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Patients with sickle cell disease (SCD) have poorly deformable red blood cells (RBC) that may impede blood flow into microcirculation. Very few studies have been able to directly visualize microcirculation in humans with SCD. Sublingual video microscopy was performed in eight healthy (HbAA genotype) and four sickle cell individuals (HbSS genotype). Their hematocrit, blood viscosity, red blood cell deformability, and aggregation were individually determined through blood sample collections. Their microcirculation morphology (vessel density and diameter) and microcirculation hemodynamics (local velocity, local viscosity, and local red blood cell deformability) were investigated. The De Backer score was higher (15.9 mm) in HbSS individuals compared to HbAA individuals (11.1 mm). RBC deformability, derived from their local hemodynamic condition, was lower in HbSS individuals compared to HbAA individuals for vessels < 20 μm. Despite the presence of more rigid RBCs in HbSS individuals, their lower hematocrit caused their viscosity to be lower in microcirculation compared to that of HbAA individuals. The shear stress for all the vessel diameters was not different between HbSS and HbAA individuals. The local velocity and shear rates tended to be higher in HbSS individuals than in HbAA individuals, notably so in the smallest vessels, which could limit RBC entrapment into microcirculation. Our study offered a novel approach to studying the pathophysiological mechanisms of SCD with new biological/physiological markers that could be useful for characterizing the disease activity.
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http://dx.doi.org/10.3390/ijms24043621 | DOI Listing |
BMC Prim Care
August 2025
Faculty of Medicine, University of Lubumbashi, Lubumbashi, Democratic Republic of the Congo.
Introduction: Sickle cell disease (SCD) is an inherited genetic disorder that represents a major public health challenge in sub-Saharan Africa, particularly in the Democratic Republic of the Congo (DRC). This disease, characterized by an amino acid substitution in the beta-globin chain, requires early management to reduce serious complications and improve patients' quality of life. This study aimed to test the feasibility of integrating newborn screening for SCD into primary health care in Bukavu, in the DRC, to improve health outcomes for affected infants and reduce serious complications associated with the disease.
View Article and Find Full Text PDFFront Biosci (Schol Ed)
June 2025
Centro de Investigação em Antropologia e Saúde (CIAS), Universidade de Coimbra, 3000 Coimbra, Portugal.
Background: While an increase in fetal hemoglobin (HbF) has no consequences in healthy adults, clinical benefits can be promoted in sickle cell disease (SCD) and β-thalassemia patients. Single-nucleotide polymorphisms (SNPs) in three genomic regions: the gene cluster, the gene, and the () intergenic region, have been associated with HbF regulation. Therefore, the present study aimed to examine the potential association of SNPs in (rs11886868 and rs1427407), (rs66650371 and rs4895441), (rs7482144), and (rs7924684) with HbF levels in an adult population sample from São Tomé e Príncipe (Central Africa).
View Article and Find Full Text PDFWest Afr J Med
January 2025
Haematology and Immunology Department, University of Nigeria Teaching Hospital, Ituku Ozalla, Enugu State, Nigeria.
Background: According to the World Health Organization, about 5% of the world's population has the abnormal haemoglobin gene with Nigeria having the highest burden of sickle cell disease (SCD). Concerted efforts should target the reduction of SCD prevalence.
Objective: This study aimed to determine the various factors that contribute to the non-dwindling prevalence of SCD in Nigeria.
West Afr J Med
December 2024
Department of Paediatrics, College of Medicine, University of Benin Teaching Hospital (UBTH), Benin, Edo State, Nigeria.
Introduction: Sickle Cell Anaemia (SCA) is the homozygous state of heamoglobin SS gene. It is highly prevalent in Sub-saharan Africa with Nigeria ranking highest for people living with the disease. Blood pressure recording in individuals with SCA has been known to be influenced by certain factors such as age, sex, body mass index and hemoglobin level.
View Article and Find Full Text PDFFood Chem Toxicol
August 2025
Department of Pathology, The Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China; Zhengzhou Key Laboratory of Gynecological Disease's Early Diagnosis, Zhengzhou, China. Electronic address:
Acrylamide (AA), a potential carcinogen mainly present in thermally processed carbohydrate-rich foods, and cigarette smoke, forms acrylamide-hemoglobin adducts (HbAA) and its metabolite glycidamide-hemoglobin adducts (HbGA). Female reproductive lifespan (from menarche to menopause) is the key to women's health management and disease prevention. The reproductive lifespan of US women has shifted, but epidemiological research on its causes is lacking.
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