This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.These studies will test the hypothesis that routine dietary protein and/ or energy supplementation will improve clinical conditions and reduce morbidity in adults with homozygous sickle cell disease (HbSS). We hypothesize that the characteristic deficit in body weight in HbSS adults. results from greater energy and protein needs for individuals with the disease energy needs arise because the shortened life span of the 'sickled' erythrocytes accelerates hemoglobin energy expenditure is ultimately increased. Furthermore, the increased protein turnover is associated with an increased net loss of protein, therefore increasing protein requirement. A corollary to this hypothesis is that dietary protein and/or energy supplementation will proved the extra energy and protein required to maintain elevated rates of protein turnover and energy expenditure, thereby allowing optimal lean tissue deposition and repair. Consequently body weight and body composition will be normalized and clinical condition improved.
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