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https://www.readbyqxmd.com/read/28745855/managing-acute-complications-of-sickle-cell-disease-in-pediatric-patients-digest
#1
Sathyaseelan Subramaniam, Jennifer H Chao, Pradip Chaudhari
Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management...
November 22, 2016: Pediatric Emergency Medicine Practice
https://www.readbyqxmd.com/read/28743191/successful-utilization-of-an-electronic-pain-diary-in-a-multinational-phase-3-interventional-study-of-pediatric-sickle-cell-anemia
#2
Lori E Heath, Matthew M Heeney, Carolyn C Hoppe, Samuel Adjei, Tsiri Agbenyega, Mohamed Badr, Nicoletta Masera, Chunmei Zhou, Patricia B Brown, Joseph A Jakubowski, Carlton Dampier
BACKGROUND/AIMS: Patients with sickle cell anemia can experience recurrent pain episodes, which affect quality of life. The reported prevalence of pain is higher in studies using patient diaries than in healthcare facility utilization data. Determining Effects of Platelet Inhibition on Vaso-Occlusive Events was a multinational study that assessed the efficacy and safety of prasugrel in reducing the rate of vaso-occlusive events in children with sickle cell anemia (NCT01794000) and included an electronic patient-reported outcome diary to record pain occurrence...
July 1, 2017: Clinical Trials: Journal of the Society for Clinical Trials
https://www.readbyqxmd.com/read/28738269/an-anti-inflammatory-effect-of-red-microalga-polysaccharides-in-coronary-artery-endothelial-cells
#3
Oshrat Levy-Ontman, Mahmoud Huleihel, Rachel Hamias, Talya Wolak, Esther Paran
BACKGROUND AND AIMS: Polysaccharides (PSs) produced by the red microalga Porphyridium sp. were reported to exhibit anti-inflammatory bioactivities in the human skin. The primary goal of the present research was to assess whether PSs attenuate inflammatory processes by interfering with tumour necrosis factor-alpha (TNF-α)-induced inflammation, in human coronary artery endothelial cells (HCAECs). METHODS: Functional and inflammatory markers were quantified in TNF-α-stimulated HCAECs, with and without pre-treatment with PSs...
July 16, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28736310/non-bold-contrast-for-laminar-fmri-in-humans-cbf-cbv-and-cmr02
#4
REVIEW
Laurentius Huber, Kâmil Uludağ, Harald E Möller
Functional magnetic resonance imaging (fMRI) using the blood oxygenation level-dependent (BOLD) contrast indirectly probes neuronal activity changes via evoked cerebral blood flow (CBF), cerebral blood volume (CBV) and cerebral metabolic rate of oxygen (CMR02) changes. The gradient-echo BOLD signal is mostly sensitive to ascending veins in the tissue and to pial veins. Thereby, the achievable spatial specificity to neuronal activation is limited. Furthermore, the non-linear interaction of CBF, CBV and CMR02 can hamper quantitative interpretations of the BOLD signal across cortical depths with different baseline physiology...
July 20, 2017: NeuroImage
https://www.readbyqxmd.com/read/28735981/autoimmune-liver-disease-in-children-with-sickle-cell-disease
#5
Suttiruk Jitraruch, Emer Fitzpatrick, Maesha Deheragoda, Annamaria Deganello, Giorgina Mieli-Vergani, Susan Height, David Rees, Nedim Hadzic, Marianne Samyn
OBJECTIVE: To assess the incidence, clinical features, and outcome of autoimmune liver disease (AILD) in patients with sickle cell disease (SCD). STUDY DESIGN: Single center retrospective review of patients with SCD with AILD referred between 1999 and 2015. RESULTS: Thirteen of 77 (17%) patients with SCD with hepatic dysfunction were diagnosed with AILD (median age 11, range, 3.4-16 years) with a female preponderance (77%). Acute hepatitis and insidious onset were the commonest presentations...
July 20, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28724841/sphingosine-1-phosphate-and-atherosclerosis
#6
Makoto Kurano, Yutaka Yatomi
Sphingosine 1-phosphate (S1P) is a potent lipid mediator that works on five kinds of S1P receptors located on the cell membrane. In the circulation, S1P is distributed to HDL, followed by albumin. Since S1P and HDL share several bioactivities, S1P is believed to be responsible for the pleiotropic effects of HDL. Plasma S1P levels are reportedly lower in subjects with coronary artery disease, suggesting that S1P might be deeply involved in the pathogenesis of atherosclerosis. In basic experiments, however, S1P appears to possess both pro-atherosclerotic and anti-atherosclerotic properties; for example, S1P possesses anti-apoptosis, anti-inflammation, and vaso-relaxation properties and maintains the barrier function of endothelial cells, while S1P also promotes the egress and activation of lymphocytes and exhibits pro-thrombotic properties...
