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https://www.readbyqxmd.com/read/29668544/splenectomy-to-optimize-hemoglobin-s-control-in-children-with-sickle-cell-anemia-on-chronic-transfusion-therapy-for-stroke-prevention
#1
L Vandy Black, Jolie S Ramesar, Catherine H Boston
Chronic transfusion therapy with the goal of maintaining a hemoglobin (Hb) S <30% is the primary recommended treatment for children with sickle cell anemia and a history of overt stroke or abnormal transcranial Doppler examination. We report chronic hypersplenism as a cause of poor HbS% control in 3 children on chronic transfusion therapy for stroke prevention. Splenectomy resulted in a 39.77% (95% confidence interval, 34.3-45.3, P<0.0001) mean reduction in HbS% with no perioperative or infectious complications suggesting the need for additional research into splenectomy as a therapeutic option for select high-risk children to optimize transfusion therapy for stroke prevention...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29666400/the-corticosteroid-compounds-prednisolone-and-vamorolone-do-not-alter-the-nociception-phenotype-and-exacerbate-liver-injury-in-sickle-cell-mice
#2
Luis E F Almeida, Jesse M Damsker, Sarah Albani, Nina Afsar, Sayuri Kamimura, Drew Pratt, David E Kleiner, Martha Quezado, Heather Gordish-Dressman, Zenaide M N Quezado
Clinicians often hesitate prescribing corticosteroids to treat corticosteroid-responsive conditions in sickle cell disease (SCD) patients because their use can be associated with complications (increased hospital readmission, rebound pain, strokes, avascular necrosis, acute chest syndrome). Consequently, SCD patients may receive suboptimal treatment for corticosteroid-responsive conditions. We conducted a preclinical trial of dissociative (vamorolone) and conventional (prednisolone) corticosteroid compounds to evaluate their effects on nociception phenotype, inflammation, and organ dysfunction in SCD mice...
April 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29664857/restrictive-versus-liberal-strategy-for-red-blood-cell-transfusion-a-systematic-review-and-meta-analysis-in-orthopaedic-patients
#3
Wan-Jie Gu, Xiao-Ping Gu, Xiang-Dong Wu, Hao Chen, Joey S W Kwong, Lu-Yang Zhou, Shuo Chen, Zheng-Liang Ma
BACKGROUND: Current guidelines recommend restrictive criteria for red blood-cell transfusion in most clinical settings. However, patients undergoing orthopaedic surgery may require distinct transfusion criteria since benefits and potential harm often vary considerably based on patient characteristics and surgical procedures. We aimed to assess the efficacy and safety of restrictive transfusion in patients undergoing orthopaedic surgery, especially in important subgroups. METHODS: Electronic databases were searched to identify randomized controlled trials investigating restrictive (mostly a hemoglobin level of 8...
April 18, 2018: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/29653202/clinicopathological-feature-and-outcome-of-pythiosis
#4
Maria Nina Chitasombat, Noppadol Larbcharoensub, Ariya Chindamporn, Theerapong Krajaejun
OBJECTIVES: Vascular pythiosis is a life-threatening infection, caused by Pythium insidiosum. We presented the clinical presentation, serodiagnosis, pathology, and outcome from our institution. METHODS: We retrospectively analyzed patients with proven vascular pythiosis at Ramathibodi Hospital, Mahidol University, Bangkok, Thailand from January 2006 to December 2016. RESULTS: Thirteen patients were analyzed, eight thalassemias. Five had aplastic anemia, myelodysplasia, acute leukemia, cirrhosis, and alcoholism...
April 10, 2018: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/29628940/moderate-to-severe-anemia-is-associated-with-poor-functional-outcome-in-acute-stroke-patients-treated-with-mechanical-thrombectomy
#5
Cetin Kursad Akpinar, Erdem Gurkas, Emrah Aytac
Background: Anemia will negatively affect cerebral collaterals and penumbra. Eventually, it may cause worse clinical outcomes and even increase mortality rates in stroke patients. Anemia has recently been suggested to be an independent risk factor for ischemic stroke. Therefore, we aimed to investigate the effects of the presence of anemia on clinical outcomes in ischemic stroke patients undergoing mechanical thrombectomy. Methods: This was a retrospective study involving the prospectively and consecutively collected data of 90 adult patients between January 2015 and August 2016...
