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Anemia
- most common hematologic disorder of childhood
- RBC and or Hgb depletion
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when anemia develops slowly the child
is able to adapt.
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s/sx of anemia
- fatigue
- bruising (clotting factors)
- pale
- muscle weakness
- flat growth chart
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treat anemia
- transfusions after hemorrhage
- nutritional interventions for deficiencies
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iron deficiency anemia
- most prevalent due to inadequate dietary iron.
- prevent: iron fortified for infants. NO COW MILK and iron supplements for premies
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sickle cell anemia
- Autosomal recessive disorder (aa)
- defect in RBC. sickled
- Production does not equal destruction
- vasoocclusion > ischemia > hypoxia > tissue and organ damage
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splenic sequestration
- acute exacerbations of sickle cell crisis
- blood pools in the spleen > splenectomy > decreased immunity
- profound anemia, hypovolemia, shock
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sickle cell crisis
- anything that increases body's need for O2 or alters the transport of O2
- trauma, infection, stress, dehydration, and hypothermia
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vasoocclusive thombotic
- most common sickle cell crisis
- occlusion >ischemia >infarction
- fever, PAIN, tissue engorgement
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Prevention and treatment of sickle cell's success depends on
- pt and family education.
- recognize s/sx of crisis, help children adjust, and manage the pain
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aplastic anemia
- bone marrow failure where RBC, WBC, platelets are depressed.
- definitive diagnosis: bone marrow is red > yellow fatty marrow.
- tx: immunosuppression therapy and bone marrow transplant
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RH incompatibility
mom - baby + so she receives RhoGam so antibodies don't attack baby #2
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hemophilia
- clotting disorder
- Hemophilia a: decrease factor 8. more common
- hemophilia b: Christmas disease factor 9
- Labs: low levels of factor 8,9, and prolonged PTT
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hemophilia effects
- males. X linked
- females are carriers
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primary tx for hemophilia
- replace missing clotting factors
- prevent bleeding and decrease risk of injuries for children with disease.
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blood transfusion therapy
- monitor VS q 15min
- infuse over 4 hours max
- type and screen: match blood type
- type and cross: agglutination (clotting)
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transfusion reactions
hemolytic, fever (pre medicate), hypothermia (warm underarm), allergies, air emboli, and hyperkalemia
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delayed transfusion reactions
- transmission of infection
- delayed hemolytic reaction: destruction of RBC
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apheresis
removal of blood > separation of blood into its components
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thalassemia
- Beta chain most commonly affected
- defect in the RBC > weak RBC > shortened RBC life
- both patents must be carriers (Aa) to have baby with disease
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Thalassemia major
- cooley anemia (aa)
- 1:4 chance if both parents are carriers
- severe anemia, need transfusions to survive
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4 types of deformed beta thalassemia
- minor: asymptomatic
- trait: mild microcytic anemia
- intermediate: moderate/severe anemia and splenomegaly
- major: transfusions needed
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s/sx of thalassemia in children
- pallor
- failure to thrive
- large spleen and liver
- severe anemia (hgb:under 6)
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