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https://www.readbyqxmd.com/read/29781571/new-approach-to-accurate-interpretation-of-sickle-cell-disease-newborn-screening-by-applying-multiple-of-median-cutoffs-and-ratios
#1
Bichr Allaf, Franck Patin, Jacques Elion, Nathalie Couque
BACKGROUND: The main goal of newborn screening (NBS) for sickle cell disease (SCD) is to detect affected neonates so that specific preventive care can be implemented. High-performance liquid chromatography (HPLC) used for NBS has high sensitivity and specificity, but we lack guidelines for quantitative hemoglobin (Hb) fraction interpretation. The purpose of this study was to determine cutoff values to standardize quantitative interpretation in SCD NBS for different clinical situation such as, red blood cell transfusion or beta-thalassemia, which can be real screening pitfalls...
May 21, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29781568/diastolic-dysfunction-is-associated-with-exercise-impairment-in-patients-with-sickle-cell-anemia
#2
Tarek Alsaied, Omar Niss, Adam W Powell, Robert J Fleck, James F Cnota, Clifford Chin, Punam Malik, Charles T Quinn, Michael D Taylor
BACKGROUND: Left ventricular diastolic dysfunction (DD) is an independent risk factor for mortality in sickle cell anemia (SCA) and is associated with increased extracellular volume (ECV) on cardiac MRI (CMR). Exercise impairment is common in SCA, but its causes and prognostic value are not well understood. OBJECTIVE: To study the effects of DD and ECV on cardiopulmonary exercise test (CPET) in patients with SCA. METHODS AND RESULTS: As part of a prospective study to characterize the cardiomyopathy of SCA (NCT02410811), 20 children and adults with SCA underwent CMR, echocardiography, and cycle ergometer CPET (age range 8-43 years)...
May 21, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29778312/biomarker-signatures-of-sickle-cell-disease-severity
#3
Mengtian Du, Sarah Van Ness, Victor Gordeuk, Sayed M Nouraie, Sergei Nekhai, Mark Gladwin, Martin H Steinberg, Paola Sebastiani
Identifying sickle cell disease patients at high risk of complications could lead to personalized treatment and better prognosis but despite many advances prediction of the clinical course of these patients remains elusive. We propose a system-type approach to discover profiles of multiple, common biomarkers that correlate with morbidity and mortality in sickle cell disease. We used cluster analysis to discover 17 signatures of 17 common circulating biomarkers in 2320 participants of the Cooperative Study of Sickle Cell Disease, and evaluated the association of these signatures with risk for stroke, pain, leg ulceration, acute chest syndrome, avascular necrosis, seizure, death, and trend of fetal hemoglobin and hemolysis using longitudinally collected data...
May 16, 2018: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29773954/pain-catastrophizing-is-associated-with-poorer-health-related-quality-of-life-in-pediatric-patients-with-sickle-cell-disease
#4
Nitya Bakshi, Ines Lukombo, Inna Belfer, Lakshmanan Krishnamurti
Background: Sickle cell disease (SCD) is an inherited disorder of the red blood cells and is associated with chronic multisystem involvement. While SCD has been associated with poorer health-related quality of life (HRQoL), there is a paucity of data on the relationship of psychological covariates other than anxiety and depression and quality of life (QoL) in children with SCD. Materials and methods: We performed a cross-sectional study of psychological factors, HRQoL, and pain-related outcomes in participants with SCD and race-matched controls as part of a larger study of experimental pain phenotyping...
2018: Journal of Pain Research
https://www.readbyqxmd.com/read/29773497/indications-for-red-cell-transfusions-in-pediatric-patients
#5
REVIEW
Jill M Cholette, Suzie A Noronha, Jerard Seghatchian, Neil Blumberg
Red cell transfusions are amongst the most common therapeutic procedures in seriously ill children, particularly in the inpatient setting. This is despite the fact that there is no evidence base for most clinical settings, with the exception of patients with hemoglobinopathies, particularly thalassemia and sickle cell anemia. Obviously exsanguinating hemorrhage and life threatening anemia are urgent indications for which no other therapeutic approach is currently available. Most transfusions are, however, given prophylactically to prevent the complications of hypoxia or hemodynamic stability, based upon expert opinion and a faith in the oxygen carrying capacity and beneficial hemodynamic properties of transfused red cells...
