Engaging Parents of Children With Sickle Cell Disease in Shared Decision-Making for Hydroxyurea: The ENGAGE-HU Study.

Background: Guidelines recommend that hydroxyurea be offered to children with sickle cell disease (SCD) as early as 9 months of age using shared decision-making. To help clinicians implement shared decision-making with parents, we developed the Hydroxyurea Shared Decision-Making (H-SDM) toolkit. We evaluated its effectiveness on parent decisional uncertainty, perceptions of shared decision-making, hydroxyurea knowledge, and the likelihood of being offered and prescribed hydroxyurea.

Social determinants of health and treatment center affiliation: analysis from the sickle cell disease implementation consortium registry.

Background: Adults with sickle cell disease (SCD) suffer early mortality and high morbidity. Many are not affiliated with SCD centers, defined as no ambulatory visit with a SCD specialist in 2 years. Negative social determinants of health (SDOH) can impair access to care.

Engaging Caregivers and Providers of Children With Sickle Cell Anemia in Shared Decision Making for Hydroxyurea: Protocol for a Multicenter Randomized Controlled Trial.

Background: Sickle cell anemia (SCA) is a genetic blood disorder that puts children at a risk of serious medical complications, early morbidity and mortality, and high health care utilization. Until recently, hydroxyurea was the only disease-modifying treatment for this life-threatening disease and has remained the only option for children younger than 5 years. Evidence-based guidelines recommend using a shared decision-making (SDM) approach for offering hydroxyurea to children with SCA (HbSS or HbS/β0 thalassemia) aged as early as 9 months.

Sickle Cell Disease: A Primer for Primary Care Providers.

Sickle cell disease is an autosomal recessive disorder with significant global impact. This disorder causes the production of a dysfunctional hemoglobin, which leads to sickling of erythrocytes and ultimately hemolysis, endothelial dysfunction, vaso-occlusion, and sterile inflammation. These cellular level processes produce end-organ changes that ultimately result in specific risks and preventive care needs, unique emergency situations, and long-term complications for patients.

Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood.

Background: Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease-modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions. To further advance knowledge of SCD, St. Jude Children's Research Hospital established the Sickle Cell Clinical Research and Intervention Program (SCCRIP), to conduct research in a clinically evaluated cohort of individuals with SCD across their lifetime.

Translating sickle cell guidelines into practice for primary care providers with Project ECHO.

Background: Approximately 100,000 persons with sickle cell disease (SCD) live in the United States, including 15,000 in the Midwest. Unfortunately, many patients experience poor health outcomes due to limited access to primary care providers (PCPs) who are prepared to deliver evidence-based SCD care. Sickle Treatment and Outcomes Research in the Midwest (STORM) is a regional network established to improve care and outcomes for individuals with SCD living in Indiana, Illinois, Michigan, Minnesota, Ohio, and Wisconsin.

Neuroprotection by cord blood stem cells against glutamate-induced apoptosis is mediated by Akt pathway.

The neurotransmitter glutamate mediates excitatory synaptic transmission in the brain and spinal cord. In pathological conditions massive glutamate release reaches near millimolar concentrations in the extracellular space and contributes to neuron degeneration and death. In the present study, we demonstrate a neuroprotective role for human umbilical cord blood stem cells (hUCB) against glutamate-induced apoptosis in cultured rat cortical neurons.

Axonal remyelination by cord blood stem cells after spinal cord injury.

Human umbilical cord blood stem cells (hUCB) hold great promise for therapeutic repair after spinal cord injury (SCI). Here, we present our preliminary investigations on axonal remyelination of injured spinal cord by transplanted hUCB. Adult male rats were subjected to moderate SCI using NYU Impactor, and hUCB were grafted into the site of injury one week after SCI.

Neonatal neutropenia and bacterial sepsis associated with placental transfer of maternal neutrophil-specific autoantibodies.

Background: Passively acquired neonatal neutropenia is an infrequently reported complication of maternal autoimmune neutropenia (AIN). Two affected siblings are described. The firstborn developed Citrobacter meningitis and was permanently disabled.

CD62 expression during thrombus formation.

A large right atrial mass was discovered in a 16-year-old female patient receiving chemotherapy treatment for Hodgkin disease. The patient was participating in a platelet function research study. During laboratory examination of platelet activation, increasing CD62 and CD63 expression were found to mirror the clot formation.

Differences in adhesion receptor expression between immature and older platelets and red blood cells of neonates and adults.

Purpose: To examine the hypothesis that reticulated platelets and reticulocytes show elevated adhesion receptor expression compared with mature cells in both adult and neonatal cells.

Methods: Flow cytometry was used to examine laminin, fibronectin (VLA-6), and thrombospondin (glycoprotein IV [GPIV]) expression in reticulated red cells, reticulated platelets, and older peripherally circulating mature red cells and mature platelets in seven newborn cord blood samples and blood samples from eight adult volunteers.

Results: The difference in the neonatal reticulated platelet percentage of 9.