July 20, 2017: Journal of Atherosclerosis and Thrombosis
https://www.readbyqxmd.com/read/28711132/additional-conditions-amenable-to-observation-care
#7
REVIEW
Matthew A Wheatley
ED observation units (EDOUs) are designed for patients who require diagnostics or therapeutics beyond the initial ED visit to determine the need for hospital admission. Best evidence is that this care be delivered via ordersets or protocols. Occasionally, patients present with conditions that are amenable to EDOU care but fall outside the commonly used protocols. This article details a few of these conditions: abnormal uterine bleeding, allergic reaction, alcohol intoxication, acetaminophen overdose and sickle cell vaso-occlusive crisis...
August 2017: Emergency Medicine Clinics of North America
https://www.readbyqxmd.com/read/28707371/individuals-with-sickle-cell-disease-have-a-significantly-greater-vasoconstriction-response-to-thermal-pain-than-controls-and-have-significant-vasoconstriction-in-response-to-anticipation-of-pain
#8
Maha Khaleel, Mammen Puliyel, Payal Shah, John Sunwoo, Roberta M Kato, Patjanaporn Chalacheva, Wanwara Thuptimdang, Jon Detterich, John C Wood, Jennie Tsao, Lonnie Zeltzer, Richard Sposto, Michael C K Khoo, Thomas D Coates
The painful vaso-occlusive crises (VOC) that characterize sickle cell disease (SCD) progress over hours from the asymptomatic steady-state. SCD patients report that VOC can be triggered by stress, cold exposure and pain itself. We anticipated that pain could cause neural-mediated vasoconstriction, decreasing regional blood flow and promoting entrapment of sickle cells in the microvasculature. Therefore, we measured microvascular blood flow in the fingers of both hands using plethysmography and laser-Doppler flowmetry while applying a series of painful thermal stimuli on the right forearm in 23 SCD patients and 25 controls...
July 13, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28699283/prospective-evaluation-of-chronic-organ-damage-in-adult-sickle-cell-patients-a-seven-year-follow-up-study
#9
Charlotte F J van Tuijn, Marein Schimmel, Eduard J van Beers, Erfan Nur, Bart J Biemond
Organ damage in sickle cell disease (SCD) is a crucial determinant for disease severity and prognosis. In a previous study, we analyzed the prevalence of SCD-related organ damage and complications in adult sickle cell patients. We now describe a seven-year follow-up of this cohort. All patients from the primary analysis in 2006 (n=104), were included for follow-up. Patients were screened for SCD-related organ damage and complications (microalbuminuria, renal failure, elevated tricuspid regurgitation flow velocity (TRV) (≥2...
July 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28698351/subdissociative-intranasal-ketamine-plus-standard-pain-therapy-versus-standard-pain-therapy-in-the-treatment-of-paediatric-sickle-cell-disease-vaso-occlusive-crises-in-resource-limited-settings-study-protocol-for-a-randomised-controlled-trial
#10
James R Young, Hendry Robert Sawe, Juma A Mfinanga, Ernest Nshom, Ethan Helm, Charity G Moore, Michael S Runyon, Stacy L Reynolds
INTRODUCTION: Pediatric sickle cell disease, highly prevalent in sub-Saharan Africa, carries great morbidity and mortality risk. Limited resources and monitoring make management of acute vaso-occlusive crises challenging. This study aims to evaluate the efficacy and safety of subdissociative intranasal ketamine as a cheap, readily available and easily administered adjunct to standard pain therapy. We hypothesise that subdissociative, intranasal ketamine may significantly augment current approaches to pain management in resource-limited settings in a safe and cost-effective manner...
July 10, 2017: BMJ Open
https://www.readbyqxmd.com/read/28697028/sciatic-popliteal-fossa-catheter-for-pediatric-pain-management-of-sickle-cell-crisis-a-case-report
#11
Garret Weber, Sherry Liao, Micah Alexander Burns
Sickle cell crisis, or vaso-occlusive crisis (VOC), is a major cause of hospitalizations for adults and children with sickle cell disease, and is associated with increased morbidity and mortality. Despite prompt pharmacological treatment and multimodal pain management, acute pain during a VOC is often not adequately controlled in the pediatric population. We placed a continuous popliteal sciatic nerve block under ultrasound guidance in a pediatric patient for localized refractory pain during a VOC, resulting in improved pain control with preserved sensorimotor function...
July 10, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28695380/macular-capillary-recovery-in-systemic-lupus-erythematosus-complicated-by-kikuchi-fujimoto-disease
#12
Reiko Kinouchi, Motoshi Kinouchi, Akihiro Ishibazawa, Akitoshi Yoshida
PURPOSE: Few case reports have described vaso-occlusive retinopathy in systemic lupus erythematosus (SLE) using optical coherence tomography (OCT) angiography. Here we report the clinical features of a patient with SLE, complicated by Kikuchi-Fujimoto disease, who developed vaso-occlusive retinopathy. We then describe the subsequent recovery of the macular capillaries as assessed by OCT angiography. CASE: A 16-year-old male was referred to us with fever, a 1-month history of violaceous red papules and erythematous plaques on his face and a painful nodule in his right neck...
