1,726,187 research outputs found
Nutritional status, hospitalization and mortality among patients with sickle cell anemia in Tanzania.
Get PDFBACKGROUND: Reduced growth is common in children with sickle cell anemia, but few data exist on associations with long-term clinical course. Our objective was to determine the prevalence of malnutrition at enrollment into a hospital-based cohort and whether poor nutritional status predicted morbidity and mortality within an urban cohort of Tanzanian sickle cell anemia patients. DESIGN AND METHODS: Anthropometry was conducted at enrollment into the sickle cell anemia cohort (n=1,618; ages 0.5-48 years) and in controls who attended screening (siblings, walk-ins and referrals) but who were found not to have sickle cell anemia (n=717; ages 0.5-64 years). Prospective surveillance recorded hospitalization at Muhimbili National Hospital and mortality between March 2004 and September 2009. RESULTS: Sickle cell anemia was associated with stunting (OR=1.92, P<0.001, 36.2%) and wasting (OR=1.66, P=0.002, 18.4%). The greatest growth deficits were observed in adolescents and in boys. Independent of age and sex, lower hemoglobin concentration was associated with increased odds of malnutrition in sickle cell patients. Of the 1,041 sickle cell anemia patients with a body mass index z-score at enrollment, 92% were followed up until September 2009 (n=908) or death (n=50). Body mass index and weight-for-age z-score predicted hospitalization (hazard ratio [HZR]=0.90, P=0.04 and HZR=0.88, P=0.02) but height-for-age z-score did not (HZR=0.93, NS). The mortality rate of 2.5 per 100 person-years was not associated with any of the anthropometric measures. CONCLUSIONS: In this non-birth-cohort of sickle cell anemia with significant associated undernutrition, wasting predicted an increased risk of hospital admission. Targeted nutritional interventions should prioritize treatment and prevention of wasting
Hematological and Genetic Predictors of Daytime Hemoglobin Saturation in Tanzanian Children with and without Sickle Cell Anemia.
Get PDFLow hemoglobin oxygen saturation (SpO2) is common in Sickle Cell Anemia (SCA) and associated with complications including stroke, although determinants remain unknown. We investigated potential hematological, genetic, and nutritional predictors of daytime SpO2 in Tanzanian children with SCA and compared them with non-SCA controls. Steady-state resting pulse oximetry, full blood count, transferrin saturation, and clinical chemistry were measured. Median daytime SpO2 was 97% (IQ range 94-99%) in SCA (N = 458), lower (P < 0.0001) than non-SCA (median 99%, IQ range 98-100%; N = 394). Within SCA, associations with SpO2 were observed for hematological variables, transferrin saturation, body-mass-index z-score, hemoglobin F (HbF%), genotypes, and hemolytic markers; mean cell hemoglobin (MCH) explained most variability (P < 0.001, Adj r (2) = 0.09). In non-SCA only age correlated with SpO2. α-thalassemia 3.7 deletion highly correlated with decreased MCH (Pearson correlation coefficient -0.60, P < 0.0001). In multivariable models, lower SpO2 correlated with higher MCH (β-coefficient -0.32, P < 0.001) or with decreased copies of α-thalassemia 3.7 deletion (β-coefficient 1.1, P < 0.001), and independently in both models with lower HbF% (β-coefficient 0.15, P < 0.001) and Glucose-6-Phosphate Dehydrogenase genotype (β-coefficient -1.12, P = 0.012). This study provides evidence to support the hypothesis that effects on red cell rheology are important in determining SpO2 in children with SCA. Potential mechanisms and implications are discussed
Interview with Isaac Haigler - OH 317
Get PDFIn his interview with Michael Cooke, Isaac Haigler discusses his work with the Sickle Cell Anemia Foundation in Orangeburg County, South Carolina. Mr. Haigler discusses his educational and military background, his history with sickle cell anemia awareness, his work with the Foundation, the Foundation’s goals, educating the community about sickle cell anemia, the Foundation’s impact on the black community of Orangeburg County, the community’s response to the Foundation, the causes of the continued existence of the Foundation, the impact of the Department of Health and Environmental Control (DEHC) on the Foundation, and his dedication to the sickle cell anemia cause and the Black community. This interview was conducted as a part of the Sickle Cell Anemia Foundation of SC Project.https://digitalcommons.winthrop.edu/oralhistoryprogram/1475/thumbnail.jp
Interview with Ruth Erline Mathis Martin - OH 328
Get PDFIn her June 1984 interview with Michael Cooke, Ruth Erline Mathis Martin (1934-2020) discussed her time as a DHEC employee in the sickle cell anemia program. She covered topics of DHEC’s involvement with community groups, sickle cell anemia community groups and their success and failures, and the James R. Clark Sickle Cell Foundation. This interview was conducted for inclusion into the Louise Pettus Archives and Special Collections Oral History Program.https://digitalcommons.winthrop.edu/oralhistoryprogram/1293/thumbnail.jp
Interview with Louvenia Barksdale - OH 325
Get PDFThis collection includes an interview of Louvenia D. Barksdale (1913-1990), who founded the Sickle Cell Foundation in 1974 (which was renamed the L.D. Barksdale Sickle Cell Anemia Foundation in her honor). In this interview, Ms. Barksdale discusses her early life, education, and experience as an African American educator in Spartanburg, South Carolina. Ms. Barksdale also discusses her initial interest in sickle cell disease, the formation of her organization, the South Carolina Genetic Grant, the functions of the L.D. Barksdale Sickle Cell Anemia Foundation (particularly testing, screening, transportation, and caring for the community), the difficulties of running the organization, donations from other organizations, the educational campaign about sickle cell disease, and the experience of sickle cell patients. She also describes the connection between her foundation and Dr. James R. Clark (a well-known sickle cell anemia expert from South Carolina).https://digitalcommons.winthrop.edu/oralhistoryprogram/1292/thumbnail.jp
