydrated

64
Q

Why would prophylactic treatment be prescribed to avoid VOC

A

Prevents streptococcus

65
Q

For VOC prevention if a person is allergic to penicillin what can we prescribe

A

Erythromycin

66
Q

T or f do not withhold fluids from a child at night in regards to preventing VOC

A

True.
We want to ensure hydration

67
Q

What are other things we can do to prevent VOC (3)

A

Up 2 date immunizations
Refer fam for genetic counseling
Provide emotional support

68
Q

Prognosis of sickle cell

A

Most people live to be in their 50s
Greatest risk for pt under 5 to have a crisis
Death due to overwhelming infection

69
Q

Beta thalassemia major

A

Life threatening anemia
Ultimately increase destruction of RBC
Also my produce fetal Hgb in adulthood

70
Q

Beta thalassemia major RBC life span

A

30-60 days

71
Q

Clinical manifestations of beta thalassemia at first

A

Pale, fussy , poor apetite and a lot of infections related to anemia

72
Q

When does beta thalassemia typically show up

A

Within first 2 y of life

73
Q

What lab signs will be increased with bet thalassemia

A

Retic count

74
Q

What does severe anemia cause (beta thalassemia)

A

Chronic hypoxi
Small stature (FTT)
adolescents (delayed puberty)
Bronzed skin tone
Hepatosplenomegaly
Cardiomegaly
Bone changes

75
Q

Why do you see bronzed skin tone in beta thalassemia ?

A

High bilirubin (bile)

76
Q

Why is liver enlarged in beta thalassemia

A

Due to liver storing excess iron from chelation

77
Q

Why is spleen enlarged in beta thalassemia

A

And the spleen is hyperactive due to pooling of blood RBC die faster spleen works harder. Also RBC gets stuck in spleen due to small and misshaped

78
Q

In untreated children in beta thalassemia what kind of bone changes do we see?

A

Prominent frontal bossing
Changes to nose, and maxilla , jaw bones
Generalized osteoporosis

79
Q

Prenatal Testing for beta thalassemia

A

Chorionic villus
Amniocentesis

80
Q

After birth diagnostic testing for beta thalassemia

A

No screening if positive-
CBC (to see chronic anemia )
But ultimately hemoglobin electrophoresis
X-rays to see bone involvement

81
Q

Goal of children with beta thalassemia major ?

A

To keep hemoglobin above 9.5 gm/dl

82
Q

How often would a child with beta thalassemia major receive packed red blood cellls

A

Every 3-5 wks

83
Q

Advantages of packed RBC of beta thalassemia major

A

Improve well being (phys&mental)
Decrease cardiomegaly/hepatosplenomegaly
Prevent bone changes
Promote normal or near normal growth and development (avg growth chart)
Decrease infection

84
Q

Beta thalassemia major

A

Cooley anemia

85
Q

What is the safe level of lead in the blood

A

There is no safe level

86
Q

What can lead cause

A

Anemia

87
Q

How can we get lead into our blood stream by ingestion

A

Crib rails
Window seals
Older plumbing(water source)
Contaminated soil(drop.)
Paint chips
Etc

88
Q

How can we get lead into our blood stream by inhalation

A

Saying, lead pain in nearby vicinity

89
Q

General sings of lead exposure

A

Anemia s/s
Cramping , Abdominal

90
Q

What age is a risk factor for lead poisoning

A

Younger than 6 ( age when they put things in their mouth

91
Q

What population is also at risk for lead poisoning

A

Living in poverty
living in Urban areas
Living in older homes
Children with anemia

92
Q

Why are children with anemia more prone to lead poisoning

A

Led binds to hemoglobin = at risk for getting more led

93
Q

Clinical manifestations of led poisoning

A

Anemia
Cramping , abd pain
Vomiting
Constipation
Anorexia
Headache
Lethargy
Impaired growth

94
Q

Early CNS manifestations of led poisoning

A

Hyperactivity
Agggression
Impulsiveness
Decreased interest in play
Irritability
Short retention span

95
Q

Late manifestations of lead poisoning

A

Mental retardation (decreased IQ)
Paralysis
Blindness
Convulsions
Coma
Death

96
Q

Universal screening for lead poisoning

A

Blood lead level test BLL

97
Q

When does a BLL get done

A

Children at ages 1 and 2
Any child who hasn’t got screened at ages 3 and 6 years if never screened

98
Q

Target screening AD LIB)

A

Will always be screened during well checks
For high risk population
If in a high risk group
If fam cannot respond to no to personal risk questions

99
Q

K

A
100
Q

Identifying the sources of lead in the child’s environment

A

If they lie in old home
Assess where parents work (construction, pottery/ paints)

101
Q

How can we eliminate lead sources when we identify them in the child’s environment

A

If in an old home teach to wet pop areas instead of vacuuming
Wash and dry child’s hands frequently especially before they eat
Wash toys and pacifiers frequently

Ensure the exposure isnt coming from parents occupation

102
Q

Hemophilia

A

X -linked
Mom carries it
And male will have the disease ( males dont have second X chromosome to cancel it out)
Resulting of absence factor 8&9

103
Q

Two type of hemophilia

A

A- Factor 8
B- Factor 9

104
Q

Factor 8 deficiency

A

Hemophilia A classic (75%)

105
Q

Factor 9 deficiency

A

Hemophilia b
Christmas disease
(25%)

106
Q

Where can bleeding happen in hemophilia

A

Anywhere in the body
(Internal or external)

107
Q

How can hemophilia be labeled?

A

Mild moderate or severe

108
Q

Mild clotting factor

A

6-50%

109
Q

Moderate clotting factor

A

1-5%

110
Q

Severe clotting factor

A

Less than 1%

111
Q

Diagnosis of hemophilia

A

History of bleeding episodes
Genetic testing , ptt to test clotting time

112
Q

What will PTT ( partial thromboblastin time) come back as positive hemophilia

A

Less than 25 % clotting factor

113
Q

Hemarthrosis

A

Hemophilia
Bleeding in the joints