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"Sickle Cell Anemia"

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https://www.readbyqxmd.com/read/29449005/association-between-oxidative-stress-genetic-factors-and-clinical-severity-in-children-with-sickle-cell-anemia
#1
Céline Renoux, Philippe Joly, Camille Faes, Pauline Mury, Buse Eglenen, Mine Turkay, Gokce Yavas, Ozlem Yalcin, Yves Bertrand, Nathalie Garnier, Daniela Cuzzubbo, Alexandra Gauthier, Marc Romana, Berenike Möckesch, Giovanna Cannas, Sophie Antoine-Jonville, Vincent Pialoux, Philippe Connes
OBJECTIVES: To investigate the associations between several sickle cell disease genetic modifiers (beta-globin haplotypes, alpha-thalassemia, and glucose-6-phosphate dehydrogenase deficiency) and the level of oxidative stress and to evaluate the association between oxidative stress and the rates of vaso-occlusive events. STUDY DESIGN: Steady-state oxidative and nitrosative stress markers, biological variables, genetic modulators, and vaso-occlusive crisis events requiring emergency admissions were measured during a 2-year period in 62 children with sickle cell anemia (58 SS and 4 Sβ 0 )...
February 13, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29447985/selected-terpenes-from-leaves-of-ocimum-basilicum-l-induce-hemoglobin-accumulation-in-human-k562-cells
#2
Feriotto Giordana, Marchetti Nicola, Costa Valentina, Piera Torricelli, Simone Beninati, Federico Tagliati, Carlo Mischiati
Re-expression of fetal hemoglobin (HbF) was proposed as a possible therapeutic strategy for β-haemoglobinopathies. Although several inducers of HbF were tested in clinical trials, only hydroxyurea (HU) received FDA approval. Despite it produced adequate HbF levels only in half of HU-treated SCD patients, and was ineffective at all in β-thalassemia patients, beneficial effects of this approach suggested to continue in this direction identifying further molecules capable of inducing HbF. We tested the potential of essential oil isolated from Ocimum basilicum L...
February 12, 2018: Fitoterapia
https://www.readbyqxmd.com/read/29447888/pathogenesis-of-chronic-rhinosinusitis-in-patients-affected-by-%C3%AE-thalassemia-major-and-sickle-cell-anaemia-post-allogenic-bone-marrow-transplant
#3
F Martino, R Di Mauro, K Paciaroni, J Gaziev, C Alfieri, L Greco, R Floris, S Di Girolamo, M Di Girolamo
OBJECTIVES: Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29437860/antibiotic-prophylaxis-for-children-with-sickle-cell-anemia
#4
Sarah L Reeves, Alison C Tribble, Brian Madden, Gary L Freed, Kevin J Dombkowski
BACKGROUND: Children with sickle cell anemia (SCA) are at increased risk for invasive pneumococcal disease; antibiotic prophylaxis significantly reduces this risk. We calculated the proportion of children with SCA who received ≥300 days of antibiotic prophylaxis and identified predictors of such receipt. METHODS: Children aged 3 months to 5 years with SCA were identified by the presence of 3 or more Medicaid claims with a diagnosis of SCA within a calendar year (2005-2012) in Florida, Illinois, Louisiana, Michigan, South Carolina, and Texas...
February 5, 2018: Pediatrics
https://www.readbyqxmd.com/read/29411937/nephrin-as-a-biomarker-of-sickle-cell-glomerulopathy-in-malawi
#5
J Brett Heimlich, Godwin Chipoka, Laila Elsherif, Emeraghi David, Graham Ellis, Portia Kamthunzi, Robert Krysiak, Pilirani Mafunga, Qingning Zhou, Jianwen Cai, Satish Gopal, Nigel S Key, Kenneth I Ataga
BACKGROUND: Glomerulopathy is an increasingly identified complication in young patients with sickle cell disease (SCD). Hyperfiltration and albuminuria followed by declining glomerular filtration rates and eventual end-stage renal disease (ESRD) is assumed to be the typical progression of glomerular disease. There are only a few reported biomarkers to identify early-stage renal disease in SCD. PROCEDURES: We detail the renal profile of 101 children with SCD in Malawi and propose a novel urinary biomarker for the identification of early renal disease...
