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Sickle cell disease thrombosis

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https://www.readbyqxmd.com/read/28811305/red-blood-cells-in-thrombosis
#1
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 RBCs can promote thrombus formation and enhance thrombus stability. These findings suggest RBCs may contribute to thrombosis pathophysiology and reveal potential strategies for therapeutically targeting RBCs to reduce thrombosis...
August 15, 2017: Blood
https://www.readbyqxmd.com/read/28771279/superoxide-dismutase-2-is-dispensable-for-platelet-function
#2
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...
August 3, 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
#3
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
#4
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...
July 18, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28692445/normal-cerebral-vascular-pulsations-in-humans-changes-with-age-and-implications-for-microvascular-disease
#5
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)...
July 7, 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28599856/-bilateral-pulmonary-embolism-mimicking-acute-chest-syndrome-in-an-adolescent-with-sickle-cell-disease
#6
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
#7
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
#8
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
#9
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
#10
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...
April 3, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28249049/platelet-independent-adhesion-of-calcium-loaded-erythrocytes-to-von-willebrand-factor
#11
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
#12
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
https://www.readbyqxmd.com/read/27924667/contraceptive-practices-in-women-with-sickle-cell-disease
#13
Natália S Carvalho, Josefina Pellegrini Braga, Márcia Barbieri, Maria R Torloni, Maria S Figueiredo, Cristina A F Guazzelli
This was a cross-sectional study of sexually active women with sickle-cell disease (SCD) managed at São Paulo Federal University during a one-year period. A total of 54 women were included. Mean age was 32.0 (11.2, standard deviation) years and almost 95% were black or of mixed racial ancestry. Almost 80% reported a history of multiple blood transfusions, 50% had kidney disease and 17% had a history of thrombosis. Over 80% of them had used some form of contraception, mostly combined hormonal contraceptive (52%) or progestin-only contraceptives (46%)...
December 7, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/27913540/sickle-cell-disease-an-inherited-thrombophilia
#14
REVIEW
Ted Wun, Ann Brunson
Activation of the hemostatic system occurs in patients with sickle cell disease. The extent to which this activation contributes to sickle cell pathophysiology is uncertain. Clinical trials of anticoagulants or platelet inhibitors have demonstrated the ability to decrease biomarkers of hemostatic activation, but this has generally not resulted in improvement in clinically relevant outcomes. Venous thromboembolism (VTE: deep venous thrombosis and pulmonary embolism) has been until recently an underappreciated complication of sickle cell disease, with incident event and recurrence rates consistent with a strong thrombophilia...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27813144/arterio-venous-fistula-for-automated-red-blood-cells-exchange-in-patients-with-sickle-cell-disease-complications-and-outcomes
#15
Marianne Delville, Sandra Manceau, Nassim Ait Abdallah, Jan Stolba, Sameh Awad, Thibaud Damy, Barnabas Gellen, Laurent Sabbah, Karima Debbache, Vincent Audard, Jean-Louis Beaumont, Cécile Arnaud, Christelle Chantalat-Auger, Françoise Driss, François Lefrère, Marina Cavazzana, Gilbert Franco, Frederic Galacteros, Jean-Antoine Ribeil, Justine Gellen-Dautremer
Erythrocytapheresis (ER) can improve outcome in patients with sickle cell disease (SCD). A good vascular access is required but frequently it can be difficult to obtain for sickle cell patients. Arterio-venous fistulas (AVFs) have been suggested for ER in SCD supported by limited evidence. We report the largest cohort of ER performed with AVFs from three French SCD reference centers. Data of SCD patients undergoing ER with AVFs in the French SCD reference center were retrospectively collected. The inclusion criteria were: SS or Sβ-Thalassemia and AVF surgery for ER...
