METABOLIC CONSEQUENCES OF ERYTHROCYTE TRANSFUSION IN SICKLE CELL ANEMIA

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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. HYPOTHESIS: The sequence of metabolic events accompanying chronic erythrocyte transfusions (TR) in sickle cell anemia (SCA) is unknown. Although it has been reported that resting energy expenditure (EE) and protein turnover are increased in SCA, underlying physiological mechanism(s) are not completely understood. The physiological basis for the proposed research is that increased intravascular volume, decreased erythrocyte activity, increased blood viscosity, and changes in hormonal activity could collectively contribute to changes in energy metabolism and protein turnover. The central hypothesis of this GCRC protocol is that resting energy expenditure and whole body protein turnover will decrease following a single TR, and this effect will be similar after repeated TR. SPECIFIC AIMS: The central hypothesis will be tested by pursuing the following specific aims: 1) Determine how single and repeated TR affect energy expenditure and protein turnover in SCA adolescents. The working hypothesis is that components of EE and whole body protein turnover will decrease following single TR and this effect will be similar after repeated TR. 2) Identify underlying physiological mechanisms responsible for changes in energy and protein metabolism following TR.