Treatment of sickling disorders.
Sudden death in military recruits with sickle cell trait appears to be related to hyperthermia and its consequences and can probably be prevented by use of sensible precautions and heightened awareness of the risk. Sickle cell disease can be treated by decreasing the proportion of sickle cells through transfusion; indications and pathophysiology of such transfusions are beginning to become clear. Sickle cell disease can be prevented if erythrocytes can be prevented from sickling. Dilution of hemoglobin S within erythrocytes, by stimulating fetal hemoglobin production, increasing cell water, or inducing iron deficiency, can achieve that goal in some patients, but risks and benefits of such treatment are still incompletely understood.
Charache
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Johns Hopkins University, Baltimore, MD 21209-4519, USA.
D000755:Anemia, Sickle Cell / Q000401:mortality / Q000628:therapy*; D000986:Antisickling Agents / Q000627:therapeutic use; D001781:Blood Component Removal / Q000379:methods; D001803:Blood Transfusion; D002087:Butyrates / Q000627:therapeutic use; D003022:Clotrimazole / Q000627:therapeutic use; D004912:Erythrocytes; D005319:Fetal Hemoglobin / Q000187:drug effects; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D015996:Survival Rate; D017086:beta-Thalassemia / Q000401:mortality / Q000628:therapy*
D000986:Antisickling Agents; D002087:Butyrates; D005319:Fetal Hemoglobin; D003022:Clotrimazole; D006918:Hydroxyurea