ARGININE SUPPLEMENTATION IN SICKLE CELL ANEMIA-PHYSIOLOGICAL AND PROPHYLATIC

这个子项目是众多研究子项目之一

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. Sickle cell disease is a serious inherited disease of the red blood cells resultiing in sever anemia and episodes of occlusion of the blood vessels. This vaso-occlusion affects every organ system in the body and results in recurrent episodes of pain, overwhelming infections and progressive organ damage. Nitric oxide is a substance produced by arginine and has many functions that could impact favorably in vaso-occlusion in sickle cell disease. Arginine is an aminoacid that has been shown to raise nitric oxide and have benificial results in subjects with sickle cell disease, and will help to determine if daily arginine is a benificial investigational drug for subjects with sickle cell disease. This study is a double-blind, placebo-controlled trial in which eligible subjects (children, adolescents, and adults) with sickle cell disease will receive either arginine or placebo by mouth during 12 weeks. Subjects will be randomized to recieve placebo or one of two doses of arginine (0.04g/kg/day).