PULMONARY HYPERTENSION AND THE HYPOXIC RESPONSE IN SICKLE CELL DIESEASE:

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 本研究旨在确定患有镰状细胞病的儿童和青少年中肺动脉高压的患病率和危险因素，并确定低氧反应在其发病机制中的作用。在这方面，由i)低氧诱导因子(HIF)--丘瓦什红细胞增多症(CP)介导的增殖性血管反应是另一种血液系统疾病，其特征是PHT风险增加。高通量微阵列和基因分型技术将被用来鉴定在伴有和不伴有PHT的SCD和CP患者的低氧病理反应中涉及的候选基因多态性。

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 i)hypoxia inducible factor (HIF)- with Chuvash polycythemia (CP), 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.