Chapter 24: Hematologic & Immunologic Dysfunction Flashcards

1
Q

What are the main 3 hematologic differences in pediatric patients?

A
  1. hematopoiesis: formation of blood that begins before birth
  2. WBC count is highest at birth
  3. platelet levels are lower in newborns
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2
Q

What is anemia?

3 aspects

A
  • most common hematologic disorder of childhood
  • decrease in the number of RBCs/Hgb – Hgb < 10 or 11 g/dL –> decreased oxygen-carrying capacity of blood
  • 2 classifications:
    1. etiology – RBC/Hgb depletion
    2. morphology – RBC characteristics that alter function
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3
Q

How does anemia affect the circulatory system?

4 effects

A
  • hemodilution: decreased concentration of RBCs
  • decreased peripheral resistance –> more blood returns to the heart
  • increased cardiac circulation and turbulence –> murmur or HF d/t increased workload
  • slowed growth d/t decreased cellular metabolism
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4
Q

What are some treatments for anemia?

5 treatments

A
  • transfusion
  • nutritional intervention for deficiency anemias
  • IV fluids to replace blood volume
  • oxygen
  • rest
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5
Q

What is iron-deficiency anemia?

2 aspects

A
  • decreased oxygen-carrying capacity of blood d/t inadequate supply of iron
  • generally preventable
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6
Q

What are some possible causes of iron-deficiency anemia based on the patient’s age group?

3 age groups

A
  • premature infants – low fetal supply of iron
  • 12 - 36 months – excessive cow’s milk (poor iron source and interferes with iron absorption)
  • adolescents – rapid growth, poor eating habits, or menses
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7
Q

What are some treatments for iron-deficiency anemia?

3 treatments

A
  • infants – no cow’s milk, iron-fortified foods/formula
  • dietary iron
  • iron supplements
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8
Q

What are 3 education topics when giving PO iron?

A
  • may stain teeth – drink with straw
  • take vitamin C with iron – facilitates absorption of iron
  • dark tarry (green/black) stools
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9
Q

What is sickle-cell anemia?

3 aspects

A
  • partial or complete replacement of normal Hgb with abnormal Hgb (Hgb S) that is in a sickle shape
  • sickle-cell shaped RBCs
  • these obstruct blood flow –> engorgement and tissue ischemia –> hypoxia occurs –> damage to and impaired function of tissues
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10
Q

Which population more commonly experiences sickle-cell anemia?

A

African Americans

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11
Q

What kind of hereditary pattern does sickle-cell anemia follow?

A

autosomal recessive

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12
Q

What is one advantage of sickle-cell anemia when compared to non-sickle-celled RBCs?

A

in areas where malaria is common, people with sickle-cell anemia tend to have a survival advantage

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13
Q

What are some treatments for sickle-cell anemia?

3 treatments

A
  • bone marrow transplants
  • transfusions
  • hydration – can prevent sickling
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14
Q

What is a sickle cell crisis?

A

pain that results from sickling of RBC d/t obstruction of blood flow

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15
Q

What are some factors that can precipitate sickle cell crisis?

6 factors

A
  • anything that increases the body’s need for oxygen
  • anything that alters transport of oxygen
  • trauma
  • fever
  • infection
  • physical or emotional stress
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16
Q

What are the 3 types of sickle cell crisis?

A
  1. vaso-occlusive (VOC) thrombotic episode
  2. splenic sequestration
  3. aplastic crisis
17
Q

What is a vaso-occlusive thrombotic episode?

4 aspects

A
  • most common type of crisis
  • very painful
  • stasis of blood with clumping of cells –> ischemia –> infarction
  • s/s:
    – fever
    – pain
    – tissue engorgement
18
Q

What is splenic sequestration?

4 aspects

A
  • life-threatening type of crisis – death can occur within hours
  • large volume of blood pools in spleen –> decreases blood volume –> shock
  • s/s:
    – profound anemia
    – hypovolemia
    – shock
  • treatment – splenectomy
19
Q

What is aplastic crisis?

3 aspects

A
  • diminished production of RBCs with increased destruction of RBCs
  • triggered by viral infection or depletion of folic acid
  • s/s:
    – profound anemia
    – pallor
20
Q

What are some treatments for sickle-cell anemia?

6 treatments

A
  • prevent sickling – rest, hydration, electrolytes, pain meds, transfusions, antibiotics
  • encourage childhood vaccines –> decreases infection
  • treat infections
  • prophylactic antibiotics for children 2 months - 5 y.o.
  • diet – iron and hydration
  • splenectomy
21
Q

What is thalassemia? What is the difference between the 2 types?

A
  • anemia that results from defective synthesis of Hgb, structurally-impaired RBCs, and a shortened life of RBCs
  • 2 types:
    1. alpha – alpha chains affected; more common in Asian and African pop.
    2. beta – most common; 4 forms; more common in Greek, Italian, and Syrian pop.
22
Q

What are the s/s of thalassemia?

11 s/s

A
  • chronic hypoxia
  • h/a
  • irritability
  • precordial and bone pain
  • exercise intolerance
  • anorexia
  • epistaxis
  • pallor
  • FTT
  • hepatosplenomegaly
  • severe anemia (Hgb < 6 g/dL)
23
Q

How is thalassemia treated?

2 treatments

A
  • transfusions
  • iron-chelating drugs (Desferal) which bind excess iron for excretion – use for hemosiderosis (iron overload)
24
Q

What is hemophilia?

3 aspects

A
  • group of hereditary bleeding disorders that result from deficiencies of clotting factors
  • X-linked recessive
  • 3 types:
    – hemophilia A
    – hemophilia B
    – von Willebrand disease (vWD)
25
Q

Differentiate between the 3 types of hemophilia?

A
  • hemophilia A:
    – classic hemophilia
    – deficiency of factor VIII
  • hemophilia B:
    – Christmas disease
    – deficiency of factor IX
  • von Willebrand disease (vWD):
    – deficiency, abnormality, or absence of vWF
    – deficiency of factor VIII
    – affects both males and females
26
Q

What are some s/s of hemophilia?

10 s/s

A
  • bleeding into joint spaces –> impaired mobility –> bony changes –> disability
  • warmth
  • pain
  • bruising
  • decreased movement
  • epistasis
  • bleeding in GI tract
  • excessive bleeding after procedures
  • hemorrhaging
  • bleeding in spinal cord –> paralysis
27
Q

What are some treatments for hemophilia?

8 treatments

A
  • replacement of missing clotting factors
  • desmopressin (DDAVP) – increases factor VIII
  • transfusions
  • meds – steroids, NSAIDs
  • exercise
  • physical therapy
  • advise to shave only with electric razors
  • apply pressure and ice for 15 minutes for superficial bleeding