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https://www.readbyqxmd.com/read/29710269/association-of-sickle-cell-trait-with-ischemic-stroke-among-african-americans-a-meta-analysis
#1
Hyacinth I Hyacinth, Cara L Carty, Samantha R Seals, Marguerite R Irvin, Rakhi P Naik, Gregory L Burke, Neil A Zakai, James G Wilson, Nora Franceschini, Cheryl A Winkler, Victor A David, Jeffrey B Kopp, Suzanne E Judd, Robert J Adams, W T Longstreth, Leonard Egede, Daniel T Lackland, Herman Taylor, JoAnn E Manson, Virginia Howard, Matthew Allison, Beatrice E Gee, Adolfo Correa, Monika M Safford, Donna K Arnett, George Howard, Alexander P Reiner, Mary Cushman
Importance: African Americans and individuals of African ancestry have a higher risk of stroke compared with non-Hispanic white individuals. Identifying the source of this disparity could provide an opportunity for clinical stroke risk stratification and more targeted therapy. Whether sickle cell trait (SCT) is an indicator of increased risk of ischemic stroke among African Americans is still unclear. Objective: To examine whether SCT is associated with a higher risk of incident ischemic stroke among African Americans...
April 23, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29675950/risk-factors-for-red-blood-cell-alloimmunization-in-the-recipient-epidemiology-and-donor-evaluation-study-reds-iii-database
#2
Matthew S Karafin, Matt Westlake, Ronald G Hauser, Christopher A Tormey, Philip J Norris, Nareg H Roubinian, Yanyun Wu, Darrell J Triulzi, Steve Kleinman, Jeanne E Hendrickson
Despite the significance of red blood cell (RBC) alloimmunization, the lack of standardized registries in the US has prevented the completion of large studies. Data from 3·5 years of the Recipient Epidemiology and Donor Evaluation Study-III (REDS-III) recipient database, containing information from 12 hospitals, were studied. A RBC alloantibody responder had an antibody identified at any point during the study, and a non-responder had a negative antibody screen at least 15 days post-RBC transfusion. Demographics, blood type, ICD9/10 codes, and other potential correlates were evaluated...
April 19, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29667731/hemoglobin-s-monitoring-on-tosoh-g8-in-hemoglobin-a1c-mode-in-case-of-urgent-red-blood-cell-exchange
#3
Sophie Van Aelst, Helena Claerhout, Elke Nackers, Koen Desmet, Davy Kieffer
BACKGROUND: Pre- and post-transfusion hemoglobin S (HbS) levels are used to document the efficacy of red blood cell exchange (RCE) in patients with sickle cell disease (SCD). In case of urgent RCE a 24/7 short turn-around time (STAT) analysis, with the ability to identify and quantify HbS, is warranted. The use of TOSOH G8 (Tosoh Europe) is evaluated for this purpose, using the variant HbA1c mode. METHODS: Analytical performance of the HbS analysis on TOSOH G8 in variant HbA1c mode was evaluated, including assessment of imprecision and linearity for HbS...
April 18, 2018: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/29660907/pulse-wave-velocity-is-lower-in-trained-than-in-untrained-sickle-cell-trait-carriers
#4
Valentin Ouédraogo, Philippe Connes, Julien Tripette, Arnaud Jean Florent Tiendrébéogo, Abdou Khadir Sow, Mor Diaw, Moussa Seck, Mountaga Diop, Magid Hallab, Rhonda Belue, Abdoulaye Samb, Abdoulaye Ba, Georges Lefthériotis
BACKGROUND: Sickle cell trait (SCT) is a benign condition of sickle cell disease. Nevertheless, previous reports showed that SCT carriers have increased blood viscosity and decreased vascular reactivity compared to non-SCT carrier. The benefit of regular exercise on vascular function has been well documented in the general population but no study focused on the SCT population. PURPOSE: The aim of our study was to compare arterial stiffness and blood viscosity between trained and untrained SCT carriers, as well as a group of untrained non-SCT...