May 10, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29768930/a-strategic-planning-tool-for-increasing-african-american-blood-donation
#6
Ashley Singleton, Regena Spratling
Historically, African Americans (AAs) have been underrepresented as blood donors. Having a lack of racially diverse blood donors contributes to transfusion complications, particularly in patients with sickle cell disease, who are both disproportionately AA and the recipients of frequent transfusions. Increasing AA blood donation is a complex public health issue. This review article serves to fill a gap in translating research regarding known hindrances and facilitators of AA blood donation to improve real-world donation practice and ultimately, patient outcomes...
May 1, 2018: Health Promotion Practice
https://www.readbyqxmd.com/read/29768104/knowledge-beliefs-attitudes-and-behaviors-regarding-sickle-cell-disease-implications-for-prevention
#7
Marcella Smith, Gracie Brownell
Sickle cell disease (SCD) is a widespread inherited blood disorder, which leaves lasting effects on the health, social functioning, and finances of individuals, families, communities, and health care systems. A nonexperimental, cross-sectional research design was used to assess 415 college students' knowledge about SCD. Data was obtained through an online survey derived from a modified version of the SCD Knowledge Assessment Tool. The majority of participants (79%) reported previous SCD knowledge; however, 21% of the participants reported no previous SCD knowledge...
May 16, 2018: Social Work in Public Health
https://www.readbyqxmd.com/read/29767851/risk-factors-for-vitamin-d-deficiency-in-sickle-cell-disease
#8
Jin Han, Xu Zhang, Santosh L Saraf, Michel Gowhari, Robert E Molokie, Johara Hassan, Shivi Jain, Binal N Shah, Taimur Abbasi, Roberto F Machado, Victor R Gordeuk
Vitamin D deficiency (VDD), 25-OHD levels <20 ng/ml, is prevalent among patients with sickle cell disease (SCD) and is linked to acute and chronic pain and bone fracture in this population. There is limited literature regarding VDD-associated risk factors for SCD. We examined potential clinical and genomic parameters associated with VDD in 335 adults with SCD in a cross-sectional study. VDD was present in 65% of adult SCD patients, and 25-OHD levels independently and positively correlated with older age (P < 0·001) and vitamin D supplementation (P < 0·001)...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29767446/hydroxycarbamide-adherence-and-cumulative-dose-associated-with-hospital-readmission-in-sickle-cell-disease-a-6-year-population-based-cohort-study
#9
Jifang Zhou, Jin Han, Edith A Nutescu, Victor R Gordeuk, Santosh L Saraf, Gregory S Calip
Sickle cell disease (SCD) is a congenital haemoglobinopathy that causes frequent acute care/emergency room visits and hospital admissions for affected individuals. Evidence from population-based studies demonstrating the role of hydroxycarbamide (HC, also termed hydroxyurea) in reducing hospital readmission rates is limited. Our objective was to describe the use of HC and its association with acute care utilization and readmission rates using a large, nationally-representative US health insurance claims database over a 6-year period between 2009 and 2014...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29766032/serum-free-erythroid-differentiation-for-efficient-genetic-modification-and-high-level-adult-hemoglobin-production
#10
Naoya Uchida, Selami Demirci, Juan J Haro-Mora, Atsushi Fujita, Lydia N Raines, Matthew M Hsieh, John F Tisdale
In vitro erythroid differentiation from primary human cells is valuable to develop genetic strategies for hemoglobin disorders. However, current erythroid differentiation methods are encumbered by modest transduction rates and high baseline fetal hemoglobin production. In this study, we sought to improve both genetic modification and hemoglobin production among human erythroid cells in vitro . To model therapeutic strategies, we transduced human CD34+ cells and peripheral blood mononuclear cells (PBMCs) with lentiviral vectors and compared erythropoietin-based erythroid differentiation using fetal-bovine-serum-containing media and serum-free media...
