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https://www.readbyqxmd.com/read/29773497/indications-for-red-cell-transfusions-in-pediatric-patients
#1
REVIEW
Jill M Cholette, Suzie A Noronha, Jerard Seghatchian, Neil Blumberg
Red cell transfusions are amongst the most common therapeutic procedures in seriously ill children, particularly in the inpatient setting. This is despite the fact that there is no evidence base for most clinical settings, with the exception of patients with hemoglobinopathies, particularly thalassemia and sickle cell anemia. Obviously exsanguinating hemorrhage and life threatening anemia are urgent indications for which no other therapeutic approach is currently available. Most transfusions are, however, given prophylactically to prevent the complications of hypoxia or hemodynamic stability, based upon expert opinion and a faith in the oxygen carrying capacity and beneficial hemodynamic properties of transfused red cells...
May 10, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29628940/moderate-to-severe-anemia-is-associated-with-poor-functional-outcome-in-acute-stroke-patients-treated-with-mechanical-thrombectomy
#2
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/29545349/proteomic-analysis-of-plasma-from-children-with-sickle-cell-anemia-and-silent-cerebral-infarction
#3
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/29519371/mortality-in-children-adolescents-and-adults-with-sickle-cell-anemia-in-rio-de-janeiro-brazil
#4
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/29465435/clinical-manifestations-of-sickle-cell-disease-in-india-misconceptions-and-reality
#5
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...
May 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29379566/recurrent-stroke-in-a-child-with-trma-syndrome-and-slc19a2-gene-mutation
#6
Parvaneh Karimzadeh, Toktam Moosavian, Hamidreza Moosavian
Here we report a 5-month-old boy with thiamine Responsive Megaloblastic Anemia syndrome (TRMA syndrome) with several attacks of stroke, admitted to Mofid Children's Hospital, Tehran, Iran, in 2016. In addition to the cardinal clinical manifestations of the syndrome, other manifestations comprise thiamine-responsive megaloblastic anemia, diabetes mellitus, and sensor neural hearing loss. The patient showed the ischemic attack of stroke. Megaloblastic anemia and diabetes were diagnosed at 8 months and was successfully treated with vitamin and insulin prescription...
2018: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/29296825/disease-severity-and-slower-psychomotor-speed-in-adults-with-sickle-cell-disease
#7
Dana R Jorgensen, Andrea Metti, Meryl A Butters, Joseph M Mettenburg, Caterina Rosano, Enrico M Novelli
Psychomotor slowing is common in children with sickle cell disease (SCD), but little is known about its severity in adults. We conducted a cross-sectional study to quantify psychomotor speed, measured with the digit symbol substitution test (DSST), in relationship with disease severity in adults with SCD attending an outpatient clinic (n = 88, age 36.3 years). Genotype was used to group patients in "severe" (homozygous for hemoglobin S or compound heterozygous with β0 thalassemia) or "moderate" groups (compound heterozygous for HbS, with either HbC or β+ thalassemia)...
September 26, 2017: Blood Advances
https://www.readbyqxmd.com/read/29260615/preliminary-evidence-for-cerebral-capillary-shunting-in-adults-with-sickle-cell-anemia
#8
Meher R Juttukonda, Manus J Donahue, Larry T Davis, Melissa C Gindville, Chelsea A Lee, Niral J Patel, Adetola A Kassim, Sumit Pruthi, Jeroen Hendrikse, Lori C Jordan
Elevated flow velocities in adults with sickle cell anemia (SCA) may cause rapid erythrocyte transit through capillaries. This phenomenon could present as dural venous sinus hyperintensity on arterial spin labeling (ASL)-MRI and could be indicative of capillary shunting. Here, the prevalence of ASL venous hyperintensities and association with relevant physiology in adults with SCA was investigated. SCA ( n = 46) and age-matched control ( n = 16) volunteers were recruited for 3.0 T MRI. Pseudo-continuous ASL-MRI was acquired for cerebral blood flow (CBF) calculation and venous hyperintensity determination; venous signal intensity and a categorical venous score (three raters; 0 = no hyperintensity, 1 = focal hyperintensity, and 2 = diffuse hyperintensity) were recorded...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/29238644/impact-of-preoperative-anemia-on-outcomes-in-adults-undergoing-elective-posterior-cervical-fusion
#9
Kevin Phan, Alexander E Dunn, Jun S Kim, John Di Capua, Sulaiman Somani, Parth Kothari, Nathan J Lee, Joshua Xu, James E Dowdell, Samuel K Cho
Study Design: Retrospective analysis of prospectively collected data. Objectives: Few studies have investigated the role of preoperative anemia on postoperative outcomes of posterior cervical fusion. This study looked to investigate the potential relationship between preoperative anemia and postoperative outcomes following posterior cervical spine fusion. Methods: Data from patients undergoing elective posterior cervical fusions between 2005 and 2012 was collected from the American College of Surgeons National Surgical Quality Improvement Program database using inclusion/exclusion criteria...
