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Sickle cell disease thrombosis

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https://www.readbyqxmd.com/read/29372059/factor-v-leiden-g1691a-and-prothrombin-g20210a-mutations-among-palestinian-patients-with-sickle-cell-disease
#1
Fekri Samarah, Mahmoud A Srour
Background: Vascular thrombosis is an important pathophysiological aspect of sickle cell disease (SCD). This study aimed to investigate the prevalence and clinical impact of factor V Leiden G1691A (FVL) and prothrombin G20210A mutations among Palestinian sickle cell disease (SCD) patients. Methods: A total of 117 SCD patients, including 59 patients with sickle cell anemia (SS), 33 patients with sickle β-thalassemia and 25 individuals with sickle cell trait (AS) were studied...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29337222/blockade-of-placental-growth-factor-reduces-vaso-occlusive-complications-in-murine-models-of-sickle-cell-disease
#2
Jian-Ming Gu, Shujun Yuan, Derek Sim, Keith Abe, Perry Liu, Martin Rosenbruch, Peter Bringmann, Katalin Kauser
Vaso-occlusive crisis (VOC) is the most common and debilitating complication of sickle cell disease (SCD); recurrent episodes cause organ damage and contribute to early mortality. Plasma placental growth factor (PlGF) levels are elevated in SCD and can further increase under hypoxic conditions in SCD mice. Treatment with a PlGF-neutralizing antibody (anti-PlGF Ab) in SCD mice reduced levels of monocyte chemoattractant protein-3, eotaxin, macrophage colony-stimulating factor, plasminogen activator inhibitor-1 significantly, and macrophage-derived chemokine and macrophage inflammatory protein-3β moderately; this may contribute to inhibition of leukocyte recruitment, activation, and thrombosis...
January 11, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29301288/use-of-pleuroperitoneal-shunt-in-chylothorax-related-to-central-line-associated-thrombosis-in-sickle-cell-disease
#3
Elizabeth Spiwak, Chad Wiesenauer, Arun Panigrahi, Ashok Raj
Central vein thrombosis as a cause of chylothorax is uncommon, and in a few cases in the literature was related to thrombotic complications of central venous access devices (CVAD). Superior vena cava (SVC) occlusion-induced chylothorax has been described in adult sickle cell disease (SCD) in a setting of chronic indwelling CVAD. There are limited reports on chylothorax induced by central venous thrombosis secondary to chronic CVAD in children with SCD. We describe an 8-year-old male patient, with a history of SCD, maintained on long term erythrocytapheresis for primary prevention of stroke, and whose clinical course was complicated by chylothorax which was successfully treated with a pleuroperitoneal shunt...
January 2, 2018: Children
https://www.readbyqxmd.com/read/29152296/catheter-related-right-atrial-thrombus-in-sickle-cell-disease
#4
Chen Zhao, Bharath Sathya, Rosa Nadal Rios, Andrew E Arai, Alessandra Brofferio, Swee Lay Thein, A Parker Ruhl
Catheter-related right atrial thrombus (CRAT) can occur in patients with sickle cell disease, particularly if additional risk factors for thrombosis are present. Cardiac MRI may differentiate thrombi from other types of atrial masses. Treatment should include anticoagulation and the timing of catheter removal should balance the potential risk of embolization.
November 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28905364/a-clinical-risk-score-for-pulmonary-artery-thrombosis-during-acute-chest-syndrome-in-adult-patients-with-sickle-cell-disease
#5
Anaïs Winchenne, Jérôme Cecchini, Jean-François Deux, Nicolas De Prost, Keyvan Razazi, Guillaume Carteaux, Frederic Galacteros, Anoosha Habibi, Pablo Bartolucci, Giovanna Melica, Mehdi Khellaf, Marc Michel, Bernard Maitre, Armand Mekontso Dessap
Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Patients with SCD were investigated by MDCT during ACS. A logistic regression was performed to determine independent risks factors for PAT and to build the PAT-ACS risk score...
