A complete description of the pathophysiology of sickle cell disease requires a physiologically meaningful measurement of red cell deformability. We have designed and built a system which allows one to determine filtration characteristics of erythrocytes. A dilute red cell suspension is forced through a 3.0-micron polycarbonate Nuclepore membrane with a constant positive pressure of 20 mm Hg. Under these conditions blockage of the pores in the polycarbonate membrane is insignificant and flow is linear. We use the relative number of cells filtered through the membrane as a means of approximating the means deformability of cells in the suspension. Using this system we have compared erythrocytes from various mammals and shown that our technique is sensitive in detecting not only differences in cell deformabilities between mammalian species but also changes in cell deformability of human red cells due to exchange transfusion and application of drugs. There was a positive correlation between cell filtrability and percentage cell recovery (coefficient of correlation, 0.65) and a negative correlation between cell size and filtrability (coefficient of correlation, -0.61). The filtrabilities of normal volunteers and sickle cell disease patients were found to be 71.8 +/- 6.6 and 53.6 +/- 5.0%, respectively. This system is sensitive and reliable, and should be useful in evaluating both the contribution of filtrability to the viability of red cells in vivo and potential therapeutic agents for sickle cell disease.
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http://dx.doi.org/10.1016/0026-2862(87)90002-1 | DOI Listing |
Sickle cell disease (SCD) is the most common genetic disease in the world and a societal challenge. SCD is characterized by multi-organ injury related to intravascular hemolysis. To understand tissue-specific responses to intravascular hemolysis and exposure to heme, we present a transcriptomic atlas in the primary target organs of HbSS vs HbAA transgenic SCD mice.
View Article and Find Full Text PDFBackground And Objective: Sickle cell disease (SCD) is a vascular disease that may affect the retina. This study aimed to evaluate differences in average velocity (AV, mm/s), blood flow (BF, μL/min) and vessel diameter (VD, μm) from the temporal retinal arcades in SCD compared to healthy eyes using Doppler optical coherence tomography (DOCT).
Patients And Methods: A cross-sectional study was conducted between 2021 and 2023.
Front Public Health
January 2025
Center of Excellence for Sickle Cell Disease Research and Training (CESRTA), University of Abuja, Abuja, Nigeria.
Introduction: Newborn Screening (NBS) is a public health program designed to identify and provide early interventions for infants with genetic disorders such as Sickle Cell Disease (SCD). Lack of awareness and unwillingness to participate in the NBS by caregivers and some healthcare workers are major contributing factors impeding NBS for SCD.
Objective: To evaluate the level of awareness and acceptance of NBS for SCD and the determinant factors influencing caregivers in Gwagwalada Area Council of the Federal Capital Territory, Abuja, Nigeria.
Ann Am Thorac Soc
January 2025
University of Illinois in Chicago, Medicine, Chicago, Illinois, United States;
Ann Am Thorac Soc
January 2025
National Heart Lung and Blood Institute Division of Intramural Research, Bethesda, Maryland, United States.
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