PULMONARY HYPERTENSION AND THE HYPOXIC RESPONSE IN SICKLE CELL DIESEASE:

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。子项目和 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。 该研究旨在确定镰状细胞病儿童和青少年肺动脉高压的患病率和危险因素，并确定缺氧反应在其发病机制中的作用。 在这方面，缺氧诱导因子调节途径介导的增殖性血管反应和一氧化氮清除将在SCD患者和Chuvash红细胞增多症患者之间进行比较，Chuvash红细胞增多症是另一种以PHT风险增加为特征的血液学疾病。 高通量微阵列和基因分型技术将被用来确定候选基因多态性参与病理反应缺氧SCD和CP患者有和没有PHT。

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. The research is designed to determine the prevalence and risk factors of pulmonary hypertension in children and adolescents with sickle cell disease, and to determine the role of hypoxic response in its pathogenesis. In this regard, proliferative vascular responses mediated by hypoxia inducible factor-regulated pathways and nitric oxide scavenging will be compared between patients with SCD and patients with Chuvash polycythemia, another hematological disorder characterized by increased risk for PHT. High throughput microarray and genotyping technologies will be employed to identify candidate gene polymorphisms involved in pathologic responses to hypoxia in SCD and CP patients with and without PHT.