February 2018: Interventional Neurology
https://www.readbyqxmd.com/read/29614631/simple-chronic-transfusion-therapy-a-crucial-therapeutic-option-for-sickle-cell-disease-improves-but-does-not-normalize-blood-rheology-what-should-be-our-goals-for-transfusion-therapy
#6
Jon A Detterich
Sickle cell anemia is characterized by a mutation resulting in the formation of an abnormal beta-hemoglobin called hemoglobin S. Hemoglobin S polymerizes upon deoxygenation, causing impaired red blood cell deformability and increased blood viscosity at equivalent hematocrits. Thus, sickle cell disease is a hemorheologic disease that results in various pathologic processes involving multiple organ systems including the lungs, heart, kidneys and brain. Red blood cell mechanics and the perturbations on blood flow-endothelial interaction underlie much of the pathology found in sickle cell disease...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29606044/impact-of-preoperative-endovascular-embolization-on-immediate-meningioma-resection-outcomes
#7
Michael G Brandel, Robert C Rennert, Arvin R Wali, David R Santiago-Dieppa, Jeffrey A Steinberg, Christian Lopez Ramos, Peter Abraham, J Scott Pannell, Alexander A Khalessi
OBJECTIVE Preoperative embolization of meningiomas can facilitate their resection when they are difficult to remove. The optimal use and timing of such a procedure remains controversial given the risk of embolization-linked morbidity in select clinical settings. In this work, the authors used a large national database to study the impact of immediate preoperative embolization on the immediate outcomes of meningioma resection. METHODS Meningioma patients who had undergone elective resection were identified in the National (Nationwide) Inpatient Sample (NIS) for the period 2002-2014...
April 2018: Neurosurgical Focus
https://www.readbyqxmd.com/read/29593859/a-threshold-trajectory-was-revealed-by-isolating-the-effects-of-hemoglobin-rate-of-rise-in-anemia-of-chronic-kidney-disease
#8
Gregory Fusco, Ali Hariri, Carlos Vallarino, Ajay Singh, Peter Yu, Lesley Wise
Background: To assess cardiovascular risk among various hemoglobin (Hb) rates of rise (RoR) in chronic kidney disease (CKD) patients with anemia who have initiated therapy with erythropoiesis stimulating agents (ESAs). Methods: Observational cohort of CKD patients initiating ESA therapy from the Centricity® database, 1990-2011. Proportional hazards models tested the hypothesis that a slower Hb RoR (0 < g/dl/month ⩽ 0.125) is associated with a lower cardiovascular (CV) incidence [composite of fatal/nonfatal myocardial infarction (MI) and stroke] compared with faster RoR (0...
October 2017: Therapeutic Advances in Drug Safety
https://www.readbyqxmd.com/read/29590102/common-%C3%AE-globin-variants-modify-hematologic-and-other-clinical-phenotypes-in-sickle-cell-trait-and-disease
#9
Laura M Raffield, Jacob C Ulirsch, Rakhi P Naik, Samuel Lessard, Robert E Handsaker, Deepti Jain, Hyun M Kang, Nathan Pankratz, Paul L Auer, Erik L Bao, Joshua D Smith, Leslie A Lange, Ethan M Lange, Yun Li, Timothy A Thornton, Bessie A Young, Goncalo R Abecasis, Cathy C Laurie, Deborah A Nickerson, Steven A McCarroll, Adolfo Correa, James G Wilson, Guillaume Lettre, Vijay G Sankaran, Alex P Reiner
Co-inheritance of α-thalassemia has a significant protective effect on the severity of complications of sickle cell disease (SCD), including stroke. However, little information exists on the association and interactions for the common African ancestral α-thalassemia mutation (-α3.7 deletion) and β-globin traits (HbS trait [SCT] and HbC trait) on important clinical phenotypes such as red blood cell parameters, anemia, and chronic kidney disease (CKD). In a community-based cohort of 2,916 African Americans from the Jackson Heart Study, we confirmed the expected associations between SCT, HbC trait, and the -α3...
