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Correspondence
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Volume 331:746-747 September 15, 1994 Number 11
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Induction of Fetal Hemoglobin in the Presence of Increased 3-Hydroxybutyric Acid Associated with ß-Ketothiolase Deficiency

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 by Parsonnet, J.
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To the Editor: Elevated levels of fetal hemoglobin may diminish the severity of the {beta}-hemoglobinopathies sickle cell disease and {beta}-thalassemia. In {beta}-thalassemia, this effect is related to a reduction in the overall imbalance in the ratio of {alpha}-globin and non-{alpha}-globin chains. In sickle cell anemia, an elevated level of fetal hemoglobin results in a reciprocal decrease in the intracellular concentration of hemoglobin S and inhibits polymer formation. The discovery that butyric acid is an inducer of fetal hemoglobin arose from the observation that the switch from hemoglobin F to hemoglobin A is delayed in infants of diabetic mothers. In . . . [Full Text of this Article]

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