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https://www.readbyqxmd.com/read/28520780/towards-a-point-of-care-strip-test-to-diagnose-sickle-cell-anemia
#1
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/28518049/red-blood-cell-storage-time-and-transfusion-current-practice-concerns-and-future-perspectives
#2
REVIEW
María García-Roa, María Del Carmen Vicente-Ayuso, Alejandro M Bobes, Alexandra C Pedraza, Ataúlfo González-Fernández, María Paz Martín, Isabel Sáez, Jerard Seghatchian, Laura Gutiérrez
Red blood cells (RBCs) units are the most requested transfusion product worldwide. Indications for transfusion include symptomatic anaemia, acute sickle cell crisis, and acute blood loss of more than 30% of the blood volume, with the aim of restoring tissue oxygen delivery. However, stored RBCs from donors are not a qualitative equal product, and, in many ways, this is a matter of concern in the transfusion practice. Besides donor-to-donor variation, the storage time influences the RBC unit at the qualitative level, as RBCs age in the storage bag and are exposed to the so-called storage lesion...
May 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28512745/implementation-of-multi-disciplinary-care-reduces-maternal-mortality-in-women-with-sickle-cell-disease-living-in-low-resource-setting
#3
Eugenia Vicky Asare, Edeghonghon Olayemi, Theodore Boafor, Yvonne Dei-Adomakoh, Enoch Mensah, Harriet Ghansah, Yvonne Osei-Bonsu, Selina Crabbe, Latif Musah, Charles Hayfron-Benjamin, Brittany Covert, Adetola A Kassim, Andra James, Mark Rodeghier, Michael R DeBaun, Samuel A Oppong
Sickle cell disease (SCD) is associated with adverse pregnancy outcome. In women with SCD living in low-resource settings, pregnancy is associated with significantly increased maternal and perinatal mortality rates. We tested the hypothesis that implementing a multidisciplinary obstetric and hematology care team in a low-resource setting would significantly reduce maternal and perinatal mortality rates. We conducted a before-and-after study, at the Korle-Bu Teaching Hospital in Accra, Ghana, to evaluate the effect of a multidisciplinary obstetric-hematology care team for women with SCD in a combined SCD-Obstetric Clinic...
May 16, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28512559/spirometric-evaluation-of-pulmonary-function-in-nigerian-children-underwent-bone-marrow-transplantation-for-sickle-cell-anemia
#4
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/28512557/increased-vasoocclusive-crises-in-o-blood-group-sickle-cell-disease-patients-association-with-underlying-thrombospondin-levels
#5
M Al Huneini, S Alkindi, V Panjwani, K Al Falahi, B Al Balushi, D Gravell, C H Ho, R Krishnamoorthy, A V Pathare
OBJECTIVES: To explore the incidence of vaso-occlusive crisis (VOC) in Blood Group "O" sickle cell disease (SCD) patients, and correlate it with the blood group and thrombospondin (TSP) levels. METHODS: In 89 consecutive SCD patients, blood samples were obtained for von Williebrand factor (vWF:Ag) antigen, collagen binding activity (CBA), ristocetin binding activity (RCo), blood group typing, C-reactive protein (CRP), high performance liquid chromatography (HPLC), Serum TSP 1 and TSP 2 levels, complete blood counts (CBC), lactic dehydrogenase (LDH) levels, liver function (LFT) and renal function tests (RFT) during VOC episodes and in steady state conditions...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28511389/variability-of-iron-load-in-patients-of-sickle-cell-anaemia-hbss-a-study-from-eastern-india
#6
Pranati Mohanty, Rabindra Kumar Jena, Sudha Sethy
INTRODUCTION: Sickle Cell Anaemia (SCA) is one of the commonest haemoglobinopathies due to a point mutation (A→T) of the β-globin gene. Out of five haplotypes, the Arab-Indian haplotype present in India is one of the least severe phenotype and least studied also. It is characterized by lifelong haemolytic anaemia requiring red cell transfusion leading to iron overload. In contrast, there is very high incidence of deficiency of iron, folic acid and vitamin B12. AIM: Our objective was to access the Iron status of SCA patients and to find its correlation with various parameters like red cell transfusion, haemolysis and serum hepcidin...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28511346/potential-therapeutic-action-of-nitrite-in-sickle-cell-disease
#7
Nadeem Wajih, Swati Basu, Anuj Jailwala, Hee Won Kim, David Ostrowski, Andreas Perlegas, Crystal A Bolden, Nancy L Buechler, Mark T Gladwin, David L Caudell, Elaheh Rahbar, Martha A Alexander-Miller, Vidula Vachharajani, Daniel B Kim-Shapiro
Sickle cell disease is caused by a mutant form of hemoglobin that polymerizes under hypoxic conditions, increasing rigidity, fragility, calcium influx-mediated dehydration, and adhesivity of red blood cells. Increased red cell fragility results in hemolysis, which reduces nitric oxide (NO) bioavailability, and induces platelet activation and inflammation leading to adhesion of circulating blood cells. Nitric Oxide inhibits adhesion and platelet activation. Nitrite has emerged as an attractive therapeutic agent that targets delivery of NO activity to areas of hypoxia through bioactivation by deoxygenated red blood cell hemoglobin...
May 10, 2017: Redox Biology
https://www.readbyqxmd.com/read/28510346/lack-of-effect-of-rivipansel-on-qtc-interval-in-healthy-adult-african-american-male-subjects
#8
Brinda K Tammara, Anna Plotka, Frank E Shafer, David R J Readett, Steve Riley, Joan M Korth-Bradley
Rivipansel is a pan-selectin inhibitor in phase 3 development for the treatment of vaso-occlusive crises in patients with sickle cell disease. This single-dose, randomized, 3-period, 3-treatment (400 mg moxifloxacin open-label, 4 g rivipansel-blinded, and placebo-blinded) crossover study evaluated the effect of rivipansel on the QT/QTc interval in 48 healthy male African American subjects (age, 21-53 years; weight, 60-115 kg). Time-matched, placebo-adjusted change from baseline QT interval using Fridericia's correction method (QTcF) was determined using a repeated-measures mixed-effects model...
