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Sickle cell diseases

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https://www.readbyqxmd.com/read/28538513/attention-deficit-hyperactivity-disorder-in-children-with-sickle-cell-disease-referred-for-an-evaluation
#1
Melissa A Acquazzino, Meghan Miller, Matthew Myrvik, Robert Newby, John Paul Scott
Neuropsychological deficits, including difficulties with attention, are well described in children with sickle cell disease (SCD). Very little is known about attention deficit hyperactivity disorder (ADHD) in children with SCD. The objective of this study was to determine the proportion of ADHD in children with SCD referred for neuropsychological evaluation. This prospective, cross-sectional study included patients (age, 4 to 18 y) with SCD and completion of a neuropsychological evaluation between December 2013 and March 2016...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538505/thalassemia-major-and-priapism-a-case-report-of-an-adolescent
#2
Sibel Öz, Serhan Küpeli, Gülay Sezgin, İbrahim Bayram
Priapism is defined as a prolonged pathologic penile erection without sexual stimulation. In children, priapism secondary to sickle cell disease or hematological malignancy is a frequent condition. Appropriate treatment of priapism varies; the treatment is primarily etiological, conservative management. In the present report, we aimed to present a case of asplenic thalassemia major who developed priapism, improved with hydration and ibuprofen treatment. Clinicians should take into account that priapism can be encountered in patients with thalassemia major...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538501/invasive-pneumococcal-disease-in-patients-with-sickle-cell-disease
#3
Pournima Navalkele, Bülent Özgönenel, Eric McGrath, Paul Lephart, Sharada Sarnaik
BACKGROUND: Patients with sickle cell disease (SCD) are at risk of fatal sepsis with encapsulated bacteria, such as Streptococcus pneumoniae, because of the inherent autosplenectomy that occurs in SCD. This risk is thwarted with oral penicillin prophylaxis during the first 5 years of life, and with stringent vaccination against S. pneumoniae alongside routine childhood immunization. But compared with the general African American pediatric population, the rate of invasive pneumococcal disease (IPD) in patients with SCD still remains high, resulting in hospitalization and fatality...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538131/gene-therapy-in-a-patient-with-sickle-cell-disease
#4
Philippe Leboulch, Marina Cavazzana
No abstract text is available yet for this article.
May 25, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28538130/gene-therapy-in-a-patient-with-sickle-cell-disease
#5
LETTER
(no author information available yet)
No abstract text is available yet for this article.
May 25, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28534764/traveling-to-angola-to-validate-a-paper-based-sickle-cell-disease-test
#6
Nathaniel Z Piety
When I started graduate school, I had no idea that it would take me halfway around the world to a small clinic in Cabinda, Angola, but I'm glad that it did. Since 2011, I have been a part of the team of engineers and clinicians, led by Dr. Sergey Shevkoplyas, working to develop a simple, low-cost, paper-based test for sickle cell disease-a common inherited blood disorder that is most prevalent in sub-Saharan Africa (Figure S1).
May 2017: IEEE Pulse
https://www.readbyqxmd.com/read/28534763/paper-based-diagnostics-rethinking-conventional-sickle-cell-screening-to-improve-access-to-high-quality-health-care-in-resource-limited-settings
#7
Nathaniel Z Piety, Sergey S Shevkoplyas
Every year, hundreds of thousands of children worldwide are born with sickle cell disease, a genetic disorder that impacts the hemoglobin molecules in blood. If left undiagnosed and untreated, most affected children will die before reaching the age of five. However, highly accurate diagnostic methods and effective treatment regimens for sickle cell disease have been known for many years, and children who receive early diagnosis and subsequent comprehensive care survive well into adulthood-as evidenced by the tremendous success of universal newborn screening programs in North America and Europe...
