scholarly journals Legitimate suffering: a case of belonging and sickle cell trait in Brazil

BioSocieties ◽  
2021 ◽  
Author(s):  
Melissa Creary
Keyword(s):  
Lived Experience ◽  
Sickle Cell ◽  
Blood Cells ◽  
Sickle Cell Trait ◽  
Genetic Disorder ◽  
The Other ◽  
Patient Organization ◽  
Cell Disease ◽  
Racial Inequalities ◽  
Patient Activism

AbstractPatient activism organizations are formed around and seek legitimacy via both biological and biographical identities (Fassin, in: Theory Cult Soc 26(5):44–60, 2009). In the case of sickle cell disease (SCD) in Brazil, two different modes of suffering authenticate the lived experience—one is based on the disease state, the other is based on the ways in which racial inequalities and disadvantage contribute to its own suffering while also entangled with disease-based suffering. SCD is a rare genetic disorder that affects red blood cells and whose hallmark symptom is pain. This paper places an ethnographic focus on the failed mobilization of suffering by an organization leader in attempts to make claims for inclusion. The leader’s social and biological identities of mother, sickle cell trait carrier, middle class, and mulata disrupted biosocial cohesion. This disruption reveals a hierarchy of suffering, where some indices of suffering are delegitimized. This hierarchy illuminates how exclusion and representation work within a patient organization whose membership embody both physical and social distress.

scholarly journals Valsartan impedes epinephrine-induced ICAM-4 activation on normal, sickle cell trait and sickle cell disease red blood cells

PLoS ONE ◽  
2019 ◽  
Vol 14 (5) ◽  
pp. e0216467
Author(s):  
Jing Zhang ◽  
Sasia-Marie Jones ◽  
George Lykotrafitis ◽  
Biree Andemariam
Keyword(s):  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Sickle Cell Trait ◽  
Cell Disease

scholarly journals Red blood cell mannoses as phagocytic ligands mediating both sickle cell anaemia and malaria resistance

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Huan Cao ◽  
Aristotelis Antonopoulos ◽  
Sadie Henderson ◽  
Heather Wassall ◽  
John Brewin ◽  
...  
Keyword(s):  
Molecular Weight ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Cells ◽  
Sickle Cell Anaemia ◽  
Sickle Cell Trait ◽  
Mannose Receptor ◽  
Cell Disease ◽  
Infected Rbcs ◽  
High Mannose

AbstractIn both sickle cell disease and malaria, red blood cells (RBCs) are phagocytosed in the spleen, but receptor-ligand pairs mediating uptake have not been identified. Here, we report that patches of high mannose N-glycans (Man5-9GlcNAc2), expressed on diseased or oxidized RBC surfaces, bind the mannose receptor (CD206) on phagocytes to mediate clearance. We find that extravascular hemolysis in sickle cell disease correlates with high mannose glycan levels on RBCs. Furthermore, Plasmodium falciparum-infected RBCs expose surface mannose N-glycans, which occur at significantly higher levels on infected RBCs from sickle cell trait subjects compared to those lacking hemoglobin S. The glycans are associated with high molecular weight complexes and protease-resistant, lower molecular weight fragments containing spectrin. Recognition of surface N-linked high mannose glycans as a response to cellular stress is a molecular mechanism common to both the pathogenesis of sickle cell disease and resistance to severe malaria in sickle cell trait.

scholarly journals High mannose N-glycans on red blood cells as phagocytic ligands, mediating both sickle cell anaemia and resistance to malaria

2020 ◽  
Author(s):  
Huan Cao ◽  
Aristotelis Antonopoulos ◽  
Sadie Henderson ◽  
Heather Wassall ◽  
John Brewin ◽  
...  
Keyword(s):  
Molecular Weight ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Sickle Cell Anaemia ◽  
Sickle Cell Trait ◽  
Mannose Receptor ◽  
Cell Disease ◽  
High Mannose ◽  
Haemoglobin S