February 7, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29411418/feasibility-trial-for-primary-stroke-prevention-in-children-with-sickle-cell-anemia-in-nigeria-spin-trial
#6
Najibah A Galadanci, Shehu Umar Abdullahi, Leah D Vance, Abdulkadir Musa Tabari, Shehi Ali, Raymond Belonwu, Auwal Salihu, Aisha Amal Galadanci, Binta Wudil Jibir, Halima Bello-Manga, Kathleen Neville, Fenella J Kirkham, Yu Shyr, Sharon Phillips, Brittany V Covert, Adetola A Kassim, Lori C Jordan, Muktar H Aliyu, Michael R DeBaun
No abstract text is available yet for this article.
March 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29405852/james-herrick-1861-1954-consultant-physician-and-cardiologist
#7
Todd M Olszewski
In 1910, James Bryan Herrick published the first clinical and laboratory description of sickle cell anemia. Two years later, he published a case report on coronary thrombosis. Together, these case reports solidified his reputation as one of the premier diagnosticians of his generation. Now regarded as a central figure in the history of American medicine, Herrick played an integral role in the clinical adoption of the electrocardiograph and the professionalization of cardiology in the United States. Although a full decade passed before the medical profession recognized his clinical description of coronary thrombosis and myocardial infarction, it has had profound implications for cardiovascular medicine and prevention over the past hundred years...
January 1, 2018: Journal of Medical Biography
https://www.readbyqxmd.com/read/29399706/differential-diagnostic-perspectives-provided-by-en-face-microscopic-examination-of-articular-surface-defects
#8
Bruce M Rothschild
Surface defects have a central position in diagnosis of articular pathology. Recognizing the limitations of standard radiologic techniques and those imposed by positioning and averaging artifacts on CT evaluation, direct visualization of surface defects was pursued to identify disease characteristics that would facilitate interpretation of radiologic findings. Epi-illumination surface microscopy was utilized to examine macroscopically recognized articular surface defects in individuals in the Hamann-Todd, Terry, and Huntington human skeletal collections with previously verified diagnoses of rheumatoid arthritis, spondyloarthropathy, juvenile inflammatory arthritis (JIA), calcium pyrophosphate deposition disease (CPPD), gout, metastatic cancer, multiple myeloma, septic arthritis, tuberculosis, fungal arthritis, histiocytosis and sickle cell anemia (Rothschild and Rothschild Clin Infect Dis 20(5):1402-1408, 1995; Rothschild et al...
February 4, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29398806/lower-transcranial-doppler-flow-velocities-in-sickle-cell-anemia-patients-on-hydroxyurea-myth-or-fact
#9
Sawsan M Moeen, Ahmad F Thabet, Hosam A Hasan, Medhat A Saleh
Transcranial Doppler (TCD) detects stroke risk in patients with sickle cell anemia (SCA). Hydroxyurea therapy has the ability to induce increased levels of fetal hemoglobin in sickle cells thus decreasing tendency for red cell sickling. This study aimed to evaluate TCD findings in SCA patients on hydroxyurea and correlate the time-averaged mean velocity (TAMV) with their hematological parameters. Forty SCA patients of both sexes, aged 16-22 years with no history of stroke were screened with TCD for an elevated TAMV, divided into: Group T (20 patients on blood transfusion); and Group H (20 patients on daily hydroxyurea)...
