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5 Cards in this Set

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Sickle Cell
Caused by what?
*Autosomal recessive disorder =
abnormal hemoglobin leads to chronic hemolytic anemia
*Rate of sickling influenced by concentration of HgB S and presence of other HgB within the cell
.
Sickle Cell
Signs and Symptoms
*Onset first year of life when HgB F falls
*Acute painful episodes = acute vaso-occlusion from clusters of sickled RBC’s
.
Sickle Cell
Classic Lab findings
*HCT 20-30%
*Irreversible sickled cells on smear
*Reticulocytosis
*Nucleated RBC’s
.
Sickle Cell
Complications
Repeated episodes affect:
heart
lungs
liver
Ischemic necrosis of bone
Hepatomegaly
Death from organ failure
Sickle Cell
Typical Tx
*No specific tx
*PO Folic acid
*Transfusions
*Acute episode: Infection tx’d, Fluids, and oxygen given
.