AbstractIn both sickle cell disease (SCD) and malaria, red blood cells (RBCs) are phagocytosed in the spleen, but receptor-ligand pairs mediating uptake have not been identified. Here, we report that patches of high mannose N-glycans (Man5-9GlcNAc2), expressed on diseased or oxidized RBC surfaces, bind the mannose receptor (CD206) on phagocytes to mediate clearance. Extravascular haemolysis in SCD correlates with high mannose glycan levels on RBCs. Infection of RBCs with Plasmodium falciparum expose surface mannose N-glycans on healthy RBCs, which occurred at significantly higher levels on RBCs from subjects with sickle cell trait compared to those lacking haemoglobin S. The glycans were associated with high molecular weight complexes and protease-resistant, lower molecular weight fragments containing spectrin. Recognition of surface N-linked high mannose glycans, a novel response to cellular stress, is the first molecular mechanism common to both the pathogenesis of SCD and resistance to severe malaria in sickle cell trait.

scholarly journals The Pattern of Sickle Cell Disease in Sickle Cell Patients from Northwestern Nigeria

2016 ◽  
Vol 8 ◽  
pp. CMT.S38164 ◽  
Author(s):  
Saganuwan Alhaji Saganuwan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Genetic Disorder ◽  
Hematological Parameters ◽  
Clinical Signs ◽  
Renal Diseases ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Significant Difference

Sickle cell disease is caused due to a genetic disorder, which accounts for people dying at an early age in Nigeria. A retrospective study of sickle cell disease patients was carried out with a view to determining the disease pattern in sickle cell patients from the Northwestern Nigeria. Case notes of 319 sickle cell patients were collected and reviewed retrospectively. The prevalence of sickle cell trait, comorbidity of sickle cell disease and malaria, and the effects of sickle cell disease and age on the weight and hematological parameters of sickle cell patients were determined and analyzed. Results showed the prevalence rate of sickle cell trait to be 61.8% (197) and that of non-sickle cell trait to be 38.2% (122). The sickle cell trait comprised 96 males (48.7%) and 101 females (51.3%). Among these patients, 51 (41.8%) males and 71 (58.2%) females had malaria. However, 35.4% (113) of sickle cell patients and 7.5% (24) of malaria patients showed anemia. Genotyping revealed 32 AS (16.2%), 102 SS (51.8%), SS+F (3.6%), and 56 SC (28.4%). The associated prevalence rates of clinical signs were pain/crisis 45.1% (89), pneumonia 28.4% (56), gastric disorders 9.1% (18), central nervous system (CNS) disorders 4.1% (8), renal diseases 2.5% (5), musculo-skeletal disorders 2.5% (5), conjunctivitis 0.5% (1), acute chest syndrome 0.5% (1), cholecystitis 0.5% (1), hemophilia 0.5% (1), fever 0.5% (1), priapism 2.0% (4), splenomegaly 2.0% (4), and epistaxis 1.5% (3). Few patients lived up to 49 years. There was significant difference ( P < 0.05) in hematological parameters of the patients from various age groups. The use of anti-sickling, hematonic, analgesic, anti-inflammatory, and antimalarial drugs in the treatment of the affected disease in patients might have improved their quality of life.

scholarly journals Frequencies of Abnormal Haemoglobin Electrophoretic Patterns in Hemolytic Anemia cases in District Swat.

