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Peds Exam (5900) #3 > RBC Diseases > Flashcards

RBC Diseases Flashcards

1
Q

Disorder defined as:

  • Decreased RBCs
  • Decreased quantity of hemoglobin
  • Volume of packed RBCs to below normal
A

Anemia

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2
Q

Causes of Anemias

A
  • Loss or destruction of existing RBCs
  • Impaired or decreased rate of production
  • Underlying disorder
    • Lead poisoning
    • Hypersplenism
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3
Q

Types of Anemia

A
  • Iron Deficiency Anemia
  • Aplastic Anemia
  • Sickle Cell Anemia
  • Lead Poisoning
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4
Q

Causes of Iron Deficiency Anemia

A
  • Blood loss
  • Malabsorption
  • Poor nutritional intake
  • Increased internal demands
    • rapid growth periods
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5
Q

Most common type of anemia & most common nutritional deficiency in children?

A

Iron Deficiency Anemia

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6
Q

Who is at risk of poor nutritional intake of iron?

A
  • Rapidly growing adolescents
  • Adolescent females with heavy menses
  • Infants who do not take in adequate solid foods after age 6 months
  • Infants who are fed only breast milk or formula not fortified w/ iron
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7
Q

neonatal stores of iron are depleted by what age?

A

6 months

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8
Q

Iron Deficiency Anemia – Clinical Manifestations?

A
  • Pallor
  • Fatigue
  • Irritability
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9
Q

Prolonged Iron Deficiency Anemia – Clinical Manifestations?

A
  • Nail bed deformities
  • Growth retardation
  • Developmental delays
  • Tachycardia/heart murmurs
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10
Q

Oral Iron supplementation may cause which side effects?

A

constipation/GI distress

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11
Q

Iron Deficiency Anemia - Therapy

A
  • Oral elemental iron preparations
    • Ferrous sulfate: 1-2 months
  • Diet high in iron – in conjunction w/ oral supplements
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12
Q

Screening for iron deficiency should be done at what intervals

A

at 9-12 months of age, 15-18 months of age & again at adolescence

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13
Q

Failure of bone marrow to produce adequate amounts of circulating blood cells causes which type of anemia?

A

Aplastic Anemia

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14
Q

All formed elements of the blood are simultaneously depressed

A

pancytopenia

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15
Q

What is pancytopenia?

A
  • All formed elements of the blood are simultaneously depressed
  • associated with Aplastic Anemia
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16
Q

Aplastic Anemia

A

Failure of bone marrow to produce adequate amounts of circulating blood cells

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17
Q

Causes of Aplastic Anemia?

A 
Congenital:
  - Fanconi’s anemia 
Idiopathic:
   - From a drug reaction
   - infection (such as viral hepatitis or mononucleosis)
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18
Q

Fanconi’s anemia

A
  • a rare recessive syndrome

- those affected are at risk for AML so bone marrow transplant treatment of choice

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19
Q

drug causes of Aplastic Anemia?

A

sulfonamides, benzene solvents (in glue, etc.) or lead

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20
Q

Aplastic Anemia – Clinical Manifestations (RBCs affected)?

A

Pallor, weakness, dizziness, headache, fatigue, tachycardia

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21
Q

Aplastic Anemia – Clinical Manifestations (WBCs affected)?

A

at risk for infection (bacterial, viral & fungal)

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22
Q

Aplastic Anemia – Clinical Manifestations (Platelets affected)?

A
  • at risk for bleeding & clotting problems

- Petechiae, purpura, bleeding, bruising

23
Q

Immunosuppressive drug therapy for Aplastic Anemia?

A 
Antithymocyte globulin (ATG)
Cyclosporine
24
Q

Cure for Aplastic Anemia?

