Predictors and Outcomes in Patients With Sickle Cell Disease
Condition: Sickle Cell Disease Intervention: Sponsors: St. Jude Children's Research Hospital; Methodist Comprehensive Sickle Cell Center; University of Memphis; National Heart, Lung, and Blood Institute (NHLBI) Not yet recruiting
Conclusions: Low-risk febrile patients with SCD can be safely discharged from the ED. An automated notification system within the electronic medical record system can facilitate patient follow-up after ED discharge. Future quality improvement efforts aimed to further reduce admissions in this population should target patients with modifiable risk factors for serious bacterial infection.
The aims of the current study were to investigate whether SCD incurs an additional risk for poor sleep over and above the influence of sociodemographic factors (ie, race and sex) during adolescence, and to explore the relationships between sociodemographic, physical (ie, age and pubertal status), and disease-related factors (ie, SCD genotype and hydroxyurea use) on sleep problem risk during adolescence. Black adolescents (age, 12 to 17 y) with SCD (n=53) were recruited from regional pediatric SCD clinics in the southeast and a sample of healthy black adolescents (n=160) were recruited from middle and high schools. R...
Pain is a clinical hallmark of sickle cell disease (SCD), and is rarely optimally managed. Cognitive-behavioral therapy (CBT) for pain has been effectively delivered through the Internet in other pediatric populations. We tested feasibility and acceptability of an Internet-delivered CBT intervention in 25 adolescents with SCD (64% female, mean age=14.8 y) and their parents randomized to Internet CBT (n=15) or Internet Pain Education (n=10). Participants completed pretreatment/posttreatment measures. Eight dyads completed semistructured interviews to evaluate treatment acceptability. Feasibility indicators included r...
Although vitamin D deficiency (VDD) has been linked to anemia among sickle cell disease (SCD), its relationship with hemolysis is unclear. Serum 25-hydroxyvitamin D and biomarkers of hemolysis (hemoglobin [Hb]/hematocrit, reticulocyte percentage, absolute reticulocyte, and lactate dehydrogenase [LDH] levels) in 36 hydroxyurea-naive SCD children were quantified. Correlations were significantly positive with Hb/hematocrit (r=0.40, P=0.017; r=0.45, P=0.006, respectively); inverse with reticulocyte percentage, absolute reticulocyte, and LDH (r=−0.44, P=0.008; r=−0.47, P=0.007; r=−0.45, P=0.007, respectively)....
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ConclusionThere have only been scarce reports of avascular necrosis in patients with sickle cell trait. This manuscript presents such a case and includes the trials and tribulations associated with its management.
Under physiologic conditions hematopoiesis takes place in the bone marrow, and the skeleton provides the structural and supportive network necessary for normal hematopoiesis. Chronic disorders affecting hematopoiesis such as sickle cell anemia and thalassemia demonstrate striking skeletal phenotypes including bone loss and increased fracture risk. There is mounting evidence that anemia in older populations may also be associated with bone fragility. Given the interconnectedness of bone and hematopoietic cells, it is important to review the potential clinical implications and opportunities for therapeutic intervention.
This study investigated the association of theSOD2polymorphism and superoxide dismutase (SOD) activity with vaso-occlusive crisis (VOC) and acute splenic sequestration (ASS) in children with sickle cell anemia (SCA). One hundred ninety-five children aged 1-9 years old were analyzed. The TC and CC genotypes were associated with lower SOD activity compared with the TT genotype (p=0.0321;p=0.0253, respectively). Furthermore, TC/CC were more frequent in patients with VOC or ASS (p=0.0285;p=0.0090, respectively). These results suggest that theSOD2polymorphism associated with low SOD activity could be involved in SCA physiopathology.
Sickle cell disease (SCD) is a life-threatening chronic condition primarily caused by genetic mutation. The disease is characterized by intermittent vaso-occlusive events and chronic hemolytic anemia. Acute complications in patients with SCD are difficult to manage due to the pathophysiological nature of the disease. Transfusion therapy is the cornerstone of management of acute complications and significantly reduces SCD morbidity and mortality. Red cell exchange (RCE), which is characterized by low iron accumulation and volume overload, has been widely used for transfusion therapy in recent years.