Alloimmunization in sickle cell disease: changing antibody specificities and association with chronic pain and decreased survival.

Background: Alloimmunization remains a significant complication of transfusion and has been associated with multiple factors, including inflammation, an important pathophysiologic mechanism in sickle cell disease (SCD). We explored whether alloimmunization is associated with disease severity in SCD.

Study Design And Methods: Adult SCD patients were enrolled in a study of outcome-modifying genes in SCD.

A model for developing, evaluating, and disseminating best practices in education and training.

With the shift toward team-based translational science came recognition that existing strategies for training individual investigators and retaining them in the biomedical workforce would be inadequate. To support this shift, it is important to: develop innovative strategies to educate and train diverse members of research teams; evaluate those programs; and disseminate best practices broadly. We have developed a four-phase model to facilitate the development, evaluation, and widespread dissemination of innovative strategies to train the biomedical research workforce.

Detecting the emergence of chronic pain in sickle cell disease.

Context: Sickle cell disease (SCD) is an inherited hematological disease marked by intense pain. Early in life the pain is episodic, but it becomes increasingly chronic in many cases. Little is known about this emergence of a chronic pain state.

MYH9 and APOL1 are both associated with sickle cell disease nephropathy.

Renal failure occurs in 5-18% of sickle cell disease (SCD) patients and is associated with early mortality. At-risk SCD patients cannot be identified prior to the appearance of proteinuria and the pathobiology is not well understood. The myosin, heavy chain 9, non-muscle (MYH9) and apolipoprotein L1 (APOL1) genes have been associated with risk for focal segmental glomerulosclerosis and end-stage renal disease in African Americans.

Association of soluble fms-like tyrosine kinase-1 with pulmonary hypertension and haemolysis in sickle cell disease.

The pathophysiology of pulmonary hypertension (PHT) in sickle cell disease (SCD) is probably multifactorial. Soluble fms-like tyrosine kinase-1 (sFLT-1) is a member of the vascular endothelial growth factor receptor (VEGFR) family. By adhering to and inhibiting VEGF and placenta growth factor, it induces endothelial dysfunction.