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.Sickle cell disease (SCD) is one of the most prevalent genetic disorders in the United States. Based on the National Center for Health Statistics report(1989-1993), there were on the average an estimated 75,000 hospitalizations per year of children and adults with SCD. The largest proportion of SCD occurs in blacks, both living in Africa and in countries with a slavw trade history. Despite the increasing awareness that nutritional factors play a crucial role in the expression of a variety of chronic diseases, their roles in sickle cell disease pathophysiology and treatment have not been fully examined. The purpose of the study is to conduct a comparative assessment of the nutritional status, energy expenditure, and hematological analysis of Black male and female, adult sickle cell patients and also between sickle cell and non-sickle patients. The study will be conducted at the General Clinical Research Center (GCRC) at Howard University Hospital over a twenty-four (24) hour period. Twenty (20) homozygous sickle cell patients (HbSS) will be selected at random from the Howard University Hospital Sickle Cell Clinic. Groups I and II will be contols without sickle cell disease; ten (10) males and females respectively. Nutitional status will be determine by conducting dietary analysis, anthropometric measurement, body composition and biochemical assessment. Dietary data will be gleaned from a weighed food intake. Body composition will be determine with the use of dual-energy X-ray absorptiometry (DEXA). Anthropometric data - height, weight and body mas index(BMI) will also be determined. Total energy expenditure will be measured by indirect calorimetry. Also, hematological parameters will be measured - red blood cell indices, reticulocyte count, HbSS and fetal hemoglobin(HbF). The data will be analyzed by computer using Statistical Package for Social Sciences (SPSS) for Windows, Version 13.0(SPSS Inc., Chicago, IL). Descriptive statistics will be used in data analysis. Data will be presented as means ? standard errors of the mean (SEM). One way analysis of variance (ANOVA) and Tukey's post hoc tests will be used to assess differences between subjects. Pearson's correlation will be used to determine correlation between selected variables. Statistical significance will be determine at the 5% level.
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