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Sickle cell disease

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https://www.readbyqxmd.com/read/27913540/sickle-cell-disease-an-inherited-thrombophilia
#1
Ted Wun, Ann Brunson
Activation of the hemostatic system occurs in patients with sickle cell disease. The extent to which this activation contributes to sickle cell pathophysiology is uncertain. Clinical trials of anticoagulants or platelet inhibitors have demonstrated the ability to decrease biomarkers of hemostatic activation, but this has generally not resulted in improvement in clinically relevant outcomes. Venous thromboembolism (VTE: deep venous thrombosis and pulmonary embolism) has been until recently an underappreciated complication of sickle cell disease, with incident event and recurrence rates consistent with a strong thrombophilia...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913539/prevention-of-central-nervous-system-sequelae-in-sickle-cell-disease-without-evidence-from-randomized-controlled-trials-the-case-for-a-team-based-learning-collaborative
#2
Michael R DeBaun, Allison A King
Since 1998, the National Institutes of Health has funded 5 randomized controlled trials (RCTs) for primary and secondary prevention of strokes in children with sickle cell anemia (SCA). In a systematic fashion, these trials have significantly advanced the care of children with SCA. In the absence of an RCT, clinicians are often compelled to make decisions at the bedside, based on experience, observational studies, and principles of hematology. We will provide an initial example that describes how a team-based, learning collaborative developed a multisite standard care protocol with a low budget (<$10 000 per year) to overcome the intrinsic limitations of advancing the care of neurologic complications in sickle cell disease (SCD)...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913538/sickle-cell-disease-when-and-how-to-transfuse
#3
Jo Howard
Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome, comes from observational data...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913516/immunoregulatory-networks-in-sickle-cell-alloimmunization
#4
Karina Yazdanbakhsh
Red blood cell (RBC) transfusions are critical for treatment and prevention of complications of sickle cell disease (SCD), and most SCD patients will receive 1 or more transfusions by age 20. However, SCD alloimmunization remains a serious complication of transfusions that can lead to life-threatening acute and delayed transfusion reactions. Alloimmunization rates are higher in SCD patients most likely due to RBC antigenic differences between largely white donors vs mainly African-American recipients and frequency of transfusions...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913515/cellular-immune-responses-in-red-blood-cell-alloimmunization
#5
James C Zimring, Krystalyn E Hudson
In excess of 340 blood group antigens have now been described that vary between individuals. Thus, any unit of blood that is nonautologous represents a significant dose of alloantigen. Most blood group antigens are proteins, which differ by a single amino acid between donors and recipients. Approximately 1 out of every 70 individuals are transfused each year (in the United States alone), which leads to antibody responses to red blood cell (RBC) alloantigens in some transfusion recipients. When alloantibodies are formed, in many cases, RBCs expressing the antigen in question can no longer be safely transfused...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27909222/associations-between-environmental-factors-and-hospital-admissions-for-sickle-cell-disease
#6
Frédéric B Piel, Sanjay Tewari, Valentine Brousse, Antonis Analitis, Anna Font, Stephan Menzel, Subarna Chakravorty, Swee Lay Thein, Baba Inusa, Paul Telfer, Marianne de Montalembert, Gary W Fuller, Klea Katsouyanni, David C Rees
Sickle cell disease (SCD) is an increasing global health burden. This inherited disease is characterised by a remarkable phenotypic heterogeneity, which can only partly be explained by genetic factors. Environmental factors are likely to play an important role but studies of their impact on disease severity are limited and their results are often inconsistent. This study investigated associations between a range of environmental factors and hospital admissions of young patients with SCD in London and in Paris between 2008 and 2012...
December 1, 2016: Haematologica
https://www.readbyqxmd.com/read/27906010/sickle-cell-vaso-occlusive-crisis-it-s-a-gut-feeling
#7
Seah H Lim, Loren Fast, Alison Morris
Insights in the pathogenesis of vaso-occlusive crisis in patients with sickle cell disease have changed significantly in the last decade. Various laboratory and clinical evidence have provided support to the pivotal role of activated neutrophils in this process. A recent study in murine sickle cell disease indicated that the intestinal microbiota is responsible for regulating the number of aged neutrophils, a subset of neutrophils that are overly activated. Reduction of these neutrophils in vivo protected the mice from fatal TNFα-induced vaso-occlusive crisis...