September 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28888622/the-utility-of-thromboelastography-and-thrombin-generation-in-assessing-the-prothrombotic-state-of-adults-with-sickle-cell-disease
#6
Marije Wijnberge, Kiran Parmar, Rachel Kesse-Adu, Jo Howard, Alexander T Cohen, Beverley J Hunt
INTRODUCTION: Previous studies have suggested a chronic hypercoagulable state in SCD, and that thrombosis also plays a role in the pathophysiology of sickle cell vaso-occlusive pain crises (VOC). Studies looking at thrombin generation have produced conflicting results. In this study we aimed to assess and compare whole blood thromboelastography (TEG) and plasma Calibrated Automated Thrombogram (CAT) in SCD versus healthy controls and in four different SCD subgroups. MATERIALS AND METHODS: In this prospective observational study, TEG and 1pM TF activated CAT assays were performed in citrated blood samples from 77 adult (18-66years old) SCD patients (HbSS and HbSB) and 22 healthy (HbAA) ethnically-matched controls...
October 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28878012/synthetic-oligosaccharides-can-replace-animal-sourced-low-molecular-weight-heparins
#7
Yongmei Xu, Kasemsiri Chandarajoti, Xing Zhang, Vijayakanth Pagadala, Wenfang Dou, Debra Moorman Hoppensteadt, Erica M Sparkenbaugh, Brian Cooley, Sharon Daily, Nigel S Key, Diana Severynse-Stevens, Jawed Fareed, Robert J Linhardt, Rafal Pawlinski, Jian Liu
Low-molecular weight heparin (LMWH) is used clinically to treat clotting disorders. As an animal-sourced product, LMWH is a highly heterogeneous mixture, and its anticoagulant activity is not fully reversible by protamine. Furthermore, the reliability of the LMWH supply chain is a concern for regulatory agencies. We demonstrate the synthesis of heparin dodecasaccharides (12-mers) at the gram scale. In vitro experiments demonstrate that the anticoagulant activity of the 12-mers could be reversed using protamine...
September 6, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28837214/combined-genotypes-of-the-mbl2-gene-related-to-low-mannose-binding-lectin-levels-are-associated-with-vaso-occlusive-events-in-children-with-sickle-cell-anemia
#8
Fernanda Silva Medeiros, Taciana Furtado de Mendonça, Katiuscia Araújo de Miranda Lopes, Laís Medeiros da Câmara França, Andreia Soares da Silva, Luydson Richardson Silva Vasconcelos, Maria do Carmo Valgueiro Costa de Oliveira, Ana Cláudia Mendonça Dos Anjos, Betânia Lucena Domingues Hatzlhofer, Marcos André Cavalcanti Bezerra, Aderson da Silva Araújo, Patrícia Moura, Maria do Socorro de Mendonça Cavalcanti
Sickle cell anemia (SCA) presents heterogenous clinical manifestations that cannot be explained solely by alterations to hemoglobin (Hb); other components such as endothelial adhesion, thrombosis and inflammation may be involved. The mannose-binding lectin (MBL) has an important role in innate immunity and inflammatory diseases. In this report, we describe an association between MBL2 polymorphism related to low production of serum MBL and the frequency of vasoocclusive events (FVOE) in children ≤ 5 years old with SCA (p = 0...
July 2017: Genetics and Molecular Biology
https://www.readbyqxmd.com/read/28811305/red-blood-cells-in-thrombosis
#9
REVIEW
James R Byrnes, Alisa S Wolberg
Red blood cells (RBCs) have historically been considered passive bystanders in thrombosis. However, clinical and epidemiological studies have associated quantitative and qualitative abnormalities in RBCs, including altered hematocrit, sickle cell disease, thalassemia, hemolytic anemias, and malaria, with both arterial and venous thrombosis. A growing body of mechanistic studies suggests that RBCs can promote thrombus formation and enhance thrombus stability. These findings suggest that RBCs may contribute to thrombosis pathophysiology and reveal potential strategies for therapeutically targeting RBCs to reduce thrombosis...
October 19, 2017: Blood
https://www.readbyqxmd.com/read/28771279/superoxide-dismutase-2-is-dispensable-for-platelet-function
#10
Trevor P Fidler, Jesse W Rowley, Claudia Araujo, Luc H Boudreau, Alex Marti, Rhonda Souvenir, Kali Dale, Eric Boilard, Andrew S Weyrich, E Dale Abel
Increased intracellular reactive oxygen species (ROS) promote platelet activation. The sources of platelet-derived ROS are diverse and whether or not mitochondrial derived ROS, modulates platelet function is incompletely understood. Studies of platelets from patients with sickle cell disease, and diabetes suggest a correlation between mitochondrial ROS and platelet dysfunction. Therefore, we generated mice with a platelet specific knockout of superoxide dismutase 2 (SOD2-KO) to determine if increased mitochondrial ROS increases platelet activation...
