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Sickle cell anemia

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https://www.readbyqxmd.com/read/28542880/suicidal-death-of-erythrocytes-in-cancer-and-its-chemotherapy-a-potential-target-in-the-treatment-of-tumor-associated-anemia
#1
REVIEW
Elisabeth Lang, Rosi Bissinger, Syed M Qadri, Florian Lang
In analogy to apoptosis of nucleated cells, erythrocytes may enter eryptosis characterized by cell shrinkage and cell membrane scrambling. Eryptotic erythrocytes are rapidly cleared from circulating blood and may adhere to the vascular wall. Stimulation of eryptosis thus impairs microcirculation and leads to anemia as soon as the loss of erythrocytes cannot be fully compensated by enhanced erythropoiesis. Signaling stimulating eryptosis includes increase of cytosolic Ca(2+) -activity, ceramide, caspases, calpain, p38-kinase, protein-kinase C, Janus-activated kinase 3, casein-kinase 1α, and cyclin-dependent kinase 4...
May 23, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28542469/biophysical-markers-of-the-peripheral-vasoconstriction-response-to-pain-in-sickle-cell-disease
#2
Patjanaporn Chalacheva, Maha Khaleel, John Sunwoo, Payal Shah, Jon A Detterich, Roberta M Kato, Wanwara Thuptimdang, Herbert J Meiselman, Richard Sposto, Jennie Tsao, John C Wood, Lonnie Zeltzer, Thomas D Coates, Michael C K Khoo
Painful vaso-occlusive crisis (VOC), a complication of sickle cell disease (SCD), occurs when sickled red blood cells obstruct flow in the microvasculature. We postulated that exaggerated sympathetically mediated vasoconstriction, endothelial dysfunction and the synergistic interaction between these two factors act together to reduce microvascular flow, promoting regional vaso-occlusions, setting the stage for VOC. We previously found that SCD subjects had stronger vasoconstriction response to pulses of heat-induced pain compared to controls but the relative degrees to which autonomic dysregulation, peripheral vascular dysfunction and their interaction are present in SCD remain unknown...
2017: PloS One
https://www.readbyqxmd.com/read/28528302/nanomolar-anti-sickling-compounds-identified-by-ligand-based-pharmacophore-approach
#3
Odailson Santos Paz, Milena de Jesus Pinheiro, Renan Fernandes do Espirito Santo, Cristiane Flora Villarreal, Marcelo Santos Castilho
Adenosine receptors are considered as potential targets for drug development against several diseases. The discovery of subtype 2B adenosine receptors role in erythrocyte sickling process proved its importance to neglected diseases such as sickle cell anemia, which affects approximately 29.000 people around the world, but whose treatment is restricted to just one FDA approved drug (hydroxyurea). In order to widen the therapeutic arsenal available to treat sickle cell anemia patients, it is imperative to identify new lead compounds that modify the sickling course and not just its symptoms...
May 12, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28520780/towards-a-point-of-care-strip-test-to-diagnose-sickle-cell-anemia
#4
Meaghan Bond, Brady Hunt, Bailey Flynn, Petri Huhtinen, Russell Ware, Rebecca Richards-Kortum
A rapid test to identify patients with sickle cell disease could have important benefits in low-resource settings. Sickle cell anemia (SCA) affects about 300,000 newborns each year, the majority of whom are born in sub-Saharan Africa. Low-cost therapies are available to treat SCA, but most countries in sub-Saharan Africa lack robust neonatal screening programs needed to identify patients in need of treatment. To address this need, we developed and evaluated a competitive lateral flow assay that identifies patients with SCA (genotype HbSS) in 15 minutes using undiluted whole blood...
2017: PloS One
https://www.readbyqxmd.com/read/28512559/spirometric-evaluation-of-pulmonary-function-in-nigerian-children-underwent-bone-marrow-transplantation-for-sickle-cell-anemia
#5
Antonella Isgro', Marco Marziali, Katia Paciaroni, Gioia De Angelis, Cecilia Alfieri, Michela Ribersani, Festus Olusola Olowoselu, Guido Lucarelli, Javid Gaziev
No abstract text is available yet for this article.