March 28, 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29566812/transcatheter-aortic-valve-replacement-in-patients-with-low-flow-low-gradient-aortic-stenosis-the-topas-tavi-registry
#10
Henrique Barbosa Ribeiro, Stamatios Lerakis, Martine Gilard, João L Cavalcante, Raj Makkar, Howard C Herrmann, Stephan Windecker, Maurice Enriquez-Sarano, Asim N Cheema, Luis Nombela-Franco, Ignacio Amat-Santos, Antonio J Muñoz-García, Bruno Garcia Del Blanco, Alan Zajarias, John C Lisko, Salim Hayek, Vasilis Babaliaros, Florent Le Ven, Thomas G Gleason, Tarun Chakravarty, Wilson Y Szeto, Marie-Annick Clavel, Alberto de Agustin, Vicenç Serra, John T Schindler, Abdellaziz Dahou, Rishi Puri, Emilie Pelletier-Beaumont, Melanie Côté, Philippe Pibarot, Josep Rodés-Cabau
BACKGROUND: Few data exist on patients with low-flow, low-gradient aortic stenosis (LFLG-AS) undergoing transcatheter aortic valve replacement (TAVR). Also, very scarce data exist on the usefulness of dobutamine stress echocardiography (DSE) before TAVR in these patients. OBJECTIVES: The authors sought to evaluate clinical outcomes and changes in left ventricular ejection fraction (LVEF) following TAVR in patients with classical LFLG-AS. METHODS: This multicenter registry included 287 patients with LFLG-AS undergoing TAVR...
March 27, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29545349/proteomic-analysis-of-plasma-from-children-with-sickle-cell-anemia-and-silent-cerebral-infarction
#11
Sanjay Tewari, George Renney, John Brewin, Kate Gardner, Fenella Kirkham, Baba Inusa, James E Barrett, Stephan Menzel, Swee Lay Thein, Malcolm Ward, David C Rees
Silent cerebral infarction is the commonest neurological abnormality in children with sickle cell anemia, affecting 30-40% 14 year olds. There are no known biomarkers to identify children with silent cerebral infarcts and the pathological basis is also unknown. We used an unbiased proteomic discovery approach to identify plasma proteins differing in concentration between children with and without silent cerebral infarcts. Clinical parameters and plasma samples were analysed from 51 children (mean age 11.8 years, range 6-18) with sickle cell anemia (HbSS)...
March 15, 2018: Haematologica
https://www.readbyqxmd.com/read/29520844/children-with-sickle-cell-anemia-with-normal-tcd-and-without-silent-infarcts-have-a-low-incidence-of-new-strokes
#12
Lori C Jordan, Dionna O Roberts Williams, Mark J Rodeghier, Brittany Covert, Maria R Ponisio, James F Casella, Robert C McKinstry, Michael J Noetzel, Fenella J Kirkham, Emily R Meier, Beng Fuh, Melissa McNaull, Sharada Sarnaik, Suvankar Majumdar, Timothy L McCavit, Michael R DeBaun
In a prospective cohort study, we tested the hypothesis that children with sickle cell anemia (SCA) with normal transcranial Doppler ultrasound (TCD) velocities and without silent cerebral infarcts (SCIs) would have a lower incidence rate of new neurological events (strokes, seizures or transient ischemic attacks) compared to children with normal TCD measurements and SCIs, not receiving regular blood transfusions. Non-randomized participants from the Silent Cerebral Infarct Transfusion (SIT) Trial who had screening magnetic resonance imaging (MRI) of the brain and normal TCD measurements were included...
