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18 Cards in this Set
- Front
- Back
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what is the inheritance pattern of the sickling gene?
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autosomal recessive
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what is the advantage of sickle cell trait?
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it confers a survival advantage to malaria
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what is the pathophysiology of the sickling gene?
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a point mutation in the beta allele on chromosome 11 that causes a GAG to become a GTG. this results in the insertion of a valine instead of a glutamate in the beta chain. this glutatmate to valine switch allows for the polymerization of sickle hemoglobin when deoxygenated
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what is the life span of RBCs with HbS?
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< 20 days
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what is the biggest complication associated with sickle cell disease?
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vaso-occlusion
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what are 3 sickling promoters?
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- vascular stasis
- acidosis - dehydration |
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what are 3 sickling inhibitors?
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- iron deficiency
- alpha thalassemia - HbF & HbA2 |
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what is an aplastic crisis? what causes it?
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- halted RBC production which leads to severe anemia with a dramatic decrease in reticulocytes
- Parvovirus usually causes it |
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what is hand-foot syndrome? what disease is it associated with?
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- painful swelling of the hands and feet due to vaso-occlusion
- sickle cell disease |
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how does sickle cell disease cause stroke? (hint there are 2 ways)
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1) occlusion of vessels to and from the brain
2) repetitive damage done by sickle cells can lead to intimal thickening and occlusion |
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what is the leading cause of death in patients with sickle cell disease?
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acute chest syndrome
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what is acute chest syndrome?
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an acute onset of fever, respiratory symptoms, and new infiltrate on check x-ray. can be caused by an infection, lung infarct, or fat emboli
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T or F: when a patient with sickle cell disease presents with pain in the abdomen it most likely an abdominal crisis and not a surgical abdomen.
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True
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what is priapism? what causes it? who gets it?
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- painful erections
- vaso-occlusion by sickled cells preventing penile venous drainage - commonly seen in children and adolescents with sickle cell disease |
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why are patients with sickle cell disease more prone to meningitis and sepsis? what do we do about it?
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- sickle cell patients do not opsonize bacteria well leaving them vulnerable to infections with encapsulated organisms
- we give them prophylaxis penicillin until about the age of 5 |
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what does hydroxyurea do?
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- it increases the amount of HbF, increases RBC volume & hydration, and decreases neutrophils, monocytes, and retics
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who do we give hydroxyurea to?
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adults, adolescents, and older children who have sickle cell disease with a complication (frequent pain episodes, acute chest syndrome, severe vaso-occlusive episodes, severe symptomatic anemia)
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is there a cure for sickle cells disease? if so what is it? how often does it work?
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- yes
- bone marrow transplant - there is a > 90% 2 yr survival and 75-80% are cured of their sickle cell disease |
