PULMONARY HYPERTENSION IN SICKLE CELL DISEASE WITH IDENTIFICATION OF CLINICAL AS

点击翻译按钮获取中文摘要

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Pulmonary complications such as pulmonary hypertension (pHTN) have become a major source of both morbidity and mortality in patients with sickle cell disease (SCD). Achievement of the aims of this study will provide a more in-depth understanding of the development and natural history of pHTN in SCD and help identify those patients at greatest risk for pHTN. The UNC Comprehensive Sickle Cell Program Center offers a large and well-characterized patient population with which to study many aspects of pHTN in SCD. We will enroll patients already known to have pHTN, and screen others to determine whether inclusion in a Pulmonary Hypertension Registry is appropriate. Those enrolled in this registry will be followed prospectively during the 4-year study to assess the rate of progression of pHTN and to identify those factors associated with progression of pHTN. We will correlate the presence of different alleles (SNP analysis) and degrees of red cell adhesion with clinical data defining the presence and severity of pHTN.