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28507969/core-decompression-with-synthetic-grafting-as-a-joint-preservation-strategy-in-humeral-avascular-necrosis-due-to-sickle-cell-anemia-a-case-report
#6
Andrew M Steffensmeier, Karen Kirkham, John M Wiemann
INTRODUCTION: Avascular necrosis (AVN) of the femoral or humeral heads in patients with sickle cell anemia is a common and painful condition. There is currently no gold standard treatment protocol for this condition. Typically, the pain is managed with narcotics and activity restriction until there has been collapse of the subchondral bone with a degree of arthrosis sufficient to warrant total joint arthroplasty. This method entails prolonged pain for the patient and decreases the ability to function occupationally and recreationally...
November 2016: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28507438/pulmonary-hypertension-in-nigerian-adults-with-sickle-cell-anemia
#7
Valentine N Amadi, Michael O Balogun, Norah O Akinola, Rasaaq A Adebayo, Anthony O Akintomide
BACKGROUND: Sickle cell anemia (SCA) is the commonest hemoglobinopathy and is associated with high morbidity and mortality. Pulmonary hypertension (PH) is reported to play a significant role in this regard. There is very limited literature on PH in SCA in Nigeria. OBJECTIVES: The objectives of this study were to determine the prevalence of Doppler-derived PH in SCA, assess its influence on exercise capacity, and determine the correlates and predictors of measures of estimated pulmonary pressure...
2017: Vascular Health and Risk Management
https://www.readbyqxmd.com/read/28507082/association-between-diffuse-myocardial-fibrosis-and-diastolic-dysfunction-in-sickle-cell-anemia
#8
Omar Niss, Robert Fleck, Fowe Makue, Tarek Alsaied, Payal Desai, Jeffrey A Towbin, Punam Malik, Michael D Taylor, Charles T Quinn
Sickle cell anemia (SCA)-related cardiomyopathy is characterized by diastolic dysfunction and hyperdynamic features. Diastolic dysfunction portends early mortality in SCA. Diastolic dysfunction is associated with microscopic myocardial fibrosis in SCA mice, but the cause of diastolic dysfunction in humans with SCA is unknown. We used cardiac magnetic resonance (CMR) measurements of extracellular volume fraction (ECV) to discover and quantify diffuse myocardial fibrosis in 25 individuals with SCA (mean age 23±13 years), and determine the association between diffuse myocardial fibrosis and diastolic dysfunction...
May 15, 2017: Blood
https://www.readbyqxmd.com/read/28505305/efficacy-and-safety-of-ledipasvir-sofosbuvir-for-the-treatment-of-chronic-hepatitis-c-in-persons-with-sickle-cell-disease
#9
Juhi Moon, Robert H Hyland, Fangqiu Zhang, Diana M Brainard, Sophie Lanzkron, John G McHutchison, Mark Sulkowski
Patients with sickle cell disease are at high risk for chronic hepatitis C infection. Prior treatment has been limited due to the use of ribavirin causing hemolytic anemia and interferon causing cytopenias. We demonstrate the safety and efficacy of fixed dose combination, ledipasvir and sofosbuvir for 12 weeks in this population.
May 13, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28500682/red-blood-cell-transfusions-during-sickle-cell-anemia-vaso-occlusive-crises-a-report-from-the-magnesium-in-crisis-magic-study
#10
Monica L Hulbert, Julie A Panepinto, J Paul Scott, Robert I Liem, Lawrence J Cook, Timothy Simmons, David C Brousseau
BACKGROUND: Little is known about red blood cell (RBC) transfusion practices for children hospitalized for a sickle cell vaso-occlusive pain crisis (VOC). We hypothesized that transfusion would be associated with the development of acute chest syndrome (ACS), lower hemoglobin (Hb) concentration, and lack of hydroxyurea therapy. STUDY DESIGN AND METHODS: This is a secondary analysis of all children admitted for a sickle cell pain crisis enrolled in the Magnesium in Crisis (MAGiC) randomized trial; all had HbSS or S-β(0) thalassemia...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28489923/differences-of-microparticle-patterns-between-sickle-cell-anemia-and-hemoglobin-sc-patients
#11
Yohann Garnier, Séverine Ferdinand, Maryse Etienne-Julan, Gisèle Elana, Marie Petras, Lydia Doumdo, Benoit Tressières, Marie-Laure Lalanne-Mistrih, Marie-Dominique Hardy-Dessources, Philippe Connes, Marc Romana
Sickle cell anemia (SCA) and hemoglobin SC (HbSC) disease are the two most common forms of sickle cell disease (SCD), a frequent hemoglobinopathy which exhibits a highly variable clinical course. Although high levels of microparticles (MPs) have been consistently reported in SCA and evidence of their harmful impact on the SCA complication occurrences have been provided, no data on MP pattern in HbSC patients has been reported so far. In this study, we determined and compared the MP patterns of 84 HbSC and 96 SCA children, all at steady-state, using flow cytometry...