A

Bone marrow(BMT)/stem cell transplant

25
How long does it take new marrow to start to recover after BMT?
1-2 weeks
26
Why are blood products given to patients following a BMT?
old marrow is destroyed & not producing any cells so need the blood product support until new marrow takes hold
27
Lead Poisoning occurs when serum levels are?
> 10 mcg/dL, however there is no known safe level, side effects can happen at lower range
28
Effects of Lead on Body?
- Anemia due to impairment of hemoglobin synthesis - Irreversible brain damage: - Impaired growth and stature - Behavior problems - absorbed by bone and teeth, then released slowly causing problems
29
How does lead impair growth in children?
Adversely affects the absorption of vitamin D & calcium
30
Screening for Lead should be done at what intervals?
- All children at age 9-12 months & at 2 years | - 3-6 years old if at high risk & not previously screened
31
Therapeutic Management of High Lead Levels?
- Get the lead out by: - Increase iron & calcium in diet - Decease fat in diet Oral chelation - Long-term follow-up encouraged - Prevent re-exposure by removing lead or child from environment.
32
How does Oral chelation work to remove lead for the body?
- Binds the lead & increases rate of excretion | - May need second round of treatment
33
Replacement of normal hemoglobin (Hgb) with abnormal Hgb?
Sickle Cell Anemia/ Disease | - HgbS
34
Sickle Cell Anemia/ Disease is an __________ trait and primarily affects ________?
- autosomal recessive | - African Americans
35
Ethnicities mostly affected by Sickle Cell Disease?
African Americans, Mediterranean, South American, Arabian, & East Indian descent
36
How does ischemia and hypoxia occur in an individual affected with SCD?
- Sickled cells are rigid and obstruct capillary blood flow, causing microscopic obstructions that lead to engorgement and tissue ischemia - Hypoxia occurs and causes even more sickling
37
Normal RBC life span?
120 days
38
Sickling response can be reversible under conditions of ______ but the membranes become more fragile and life of the RBC is reduced to _____ versus the normal _____?
- adequate oxygenation & hydration - 10-20 days - 120 days
39
Sickle Cell Anemia – Clinical Manifestations
- directly related to tissue destructions from vaso-occlusion - Brain: strokes - Circulation: painful crises - Bones/Joints: osteomyelitis (dactylitis is common in infants) - Spleen: splenic sequestration
40
Infants tend to be asymptomatic until what age and why?
- 4-6 months of ages due to high levels of fetal Hgb
41
Sickle Cell Anemia – Common Pediatric Crises
- Vaso-Occlusive - Splenic Sequestration - Aplastic Crisis - Acute Chest Syndrome
42
Sickle Cell Anemia – S/S Pediatric Crises (Vaso-Occlusive)
- Extremely painful Fever, tissue engorgement, swelling of joints, priapism -may last days to weeks
43
Sickle Cell Anemia – S/S Pediatric Crises (Splenic Sequestration)
Profound anemia, hypovolemia & shock
44
Sickle Cell Anemia – S/S Pediatric Crises (Aplastic Crisis)
Profound anemia, pallor, fatigue
45
Sickle Cell Anemia – S/S Pediatric Crises (Acute Chest Syndrome)
Fever, cough, chest & back pain, dyspnea & hypoxemia
46
Splenic Sequestration
pooling of blood in spleen, can be life threatening
47
diminished production & increased destruction of RBCs. Can be triggered by viral infection or depletion of folic acid
Aplastic crisis
48
Aplastic crisis
diminished production & increased destruction of RBCs. Can be triggered by viral infection or depletion of folic acid
49
pooling of blood in spleen, can be life threatening
Splenic Sequestration
50
pulmonary infiltrate of abnormal blood cells leads to lower respiratory tract symptoms. Pulmonary infection, infarction may occur & lead to pulmonary failure or death
Acute Chest Syndrome
51
Acute Chest Syndrome
pulmonary infiltrate of abnormal blood cells leads to lower respiratory tract symptoms. Pulmonary infection, infarction may occur & lead to pulmonary failure or death
52
Sickle Cell Anemia – Diagnostics
- Hgb electrophoresis done as part of newborn screening - Can determine carrier or disease status - If not done as newborn, genetic testing also available
53
Sickle Cell Anemia – Therapeutic Management
- Pain control - Hydration - Oxygenation - Prevention & treatment of infection - Transfusion of RBCs - Hydroxyurea - Folic acid & Penicillin - Hematopoietic stem cell transplantation (HSCT) - Monitor lab values
54
Sickle Cell Anemia – Nursing Management
Maintain HYDRATION (oral or other as needed)

Peds Exam (5900) #3 (10 decks)

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