2020 ◽  
Vol 10 (2) ◽  
Author(s):  
Amin Ullah ◽  
Amreek Lal ◽  
Siyab Ahmad ◽  
Akhtar Nawab ◽  
Salman Khan ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Genetic Disorder ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Cell Disease ◽  
Electrophoretic Patterns ◽  
One Year ◽  
Complete Blood Counts

Background: Thalassemia is a genetic disorder due to deletion or mutation in the gene for alpha or beta chain of hemoglobin. Thalassemia, sickle cell disease, HbD, HbE, HbC and other such disorders are genetic defects associated with hemolytic anemia's and other complications. The prevalence of these are significantly growing in Pakistan especially in the northern areas of Pakistan. Its prompt detection is of paramount importance, both for the prevention of thalassemia major and clinically severe other hemoglobinopathies as well as for the epidemiological purposes.Objective: To assess the frequencies of abnormal hemoglobin variants in the suspected cases of hemolytic anemia's, in the population of district swat. Material and Methods: A total of 1832 suspected cases of hemolytic anemias admitted in SGTH/SMC were assessed after taking clinical and familial history. Whole blood samples were collected in EDTA tube; complete blood counts with peripheral smears were prepared. All samples were processed with fully automatic micro capillary hemoglobin electrophoretic movability (SEBEA micro capillary electrophoresis).Results: A normal Hb pattern was observed in 1224 (66.81%) cases and abnormalities were detected in 608 (33.18%) cases. â (beta) thalassemia trait was the commonest abnormality found in 477 (26.03 %) patients followed by â thalassemia major 53 (2.89 %), â thalassemia intermedia 24 (1.31 %), raised HbF for age review after one year 17 (0.92 %), Sickle cell trait 9 (0.49 %), Sickle cell disease 8 (0.43 %), sickle/beta-thalassemia 6 (0.32 %) HbD Trait 5 (0.27 %), HbE (0.21%) and HbC (0.10%) were common hemoglobinopathies.Conclusion: Amongst hemoglobinopathies, â- (beta) thalassemia and sick cell trait/disease is the most common of the hemoglobinopathies in the study area.

Sphingosine 1-Phosphate (S1P)/S1P Receptor 1 Pathway Has an Essential Role for Sickle Cell Disease

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4063-4063
Author(s):  
Yujin Zhang ◽  
Shushan Zhao ◽  
Hongyu Wu ◽  
Xia Hu ◽  
Renna Luo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Inflammatory Cytokines ◽  
Sickle Cell ◽  
Blood Cells ◽  
Genetic Disorder ◽  
White Blood Cells ◽  
High Morbidity ◽  
Cell Disease ◽  
S1p Receptors ◽  
Sphingosine 1 Phosphate

Abstract Sickle cell disease (SCD) is a devastating hemolytic genetic disorder associated with high morbidity and mortality. In order to understand the pathogenesis of this disease, we have conducted non-biased metabolomic screening and found that circulating sphingosine-1-phosphate (S1P) was significantly elevated in mice and patients with SCD. S1P is an important bioactive lipid signaling molecule known to regulate inflammation. Our previous study demonstrated that reduced S1P level in plasma and erythrocytes by treatment with sphingosine kinase 1 (SPHK1) inhibitor, PF-543, significantly decreased sickling cells, hemolysis and inflammation in SCD mouse model, which indicated that S1P may play an important role in an SCD complication, especially in inflammation. S1P engages five G-protein coupled receptors known as S1PR1-5. Targeting S1P signaling has been successfully applied in the treatment of the autoimmune disease-multiple sclerosis with the compound named FTY720. In order to understand the roles of S1P/S1PRs signal pathway in pathophysiology of SCD, we treated SCD mice with S1P receptors antagonist FTY720. The results showed that FTY720 successfully inhibited S1P receptors, especially S1P1 expression on immune cells from thymus and lymph node (P<0.05). Circulating white blood cells and inflammatory cytokines, such as CRP, IL-1β, TNF-α and IL-6, also decreased significantly measured by ELSIA kit. Additionally, FTY720 treatment significantly ameliorated organ damage. To investigate the roles of S1P1 receptor in SCD, we treated SCD mice with S1P1 specific antagonist, SEW2871. The results demonstrated that circulating white blood cells and inflammatory cytokines reduced significantly. Histologic studies revealed that the necrosis and congestion of multiple organs including kidney, lung and spleen were substantially reduced by SEW2841.Our studies demonstrate the elevated circulating S1P signaling via its receptor (likely S1PR1) directly contributes to inflammation and multiple tissue damage. Thus, it provides strong evidence that S1P/S1P1 pathway is likely a therapeutic target for SCD. Disclosures No relevant conflicts of interest to declare.