2017: PloS One
https://www.readbyqxmd.com/read/28484290/cardiac-structural-and-functional-changes-evaluated-by-transthoracic-and-tissue-doppler-echocardiography-in-adult-patients-with-sickle-cell-disease
#12
Mojdeh Dabirian, Ghasem Janbabaei, Hossein Karami, Maryam Nabati, Mohsen Aarabi, Morteza Namazi, Hadi Darvishi-Khezri
One of the most common genetic blood disorders, resulting from inherited red blood cell disorders, is sickle cell disease (SCD) which is responsible for high death in adult patients with left ventricular diastolic dysfunction and pulmonary hypertension. Tissue Doppler Echocardiography (TDE) and transthoracic echocardiography (TTE) are two useful tools to assess the risk of SCD. The present study was conducted to evaluate the cardiac functions and structure using TDE and TTE among adult patients suffering from sickle cell anemia compare to normal samples...
March 2017: Acta Informatica Medica: AIM
https://www.readbyqxmd.com/read/28482712/heme-mediated-cell-activation-the-inflammatory-puzzle-of-sickle-cell-anemia
#13
Caroline Conceição da Guarda, Rayra Pereira Santiago, Luciana Magalhães Fiuza, Milena Magalhães Aleluia, Júnia Raquel Dutra Ferreira, Camylla Vilas Boas Figueiredo, Setondji Cocou Modeste Alexandre Yahouedehou, Rodrigo Mota de Oliveira, Isa Menezes Lyra, Marilda de Souza Gonçalves
Hemolysis triggers the onset of several clinical manifestations of sickle cell anemia (SCA). During hemolysis, heme, which is derived from hemoglobin (Hb), accumulates due to the inability of detoxification systems to scavenge sufficiently. Heme exerts multiple harmful effects, including leukocyte activation and migration, enhanced adhesion molecule expression by endothelial cells and the production of pro-oxidant molecules. Area covered: In this review, we describe the effects of heme on leukocytes and endothelial cells, as well as the features of vascular endothelial cells related to vaso-occlusion in SCA...
May 17, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28472705/measurements-of-red-cell-deformability-and-hydration-reflect-hbf-and-hba2-in-blood-from-patients-with-sickle-cell-anemia
#14
Nermi L Parrow, Hongbin Tu, James Nichols, Pierre-Christian Violet, Corinne A Pittman, Courtney Fitzhugh, Robert E Fleming, Narla Mohandas, John F Tisdale, Mark Levine
Decreased erythrocyte deformability, as measured by ektacytometry, may be associated with disease severity in sickle cell anemia (SCA). Heterogeneous populations of rigid and deformable cells in SCA blood result in distortions of diffraction pattern measurements that correlate with the concentration of hemoglobin S (HbS) and the percentage of irreversibly sickled cells. We hypothesize that red cell heterogeneity, as well as deformability, will also be influenced by the concentration of alternative hemoglobins such as fetal hemoglobin (HbF) and the adult variant, HbA2...
April 18, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28460533/a-multidisciplinary-approach-to-impact-acute-care-utilization-in-sickle-cell-disease
#15
Rhea E Powell, Paris B Lovett, Albert Crawford, John McAna, David Axelrod, Lawrence Ward, Dianne Pulte
Sickle cell disease (SCD), an inherited red blood cell disorder, is characterized by anemia, end-organ damage, unpredictable episodes of pain, and early mortality. Emergency department (ED) visits and hospitalizations are frequent, leading to increased burden on patients and increased health care costs. This study assessed the effects of a multidisciplinary care team intervention on acute care utilization among adults with SCD. The multidisciplinary care team intervention included monthly team meetings and development of individualized care plans...