December 2017: Global Spine Journal
https://www.readbyqxmd.com/read/29107441/real-life-experience-with-hydroxyurea-in-sickle-cell-disease-a-multicenter-study-in-a-cohort-of-patients-with-heterogeneous-descent
#10
Paolo Rigano, Lucia De Franceschi, Laura Sainati, Antonio Piga, Frédéric B Piel, Maria Domenica Cappellini, Carmelo Fidone, Nicoletta Masera, Giovanni Palazzi, Barbara Gianesin, Gian Luca Forni
We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5±15years, 51.4% males). Hydroxyurea median treatment duration was 7years (range: <1year to 29years) at a mean therapeutic dose of 18±4.7mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels...
March 2018: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29103427/neuroinflammation-in-ischemic-pediatric-stroke
#11
Maja Steinlin
Over the last decades, the importance of inflammatory processes in pediatric stroke have become increasingly evident. Ischemia launches a cascade of events: activation and inhibition of inflammation by a large network of cytokines, adhesion and small molecules, protease, and chemokines. There are major differences in the neonatal brain compared to adult brain, but developmental trajectories of the process during childhood are not yet well known. In neonatal stroke ischemia is the leading pathophysiology, but infectious and inflammatory processes have a significant input into the course and degree of tissue damage...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29034755/the-alliance-with-expanding-blood-volume-and-correcting-anemia-is-an-effective-therapeutic-measure-for-the-adult-anemia-patients-of-acute-cerebral-infarction
#12
Zunyu Ke, Yu Zhao, Chuanling Wang, Zhiyou Cai
Purpose/aim of the study: Various factors are believed to be involved in the etiology of cerebral infarction. Anemia has been shown to deteriorate in association with ischemic stroke. However, the exact clinical association between anemia and ischemic stroke still remains unclear. We evaluated the clinical features of adult anemia patients with acute cerebral infarction and seek a better treatment different from the other causes of cerebral infarction, and to provide a reference for the diagnosis and treatment of these anemia patients with acute cerebral infarction...
May 2018: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28931524/degree-of-anemia-indirect-markers-of-hemolysis-and-vascular-complications-of-sickle-cell-disease-in-africa
#13
Marie Dubert, Jacques Elion, Aissata Tolo, Dapa Aly Diallo, Saliou Diop, Ibrahima Diagne, Ibrahima Sanogo, Suzanne Belinga, Odette Guifo, Guillaume Wamba, Françoise Ngo Sack, Kouakou Boidy, Ismael Kamara, Youssouf Traore, Cheick Oumar Diakite, Valérie Gbonon, Blaise Felix Faye, Moussa Seck, Indou Deme Ly, David Chelo, Roland N'Guetta, Ibrahima Bara Diop, Bamba Gaye, Xavier Jouven, Brigitte Ranque
The hyperhemolysis paradigm that describes overlapping "hyperhemolytic-endothelial dysfunction" and "high hemoglobin-hyperviscous" subphenotypes of sickle cell disease (SCD) patients is based on North American studies. We performed a transversal study nested in the CADRE cohort to analyze the association between steady-state hemolysis and vascular complications of SCD among sub-Saharan African patients. In Mali, Cameroon, and Ivory Coast, 2407 SCD patients (1751 SS or sickle β-zero-thalassemia [Sβ0 ], 495 SC, and 161 sickle β+ -thalassemia [Sβ+ ]), aged 3 years old and over, were included at steady state...
November 16, 2017: Blood
https://www.readbyqxmd.com/read/28925645/vitamin-b12-deficiency-recognition-and-management
#14
Robert C Langan, Andrew J Goodbred
Vitamin B12 deficiency is a common cause of megaloblastic anemia, various neuropsychiatric symptoms, and other clinical manifestations. Screening average-risk adults for vitamin B12 deficiency is not recommended. Screening may be warranted in patients with one or more risk factors, such as gastric or small intestine resections, inflammatory bowel disease, use of metformin for more than four months, use of proton pump inhibitors or histamine H2 blockers for more than 12 months, vegans or strict vegetarians, and adults older than 75 years...
September 15, 2017: American Family Physician
https://www.readbyqxmd.com/read/28730452/transcranial-color-doppler-in-stroke-free-adult-patients-with-sickle-cell-disease
#15
COMPARATIVE STUDY
G Graziadei, F M Casoni, F Annoni, I Cortinovis, P Ridolfi, I Gandolfi, A Marcon, E Di Pierro, M D Cappellini
The threshold velocity ≥200 cm/s at transcranial Doppler (TCD) evaluation is a useful cut-off for preventing the stroke (STOP trial) in pediatric patients with sickle cell disease (SCD), term including different types of sickle genotypes. Scanty data are available for adult SCD patients. We compared intracranial blood flow velocities between adult SCD patients and controls using transcranial color Doppler (TCCD), measuring the peak of systolic velocity (PSV) with the insonation angle correction and the pulsatility index (PI), an indicator of endothelial elasticity...
