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Blood Cells, Molecules & Diseases

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https://www.readbyqxmd.com/read/28918238/recent-advances-and-future-challenges-in-gaucher-disease
#1
EDITORIAL
Ari Zimran, Jeff Szer
No abstract text is available yet for this article.
September 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28918237/preface-to-the-special-issue-on-gaucher-disease-2017
#2
EDITORIAL
Ari Zimran, Jeff Szer
No abstract text is available yet for this article.
September 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28927633/the-use-of-mri-to-monitor-iron-overload-in-scd
#3
John C Wood
No abstract text is available yet for this article.
September 6, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28911832/association-of-silent-infarcts-in-sickle-cell-anemia-with-decreased-annexin-a5-resistance
#4
Kerry A Morrone, Lydia H Pecker, Jacob Rand, Jennifer Davila, Suzette Oyeku, Jane A Little, Xue Xiaonan, Deepa Manwani
BACKGROUND: Sickle cell anemia (SCA) is characterized by abnormally shaped, adhesive RBCs that interact with white blood cells and the endothelium, leading to chronic hemolysis, vasculopathy and a prothrombotic state. About 10% of subjects with a thrombotic event in the general population will have an associated antiphospholipid (aPL) antibody. One proposed mechanism for the thrombophilic nature of aPL antibodies is the disruption of the potent anticoagulant annexin A5 or Annexin A5 resistance (A5R)...
September 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28882478/corrigendum-to-the-expression-of-death-inducer-obliterator-dido-variants-in-myeloproliferative-neoplasms-blood-cells-mol-dis-59-2016-25-30
#5
Maria Gabriela Berzoti-Coelho, Aline Fernanda Ferreira, Natalia de Souza Nunes, Mariana Tomazini Pinto, Maurício Cristiano Rocha Júnior, Belinda Pinto Simões, Carlos Martínez-A, Elizabeth Xisto Souto, Rodrigo Alexandre Panepucci, Dimas Tadeu Covas, Simone Kashima, Fabíola Attié Castro
No abstract text is available yet for this article.
September 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28893518/curative-approaches-for-sickle-cell-disease-a-review-of-allogeneic-and-autologous-strategies
#6
Daniel E Bauer, Christian Brendel, Courtney D Fitzhugh
Despite sickle cell disease (SCD) first being reported >100years ago and molecularly characterized >50years ago, patients continue to experience severe morbidity and early mortality. Although there have been substantial clinical advances with immunizations, penicillin prophylaxis, hydroxyurea treatment, and transfusion therapy, the only cure that can be offered is hematopoietic stem cell transplantation (HSCT). In this work, we summarize the various allogeneic curative approaches reported to date and discuss open and upcoming clinical research protocols...
September 1, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28822622/novel-in-vivo-and-in-vitro-techniques-to-image-and-model-the-cerebral-vasculature-in-sickle-cell-disease
#7
Erin M Buckley, Manu O Platt, Wilbur A Lam
No abstract text is available yet for this article.
August 12, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28865304/hypocoagulability-as-assessed-by-thrombin-generation-test-in-newly-diagnosed-patients-with-multiple-myeloma
#8
LETTER
Pauline Legendre, Emma Verstraete, Michèle Martin, Amélie Poinsard, Aurore Perrot, Cyrille Hulin, Gilbert Faure, Véronique Latger-Cannard, Julien Perrin
No abstract text is available yet for this article.
August 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28822917/coexistence-of-aberrant-hematopoietic-and-stromal-elements-in-myelodysplastic-syndromes
#9
Salar Abbas, Archana Kini, Vivi M Srivastava, Marie Therese M, Sukesh C Nair, Aby Abraham, Vikram Mathews, Biju George, Sanjay Kumar, Aparna Venkatraman, Alok Srivastava
Myelodysplastic syndromes (MDS) are a group of clonal hematopoietic disorders related to hematopoietic stem and progenitor cell dysfunction. Several studies have shown the role of the bone marrow microenvironment in regulating hematopoietic stem, and progenitor function and their individual abnormalities have been associated with disease pathogenesis. In this study, we simultaneously evaluated hematopoietic stem cells (HSC), hematopoietic stem progenitor cells (HSPCs) and different stromal elements in a cohort of patients with MDS-refractory cytopenia with multilineage dysplasia (RCMD)...