April 13, 2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29625861/-targeted-newborn-screening-for-sickle-cell-anemia-sickling-test-emmel-test-boundaries-in-the-prenatal-assessment-in-west-african-area
#5
D A Diallo, A Guindo, B A Touré, Y S Sarro, M Sima, O Tessougué, M A Baraika, P Guindo, M Traoré, M Diallo, A Dorie
BACKGROUND: Newborn screening for sickle cell anemia is necessary in Africa where the disease is more frequent. Hemoglobin electrophoresis is used for screening, but is limited by a high cost and difficult access. Sickling test (Emmel test), which is more affordable and technically more accessible, is often requested for prenatal assessment of pregnant women in West African areas to reserve screening for newborns from mothers in whom the positive sickling test attests the presence of hemoglobin S...
April 3, 2018: Revue D'épidémiologie et de Santé Publique
https://www.readbyqxmd.com/read/29614636/autonomic-nervous-system-involvement-in-sickle-cell-disease
#6
Thomas D Coates, Patjanaporn Chalacheva, Lonnie Zeltzer, Michael C K Khoo
Sickle cell disease (SCD) is a genetic disorder of hemoglobin producing hemoglobin-S (HbS) and resulting in recurrent severe episodes of pain, organ damage and premature death due to vaso- occlusion. Deoxy HbS polymerizes, causing red cells to become rigid and lodge in the microvasculature if they do not escape into larger vessels before this transformation occurs. The mechanism that triggers this transition from steady state to vaso-occlusive crisis (VOC) is not known. Patients state that cold, emotional stress, and pain itself can trigger these events...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29600192/choroidal-vascular-occlusion-in-a-young-male-patient-with-sickle-cell-trait
#7
Maria Kotoula, Eleni Papageorgiou, Foteini Xanthou, Sotirios Kalampalikis, Sofia Androudi, Evangelia E Tsironi
No abstract text is available yet for this article.
2018: International Journal of Ophthalmology
https://www.readbyqxmd.com/read/29590102/common-%C3%AE-globin-variants-modify-hematologic-and-other-clinical-phenotypes-in-sickle-cell-trait-and-disease
#8
Laura M Raffield, Jacob C Ulirsch, Rakhi P Naik, Samuel Lessard, Robert E Handsaker, Deepti Jain, Hyun M Kang, Nathan Pankratz, Paul L Auer, Erik L Bao, Joshua D Smith, Leslie A Lange, Ethan M Lange, Yun Li, Timothy A Thornton, Bessie A Young, Goncalo R Abecasis, Cathy C Laurie, Deborah A Nickerson, Steven A McCarroll, Adolfo Correa, James G Wilson, Guillaume Lettre, Vijay G Sankaran, Alex P Reiner
Co-inheritance of α-thalassemia has a significant protective effect on the severity of complications of sickle cell disease (SCD), including stroke. However, little information exists on the association and interactions for the common African ancestral α-thalassemia mutation (-α3.7 deletion) and β-globin traits (HbS trait [SCT] and HbC trait) on important clinical phenotypes such as red blood cell parameters, anemia, and chronic kidney disease (CKD). In a community-based cohort of 2,916 African Americans from the Jackson Heart Study, we confirmed the expected associations between SCT, HbC trait, and the -α3...
March 28, 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29574538/identifying-factors-underlying-the-decision-for-sickle-cell-carrier-screening-among-african-americans-within-middle-reproductive-age
#9
Tilicia L Mayo-Gamble, Susan E Middlestadt, Hsien-Chang Lin, Jennifer Cunningham-Erves, Priscilla Barnes, Pamela Braboy Jackson
Guidelines recommend that African Americans know their sickle cell trait status to inform reproductive health decisions. Few studies have applied a behavioral theory to identify factors associated with sickle cell trait screening to inform intervention targets to increase this behavior. We applied a Sickle Cell Trait Screening Framework to identify factors associated with African Americans' intention to ask for sickle cell trait screening. Participants (N = 300), ages 18 to 35, completed a cross-sectional survey...
March 24, 2018: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/29533274/livedoid-vasculopathy-presenting-in-a-patient-with-sickle-cell-disease
#10
Heather Reagin, Etan Marks, Stephen Weis, Joseph Susa
We report a case in which a 43-year-old African American male with medical history of sickle cell disease (SCD) presented with a nonhealing ulcer. Biopsy revealed features of livedoid vasculopathy. Previously, livedoid vasculopathy had only been described in a patient with sickle cell trait, but never in a patient with SCD. Livedoid vasculopathy most commonly affects the distal lower extremities and is characterized by irregular, punched-out, painful ulcers that heal with stellate white scars of atrophie blanche...