Pulmonary hypertension in Nigerian adults with sickle cell anemia
No full textValentine N Amadi,1 Michael O Balogun,2 Norah O Akinola,3 Rasaaq A Adebayo,2 Anthony O Akintomide2 1Department of Internal Medicine, Federal Medical Centre, Asaba, Delta State, 2Cardiac Care Unit, Department of Medicine, 3Department of Haematology and Blood Transfusion, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria Background: Sickle cell anemia (SCA) is the commonest hemoglobinopathy and is associated with high morbidity and mortality. Pulmonary hypertension (PH) is reported to play a significant role in this regard. There is very limited literature on PH in SCA in Nigeria.Objectives: The objectives of this study were to determine the prevalence of Doppler-derived PH in SCA, assess its influence on exercise capacity, and determine the correlates and predictors of measures of estimated pulmonary pressure.Methods: A total of 92 SCA subjects had echocardiography and 6-minute self-paced walking exercise. PH was diagnosed by Doppler echocardiography on finding a tricuspid regurgitant velocity (TRV) of ≥2.5 m/s. The pulmonary flow profile was also assessed to estimate mean pulmonary arterial pressure (MPAP).Results: Doppler-derived PH was detected in 23.9% of adults with SCA. The 6-minute walking distance (6MWD) was significantly lower in SCA adults with PH than in those without PH (380.33 ± 63.17 m vs 474.28 ± 76.74 m; p = 0.014). TRV and estimated MPAP had a significant inverse correlation with the 6MWD (r = -0.442; p < 0.001 and r = -0.571; p < 0.001, respectively).Conclusion: PH as derived by Doppler is common in Nigerian adults with SCA and has a significantly negative influence on exercise capacity. Screening for PH should be encouraged to optimize management and thus improve their quality of life and life expectancy. Keywords: pulmonary hypertension, Nigerian adults, sickle cell anemia, doppler-derive
Interview with Edward Bynum - OH 322
Get PDFThis collection includes an interview of Edward Bynum (1933-2012) concerning his work with the Dr. James R. Clark Memorial Sickle Cell Anemia Foundation. Bynum discusses his education, his service in the Korean War on a hospital ship, and his medical career in hematology afterwards. Bynum discusses Dr. James R. Clark, the foundation of Dr. Clark’s sickle cell foundation, hematology testing for multiple diseases, the need for educating the public about sickle cell anemia, funding from various (mostly Black) organizations, hemoglobinopathy, and his work within Dr. Clark’s sickle cell foundation. Bynum also describes his sickle cell research and work in other parts of the U.S., his creation of the Center for Disease Control in Columbia, SC, training for technicians who can test for sickle cell, and the politics surrounding sickle cell disease.https://digitalcommons.winthrop.edu/oralhistoryprogram/1305/thumbnail.jp
Telomere Dynamics in Sickle Cell Anemia: Unraveling Molecular Aging and Disease Progression
Get PDFEmmanuel Ifeanyi Obeagu,1 Getrude Uzoma Obeagu2 1Department of Medical Laboratory Science, Kampala International University, Ishaka, Uganda; 2School of Nursing Science, Kampala International University, Ishaka, UgandaCorrespondence: Emmanuel Ifeanyi Obeagu, Department of Medical Laboratory Science, Kampala International University, Uganda, Email [email protected]: Sickle Cell Anemia (SCA) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells. While extensive research has unraveled many aspects of the genetic and molecular basis of SCA, the role of telomere dynamics in disease progression remains a relatively unexplored frontier. This review seeks to provide a comprehensive examination of telomere biology within the context of SCA, aiming to elucidate its potential impact on molecular aging and the progression of the disease. The impact of oxidative stress on telomere dynamics in SCA is explored, with a particular focus on how increased reactive oxygen species (ROS) may contribute to accelerated telomere shortening and genomic instability. Furthermore, the potential relationship between telomere dysfunction and cellular senescence in SCA is investigated, shedding light on how telomere dynamics may contribute to the premature aging of cells in this population. The review concludes by summarizing key findings and proposing potential therapeutic strategies targeting telomere dynamics to mitigate disease progression in SCA. It also identifies gaps in current understanding and suggests avenues for future research, emphasizing the importance of further investigating telomere biology to advance our understanding of molecular aging and disease progression in Sickle Cell Anemia. This comprehensive exploration of telomere dynamics in SCA offers insights into potential mechanisms of molecular aging and disease progression, paving the way for targeted therapeutic interventions and improved disease management.Keywords: sickle cell anemia, telomere, molecular aging, hemoglobinopathy, telomere shortening, oxidative stres
Interview with James Earl Padgett, Jr. - OH 316
Get PDFIn his interview with Michael Cooke, Dr. James Earl Padgett, Jr. (1930-2016) discusses his work with the Department of Health and Environmental Control of South Carolina. Dr. Padgett describes the efforts to develop a program for sickle cell anemia. He details how the Health Department set up their program to screen and counsel people who might have the disease
Sickle cell anemia.
No full textThis thesis is about the disease called sickle cell anemia, or drepanocytosis. In this thesis is described the history of the disease, pathophysiology, laboratory features, various clinical features, diferencial diagnosis, quality of life in sickle cell anemia and therapy
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