May 2017: IEEE Pulse
https://www.readbyqxmd.com/read/28532265/encephaloduroarteriosynangiosis-edas-in-young-patients-with-cerebrovascular-complications-of-sickle-cell-disease-single-institution-experience
#8
Michael Winstead, Peter P Sun, Kenneth Martin, Janice Earl, Lynne Neumayr, Carolyn Hoppe, Elliott Vichinsky
Moyamoya syndrome occurs in sickle cell disease (SCD) as a secondary complication of large-artery stenosis. Moyamoya increases the risk of stroke, but its optimal management in SCD is not established.  Encephaloduroarteriosynangiosis (EDAS) is a neurosurgical revascularization procedure for moyamoya whose use has been reported in SCD patients. We report the outcomes of 11 patients with SCD systematically evaluated for EDAS by a multidisciplinary team and compare the rate of stroke in patients who received EDAS to those who did not...
May 22, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28530652/cd34-cells-from-dental-pulp-stem-cells-with-a-zfn-mediated-and-homology-driven-repair-mediated-locus-specific-knock-in-of-an-artificial-%C3%AE-globin-gene
#9
S Chattong, O Ruangwattanasuk, W Yindeedej, A Setpakdee, K Manotham
In humans, mutations in the β-globin gene (HBB) have two important clinical manifestations: β-thalassemia and sickle cell disease. The progress in genome editing and stem cell research may be relevant to the treatment of β-globin-related diseases. In this work, we employed zinc finger nuclease (ZFN)-mediated gene integration of synthetic β-globin cDNA into HBB loci, thus correcting almost all β-globin mutations. The integration was achieved in both HEK 293 cells and isolated dental pulp stem cell (DPSCs)...
May 22, 2017: Gene Therapy
https://www.readbyqxmd.com/read/28530510/self-reported-physical-activity-and-exercise-patterns-in-children-with-sickle-cell-disease
#10
Osarhiemen A Omwanghe, Devin S Muntz, Soyang Kwon, Simone Montgomery, Opeyemi Kemiki, Lewis L Hsu, Alexis A Thompson, Robert I Liem
PURPOSE: Sickle cell disease (SCD) significantly affects physical functioning. We examined physical activity (PA) patterns in children with SCD versus a national sample and factors associated with PA and participation in physical education and organized sports. METHOD: One hundred children with SCD completed a 58-item survey with questions from the 2009-2010 National Health and Nutrition Examination Survey (NHANES) Physical Activity Questionnaire and others on physical education and sports, disease impact, and physical functioning...
May 22, 2017: Pediatric Exercise Science
https://www.readbyqxmd.com/read/28528302/nanomolar-anti-sickling-compounds-identified-by-ligand-based-pharmacophore-approach
#11
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/28527641/identifying-social-behavioral-health-needs-of-adults-with-sickle-cell-disease-in-the-emergency-department
#12
Sophia K Smith, Julia Johnston, Carlton Rutherford, Rachel Hollowell, Paula Tanabe
INTRODUCTION: Sickle cell disease (SCD) is a complex illness with many social-behavioral co-morbidities. The aim of this project was to describe unmet social-behavioral health needs for adults with SCD who presented to the emergency department for treatment of vaso-occlusive episodes (VOEs). METHODS: A descriptive study using 1:1 interviews during an ED visit for a VOE was conducted; a brief social behavioral health screening interview guide was used. A convenience sample of adults with SCD treated in the emergency department for a VOE were eligible for inclusion...
May 17, 2017: Journal of Emergency Nursing: JEN: Official Publication of the Emergency Department Nurses Association
https://www.readbyqxmd.com/read/28520780/towards-a-point-of-care-strip-test-to-diagnose-sickle-cell-anemia
#13
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/28512745/implementation-of-multi-disciplinary-care-reduces-maternal-mortality-in-women-with-sickle-cell-disease-living-in-low-resource-setting
#14
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/28512557/increased-vasoocclusive-crises-in-o-blood-group-sickle-cell-disease-patients-association-with-underlying-thrombospondin-levels
#15
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/28511346/potential-therapeutic-action-of-nitrite-in-sickle-cell-disease
#16
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
#17
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/28506281/a-telephonic-mindfulness-based-intervention-for-persons-with-sickle-cell-disease-study-protocol-for-a-randomized-controlled-trial
#18
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
#19
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/28500860/interventions-for-preventing-silent-cerebral-infarcts-in-people-with-sickle-cell-disease
#20
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
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