June 15, 2018: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/29765433/left-ventricular-structural-and-functional-changes-evaluated-by-echocardiography-and-two-dimensional-strain-in-patients-with-sickle-cell-disease
#11
Ricardo Bedirian, Andrea Ribeiro Soares, Maria Christina Maioli, Jussara Fonseca Fernandes de Medeiros, Agnaldo José Lopes, Marcia Bueno Castier
Introduction: Patients with sickle cell disease have increased left ventricular size, which is not usually accompanied by changes in systolic function indexes. We assessed echocardiographic abnormalities present in patients with sickle cell anemia (SCA) and compared echocardiographic parameters to other sickle cell diseases (OSCD). Material and methods: A blind cross-sectional study with 60 patients with SCA and 16 patients with OSCD who underwent transthoracic echocardiography was performed...
April 2018: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/29764840/how-we-diagnose-and-treat-venous-thromboembolism-in-sickle-cell-disease
#12
Arun S Shet, Theodore Wun
The incidence of venous thromboembolism (VTE) in adult patients with sickle cell disease (SCD) is high. However, overlapping features between the clinical presentation of VTE and SCD complications and a low index of suspicion for thrombosis can influence patient management decisions. VTE in SCD can therefore present management challenges to the clinical hematologist. Herein, we present three distinct clinical vignettes that are representative of our clinical practice with SCD patients. These vignettes are discussed with specific reference to the hypercoagulable state in SCD patients, recent VTE diagnosis and anticoagulant therapy guidelines from the general population, and evaluation of the risk of bleeding as a result of long term exposure to anticoagulant therapy...
May 15, 2018: Blood
https://www.readbyqxmd.com/read/29762306/prevalence-and-incidence-of-congenital-anomalies-amongst-babies-born-to-women-with-sickle-cell-disease-and-exposed-to-hydroxyurea-during-pregnancy-a-systematic-review-protocol
#13
Stephen Otieno Gwer, Kennedy Odoyo Onyango
What is the prevalence and incidence of congenital anomalies among babies born to women with sickle cell disease (SCD) and who have been exposed to hydroxyurea (HU) therapy at any time in their pregnancy?The objective of this review is to identify the proportion of babies born with congenital anomalies among babies born to mothers with SCD who have been exposed to HU therapy at any point during pregnancy and to describe the specific types of congenital anomalies encountered.
May 2018: JBI Database of Systematic Reviews and Implementation Reports
https://www.readbyqxmd.com/read/29761371/tolerability-and-efficacy-of-deferasirox-in-patients-with-transfusional-iron-overload-results-from-a-german-2-year-non-interventional-study
#14
Florian Nolte, Holger Nückel, Burkhard Schmidt, Thomas Geer, Oleg Rubanov, Holger Hebart, Andrea Jarisch, Stefan Albrecht, Christiane Johr, Christiane Schumann, Wolf-Karsten Hofmann
BACKGROUND: Iron overload (IOL) due to repetitive transfusions of packed red blood cells (pRBC) has a major impact on morbidity and mortality in patients with inherited bone marrow failure syndromes and hemoglobinopathies such as thalassemia and sickle cell disease. However, whether IOL influences the outcome of elderly patients with myeloid malignancies is not yet clear. Moreover, clinical trials have reported high drop-out rates during treatment with the oral iron chelator deferasirox (DFX)...
May 14, 2018: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/29756358/echocardiographic-parameters-to-identify-sickle-cell-patients-with-cardio-pathology
#15
Simbo Chiadika, Mary Lim-Fung, Fiorella Llanos-Chea, Astrid Serauto Canache, Wei Yang, Christina Paruthi, Xu Zhang, David D McPherson, Modupe Idowu
BACKGROUND: Sickle cell disease (SCD) affects millions of people and causes chronic hemolytic anemia leading to vasculopathies such as pulmonary hypertension and abnormalities in cardiac function that increase complications and mortality. It is therefore crucial to identify cardiac abnormalities in SCD. We aimed to assess the prevalence of echocardiographic parameters in SCD to help identify cardiopulmonary risk. METHODS: Ninety-one patients (53% male), median age of 30, body surface area (BSA) of 1...
