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Reaction of nitrite with human fetal oxyhemoglobin: A model simulation study with implications for blood flow regulation in sickle cell disease (SCD).
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The secondary objectives investigated the correlations of fetal hemoglobin (HbF) levels and other patient or treatment characteristics with the occurrence of vaso-occlusive (painful) crises, and the effect of treatment on the quality of life.
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Fetal hemoglobin level was predictive of the crisis rate, suggesting that attempts to increase the fetal hemoglobin level with pharmacological agents such as hydroxyurea might decrease the crisis rate and ultimately improve survival.
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If both parents have sickle cell trait (each has one normal and one sickle cell gene), the child has a 50% chance of inheriting sickle cell trait, 25% chance of inheriting sickle cell disease (two sickle cell genes), and 25% chance of inheriting two normal genes (having neither the trait nor the disease).
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Boston Medical Center (BMC) the treatment facility of choice for Boston-area patients with sickle cell disease.
We observed an increase of cGMP levels in the erythroid progenitor cells after treatment with CysNO, an NO donor with a half-life of about 30 minutes.
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Because of the subjective and episodic nature of pain crises, more patients need to be evaluated in a controlled fashion over a longer period before firm conclusions can be drawn about how hydroxyurea treatment affects the clinical course of sickle cell anemia.
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