February 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27443994/neurologic-manifestations-of-blood-dyscrasias
#16
REVIEW
Daniel R Couriel, Holly Ricker, Mary Steinbach, Catherine J Lee
Neurologic manifestations are common in blood diseases, and they can be caused by the hematologic disorder or its treatment. This article discusses hematologic diseases in adult patients, and categorizes them into benign and malignant conditions. The more common benign hematologic diseases associated with neurologic manifestations include anemias, particularly caused by B12 deficiency and sickle cell disease, and a variety of disorders of hemostasis causing bleeding or thrombosis, including thrombotic microangiopathy...
August 2016: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/27257766/imaging-manifestations-of-hematologic-diseases-with-renal-and-perinephric-involvement
#17
REVIEW
Andrei S Purysko, Antonio C Westphalen, Erick M Remer, Christopher P Coppa, Hilton M Leão Filho, Brian R Herts
The kidneys and perinephric tissues can be affected by a variety of hematologic disorders, which usually occur in the setting of multisystem involvement. In many of these disorders, imaging is used to evaluate the extent of disease, guide biopsy, and/or monitor disease activity and patient response to therapy. Lymphoma, leukemia, and multiple myeloma commonly manifest as multiple parenchymal or perinephric lesions. Erdheim-Chester disease and Rosai-Dorfman disease, rare forms of multisystemic histiocytosis, are often identified as perinephric and periureteral masses...
July 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/27251735/massive-splenic-infarction-and-splenic-venous-thrombosis-observed-in-a-patient-with-acute-splenic-syndrome-of-sickle-cell-traits-on-contrast-enhanced-thin-slice-computed-tomography
#18
Takana Yamakawa Hayashi, Izuru Matsuda, Kazuchika Hagiwara, Tomoko Takayanagi, Akifumi Hagiwara
We report a case of splenic infarction in a patient with sickle cell traits (SCT), focusing on the computed tomography (CT) findings. The patient was an African-American man in his twenties with no past medical history who experienced sudden left upper quadrant pain while climbing a mountain (over 3000 m above sea level). Dynamic contrast-enhanced CT revealed massive non-segmental splenic infarction accompanied with nodule-like preserved splenic tissue. The region of splenic infarction did not coincide with the arterial vascular territory and differed from the features of infarction caused by large arterial embolism...
September 2016: Abdominal Radiology
https://www.readbyqxmd.com/read/27189014/upper-extremity-deep-vein-thrombosis-a-retrospective-cohort-evaluation-of-thrombotic-risk-factors-at-a-university-teaching-hospital-antithrombosis-clinic
#19
Rebecca H Stone, Adam P Bress, Edith A Nutescu, Nancy L Shapiro
BACKGROUND: Upper-extremity deep-vein thrombosis (UEDVT) causes significant morbidity and mortality and is not well characterized in the existing literature, particularly in underrepresented minorities such as African Americans. OBJECTIVE: To describe the characteristics of a cohort of patients with UEDVT seen at an urban academic medical center. METHODS: This was a retrospective cohort study among patients with a confirmed UEDVT at the University of Illinois Hospital and Health Sciences System between 1996 and 2011...
August 2016: Annals of Pharmacotherapy
https://www.readbyqxmd.com/read/27156108/the-role-of-complement-activation-in-thrombosis-and-hemolytic-anemias
#20
REVIEW
John Chapin, Hunter S Terry, Dorothy Kleinert, Jeffrey Laurence
OBJECTIVE: The objective of this study was to describe complement activation in hemostatic and pathologic states of coagulation and in the acquired and congenital hemolytic anemias. METHODS AND RESULTS: We review published and emerging data on the involvement of the classic, alternative and lectin-based complement pathways in coagulation and the hemolytic anemias. The alternative pathway in particular is always "on," at low levels, and is particularly sensitive to hyper-activation in a variety of physiologic and pathologic states including infection, autoimmune disorders, thrombosis and pregnancy, requiring tight control predicated on a variety of soluble and membrane bound regulatory proteins...
April 2016: Transfusion and Apheresis Science
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