January 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29386039/posterior-reversible-encephalopathy-syndrome-secondary-to-asymptomatic-poststreptococcal-glomerulonephritis-in-a-child-with-sickle-cell-anemia-a-case-report
#10
Ehab Hanafy, Duaa Alshareef, Suhaila Osman, Abdullah Al Jabri, Faisal Nazim, Gihan Mahmoud
BACKGROUND: Posterior reversible encephalopathy syndrome is a neurotoxic condition that occurs as a result of the failure of posterior circulatory autoregulation in response to acute changes in blood pressure. Overperfusion with resultant disruption of the blood-brain barrier results in vasogenic edema, but not infarction. Posterior reversible encephalopathy syndrome can be the presenting feature of postinfectious glomerulonephritis, which has been reported in approximately 5% of hospitalized children, and it has been reported in very few cases of adult patients with sickle cell anemia...
February 1, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29373851/carbon-monoxide-repurposed-researchers-are-developing-myriad-ways-to-deliver-co-to-treat-sickle-cell-anemia-lung-disease-and-more
#11
Wudan Yan
In the 16th century, Paracelsus-the father of modern toxicology-wrote that "all things are poison and nothing is without poison; the dose alone makes a thing not poison." While it's conceivable that too much of a good thing, such as water or oxygen, could be fatal, the opposite-that smaller quantities of a bad thing might be beneficial-may be harder to believe. But four centuries after Paracelsus shared this idea, two researchers decided to apply the more counterintuitive notion of Paracelsus' dogma for a notoriously toxic chemical: carbon monoxide (CO)...
January 2018: IEEE Pulse
https://www.readbyqxmd.com/read/29372059/factor-v-leiden-g1691a-and-prothrombin-g20210a-mutations-among-palestinian-patients-with-sickle-cell-disease
#12
Fekri Samarah, Mahmoud A Srour
Background: Vascular thrombosis is an important pathophysiological aspect of sickle cell disease (SCD). This study aimed to investigate the prevalence and clinical impact of factor V Leiden G1691A (FVL) and prothrombin G20210A mutations among Palestinian sickle cell disease (SCD) patients. Methods: A total of 117 SCD patients, including 59 patients with sickle cell anemia (SS), 33 patients with sickle β-thalassemia and 25 individuals with sickle cell trait (AS) were studied...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29364234/measuring-deformability-and-red-cell-heterogeneity-in-blood-by-ektacytometry
#13
Nermi L Parrow, Pierre-Christian Violet, Hongbin Tu, James Nichols, Corinne A Pittman, Courtney Fitzhugh, Robert E Fleming, Narla Mohandas, John F Tisdale, Mark Levine
Decreased red cell deformability is characteristic of several disorders. In some cases, the extent of defective deformability can predict severity of disease or occurrence of serious complications. Ektacytometry uses laser diffraction viscometry to measure the deformability of red blood cells subject to either increasing shear stress or an osmotic gradient at a constant value of applied shear stress. However, direct deformability measurements are difficult to interpret when measuring heterogenous blood that is characterized by the presence of both rigid and deformable red cells...
January 12, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29363630/assessment-of-some-indicators-of-oxidative-stress-in-nigerian-sickle-cell-anemic-patients
#14
C P Okorie, Theresa Nwagha, Fidelis Ejezie
BACKGROUND: Oxidative stress, the imbalance between the productive of reactive species and antioxidant defences notably plays an important role in the pathogenesis of sickle cell disease. Generating the necessary data about oxidative stress in patients with sickle cell anemia (SCA) would help in developing cost-effective and accessible treatment interventions of SCA in resource-poor countries. AIM: The aim of this study is to evaluate the levels plasma lipid peroxidation product, malondialdehyde (MDA), serum nitric oxide (NO), and total antioxidant activity (TAOA) as indicators of oxidative stress in sickle cell individuals...
