Jane S Hankins

..We describe a pilot program created to facilitate transition from pediatric to adult care by helping adolescents with SCD identify an adult medical home...

..Pathophysiological and genetic differences affecting iron deposition and accumulation in SCD and TM warrants further investigation...

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..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...

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..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...

..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...

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

..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...

..We explored factors that influenced making decisions regarding interventions and examined the relations between treatment preference and health-related quality of life (HRQOL)...

..The relationship between themes is presented, and the relationship between themes and parent empowerment is discussed as well as the ways in which the themes reflect underlying ableism...

..042) and decrease in lactate dehydrogenase levels (P = 0.035). Urine microalbumin and cystatin C levels did not change significantly. Hydroxyurea at MTD is associated with a decrease in hyperfiltration in young children with SCA...

..A systematic investigation of the influence of the region-of-interest size and placement has not been conducted...

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..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...

..Cystatin C levels inversely reflect GFR changes and are associated with albuminuria; serial monitoring may provide a sensitive and accurate marker of nephropathy in children with SCA...

..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...

..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...

..Accurate diagnosis and understanding of Hb C-related disorders helped to avoid confusion with sickle hemoglobinopathies and aided in proper clinical management...

..Our cases suggest that older children with SCD and acute parvovirus infection should be monitored closely for splenomegaly and multi-system dysfunction...

..To improve children's ability to cope with MRI examinations and avoid sedation, our institution established preparation and support procedures (PSP)...

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..Communication within families of children with sickle cell disease is important yet has not been adequately investigated...

..Generic hydroxyurea formulations represent a potent yet inexpensive therapeutic option for children with SCA worldwide...

..Despite having a much higher HbF level, patients with HbS-HPFH have a similar age-related pattern of HbF decline and associations as patients with sickle cell anaemia or HbS-β(0) thalassaemia...

..Although hepatobiliary complications are common in SCD, we report on biloma and pneumobilia in a young patient with this condition...

..The effectiveness of CTX should be prospectively compared with that of hydroxyurea and stem cell transplantation...