March 9, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29519371/mortality-in-children-adolescents-and-adults-with-sickle-cell-anemia-in-rio-de-janeiro-brazil
#13
Clarisse Lopes de Castro Lobo, Emilia Matos do Nascimento, Leonardo José Carvalho de Jesus, Thiago Gotelip de Freitas, Jocemir Ronaldo Lugon, Samir K Ballas
OBJECTIVE: To determine the mortality rate of children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. METHODS: The number of deaths, the mortality rate and the causes of deaths in patients with sickle cell anemia who were treated and followed up at our institution for 15 years were determined and compared to data available for the Brazilian population. RESULTS: The overall number of deaths was 281 patients with a mortality rate of 16...
January 2018: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/29496702/unwinding-the-path-from-anemia-to-stroke
#14
John C Wood
No abstract text is available yet for this article.
March 1, 2018: Blood
https://www.readbyqxmd.com/read/29478940/decline-in-hemoglobin-during-hospitalization-may-be-associated-with-poor-outcome-in-acute-stroke-patients
#15
Arata Abe, Yuki Sakamoto, Yasuhiro Nishiyama, Satoshi Suda, Kentaro Suzuki, Junya Aoki, Kazumi Kimura
BACKGROUND AND PURPOSE: Anemia upon hospital admission is a known predictor of poor functional outcomes in patients with acute cerebral infarction. However, it remains unclear whether reductions in hemoglobin levels during hospitalization influence stroke outcomes. We investigated the association between in-hospital decline in hemoglobin and poor outcomes. MATERIALS AND METHODS: We retrospectively analyzed data from 480 consecutive patients who had experienced acute cerebral infarction and presented without anemia between January 2012 and March 2015...
February 22, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29477223/vitamin-b-12
#16
A David Smith, Martin J Warren, Helga Refsum
The biosynthesis of B12 , involving up to 30 different enzyme-mediated steps, only occurs in bacteria. Thus, most eukaryotes require an external source of B12 , and yet the vitamin appears to have only two functions in eukaryotes: as a cofactor for the enzymes methionine synthase and methylmalonylCoA mutase. These two functions are crucial for normal health in humans, and in particular, the formation of methionine is essential for providing methyl groups for over 100 methylation processes. Interference with the methionine synthase reaction not only depletes the body of methyl groups but also leads to the accumulation of homocysteine, a risk factor for many diseases...
2018: Advances in Food and Nutrition Research
https://www.readbyqxmd.com/read/29465435/clinical-manifestations-of-sickle-cell-disease-in-india-misconceptions-and-reality
#17
Dipty Jain, Dipika Mohanty
PURPOSE OF REVIEW: In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India. RECENT FINDINGS: The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the other and also among different communities of India...
February 19, 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29459495/aplastic-anemia-and-risk-of-incident-atrial-fibrillation-a-nationwide-cohort-study
#18
Wei-Syun Hu, Fung-Chang Sung, Cheng-Li Lin
BACKGROUND: This retrospective cohort study sought to follow up patients with aplastic anemia (AA) to evaluate their risk of developing atrial fibrillation (AF).Methods and Results:From the National Health Insurance Research Database of Taiwan, this study identified an AA cohort (n=3,921), a general population cohort (n=17,617,843) and a propensity score-matched none AA cohort (PSM non-AA cohort in brief, n=15,684) in 2000-2010. By the end of 2011, the incident AF was higher in the AA cohort than in the general population and PSM non-AA cohorts (8...
February 16, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29431886/impact-of-anemia-on-in-hospital-complications-after-ischemic-stroke
#19
C-C Wei, S-T Zhang, G Tan, S-H Zhang, M Liu
BACKGROUND AND PURPOSE: In-hospital complications after stroke represent barriers to optimal recovery and are even potentially life-threatening. Anemia is common in stroke patients and is related to poor outcome after stroke. Less is known, however, of the association of anemia with complications. We aimed to investigate the impact of anemia on a series of in-hospital complications after ischemic stroke. METHODS: Consecutive patients with ischemic stroke within 7 days were included...
February 12, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29411418/feasibility-trial-for-primary-stroke-prevention-in-children-with-sickle-cell-anemia-in-nigeria-spin-trial
#20
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
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