August 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28811072/evolution-of-hemoglobin-loci-and-their-regulatory-elements
#10
REVIEW
Sjaak Philipsen, Ross C Hardison
Across the expanse of vertebrate evolution, each species produces multiple forms of hemoglobin in erythroid cells at appropriate times and in the proper amounts. The multiple hemoglobins are encoded in two globin gene clusters in almost all species. One globin gene cluster, linked to the gene NPRL3, is preserved in all vertebrates, including a gene cluster encoding the highly divergent globins from jawless vertebrates. This preservation of synteny may reflect the presence of a powerful enhancer of globin gene expression in the NPRL3 gene...
August 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28807656/opportunities-for-model-based-precision-dosing-in-the-treatment-of-sickle-cell-anemia
#11
Min Dong, Tomoyuki Mizuno, Alexander A Vinks
Hydroxyurea is the primary pharmacotherapy to prevent complications of sickle cell anemia (SCA). Accumulated clinical experience across multiple age ranges has suggested that the use of an individualized maximum tolerated dose (MTD) will achieve optimal benefit of hydroxyurea treatment. However, the current empirical and trial-and-error approach for dose escalation often results in a lengthy titration process and is not strictly implemented in many clinics. Opportunities exist for pharmacokinetics model-based precision dosing of hydroxyurea to quickly achieve individual MTD...
August 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28847416/hydroxyurea-analytical-techniques-and-quantitative-analysis
#12
Anu Marahatta, Russell E Ware
Hydroxyurea is a potent disease-modifying therapeutic agent with efficacy for the treatment of sickle cell anemia. When administered at once-daily oral doses that lead to mild marrow suppression, hydroxyurea leads to substantial and sustained fetal hemoglobin induction, which effectively inhibits erythrocyte sickling. When escalated to maximum tolerated dose, hydroxyurea has proven laboratory and clinical effects for both children and adults with sickle cell anemia. However, there is substantial inter-patient variability with regard to the optimal dosing regimen, as well as differences in treatment-related toxicities and responses that may be explained by hydroxyurea pharmacokinetics and pharmacogenetics...
August 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28844459/the-pressing-need-for-point-of-care-diagnostics-for-sickle-cell-disease-a-review-of-current-and-future-technologies
#13
Patrick T McGann, Carolyn Hoppe
Sickle cell disease (SCD) is a common and life threatening inherited blood disorder, affecting over 300,000 newborns per year. Over 75% of SCD births occur in sub-Saharan Africa, where the lack of timely and accurate diagnosis results in premature death within the first few years of life for a majority of affected infants. Current methods to diagnosis SCD require expensive laboratory equipment and reagents, and adequately trained laboratory personnel. In addition, test results are often delayed due to transport and batching of samples in a central laboratory...
August 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28827079/application-of-genomics-for-transfusion-therapy-in-sickle-cell-anemia
#14
Stella T Chou, Connie M Westhoff
The application of genomic approaches is impacting all areas of laboratory testing including transfusion medicine. Use of DNA-based test methods is particularly applicable for red cell and platelet antigen typing as the majority of genes encoding the carrier proteins and carbohydrates are now known and were cloned in the 1990's. Many of the antigenic polymorphisms are due to single nucleotide changes (SNP's) in the respective genes and DNA-arrays that target these changes have been validated by comparison with conventional serologic typing...
August 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28818577/applications-of-cardiac-magnetic-resonance-imaging-in-sickle-cell-disease
#15
Omar Niss, Michael D Taylor
Cardiac magnetic resonance imaging (CMR) has evolved from an effective research tool to a non-invasive clinical modality with versatile applications. The accuracy of volume measurements and functional assessment and the ability to identify unique myocardial tissue characteristics non-invasively are the primary advantages of CMR. The use of CMR in sickle cell disease (SCD) has been limited clinically to myocardial iron assessment. The use of other CMR applications to characterize the cardiac pathology in SCD is slowly emerging but remains limited to research level...