May 14, 2018: Echocardiography
https://www.readbyqxmd.com/read/29755709/perioperative-management-of-sickle-cell-disease
#16
REVIEW
Kwame Ofori Adjepong, Folashade Otegbeye, Yaw Amoateng Adjepong
Over 30 million people worldwide have sickle cell disease (SCD). Emergent and non-emergent surgical procedures in SCD have been associated with relatively increased risks of peri-operative mortality, vaso-occlusive (painful) crisis, acute chest syndrome, post-operative infections, congestive heart failure, cerebrovascular accident and acute kidney injury. Pre-operative assessment must include a careful review of the patient's known crisis triggers, baseline hematologic profile, usual transfusion requirements, pre-existing organ dysfunction and opioid use...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29752305/white-matter-integrity-and-processing-speed-in-sickle-cell-anemia
#17
Hanne Stotesbury, Fenella J Kirkham, Melanie Kölbel, Philippa Balfour, Jonathan D Clayden, Sati Sahota, Simrat Sakaria, Dawn E Saunders, Jo Howard, Rachel Kesse-Adu, Baba Inusa, Maria Pelidis, Subarna Chakravorty, David C Rees, Moji Awogbade, Olu Wilkey, Mark Layton, Christopher A Clark, Jamie M Kawadler
OBJECTIVE: The purpose of this retrospective cross-sectional study was to investigate whether changes in white matter integrity are related to slower processing speed in sickle cell anemia. METHODS: Thirty-seven patients with silent cerebral infarction, 46 patients with normal MRI, and 32 sibling controls (age range 8-37 years) underwent cognitive assessment using the Wechsler scales and 3-tesla MRI. Tract-based spatial statistics analyses of diffusion tensor imaging (DTI) and neurite orientation dispersion and density imaging (NODDI) parameters were performed...
May 11, 2018: Neurology
https://www.readbyqxmd.com/read/29751732/population-screening-for-hemoglobinopathies
#18
H W Goonasekera, C S Paththinige, V H W Dissanayake
Hemoglobinopathies are the most common single-gene disorders in the world. Their prevalence is predicted to increase in the future, and low-income hemoglobinopathy-endemic regions need to manage most of the world's affected persons. International organizations, governments, and other stakeholders have initiated national or regional prevention programs in both endemic and nonendemic countries by performing population screening for α- and β-thalassemia, HbE disease, and sickle cell disease in neonates, adolescents, reproductive-age adults (preconceptionally or in the early antenatal period), and family members of diagnosed cases...
May 11, 2018: Annual Review of Genomics and Human Genetics
https://www.readbyqxmd.com/read/29749702/management-of-severe-chronic-pain-with-methadone-in-pediatric-patients-with-sickle-cell-disease
#19
Zachary LeBlanc, Chris Vance, Jason Payne, Jie Zhang, Lee Hilliard, Jeffrey D Lebensburger, Thomas H Howard
Vasocclusive pain crises are common among pediatric patients with sickle cell disease (SCD). Some patients with repeated pain crises develop chronic pain. We performed a retrospective cohort study of pediatric patients with SCD with chronic pain treated with methadone. We identified a significant reduction in pain hospitalizations following methadone treatment (0.35 ± 0.19 vs. 0.19 ± 0.17 hospitalizations/month, P = 0.016). In addition, we did not observe overt organ toxicity nor symptoms of opioid withdrawal during methadone wean...
May 11, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29749368/effect-of-inherited-red-cell-defects-on-growth-of-plasmodium-falciparum-an-in-vitro-study
#20
Vrushali Pathak, Roshan Colah, Kanjaksha Ghosh
Background & objectives: High prevalence of certain polymorphic alleles of erythrocytes in malaria endemic area has been linked to the resistance provided by these alleles against parasitic infestations. Numerous studies undertaken to demonstrate this correlation have generated conflicting results. This study was undertaken to investigate the abilities of various polymorphic erythrocytes to support in vitro growth of Plasmodium falciparum parasites. Methods: In this study under in vitro condition the ability of P...
January 2018: Indian Journal of Medical Research
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