February 28, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29514337/the-association-between-sickle-cell-trait-in-u-s-service-members-with-deployment-length-of-service-and-mortality-1992-2012
#11
Darrell E Singer, Ligong Chen, Stephanie Shao, Jonathan Goldsmith, Celia Byrne, David W Niebuhr
Introduction: Sickle cell trait (SCT) affects an estimated 5.02% of non-Hispanic blacks, 1.08% of Hispanics, and 0.1% of Whites in the U.S. military. Policies for SCT screening and occupational restrictions vary by service. Population-based studies of SCT with quantification of military-relevant outcomes are lacking. Methods: The study design was a retrospective cohort of 15,081 SCT-positive versus 60,320 SCT-negative U.S. active duty personnel enlisted from 1992 to 2012 and followed through 2013...
March 1, 2018: Military Medicine
https://www.readbyqxmd.com/read/29486174/antibody-responses-to-p-falciparum-apical-membrane-antigen-1-ama-1-in-relation-to-haemoglobin-s-hbs-hbc-g6pd-and-abo-blood-groups-among-fulani-and-masaleit-living-in-western-sudan
#12
Amre Nasr, Ayman M Saleh, Muna Eltoum, Amir Abushouk, Anhar Hamza, Ahmad Aljada, Mohamed E El-Toum, Yousif A Abu-Zeid, Gamal Allam, Gehad ElGhazali
Fulani and Masaleit are two sympatric ethnic groups in western Sudan who are characterised by marked differences in susceptibility to Plasmodium falciparum malaria. It has been demonstrated that Glucose-6-phosphate dehydrogenase (G6PD) deficiency and Sickle cell trait HbAS carriers are protected from the most severe forms of malaria. This study aimed to investigate a set of specific IgG subclasses against P. falciparum Apical Membrane Antigen 1 (AMA-1 3D7), haemoglobin variants and (G6PD) in association with malaria susceptibility among Fulani ethnic group compared to sympatric ethnic group living in Western Sudan...
June 2018: Acta Tropica
https://www.readbyqxmd.com/read/29484190/a-rare-case-of-avascular-necrosis-in-sickle-cell-trait-a-case-report
#13
William J Sanders
Background: Sickle cell trait is usually an asymptomatic presentation of a patient with slightly different hemoglobin molecule makeup than normal. It is similar to a more serious disease, sickle cell disease, in which a person's hemoglobin is mutated in such a way that causes their red blood cells to easily change shape in certain environmental and internal states; this causes red blood cells to adhere to the walls and occlude the lumen of the arteries in which they travel, leading to downstream effects secondary to ischemia...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29476712/progressive-glomerular-and-tubular-damage-in-sickle-cell-trait-and-sickle-cell-anemia-mouse-models
#14
Santosh L Saraf, Justin R Sysol, Alexandru Susma, Suman Setty, Xu Zhang, Krishnamurthy P Gudehithlu, Jose A L Arruda, Ashok K Singh, Roberto F Machado, Victor R Gordeuk
Homozygosity for the hemoglobin (Hb) S mutation (HbSS, sickle cell anemia) results in hemoglobin polymerization under hypoxic conditions leading to vaso-occlusion and hemolysis. Sickle cell anemia affects 1:500 African Americans and is a strong risk factor for kidney disease, although the mechanisms are not well understood. Heterozygous inheritance (HbAS; sickle cell trait) affects 1:10 African Americans and is associated with an increased risk for kidney disease in some reports. Using transgenic sickle mice, we investigated the histopathologic, ultrastructural, and gene expression differences with the HbS mutation...
February 2, 2018: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/29459113/sickle-cell-trait-and-diagnosis-of-type-2-diabetes
#15
Sarah Skinner, Vincent Pialoux, Bérengère Fromy, Dominique Sigaudo-Roussel, Philippe Connes
No abstract text is available yet for this article.