April 1, 2017: American Journal of Medical Quality: the Official Journal of the American College of Medical Quality
https://www.readbyqxmd.com/read/28453928/the-clinical-severity-of-hemoglobin-s-black-a-%C3%AE-%C3%AE-%C3%AE-0-thalassemia
#16
Maria I Cancio, Banu Aygun, David H K Chui, Jennifer A Rothman, J Paul Scott, Jeremie H Estepp, Jane S Hankins
Hemoglobin S/Black ((A) γδβ)(0) -thalassemia is a rare sickle cell disease (SCD) variant. On the basis of limited descriptions in the literature, the disease is reported as a mild microcytic anemia with an uncomplicated course. We report the clinical and laboratory data of nine patients whose diagnoses were confirmed by DNA-based techniques. Despite having mild anemia and high fetal hemoglobin level postinfancy, these patients developed many of the classic complications of SCD, including vaso-occlusive crisis, acute chest syndrome, avascular necrosis, and cholelithiasis...
April 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28453224/a-reappraisal-of-the-mechanisms-underlying-the-cardiac-complications-of-sickle-cell-anemia
#17
REVIEW
Parul Rai, Omar Niss, Punam Malik
Anemia, hemolysis-driven vasculopathy, and intrinsic myocardial injury have been proposed as predisposing factors to cardiac disease in sickle cell anemia (SCA). The individual impact of these mechanisms on the cardiac features of SCA and the way they influence complications such as sudden death and dysrhythmias have been unclear. Recent findings of an acquired restrictive SCA-related cardiomyopathy, driven by myocardial fibrosis, may explain some of these cardiac features. Given the complexity of cardiac pathology in SCA, using additional parameters to tricuspid regurgitant jet velocity (left atrial volume, diastolic parameters, NT-proBNP) may improve the accuracy of noninvasive screening for cardiopulmonary complications in SCA...
April 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28450986/-major-sickle-cell-syndromes-and-infections-associated-with-this-condition-in-children-in-burkina-faso
#18
Sonia Douamba, Kisito Nagalo, Laure Tamini, Ismaël Traoré, Madibèlè Kam, Fla Kouéta, Diarra Yé
INTRODUCTION: This study aims to investigate infections in children with major sickle cell syndrome. METHODS: We conducted a monocentric descriptive retrospective hospital study in Ouagadougou, Burkina Faso, over a ten-year period. All children with major sickle cell syndrome (homozygous SS and double heterozygous SC, SDPunjab, Sβ thalassemic, SOArab and SE) hospitalized for microbiologically confirmed infections were enrolled in the study. RESULTS: One hundred and thirty-three patients met our inclusion criteria...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28446324/-research-advance-of-microparticles-in-hypercoagulation-of-hemolytic-anemia-review
#19
Xie Su, Peng Cheng, Dong-Hong Deng
Microparticles (MP) are small membrane vesicles released from many different cell types in response to cellular activation or apoptosis, which have the procoagulant effect. Hemolytic anemia(HA) is a type of anemia that have a short life expectancy of red blood cells due to the destruction which exceed the hematopoietic compensatory capacity of bone marrow. Sickle cell anemia(SCD), thalassemia and paroxysmal nocturnal hemoglobinuria(PNH) are all characterized by hypercoagulation and thromboembolism (TE). Research shows that MP can promote the formation of hypercoagulative state which in turn increases the risk of thromboembolism in HA...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28440126/transcranial-doppler-screening-for-stroke-risk-in-children-with-sickle-cell-disease-a-systematic-review
#20
Sara Mazzucco, Marina Diomedi, Amrana Qureshi, Laura Sainati, Soundrie T Padayachee
Background Sickle cell disease (SCD) is one of the most common causes of stroke in children worldwide. Based on the results of the Stroke Prevention Trial in Sickle Cell Anemia (STOP), annual transcranial Doppler ultrasound (TCD) screening for affected children is standard practice. However, the need for TCD surveillance programs could override the accuracy of the screening, affecting the correct stratification of stroke risk and subsequent clinical management of the target population. Aims To shed light on this issue, a systematic review of the literature on TCD screening for children and adolescents with SCD was carried out (CRD42016050549), according to a list of clinically relevant questions, with a particular focus on screening practices in European countries...
January 1, 2017: International Journal of Stroke: Official Journal of the International Stroke Society
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