May 16, 2017: Journal of Clinical Pharmacology
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
#9
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
#10
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
#11
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/28506281/a-telephonic-mindfulness-based-intervention-for-persons-with-sickle-cell-disease-study-protocol-for-a-randomized-controlled-trial
#12
Hants Williams, Susan Silva, Leigh Ann Simmons, Paula Tanabe
BACKGROUND: One of the most difficult symptoms for persons with sickle cell disease (SCD) to manage is chronic pain. Chronic pain impacts approximately one-third of persons with SCD and is associated with increased pain intensity, pain behavior, and frequency and duration of hospital visits. A promising category of nonpharmacological interventions for managing both physical and affective components of pain are mindfulness-based interventions (MBIs). METHODS/DESIGN: The primary aim of this study is to conduct a randomized controlled study to evaluate the acceptability and feasibility, as well as to determine the preliminary efficacy, of a telephonic MBI for adults with SCD who have chronic pain...
May 15, 2017: Trials
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
#13
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/28504327/preferences-for-prenatal-diagnosis-of-sickle-cell-disorder-a%C3%A2-discrete-choice-experiment-comparing-potential-service-users%C3%A2-and-health-care-providers
#14
Melissa Hill, Eugene Oteng-Ntim, Frida Forya, Mary Petrou, Stephen Morris, Lyn S Chitty
BACKGROUND: Non-invasive prenatal diagnosis (NIPD) for sickle-cell disorder (SCD) is moving closer to implementation and studies considering stakeholder preferences are required to underpin strategies for offering NIPD in clinical practice. OBJECTIVE: Determine service user and provider preferences for key attributes of prenatal diagnostic tests for SCD and examine views on NIPD. METHOD: A questionnaire that includes a discrete choice experiment was used to determine the preferences of service users and providers for prenatal tests that varied across three attributes: accuracy, time of test and risk of miscarriage...
May 15, 2017: Health Expectations: An International Journal of Public Participation in Health Care and Health Policy
https://www.readbyqxmd.com/read/28500860/interventions-for-preventing-silent-cerebral-infarcts-in-people-with-sickle-cell-disease
#15
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Carolyn Doree, Miguel R Abboud
BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Silent cerebral infarcts are the commonest neurological complication in children and probably adults with SCD. Silent cerebral infarcts also affect academic performance, increase cognitive deficits and may lower intelligence quotient...
May 13, 2017: Cochrane Database of Systematic Reviews
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
#16
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/28500485/hydroxyurea-lactose-interaction-study-in-silico-and-in-vitro-evaluation
#17
Kunal B Bachchhao, R R Patil, C R Patil, Dipak D Patil
The Maillard reaction between hydroxyurea (a primary amine-containing drug) and lactose (used as an excipient) was explored. The adduct of these compounds was synthesized by heating hydroxyurea with lactose monohydrate at 60 °C in borate buffer (pH 9.2) for 12 h. Synthesis of the adduct was confirmed using UV-visible spectroscopy and Fourier transform infrared, differential scanning calorimetry, high-pressure liquid chromatography, and liquid chromatography-mass spectrometry studies. An in silico investigation of how the adduct formation affected the interactions of hydroxyurea with its biological target oxyhemoglobin, to which it binds to generate nitric oxide and regulates fetal hemoglobin synthesis, was carried out...
May 12, 2017: AAPS PharmSciTech
https://www.readbyqxmd.com/read/28499394/sar1a-promoter-polymorphisms-are-not-associated-with-fetal-hemoglobin-in-patients-with-sickle-cell-disease-from-cameroon
#18
Gift Dineo Pule, Valentina Josiane Ngo Bitoungui, Bernard Chetcha Chemegni, Andre Pascal Kengne, Ambroise Wonkam
BACKGROUND: Reactivation of adult hemoglobin (HbF) is currently a dominant therapeutic approach to sickle cell disease (SCD). In this study, we have investigated among SCD patients from Cameroon, the association of HbF level and variants in the HU-inducible small guanosine triphosphate-binding protein, secretion-associated and RAS-related (SAR1a) protein, previously shown to be associated with HbF after HU treatment in African American SCD patients. RESULTS: Only patients >5 years old were included; hemoglobin electrophoresis and a full blood count were conducted upon arrival at the hospital...
May 12, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28494508/look-into-my-eyes-an-unusual-first-presentation-of-sickle-cell-disease
#19
Rupali Sood, Kim Jiramongkolchai, Michael Streiff, Christopher Gonzalez, Satish Shanbhag, Sophie Lanzkron, J Fernando Arevalo, Rakhi Naik
No abstract text is available yet for this article.
May 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28493472/turf-wars-exploring-splenomegaly-in-sickle-cell-disease-in-malaria-endemic-regions
#20
REVIEW
Venée N Tubman, Julie Makani
Sickle cell disease (SCD) is a group of recessively inherited disorders of erythrocyte function that presents an ongoing threat to reducing childhood and adult morbidity and mortality around the world. While decades of research have led to improved survival for SCD patients in wealthy countries, survival remains dismal in low- and middle-income countries. Much of the early mortality associated with SCD is attributed to increased risk of infections due to early loss of splenic function. In the West, bacterial infections with encapsulated organisms are a primary concern...
May 11, 2017: British Journal of Haematology
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