ARGININE SUPPLEMENTATION IN SICKLE CELL ANEMIA-PHYSIOLOGICAL AND PROPHYLATIC

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。 镰状细胞病是一种严重的遗传性红细胞疾病，会导致严重的贫血和血管闭塞。这种血管闭塞影响到身体的每个器官系统，并导致反复发作的疼痛、压倒性感染和进行性器官损害。一氧化氮是一种由精氨酸产生的物质，具有许多功能，可以在镰状细胞疾病的血管闭塞中产生有利的影响。精氨酸是一种氨基酸，已被证明能提高一氧化氮，对镰状细胞疾病患者有有益的结果，并将有助于确定每日精氨酸是否对镰状细胞疾病患者是有益的研究药物。这项研究是一项双盲、安慰剂对照试验，符合条件的镰状细胞疾病受试者(儿童、青少年和成人)将在12周内口服精氨酸或安慰剂。受试者将被随机接受安慰剂或两种剂量的精氨酸中的一种(0.04g/kg/天)。

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).