December 1, 2016: Journal of Translational Medicine
https://www.readbyqxmd.com/read/27905689/development-of-quality-indicators-for-transition-from-pediatric-to-adult-care-in-sickle-cell-disease-a-modified-delphi-survey-of-adult-providers
#8
Amy E Sobota, Nishita Shah, Jennifer W Mack
BACKGROUND: Transition from pediatric to adult care is a vulnerable time for young adults with sickle cell disease (SCD); however, improvements in transition are limited by a lack of quality indicators. The purpose of this study was to establish quality indicators for transition in SCD and to determine the optimal timing between the final pediatric visit and the first adult provider visit. PROCEDURE: We conducted a modified Delphi survey to reach a consensus on which quality indicators are most important for a successful transition...
December 1, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27905100/the-severity-of-anaemia-depletes-cerebrovascular-dilatory-reserve-in-children-with-sickle-cell-disease-a-quantitative-magnetic-resonance-imaging-study
#9
Przemyslaw D Kosinski, Paula L Croal, Jackie Leung, Suzan Williams, Isaac Odame, Gregory M T Hare, Manohar Shroff, Andrea Kassner
Overt ischaemic stroke is one of the most devastating complications in children with sickle cell disease (SCD). The compensatory response to anaemia in SCD includes an increase in cerebral blood flow (CBF) by accessing cerebrovascular dilatory reserve. Exhaustion of dilatory reserve secondary to anaemic stress may lead to cerebral ischaemia. The purpose of this study was to investigate CBF and cerebrovascular reactivity (CVR) using magnetic resonance imaging (MRI) in children with SCD and to correlate these with haematological markers of anaemia...
December 1, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27902670/ketamine-infusion-for-pain-control-in-acute-pediatric-sickle-cell-painful-crises
#10
Jonathan M Hagedorn, Evelyn C Monico
OBJECTIVES: Sickle cell disease is the most common hemoglobin variant in the world and can present with recurrent vaso-occlusive painful crises. Pain control in these patients is often difficult and requires a multimodal approach. In patients with uncontrolled pain after traditional pain control methods, ketamine infusions have proven beneficial. Unfortunately, no source alone had described ketamine infusions for pediatric sickle cell patients. Our objective was to provide a thorough definitive reference for health care providers regarding the use of ketamine infusion for pain control in pediatric sickle cell painful crises...
November 29, 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27900941/the-kidney-in-sickle-hemoglobinopathies%C3%A2
#11
Medha Airy, Garabed Eknoyan
With improvements in the care of patients with sickle hemoglobinopathies, sickle cell disease (SCD) has evolved from a disease that was fatal in childhood into one in which most survive past their 5th decade and some into old age. As a result, the renal complications of sickle hemoglobinopathies, which are age dependent, have emerged as a common and serious complication of SCD. Approximately 14 - 18% of mortality in SCD is attributed to chronic kidney disease (CKD), which develops in 1/3 of individuals with SCD and progresses to end-stage renal disease in 4 - 18% of them...
November 30, 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27896936/health-related-quality-of-life-and-adherence-to-hydroxyurea-in-adolescents-and-young-adults-with-sickle-cell-disease
#12
Sherif M Badawy, Alexis A Thompson, Jin-Shei Lai, Frank J Penedo, Karen Rychlik, Robert I Liem
BACKGROUND: Complications related to sickle cell disease (SCD) result in significant declines in health-related quality of life (HRQOL). While hydroxyurea reduces SCD complications, adherence remains suboptimal. The study's objectives were to assess the feasibility of Internet-based electronic assessment of HRQOL in SCD clinic and to examine the relationship between HRQOL and hydroxyurea adherence in adolescents and young adults (AYAs) with SCD. PROCEDURE: A cross-sectional survey was administered on tablets to 34 AYAs (12-22 years old) in a SCD clinic from January through December 2015...
November 28, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27895843/severe-anemia-in-malawian-children
#13
Job Cj Calis, Kamija S Phiri, E Brian Faragher, Bernard J Brabin, Imelda Bates, Luis E Cuevas, Rob J de Haan, Ajib I Phiri, Pelani Malange, Mirriam Khoka, Paul Jm Hulshof, Lisette van Lieshout, Marcel Ghm Beld, Yik Y Teo, Kirk A Rockett, Anna Richardson, Dominic P Kwiatkowski, Malcolm E Molyneux, Michaël Boele van Hensbroek
BACKGROUND: Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. METHODS: We conducted a case-control study of 381 preschool children with severe anemia (hemoglobin concentration, <5.0 g per deciliter) and 757 preschool children without severe anemia in urban and rural settings in Malawi. Causal factors previously associated with severe anemia were studied. The data were examined by multivariate analysis and structural equation modeling...
