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HbF expression, but not hydroxyurea therapy, corrects the end-organ damage of SCD.

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Parental and other factors associated with hydroxyurea use for pediatric sickle cell disease.

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Medical management remains largely suboptimal, however, particularly in low-income countries where the burden of disease is heaviest.

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Nonetheless, because of the small numbers of events and of exposed patients (14 percent), we cannot draw firm conclusions about the role of cigarette smoking as a risk factor for thrombosis in this group of patients.

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Provide support for cultural competency training across the interdisciplinary team regarding care for sickle cell disease.

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Standard treatment is given for sickle cell crises, which may occur more frequently during pregnancy.

DNA-based testing to predict blood group phenotypes has enhanced availability of antigen-negative donor units and improved typing of transfused patients, but replacement of routine serologic typing for non-ABO antigens with molecular typing for patients has not been reported.

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It is possible that the increased mean corpuscular volume, which has been observed in nearly all patients treated with hydroxyurea, has a favorable influence on the rheologic behavior of the cell.

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So-called VDJ (variable, diverse, and joining gene segments) recombination events also did not appear more frequent with hydroxyurea treatment, either over time or compared with the placebo group.

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Management of comorbid conditions, however, will not be addressed except in certain situations where the comorbid condition has a direct effect on the manifestation and management of the sickle cell complication in question.

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Although many of the contributing factors and deeply seated root causes of these events differ, two common causes are clear: 1) lack of professional staff knowledge regarding the causes and signs of hyponatremia, and 2) the failure of professional staff to respond to concerns expressed by several nurses in Case 1, and by the parents in Case 2, regarding the rapidly deteriorating condition of these children.

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To date, early clinical trials of other experimental HbF-inducing drugs have demonstrated considerable short- and long-term toxicity compared with HU.

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Because of the rapid turnover of red cell precursors and the resulting breakdown of the heme products, both homozygotes and heterozygotes for beta-thalassemia may develop mild jaundice and have the propensity to gallstone formation.

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