Thromboelastographic Characterization of the Activated Clotting System in Children with Sickle Cell Trait or Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3809-3809
Author(s):  
Shveta Gupta ◽  
Roxana Carmona ◽  
Jemily Malvar ◽  
Guy Young
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Cells ◽  
Thrombin Generation ◽  
Sickle Cell Trait ◽  
Coagulation System ◽  
Cell Disease ◽  
D Dimer

Abstract Background: Recent epidemiological evidence suggests sickle cell disease (SCD) and sickle cell trait (SCT) are risk factors for venous thromboembolism. The increased in vivo markers of thrombin generation support the notion that such patients are in a chronic hypercoagulable state. In an attempt to better understand the underlying mechanism, global hemostatic assays including thrombin generation assay (TGA) and thromboelastography (TEG) have been utilized by several groups, with inconsistent results either due to small sample size or technical differences. As opposed to the bleeding disorders, the traditional methods of TEG are not sensitive to hypercoagulability. Our group developed modified methods that prolonged the baseline TEG parameters to enhance this sensitivity. These altered TEG profiles were shown to be significantly shortened by increasing concentrations of thrombin in vitro. Objectives: Global hemostatic characterization of children with SCD or SCT by using TGA and modified TEG methods. Materials and methods: In this pilot study, we obtained hemostatic data including complete blood count with differential, reticulocyte count, fibrinogen, D-dimer, thrombin antithrombin complex and prothrombin fragment 1.2 on specimens from subjects with SCD in their usual state of health, subjects with SCT and healthy controls (NC). In addition global hemostatic assays including standard and modified thromboelastography methods as well as thrombin generation assays were performed. Results: Thirty-nine African-American subjects were recruited: 12 NC, 14 SCT and 13 SCD. The median ages for the groups were 12 (Range: 5-39), 19 (Range: 2-40) and 8 (Range: 2-14) years for NC, SCT, and SCD, respectively. Females represented 58% of the NC, 57% of the SCT subjects and 38% of the SCD subjects. In vivo markers of thrombin generation and activation of fibrinolysis including D-dimer and thrombin-antithrombin complexes were higher in SCD subjects as compared to SCT and NC (p=0.001; p=0.05 respectively). Reaction (R) time, and Kinetic (K) time with modified TEG methods was significantly shorter in SCD when compared to SCT and NC (p=0.014, p=0.038) respectively. Angle (alpha) and maximum amplitude (MA) did not show any significant differences between the groups. TGA profiles did not show any difference between the three groups either. Conclusion: The in vivo use of modified thromboelastography methods is able to detect the hypercoagulability known to occur in the sickle cell disease population but a larger sickle cell trait cohort needs to be studied to determine if this group also has hypercoagulability. Importantly, this study, the first to evaluate both TEG and TGA assays in SCD or SCT population demonstrates the importance of using whole blood to differentiate these groups as the TEG assay demonstrated differences the TGA could not detect. As SCD and SCT subjects have a highly complex physiology with not only alterations in almost all the components of the coagulation system, but also variation in the quantity and function of other blood components including white blood cells, platelets and red blood cells, TEG may prove a good tool in measuring a change in the activity of the coagulation system rather than a single baseline measurement. Our results support the need for further studies using thromboelastography in SCD and SCT population in order to better understand and triage this cohort of patients for risks of thrombotic complications. Disclosures No relevant conflicts of interest to declare.