January 2018: Annals of African Medicine
https://www.readbyqxmd.com/read/29345446/the-first-south-american-case-of-pre-implantation-genetic-diagnosis-to-select-compatible-embryo-for-cord-blood-transplantation-as-treatment-for-sickle-cell-anemia
#15
Ciro D Martinhago, Kalina Rn Endo, Mariana A Oliveira, Alex Mm Dias, Gislaine S Pereira, Augusto M Azzolini, Paula Rq Estrada, Caio G Bruzaca, Ana Carolina N Martinhago
Sickle cell anemia is an inherited systemic hemoglobinopathy that affects hemoglobin production in red blood cells, leading to early morbidity and mortality. It is caused by a homozygous nucleotide substitution (c.20A>T) in the β-globin gene (HBB) that changes a glutamic acid to a valine in the protein. We present a case report of a fertile couple, both carriers of the sickle cell anemia mutation, with one affected daughter. Six cycles of assisted reproductive techniques were performed, resulting in 53 embryos in cleavage stage...
January 18, 2018: JBRA Assisted Reproduction
https://www.readbyqxmd.com/read/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#16
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29318647/realizing-effectiveness-across-continents-with-hydroxyurea-enrollment-and-baseline-characteristics-of-the-multicenter-reach-study-in-sub-saharan-africa
#17
Patrick T McGann, Thomas N Williams, Peter Olupot-Olupot, George A Tomlinson, Adam Lane, José Luís Reis da Fonseca, Robert Kitenge, George Mochamah, Ham Wabwire, Susan Stuber, Thad A Howard, Kathryn McElhinney, Banu Aygun, Teresa Latham, Brígida Santos, Léon Tshilolo, Russell E Ware
Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries...
January 10, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29313508/evaluation-of-transfusion-practices-in-sickle-cell-disease-in-senegal-cohort-study-of-1078-patients-with-sickle-cell-disease
#18
M Seck, A Tall, B F Faye, D Sy Bah, Y Guéye, A Sall, A O Touré, S Diop
Blood transfusion is an essential therapeutic tool in the treatment of sickle cell disease. Its indications and modalities vary from one country to another as a function of the clinical severity of the disease and the availability of blood products. This study seeks to evaluate the frequency and modalities of blood transfusions as well as their indications in patients with sickle cell syndrome at the Dakar hematology department from 1995 through 2015. This retrospective study reviewed the records of a cohort of 1078 patients with SS, SC, Sβ0 and Sβ + thalassemia sickle cell syndromes and collected data about the type of blood products, indications, modalities, and acute transfusion complications...
November 1, 2017: Médecine et Santé Tropicales
https://www.readbyqxmd.com/read/29313505/effectiveness-safety-and-cost-of-partial-exchange-transfusions-in-patients-with-sickle-cell-anemia-at-a-sickle-cell-disease-center-in-sub-saharan-africa
#19
P Boma Muteb, J F J Kaluila Mamba, P Muhau Pfutila, V Bilo, J D Panda Mulefu, D A Diallo
The partial exchange transfusions necessary for management of some sickle-cell complications raise the issue of effectiveness in the context of limited resources and inadequate blood safety. This study evaluated the effectiveness, safety, and cost of partial exchange transfusions in 39 patients with sickle-cell anemia in Lubumbashi, looking at the patients' age and gender and the tolerability and direct cost of the transfusions. Excel and SPSS 18 were used for data entry and analysis. Chi2 and Fisher exact tests were used for comparisons...
November 1, 2017: Médecine et Santé Tropicales
https://www.readbyqxmd.com/read/29311011/-screening-for-nephropathy-in-major-sickle-cell-syndromes-in-patients-monitored-at-the-national-reference-center-for-sickle-cell-disease-in-niamey-niger
#20
Moumouni Garba, Zeinabou Maiga Moussa Tondi, Hassan Diongoule, Samaila Aboubacar, Abdou Ide, Nadège Ruddy Biyao-Nelson, Illiassou Soumaila, Soumana Alido
BACKGROUND: Sickle cell anemia is the most common hereditary hemopathy in the world. It is a disease that attacks all the systems of the organism. The kidneys are among the most sensitive organs of this disease. The main objective of this study is to detect sickle cell nephropathy in patients followed at the National Reference Center for Sickle Cell Disease in Niamey. METHODS: It is a prospective study carried out over a period of one year (January to December 2016)...
January 5, 2018: Néphrologie & Thérapeutique
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