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BACKGROUND In a previous open-label study of hydroxyurea therapy, the synthesis of fetal hemoglobin increased in most patients with sickle cell anemia, with only mild myelotoxicity. By inhibiting sickling, increased levels of fetal hemoglobin might decrease the frequency of painful crises. METHODS In a double-blind, randomized clinical trial, we tested(More)
BACKGROUND Blood transfusions prevent recurrent stroke in children with sickle cell anemia, but the value of transfusions in preventing a first stroke is unknown. We used transcranial Doppler ultrasonography to identify children with sickle cell anemia who were at high risk for stroke and then randomly assigned them to receive standard care or transfusions(More)
CONTEXT Hydroxyurea increases levels of fetal hemoglobin (HbF) and decreases morbidity from vaso-occlusive complications in patients with sickle cell anemia (SCA). High HbF levels reduce morbidity and mortality. OBJECTIVE To determine whether hydroxyurea attenuates mortality in patients with SCA. DESIGN Long-term observational follow-up study of(More)
In the United States, sickle cell disease affects approximately 1 in 350 African-American newborn infants each year, and approximately 72,000 individuals total. Sickle cell disease is the most prevalent genetic hematologic disorder in the US. In 1972, the National Sickle Cell Disease Control Act was passed by Congress. This landmark piece of legislation(More)
CONTEXT Thirty percent of patients with sickle cell disease (SCD) develop pulmonary hypertension, a major risk factor for death in this population. A validated blood biomarker of pulmonary hypertension in SCD could provide important prognostic and diagnostic information and allow the exploration of the prevalence of pulmonary hypertension in participants in(More)
BACKGROUND The Multicenter Study of Hydroxyurea (HU) in Sickle Cell Anemia (MSH) previously showed that daily oral HU reduces painful sickle cell (SS) crises by 50% in patients with moderate to severe disease. The morbidity associated with this disease is known to have serious negative impact on the overall quality of life(QOL) of affected individuals. (More)
Data from a total of 74 pregnancies in 42 patients with sickling disorders seen at Johns Hopkins Hospital are compared with similar data from other centers. Although risks are still higher than those for women without sickle cell disease, they have diminished significantly from those reported earlier. Prophylactic transfusion therapy may decrease these(More)
The Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH), a double-blind randomized clinical trial, compared the frequency of acute vaso-occlusive (painful) crises during 2 yr of follow-up in 299 patients randomly assigned to hydroxyurea or placebo. Most patients had more than one crisis; all crises reported were included in the primary outcome(More)
Exercise during pregnancy has become an important topic with the recent popularity of physical fitness programs among the non-pregnant population. Various physiological effects of exercise have been reported in several animal species, and there are some reports of physiological effects in pregnant women which will be reviewed.
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