September 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28428899/watershed-infarct-in-beta-thalassemia-major-patient
#16
Behnaz Ansari, Mohammad Saadatnia, Ali Asghar Okhovat
Background. The mechanism of stroke in beta-thalassemia was reported previously as cardioembolic and hypercoagulable state. However, there is no report of watershed infarct in beta-thalassemia anemia. Method. We present an adult β-thalassemia major patient with manifest asymptomatic chronic left carotid occlusion who suffered watershed infarct. Result. In the presence of asymptomatic chronic left internal carotid occlusion, we assumed that severe anemia (hemoglobin = 3) at admission leads to watershed infarct...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28417646/cerebral-hemodynamic-assessment-and-neuroimaging-across-the-lifespan-in-sickle-cell-disease
#17
Lori C Jordan, Michael R DeBaun
Children and adults with sickle cell anemia (SCA) have a higher risk of strokes compared to age- and race-matched peers. Velocity in the middle cerebral or distal internal carotid artery as measured by transcranial Doppler ultrasound is a recognized method to identify children but not adults with SCA at high-risk for first stroke. For both children and adults with SCA that have had a stroke, no methods clearly identify individuals at highest risk of recurrent strokes or an initial silent stroke, the most common neurological injury...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28348599/a-rare-case-of-stroke-secondary-to-iron-deficiency-anemia-in-a-young-female-patient
#18
Kavitha Gopalratnam, Kevin Andrew Woodson, Jigarkumar Rangunwala, Kanaga Sena, Manisha Gupta
Ischemic strokes occur when there is a sudden obstruction of an artery supplying blood flow to an area of the brain, leading to a focal neurological deficit. Strokes can be thrombotic or embolic in etiology and are associated with underlying conditions such as hypertension and atherosclerosis. Possible etiologies of strokes include cardioembolic disease, hematologic disorders, connective tissue disorders, and substance abuse or can be cryptogenic. Most stroke cases are seen in patients over 65 years of age...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28052565/cerebral-hemodynamics-and-pseudo-continuous-arterial-spin-labeling-considerations-in-adults-with-sickle-cell-anemia
#19
Meher R Juttukonda, Lori C Jordan, Melissa C Gindville, Larry T Davis, Jennifer M Watchmaker, Sumit Pruthi, Manus J Donahue
Sickle cell anemia (SCA) is a genetic disorder resulting in reduced oxygen carrying capacity and elevated stroke risk. Pseudo-continuous arterial spin labeling (pCASL) measures of cerebral blood flow (CBF) may have relevance for stroke risk assessment; however, the effects of elevated flow velocity and reduced bolus arrival time (BAT) on CBF quantification in SCA patients have not been thoroughly characterized, and pCASL model parameters used in healthy adults are often applied to patients with SCA. Here, cervical arterial flow velocities and pCASL labeling efficiencies were computed in adults with SCA (n = 19) and age- and race-matched controls without sickle trait (n = 7) using pCASL in sequence with phase contrast MR angiography (MRA)...
February 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/27956157/ibrutinib-for-patients-with-rituximab-refractory-waldenstr%C3%A3-m-s-macroglobulinaemia-innovate-an-open-label-substudy-of-an-international-multicentre-phase-3-trial
#20
MULTICENTER STUDY
Meletios A Dimopoulos, Judith Trotman, Alessandra Tedeschi, Jeffrey V Matous, David Macdonald, Constantine Tam, Olivier Tournilhac, Shuo Ma, Albert Oriol, Leonard T Heffner, Chaim Shustik, Ramón García-Sanz, Robert F Cornell, Carlos Fernández de Larrea, Jorge J Castillo, Miquel Granell, Marie-Christine Kyrtsonis, Veronique Leblond, Argiris Symeonidis, Efstathios Kastritis, Priyanka Singh, Jianling Li, Thorsten Graef, Elizabeth Bilotti, Steven Treon, Christian Buske
BACKGROUND: In the era of widespread rituximab use for Waldenström's macroglobulinaemia, new treatment options for patients with rituximab-refractory disease are an important clinical need. Ibrutinib has induced durable responses in previously treated patients with Waldenström's macroglobulinaemia. We assessed the efficacy and safety of ibrutinib in a population with rituximab-refractory disease. METHODS: This multicentre, open-label substudy was done at 19 sites in seven countries in adults aged 18 years and older with confirmed Waldenström's macroglobulinaemia, refractory to rituximab and requiring treatment...
February 2017: Lancet Oncology
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