August 8, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28810169/association-of-genetic-polymorphisms-with-plasma-tfpi-level-boon-or-curse-for-dvt-patients-study-from-india
#16
Kishor Kamal, Sharma Amit, Singh Kanwaljeet, Ranjan Ravi, Pandey Hareram, Kumar Ravi, Kamal Vineet Kumar, Mishra Pravas, Saxena Renu
Low plasma TFPI levels have been associated with an increased risk of DVT; however its association with TFPI gene polymorphisms is controversial and not yet studied in India. The aim of our study was to analyze prevalence of TFPI gene polymorphisms, evaluate their effects on its plasma levels and determine its association with DVT. Plasma level and genetic polymorphisms (33T>C, 399C>T and 536C>T) of TFPI were screened in subjects (100 DVT patients and 100 controls). Mean TFPI level in patients was significantly lower than controls (Patients: 33...
August 6, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28806577/prevalence-and-predictors-of-cardiac-and-liver-iron-overload-in-patients-with-thalassemia-a-multicenter-study-based-on-real-world-data
#17
Rungroj Krittayaphong, Vip Viprakasit, Pairash Saiviroonporn, Noppadol Siritanaratkul, Suvipaporn Siripornpitak, Arunotai Meekaewkunchorn, Thawatchai Kirawittaya, Pornpun Sripornsawan, Arunee Jetsrisuparb, Jiraporn Srinakarin, Peerapon Wong, Nuttaporntira Phalakornkul, Phakatip Sinlapamongkolkul, John Wood
Prevalence of cardiac and liver iron overload in patients with thalassemia in real-world practice may vary among different regions especially in the era of widely-used iron chelation therapy. The aim of this study was to determine the prevalence of cardiac and liver iron overload in and the management patterns of patients with thalassemia in real-world practice in Thailand. We established a multicenter registry for patients with thalassemia who underwent magnetic resonance imaging (MRI) as part of their clinical evaluation...
August 5, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28783617/an-age-dependent-response-to-hydroxyurea-in-pediatric-sickle-cell-anemia-patients-with-alpha-thalassemia-trait
#18
Lisa Figueiredo, Kerry Morrone, Catherine Wei, Karen Ireland, Hillel W Cohen, Catherine Driscoll, Deepa Manwani
Hydroxyurea (HU) is a key drug therapy for individuals with sickle cell anemia (SCA), yet its clinical and hematologic responses can be variable. Various studies have reported the role of α-thalassemia as one of the most prevalent heritable traits that may modify HU response. We provide data from 62 pediatric and adolescent patients with SCA, 26 with co-inherited α-thalassemia trait. Our data suggest that altered hematologic and clinical responses to HU therapy are noted in adolescent SCA individuals with co-inherited α-thalassemia trait...
July 31, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28772256/non-syndromic-childhood-onset-congenital-sideroblastic-anemia-a-report-of-13-patients-identified-with-an-alas2-or-slc25a38-mutation
#19
Marie-Amelyne Le Rouzic, Cyrielle Fouquet, Thierry Leblanc, Mohamed Touati, Fanny Fouyssac, Christiane Vermylen, Nadja Jäkel, Jean-François Guichard, Karim Maloum, Fabienne Toutain, Patrick Lutz, Yves Perel, Hana Manceau, Caroline Kannengiesser, Jean-Pierre Vannier
The most frequent germline mutations responsible for non syndromic congenital sideroblastic anemia are identified in ALAS2 and SLC25A38 genes. Iron overload is a key issue and optimal chelation therapy should be used to limit its adverse effects on the development of children. Our multicentre retrospective descriptive study compared the strategies for diagnosis and management of congenital sideroblastic anemia during the follow-up of six patients with an ALAS2 mutation and seven patients with an SLC25A38 mutation...
July 26, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28756180/does-novel-p5-n-1-mutation-in-combination-with-g6pd-asahi-in-an-indian-male-contribute-to-budd-chiari-syndrome
#20
LETTER
Prashant Warang, Rati Devendra, Ashish Chiddarwar, Vinod Gupta, Darshana Mirgal, Anshul Jadli, Aniket Mohite, Prabhakar Kedar, Malay Mukherjee
No abstract text is available yet for this article.
July 20, 2017: Blood Cells, Molecules & Diseases
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