Increased Procoagulant Activity of Red Blood Cells from Patients with Homozygous Sickle Cell Disease and β-Thalassemia

1996 ◽  
Vol 76 (03) ◽  
pp. 322-327 ◽  
Author(s):  
Dominique Helley ◽  
Amiram Eldor ◽  
Robert Girot ◽  
Rolande Ducrocq ◽  
Marie-Claude Guillin ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Annexin V ◽  
Mean Value ◽  
Procoagulant Activity ◽  
Dependent Manner ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

SummaryIt has recently been proved that, in vitro, red blood cells (RBCs) from patients with homozygous β-thalassemia behave as procoagulant cells. The procoagulant activity of β-thalassemia RBCs might be the result of an increased exposure of procoagulant phospholipids (i. e. phosphatidylserine) in the outer leaflet of the membrane. In order to test this hypothesis, we compared the catalytic properties of RBCs of patients with β-thalassemia and homozygous sickle cell disease (SS-RBCs) with that of controls. The catalytic parameters (Km, kcat) of prothrombin activation by factor Xa were determined both in the absence and in the presence of RBCs. The turn-over number (kcat) of the reaction was not modified by normal, SS- or (3-thalassemia RBCs. The Km was lower in the presence of normal RBCs (mean value: 9.1 µM) than in the absence of cells (26 µM). The Km measured in the presence of either SS-RBCs (mean value: 1.6 µM) or β-thalassemia RBCs (mean value: 1.5 pM) was significantly lower compared to normal RBCs (p <0.001). No significant difference was observed between SS-RBCs and p-thalassemia RBCs. Annexin V, a protein with high affinity and specificity for anionic phospholipids, inhibited the procoagulant activity of both SS-RBCs and (3-thalassemia RBCs, in a dose-dependent manner. More than 95% inhibition was achieved at nanomolar concentrations of annexin V. These results indicate that the procoagulant activity of both β-thalassemia RBCs and SS-RBCs may be fully ascribed to an abnormal exposure of phosphatidylserine at the outer surface of the red cells.

Microsurgery in the Sickle Cell Trait Population: Is It Actually Safe?

2020 ◽  
pp. 1-2
Author(s):  
Michael Alperovich ◽  
Eric Park ◽  
Michael Alperovich ◽  
Omar Allam ◽  
Paul Abraham
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Free Flap ◽  
Near Infrared ◽  
Sickle Cell Trait ◽  
Cell Disease ◽  
Flap Transfer ◽  
Free Flap Transfer ◽  
Patient Reported ◽  
Deep Inferior Epigastric Perforator

Although sickle cell disease has long been viewed as a contraindication to free flap transfer, little data exist evaluating complications of microsurgical procedures in the sickle cell trait patient. Reported is the case of a 55-year-old woman with sickle cell trait who underwent a deep inferior epigastric perforator (DIEP) microvascular free flap following mastectomy. The flap developed signs of venous congestion on postoperative day two but was found to have patent arterial and venous anastomoses upon exploration in the operating room. On near-infrared indocyanine green angiography, poor vascular flow was noted despite patent anastomoses and strong cutaneous arterial Doppler signals. Intrinsic microvascular compromise or sickling remains a risk in the sickle cell trait population as it does for the sickle cell disease population. Just like in sickle cell disease patients, special care should be taken to optimize anticoagulation and minimize ischemia-induced sickling for patients with sickle cell trait undergoing microsurgery.

Microfluidic electrical impedance assessment of red blood cell mediated microvascular occlusion

Lab on a Chip ◽  
2021 ◽  
Author(s):  
Yuncheng Man ◽  
Debnath Maji ◽  
Ran An ◽  
Sanjay Ahuja ◽  
Jane A Little ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Blood Cells ◽  
Electrical Impedance ◽  
Cell Disease ◽  
Blood Disorders

Alterations in the deformability of red blood cells (RBCs), occurring in hemolytic blood disorders such as sickle cell disease (SCD), contributes to vaso-occlusion and disease pathophysiology. However, there are few...

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