October 5, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28760671/application-of-phospho-cytof-to-characterize-immune-activation-in-patients-with-sickle-cell-disease-in-an-ex-vivo-model-of-thrombosis
#11
Jeffrey Glassberg, Adeeb H Rahman, Mohammad Zafar, Caroline Cromwell, Alexa Punzalan, Juan Jose Badimon, Louis Aledort
Sickle cell disease (SCD) is a genetic disease caused by mutations in the beta globin gene, and inflammation plays a key role in driving many aspects of disease pathology. Early immune activation is believed to be associated with hemodynamic stresses and thrombus formation as cells traffic through blood vessels. We applied an extracorporeal perfusion system to model these effects ex vivo, and combined this with a phospho-CyTOF workflow to comprehensively evaluate single-cell signatures of early activation across all major circulating immune subsets...
July 28, 2017: Journal of Immunological Methods
https://www.readbyqxmd.com/read/28733096/brachial-vein-transposition-is-a-promising-ultimate-upper-limb-autologous-arteriovenous-angioaccess-despite-its-many-pitfalls
#12
Lamisse Karam, Marek Rawa, Richard Shoenfeld, Pierre Bourquelot
OBJECTIVE: The objective of this study was to retrospectively evaluate the possibility of using the brachial veins despite their deep location, small caliber, and thin wall. METHODS: There were 64 patients without superficial veins who were eligible for two-stage brachial vein transposition (BrVT); 54 patients were on hemodialysis, 9 patients had sickle cell disease, and 1 patient had long-term parenteral nutrition. Preoperative imaging was performed with color duplex ultrasound as well as venography for suspected central vein stenosis...
January 2018: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28692445/normal-cerebral-vascular-pulsations-in-humans-changes-with-age-and-implications-for-microvascular-disease
#13
Mi O Kim, Yan Li, Fangfei Wei, Jiguang Wang, Michael F O'Rourke, Audrey Adji, Alberto P Avolio
BACKGROUND: Cerebral syndromes in older humans, secondary stroke in younger persons following trauma, and sickle cell anaemia in children, are linked by unexplained microvascular damage and high cerebral pressure or flow pulsations. The aim of this study was to characterize age-related pressure and flow waveforms patterns entering the brain, to explain these in terms of disturbed physiological function, and to consider clinical implications. METHOD: Blood flow velocity waves were measured in four cerebral vascular territories by transcranial Doppler of 1020 apparently normal patients (497 men, 21-78 years)...
November 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28599856/-bilateral-pulmonary-embolism-mimicking-acute-chest-syndrome-in-an-adolescent-with-sickle-cell-disease
#14
P Mornand, F Chalard, A-S Romain, M Rohr, C Paluel-Marmont, A Niakaté, B Quinet, E Grimprel, M-H Odièvre-Montanié
Pulmonary embolism is a life-threatening and potentially lethal disease. Its incidence in children with sickle cell disease is probably underestimated and pediatric case reports in the literature are rare. Moreover, symptoms can mimic an acute chest syndrome. We report on the case of a 17-year-old boy with SS sickle cell disease, admitted for chest pain with dyspnea and tachycardia. Pulmonary angiography revealed a partial bilateral obstructive pulmonary embolism. We did not find any deep venous thrombosis or thrombophilia...
June 6, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28596657/increased-level-of-factor-viii-and-physiological-inhibitors-of-coagulation-in-patients-with-sickle-cell-disease
#15
Mohamed Chekkal, Mohamed Chakib Arslane Rahal, Khedidja Moulasserdoun, Fatima Seghier
Sickle cell disease (SCD) is a hemoglobinopathy characterized by hemolysis, oxidative stress, and vaso-occlusive crises. Thromboembolism also remains a serious complication and probably underestimated in the SCD. Our objective was to seek the existence of hemostasis abnormalities that predispose to thrombosis such as elevation of FVIII and Physiological inhibitors of coagulation deficiency. We studied 81 patients with SCD, including 32 homozygous S/S, 20 double heterozygous S/β thalassemia and 29 heterozygous S/A...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28382373/antagonists-of-the-system-l-neutral-amino-acid-transporter-lat-promote-endothelial-adhesivity-of-human-red-blood-cells
#16
Laura Beth Mann Dosier, Vikram J Premkumar, Hongmei Zhu, Izzet Akosman, Michael F Wempe, Timothy J McMahon
The system L neutral amino acid transporter (LAT; LAT1, LAT2, LAT3, or LAT4) has multiple functions in human biology, including the cellular import of S-nitrosothiols (SNOs), biologically active derivatives of nitric oxide (NO). SNO formation by haemoglobin within red blood cells (RBC) has been studied, but the conduit whereby a SNO leaves the RBC remains unidentified. Here we hypothesised that SNO export by RBCs may also depend on LAT activity, and investigated the role of RBC LAT in modulating SNO-sensitive RBC-endothelial cell (EC) adhesion...
