Winfred C Wang

..However, definitive prophylaxis and treatment for most CNS complications of sickle cell disease have yet to be determined...

..To determine whether long-term transfusion improves growth in children with sickle cell anemia...

..This review focuses on advances reported in the past 2 years...

..Mg had no effects. Further investigation of hydroxycarbamide as monotherapy in HbSC disease is warranted...

..We assessed the effect of hydroxycarbamide therapy on organ dysfunction and clinical complications, and examined laboratory findings and toxic effects...

..In the near future we expect to see exploration of the efficacy of drug combinations and an expansion in the use of the proven therapeutic, hydroxycarbamide...

..To determine prevalence of imaging abnormalities in the brain of children with sickle cell disease (SCD) and to identify clinical and methodological factors that influence prevalence estimate...

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..Infants with SCA tolerate prolonged hydroxyurea therapy with sustained hematologic benefits, fewer ACS events, improved growth, and possibly preserved organ function...

..KCl co-transporter activity declined after 3 months of magnesium pidolate (P = 0.02). A phase II study is needed to investigate the efficacy of this drug combination...

..98; all P < .001). This high correlation confirms prior reports, calibrates R2*-MRI measurements, and suggests its clinical utility for predicting HIC using R2*-MRI. This study was registered at www.clinicaltrials.gov as #NCT00675038...

..Our data document a high conversion rate to abnormal velocities among untreated children with SCA. Therapy should be considered for the prevention of conversion to abnormal TCD velocities...

..8, p  ≤  0.05) with Wechsler Abbreviated Scale of Intelligence scores, suggesting that fMRI may help clarify medical, hemodynamic, and neural factors that mediate adverse effects of SCA on neurocognitive function...

..Recent studies have documented elevated gray matter (GM) CBF in untreated children with SCA, but no measurements of white matter (WM) CBF have been reported...

..Chronic organ damage is an insidious process in patients with sickle cell anemia (SCA). Although hydroxyurea prevents acute vaso-occlusive events, its effects on the preservation of organ function remain undefined...

..Hydroxyurea can be effective in treating vasculopathy, but it might not prevent the progression of parenchymal damage in advanced disease...

..To examine the feasibility and accuracy of glomerular filtration rate (GFR) measurements in infants with sickle cell anemia (SCA)...

..We evaluated the rate of progression to SAA...

..However, the age of onset and factors contributing to this problem have not been well studied. We hypothesized that in children with SCD the failure rate with Brigance screening would be higher than in the normal population...

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..The argatroban/warfarin transition was monitored with chromogenic factor X levels. This case highlights several difficult problems in pediatric thrombosis...

..16%; P = 0·005). We report a protective benefit of elevated HbF regarding retinopathy, and our data suggests induction of HbF with hydroxycarbamide may prevent retinopathy in children...

..We performed MRI examinations of the brain in infants with SCA, who were evaluated as part of the multicenter randomized double-blinded Pediatric Hydroxyurea Phase III Clinical Trial (BABY HUG)...

..To determine whether sickle cell trait (hemoglobin AS) is associated with abnormalities in the brain of asymptomatic children...

..The Tanner stage transitions took place at appropriate ages. CONCLUSIONS: Hydroxyurea treatment had no adverse effect on height or weight gain or pubertal development in school-aged children with SCA...

..The findings in this case suggest that hydroxyurea may benefit patients who have unusual complications of sickle cell disease, such as chronic erythrocyte sickling in the liver...

..Sociodemographic factors were not associated with dietary water or sodium intake. Our results suggest that children and adolescents with SCA would benefit from education regarding increasing fluid intake and limiting high sodium foods...

..01), and there was a trend (P < .10) toward lower patient scores in language. Deficits cannot be attributed to school absence and may predict academic problems for patients with sickle cell disease...

..The presence of a hepatic abscess should be considered in the differential diagnosis of atypical abdominal pain in patients with SCD...

..Careful evaluation of CBCs and peripheral blood smears is required in cases of worsening anemia among sickle cell patients and consideration should be given to cold hemagglutinin disease as an etiology...

..Until safe and effective preventive strategies against infarct recurrence are discovered, MRI studies are best reserved for children with neurologic symptoms, neuropsychometric deficits, or elevated cerebral artery velocities...

..CONCLUSIONS: These findings warrant a controlled study of the effects of magnesium supplementation in patients with SCA receiving hydroxyurea. Potentially, such therapy could alleviate or prevent vaso-occlusive crises...

..Basal energy requirements are higher in adolescents with sickle cell anemia (SCA) than in healthy control subjects. However, no equation is available to accurately predict their energy needs...

..The HbF response to hydroxyurea is variable and complex, however, and even children with low baseline %HbF values can develop substantial increases in %HbF at MTD...

..To longitudinally assess the relationship of behavioral problems, intellectual functioning, and family functioning in children with sickle cell disease (SCD)...

..The pneumococcal-polysaccharide vaccine was 80.4% effective within 3 years after vaccination (95% CI, 39.7, 93.6). Children with sickle cell disease of all ages may benefit from PVC boosted with polysaccharide vaccination...

..001). The death rate in adolescents and young adults with SCD continues to be much higher than population-specific rates. Interventions to prevent morbidity and mortality related to SCD are urgently needed...

..These studies underscore the importance to correlate clinical course with laboratory diagnosis and to make DNA-based diagnostics more widely available for patients with unusual or complicated hemoglobin disorders...

..The methodology is illustrated by an example involving kindergarten readiness skills in children with sickle cell disease...

..We sought to determine the incidence of invasive pneumococcal disease (IPD) among individuals with sickle cell disease (SCD) before and after the introduction of the pneumococcal conjugate vaccine (PCV)...

..We compared the medical care utilization and mortality in children less than 20 years of age with SCD in four regions in the state of Tennessee with and without a comprehensive sickle cell center (CSCC)...

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