PULMONARY HYPERTENSION IN SICKLE CELL DISEASE WITH IDENTIFICATION OF CLINICAL AS

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目及 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 肺部并发症如肺动脉高压（pHTN）已成为镰状细胞病（SCD）患者发病率和死亡率的主要来源。 本研究目的的实现将提供对SCD中pHTN的发展和自然史的更深入了解，并有助于识别pHTN风险最大的患者。 镰状细胞综合项目中心提供了一个庞大且特征良好的患者群体，可以研究镰状细胞病的许多方面。 SCD中的pHTN。 我们将招募已知患有pHTN的患者，并筛选其他患者以确定是否适合纳入肺动脉高压登记。 将在4年研究期间对入组本登记研究的患者进行前瞻性随访，以评估pHTN进展率并确定与pHTN进展相关的因素。 我们将不同等位基因的存在（SNP分析）和红细胞粘附程度与定义pHTN存在和严重程度的临床数据相关联。

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.