February 16, 2018: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/29432581/14q32-and-let-7-micrornas-regulate-transcriptional-networks-in-fetal-and-adult-human-erythroblasts
#16
Samuel Lessard, Mélissa Beaudoin, Stuart H Orkin, Daniel E Bauer, Guillaume Lettre
In humans, fetal erythropoiesis takes place in the liver whereas adult erythropoiesis occurs in the bone marrow. Fetal and adult erythroid cells are not only produced at different sites, but are also distinguished by their respective transcriptional program. In particular, whereas fetal erythroid cells express γ-globin chains to produce fetal hemoglobin (HbF), adult cells express β-globin chains to generate adult hemoglobin. Understanding the transcriptional regulation of the fetal-to-adult hemoglobin switch is clinically important as re-activation of HbF production in adult erythroid cells would represent a promising therapy for the hemoglobin disorders sickle cell disease and β-thalassemia...
April 15, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29415213/risk-of-exertional-heat-illnesses-associated-with-sickle-cell-trait-in-u-s-military
#17
Darrell E Singer, Celia Byrne, Ligong Chen, Stephanie Shao, Jonathan Goldsmith, David W Niebuhr
Introduction: A number of studies have found an association between sickle cell trait (SCT) and exertional heat illnesses (EHIs) including heat stroke, a potentially fatal condition. The strength of this association varied across studies, limiting the ability to quantify potential benefits of SCT-screening policies for competitive athletics and military service members. We determined the relative rate and attributable risk of developing EHI associated with being SCT positive and the EHI health care utilization...
February 5, 2018: Military Medicine
https://www.readbyqxmd.com/read/29414256/updated-recommendations-for-athletes-with-heart-disease
#18
Rachel Lampert, Douglas P Zipes
Professional society recommendations to decrease sudden cardiac death in athletes, including eligibility requirements with disqualification for athletes with diagnosed disease as well as preparticipation screening and emergency preparedness, were updated in 2015. The update includes new sections on aortic disease, channelopathies, and sickle cell trait, as well as a change in format from the previous binary yes/no format to the more nuanced and contemporary "class and level of evidence" format. Eighty-four of the 246 recommendations now carry Class II designation-"reasonable," or "may be considered...
January 29, 2018: Annual Review of Medicine
https://www.readbyqxmd.com/read/29405994/alk-tpm3-rearrangement-in-adult-renal-cell-carcinoma-report-of-a-new-case-showing-loss-of-chromosome-3-and-literature-review
#19
Yohan Bodokh, Damien Ambrosetti, Valérie Kubiniek, Branwel Tibi, Matthieu Durand, Jean Amiel, Morgane Pertuit, Anne Barlier, Florence Pedeutour
Seven cases of translocation-associated renal cell carcinoma involving ALK (ALK-tRCC) were referenced in the last World Health Organization's classification (2016), in a group of emerging/provisional RCC. The first three cases were pediatric, medullary-based, associated with sickle-cell trait and showed a fusion of ALK with VCL. Thirteen cases have been further described. They displayed clinical, morphological and genomic heterogeneity. Most of them occurred in adults. None of the patients was affected by sickle-cell disease...
February 2018: Cancer Genetics
https://www.readbyqxmd.com/read/29403210/prevalence-of-hemoglobin-variants-and-hemoglobinopathies-using-cation-exchange-high-performance-liquid-chromatography-in-central-reference-laboratory-of-india-a-report-of-65779-cases
#20
Sandeep Warghade, Jyothi Britto, Reshma Haryan, Tejaswi Dalvi, Rajesh Bendre, Pratiksha Chheda, Sunmeet Matkar, Yogita Salunkhe, Milind Chanekar, Nilesh Shah
CONTEXT: Hemoglobinopathies constitute the world's most common genetically inherited red blood cell disorder. Screening and accurate identification of hemoglobin (Hb) variants have become increasingly important in antenatal diagnosis and prevention of Hb disorders. AIM: The aim of this study was to screen and identify Hb fractions prevalent in the Central Reference Laboratory of India. MATERIALS AND METHODS: A total of 65,779 cases were screened for hemoglobinopathies on the bio-rad variant high-performance liquid chromatography (HPLC) system by beta-thalassemia short program...
January 2018: Journal of Laboratory Physicians
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