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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
Q

How long does it take new marrow to start to recover after BMT?

A

1-2 weeks

26
Q

Why are blood products given to patients following a BMT?

A

old marrow is destroyed & not producing any cells so need the blood product support until new marrow takes hold

27
Q

Lead Poisoning occurs when serum levels are?

A

> 10 mcg/dL, however there is no known safe level, side effects can happen at lower range

28
Q

Effects of Lead on Body?

A
  • 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
Q

How does lead impair growth in children?

A

Adversely affects the absorption of vitamin D & calcium

30
Q

Screening for Lead should be done at what intervals?

A
  • All children at age 9-12 months & at 2 years

- 3-6 years old if at high risk & not previously screened

31
Q

Therapeutic Management of High Lead Levels?

A
  • 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
Q

How does Oral chelation work to remove lead for the body?

A
  • Binds the lead & increases rate of excretion

- May need second round of treatment

33
Q

Replacement of normal hemoglobin (Hgb) with abnormal Hgb?

A

Sickle Cell Anemia/ Disease

- HgbS

34
Q

Sickle Cell Anemia/ Disease is an __________ trait and primarily affects ________?

A
  • autosomal recessive

- African Americans

35
Q

Ethnicities mostly affected by Sickle Cell Disease?

A

African Americans, Mediterranean, South American, Arabian, & East Indian descent

36
Q

How does ischemia and hypoxia occur in an individual affected with SCD?

A
  • 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
Q

Normal RBC life span?

A

120 days

38
Q

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 _____?

A
  • adequate oxygenation & hydration
  • 10-20 days
  • 120 days
39
Q

Sickle Cell Anemia – Clinical Manifestations

A
  • 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
Q

Infants tend to be asymptomatic until what age and why?

A
  • 4-6 months of ages due to high levels of fetal Hgb
41
Q

Sickle Cell Anemia – Common Pediatric Crises

A
  • Vaso-Occlusive
  • Splenic Sequestration
  • Aplastic Crisis
  • Acute Chest Syndrome
42
Q

Sickle Cell Anemia – S/S Pediatric Crises (Vaso-Occlusive)

A
  • Extremely painful
    Fever, tissue engorgement, swelling of joints, priapism
    -may last days to weeks
43
Q

Sickle Cell Anemia – S/S Pediatric Crises (Splenic Sequestration)

A

Profound anemia, hypovolemia & shock

44
Q

Sickle Cell Anemia – S/S Pediatric Crises (Aplastic Crisis)

A

Profound anemia, pallor, fatigue

45
Q

Sickle Cell Anemia – S/S Pediatric Crises (Acute Chest Syndrome)

A

Fever, cough, chest & back pain, dyspnea & hypoxemia

46
Q

Splenic Sequestration

A

pooling of blood in spleen, can be life threatening

47
Q

diminished production & increased destruction of RBCs. Can be triggered by viral infection or depletion of folic acid

A

Aplastic crisis

48
Q

Aplastic crisis

A

diminished production & increased destruction of RBCs. Can be triggered by viral infection or depletion of folic acid

49
Q

pooling of blood in spleen, can be life threatening

A

Splenic Sequestration

50
Q

pulmonary infiltrate of abnormal blood cells leads to lower respiratory tract symptoms. Pulmonary infection, infarction may occur & lead to pulmonary failure or death

A

Acute Chest Syndrome

51
Q

Acute Chest Syndrome

A

pulmonary infiltrate of abnormal blood cells leads to lower respiratory tract symptoms. Pulmonary infection, infarction may occur & lead to pulmonary failure or death

52
Q

Sickle Cell Anemia – Diagnostics

A
  • Hgb electrophoresis done as part of newborn screening
  • Can determine carrier or disease status
  • If not done as newborn, genetic testing also available
53
Q

Sickle Cell Anemia – Therapeutic Management

A
  • Pain control
  • Hydration
  • Oxygenation
  • Prevention & treatment of infection
  • Transfusion of RBCs
  • Hydroxyurea
  • Folic acid & Penicillin
  • Hematopoietic stem cell transplantation (HSCT)
  • Monitor lab values
54
Q

Sickle Cell Anemia – Nursing Management

A

Maintain HYDRATION (oral or other as needed)

Peds Exam (5900) #3 (10 decks)

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