Mission Statement: Pulmonary vascular disease is a growing problem in many systemic diseases. In disease states, such as systemic sclerosis and hemolytic anemias, in which patient survival has increased dramatically in recent years, pulmonary complications, especially vascular ones, have become the leading cause of morbidity and mortality. The mechanisms involved in development of pulmonary hypertension (PH) regardless of etiology is not well-understood. The object of our studies is to define pathogenic mechanisms, essential for the development of these vascular abnormalities and use them to try to direct new therapies. Lastly, each of these entities can be associated with relative or absolute hypoxemia; thus, we have been investigating the effects of hypoxia on pulmonary vascular endothelium and methods to mitigate its effect. The projects in our research group are linked by the underlying pulmonary vascular abnormalities they engender: Approximately 6-10% of adults with sickle cell disease have pulmonary hypertension which is an independent risk factor for mortality. We have been interested in studying genetic modulators of sickle cell disease that place patients at risk for the development of PH. We are in the process of performing genome wide association studies of the largest cohort of prospectively collected SCD patients with cardiopulmonary … Continue reading
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