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https://www.readbyqxmd.com/read/29296825/disease-severity-and-slower-psychomotor-speed-in-adults-with-sickle-cell-disease
#1
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
#2
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
#3
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
#4
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...
October 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29103427/neuroinflammation-in-ischemic-pediatric-stroke
#5
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
#6
Zunyu Ke, Yu Zhao, Chuanling Wang, Zhiyou Cai
Anemia is closely associated with the occurrence and development of ischemic stroke. However, the clinical association between anemia and ischemic stroke remains unclear. We evaluated the clinical features of adult patients of acute cerebral infarction with anemia, and to provide a reference in the treatment of the anemia patients of acute cerebral infarction, and seek a better treatment different from the other non-anemia causes. We found that anemia has rendered valuable service to neuronal damage and subsequent deterioration of acute cerebral infarction in the adults...
October 15, 2017: 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
#7
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, 2,407 SCD patients (1,751 SS-Sβ(0), 495 SC and 161 Sβ(+)), aged three years old and over, were included at steady-state...
September 20, 2017: Blood
https://www.readbyqxmd.com/read/28925645/vitamin-b12-deficiency-recognition-and-management
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
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
#15
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/27896324/the-relationship-between-anemia-and-recurrence-of-ischemic-stroke-in-patients-with-trousseau-s-syndrome-a-retrospective-cross-sectional-study
#16
Mehmet Yigit, Ozgur Sogut, Eda Yigit, Kenan Ahmet Turkdogan, Onur Kaplan, Ali Dur, Ertan Sonmez, Bulut Demirel
OBJECTIVES: The relationship between cancer and thrombosis was first recognized by the French internist Armand Trousseau in 1865. Trousseau's syndrome is a spectrum of symptoms that result from recurrent thromboembolism associated with cancer or malignancy-related hypercoagulability. In this study, we investigated whether demographics, clinical features, or laboratory findings were able to predict recurrent stroke episodes in patients with Trousseau's syndrome. METHODS: In total, 178 adult patients were enrolled in this retrospective cross-sectional study...
June 2016: Turkish Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27752375/embolic-stroke-due-to-a-common-carotid-artery-thrombus-in-a-young-patient-with-severe-iron-deficiency-anemia-without-thrombocytosis
#17
David Roshal
This case report describes a 41-year-old previously healthy male who presented with stuttering transient ischemic symptoms and radiographic evidence of a left common carotid artery thrombus as well as acute and subacute ischemic infarcts in the left middle cerebral artery territory. An exhaustive stroke work-up did not provide a plausible etiology for his symptoms. His complete blood count and iron studies, however, revealed evidence of severe iron-deficiency anemia without reactive thrombocytosis. His stool guaiac test was positive...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27637966/sickle-cell-disease
#18
REVIEW
J Strouse
Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a mutation in the sixth amino acid of the β-globin gene (HBB). It is the most common serious genetic diseases in childhood, affecting approximately 1 in 2500 births and 100 000 individuals in the USA, in addition to 300 000 new cases globally each year. Central nervous system injury is the most debilitating frequent complication of SCD and includes stroke, silent cerebral infarct (SCI), and cognitive impairment. Among children with sickle cell anemia (HbSS), 11% had a stroke by age 18 years before the implementation of transcranial Doppler screening...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27113147/multiple-chronic-conditions-in-older-adults-with-acute-coronary-syndromes
#19
REVIEW
Joakim Alfredsson, Karen P Alexander
Older adults presenting with acute coronary syndromes (ACSs) often have multiple chronic conditions (MCCs). In addition to traditional cardiovascular (CV) risk factors (ie, hypertension, hyperlipidemia, and diabetes), common CV comorbidities include heart failure, stroke, and atrial fibrillation, whereas prevalent non-CV comorbidities include chronic kidney disease, anemia, depression, and chronic obstructive pulmonary disease. The presence of MCCs affects the presentation (eg, increased frequency of type 2 myocardial infarctions [MIs]), clinical course, and prognosis of ACS in older adults...
May 2016: Clinics in Geriatric Medicine
https://www.readbyqxmd.com/read/27105460/medical-complications-after-adult-spinal-deformity-surgery-incidence-risk-factors-and-clinical-impact
#20
Alex Soroceanu, Douglas C Burton, Jonathan Haim Oren, Justin S Smith, Richard Hostin, Christopher I Shaffrey, Behrooz A Akbarnia, Christopher P Ames, Thomas J Errico, Shay Bess, Munish C Gupta, Vedat Deviren, Frank J Schwab, Virginie Lafage
STUDY DESIGN: Retrospective review of a prospective multicenter database evaluating surgical adult spinal deformity (ASD) patients. OBJECTIVE: This study aims to identify risk factors for medical complications in ASD patients undergoing surgery. SUMMARY OF BACKGROUND DATA: ASD surgery is known for its high complication rate. This study examines baseline patient characteristics for predictors of medical complications in surgical ASD patients...
November 15, 2016: Spine
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