June 28, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28376286/microfluidics-for-investigating-vaso-occlusions-in-sickle-cell-disease
#17
Renita E Horton
Sickle Cell Disease (SCD) stems from a mutation in the beta globin gene. Upon deoxygenation, hemoglobin polymerizes and triggers red blood cell remodeling. This phenomenon is central to SCD pathogenesis as individuals suffering from the disease are plagued by painful vaso-occlusive crises episodes. These episodes are the result of a combination of processes including inflammation, thrombosis, and blood cell adhesion to the vascular wall which leads to blockages within the vasculature termed vaso-occlusions...
April 4, 2017: Microcirculation: the Official Journal of the Microcirculatory Society, Inc
https://www.readbyqxmd.com/read/28369826/increased-incidence-of-vte-in-sickle-cell-disease-patients-risk-factors-recurrence-and-impact-on-mortality
#18
Ann Brunson, Amy Lei, Aaron S Rosenberg, Richard H White, Theresa Keegan, Ted Wun
Previous reports show increased incidence of venous thromboembolism [VTE, deep-vein thrombosis (DVT) and pulmonary embolus (PE)] in sickle cell disease (SCD) patients but did not account for frequency of hospitalization. We determined the incidence of VTE in a SCD cohort versus matched controls. For SCD patients, risk factors for incident VTE, recurrence and the impact on mortality were also determined. Among 6237 patients with SCD, 696 patients (11·2%) developed incident-VTE: 358 (51·6%) had PE (±DVT); 179 (25·7%) had lower-extremity DVT only and 158 (22·7%) had upper-extremity DVT...
July 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28249049/platelet-independent-adhesion-of-calcium-loaded-erythrocytes-to-von-willebrand-factor
#19
Michel W J Smeets, Ruben Bierings, Henriet Meems, Frederik P J Mul, Dirk Geerts, Alexander P J Vlaar, Jan Voorberg, Peter L Hordijk
Adhesion of erythrocytes to endothelial cells lining the vascular wall can cause vaso-occlusive events that impair blood flow which in turn may result in ischemia and tissue damage. Adhesion of erythrocytes to vascular endothelial cells has been described in multiple hemolytic disorders, especially in sickle cell disease, but the adhesion of normal erythrocytes to endothelial cells has hardly been described. It was shown that calcium-loaded erythrocytes can adhere to endothelial cells. Because sickle erythrocyte adhesion to ECs can be enhanced by ultra-large von Willebrand factor multimers, we investigated whether calcium loading of erythrocytes could promote binding to endothelial cells via ultra-large von Willebrand factor multimers...
2017: PloS One
https://www.readbyqxmd.com/read/27936141/hbs-binding-to-gp1b%C3%AE-activates-platelets-in-sickle-cell-disease
#20
Gowtham K Annarapu, Rashi Singhal, Avinash Gupta, Sheetal Chawla, Harish Batra, Tulika Seth, Prasenjit Guchhait
Intravascular hemolysis increases the risk of thrombosis in hemolytic disorders. Our previous study showed that the binding of adult hemoglobin (HbA) to glycoprotein (GP) 1bα induced the activation of platelets. The elevated plasma Hb or platelet surface bound Hb positively correlated with platelet activation in patients with paroxysmal nocturnal hemoglobinuria (PNH). Furthermore, this study shows that the sickle Hb [HbS, occurs due to single nucleotide polymorphism at A>T of β-globin gene of Hb and causes sickle cell disease (SCD)] also bound to GP1bα and activated platelets in a concentration-dependent manner...
2016: PloS One
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