September 2016: Malawi Medical Journal: the Journal of Medical Association of Malawi
https://www.readbyqxmd.com/read/27891535/gene-therapy-for-hemoglobin-disorders-a-mini-review
#14
Parul Rai, Punam Malik
Gene therapy by either gene insertion or editing is an exciting curative therapeutic option for monogenic hemoglobin disorders like sickle cell disease and β-thalassemia. The safety and efficacy of gene transfer techniques has markedly improved with the use of lentivirus vectors. The clinical translation of this technology has met with good success, although key limitations include number of engraftable transduced hematopoietic stem cells and adequate transgene expression that results in complete correction of β0 thalassemia major...
2016: Journal of Rare Diseases Research & Treatment
https://www.readbyqxmd.com/read/27891273/orbital-infarction-due-to-sickle-cell-disease-without-orbital-pain
#15
Cameron L McBride, Kim-Binh T Mai, Kartik S Kumar
Sickle cell disease is a hemoglobinopathy that results in paroxysmal arteriolar occlusion and tissue infarction that can manifest in a plurality of tissues. Rarely, these infarcted crises manifest in the bony orbit. Orbital infarction usually presents with acute onset of periorbital tenderness, swelling, erythema, and pain. Soft tissue swelling can result in proptosis and attenuation of extraocular movements. Expedient diagnosis of sickle cell orbital infarction is crucial because this is a potentially sight-threatening entity...
2016: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/27887664/translating-sickle-cell-guidelines-into-practice-for-primary-care-providers-with-project-echo
#16
Lisa M Shook, Christina B Farrell, Karen A Kalinyak, Stephen C Nelson, Brandon M Hardesty, Angeli G Rampersad, Kay L Saving, Wanda J Whitten-Shurney, Julie A Panepinto, Russell E Ware, Lori E Crosby
BACKGROUND: Approximately 100,000 persons with sickle cell disease (SCD) live in the United States, including 15,000 in the Midwest. Unfortunately, many patients experience poor health outcomes due to limited access to primary care providers (PCPs) who are prepared to deliver evidence-based SCD care. Sickle Treatment and Outcomes Research in the Midwest (STORM) is a regional network established to improve care and outcomes for individuals with SCD living in Indiana, Illinois, Michigan, Minnesota, Ohio, and Wisconsin...
2016: Medical Education Online
https://www.readbyqxmd.com/read/27885941/gstt1-rs4025935-null-genotype-is-associated-with-increased-risk-of-sickle-cell-disease-in-the-populations-of-tabuk-northwestern-region-of-saudi-arabia
#17
Faisel Abu-Duhier, Rashid Mir
BACKGROUND: Glutathione system plays an important role in the protection of cells and tissue against damage from oxidative stress. Impairment of the glutathione system due to genetic polymorphism of GST genes may increase the risk and severity of sickle cell disease (SCD). Present study was, therefore, undertaken to examine the relative impact of the genetic polymorphism of GSTT1 and GSTM1 (rs4025935 and rs71748309) on susceptibility and hematological aspects of the patients with SCD...
November 25, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27884835/sickle-cell-disease-the-price-of-cure
#18
Irene Roberts, Josu de la Fuente
No abstract text is available yet for this article.
November 24, 2016: Blood
https://www.readbyqxmd.com/read/27883292/il1a-rs1800587-associates-with-chronic-noncrisis-pain-in-sickle-cell-disease
#19
Xiaoyu Hu, Ellie H Jhun, Yingwei Yao, Ying He, Robert E Molokie, Diana J Wilkie, Zaijie J Wang
AIM: Pain is prevalent in sickle cell disease (SCD) patients who display great heterogeneity in pain severity and frequency. Hypothesizing that inflammatory factors are involved in the pathogenesis of SCD pain, we focused on the IL1A C/T polymorphism rs1800587 that is an SNP located in a cis-transcriptional regulatory region. METHODS: We genotyped IL1A rs1800587 and performed association studies with phenotype data obtained by a multidimensional pain assessment tool using the PAINReportIt(®) Questionnaire...
November 24, 2016: Pharmacogenomics
https://www.readbyqxmd.com/read/27880985/daily-pain-in-adults-with-sickle-cell-disease-a-different-perspective
#20
Charlotte F J van Tuijn, Joep W R Sins, Karin Fijnvandraat, Bart J Biemond
Previous reports demonstrated that patients with sickle cell disease (SCD) experience pain on more than half of the observed days. Yet, these high incidences do not seem to match observations in our population. In this prospective cohort study we aimed to assess the frequency and characteristics of daily, self-reported pain among adult SCD patients in the Netherlands. Consecutive patients were enrolled during routine outpatient visits and followed up to 6 months. A total of 55 patients completed 5 982 diary observation days...
November 23, 2016: American Journal of Hematology
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