July 10, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28686106/neonatal-screening-improves-sickle-cell-disease-clinical-outcome-in-belgium
#13
Phu-Quoc Lê, Alina Ferster, Laurence Dedeken, Christiane Vermylen, Anna Vanderfaeillie, Laurence Rozen, Catherine Heijmans, Sophie Huybrechts, Christine Devalck, Frédéric Cotton, Olivier Ketelslegers, Marie-Françoise Dresse, Jean-François Fils, Béatrice Gulbis
Objectives To compare the outcomes of sickle cell disease patients diagnosed through neonatal screening with those who were not. Methods In an observational multicenter study in Belgium, 167 screened and 93 unscreened sickle cell disease patients were analyzed for a total of 1116 and 958 patient-years of follow-up, respectively. Both groups were compared with propensity score analysis, with patients matched on three covariates (gender, genotype, and central Africa origin). Bonferroni correction was applied for all comparisons...
January 1, 2017: Journal of Medical Screening
https://www.readbyqxmd.com/read/28678930/ruthenium-complex-improves-the-endothelial-function-in-aortic-rings-from-hypertensive-rats
#14
Izabela Pereira Vatanabe, Carla Nascimento Dos Santos Rodrigues, Tereza Cristina Buzinari, Thiago Francisco de Moraes, Roberto Santana da Silva, Gerson Jhonatan Rodrigues
Background: The endothelium is a monolayer of cells that extends on the vascular inner surface, responsible for the modulation of vascular tone. By means of the release of nitric oxide (NO), the endothelium has an important protective function against cardiovascular diseases. Objective: Verify if cis- [Ru(bpy)2(NO2)(NO)](PF6)2 (BPY) improves endothelial function and the sensibility of conductance (aorta) and resistance (coronary) to vascular relaxation induced by BPY...
June 29, 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28678524/the-effects-of-food-essential-oils-on-cardiovascular-diseases-a-review
#15
Sania Saljoughian, Shahin Roohinejad, Alaa El-Din A Bekhit, Ralf Greiner, Alireza Omidizadeh, Nooshin Nikmaram, Amin Mousavi Khaneghah
Essential oils (EO) are complex secondary metabolites, which are produced by aromatic plants and identified by their powerful odors. Present studies on EO and their isolated ingredients have drawn the attention of researchers to screen these natural products and evaluate their effect on the cardiovascular system. Some EO, and their active ingredients, have been reported to improve the cardiovascular system significantly by affecting vaso-relaxation, and decreasing the heart rate and exert a hypotension activity...
February 10, 2017: Critical Reviews in Food Science and Nutrition
https://www.readbyqxmd.com/read/28669521/management-of-delayed-hemolytic-transfusion-reaction-in-sickle-cell-disease-prevention-diagnosis-treatment
#16
F Pirenne, P Bartolucci, A Habibi
Transfusion remains a key treatment of sickle cell disease complications. However, delayed hemolytic transfusion reaction, the most serious complication of transfusion, may be life-threatening if hyperhemolysis develops. This syndrome is generally underdiagnosed because its biological and clinical features resemble those of vaso-occlusive crisis, and red blood cell antibodies are frequently absent. Further transfusions may aggravate the symptoms, leading to severe multiple organ failure and death. It is therefore essential to prevent, diagnose and treat this syndrome efficiently...
June 29, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28662607/integrating-fat-embolism-syndrome-scoring-indices-in-sickle-cell-disease-a-practice-management-review
#17
Keneisha Bailey, Jagila Wesley, Adebayo Adeyinka, Louisdon Pierre
Fat embolism syndrome (FES) has been described in the literature as a rare complication of sickle cell disease (SCD). A review article published in 2005 reported 24 cases of FES associated with SCD. In many cases, a definitive diagnosis of FES in SCD is made on autopsy because of the lack of early recognition and the paucity of sensitive and specific testing for this syndrome. Patients with FES usually have a fulminant, rapidly deteriorating clinical course with mortality occurring within the first 24 hours...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28657766/a-suspected-delayed-hemolytic-transfusion-reaction-mediated-by-anti-joa
#18
Ryan P Jajosky, Wendy C Lumm, Scott C Wise, Roni J Bollag, James F Shikle
A 32-year-old African-American woman with a history of sickle cell disease presented for surgical evaluation of left total hip arthroplasty due to avascular necrosis of the femoral head. In anticipation of a complex orthopedic procedure, pre-surgical blood work was ordered. The patient's Fenwal blood sample typed as group O, D+. Although the patient had a history of anti-Fya, the antibody identification was inconclusive, so the workup was sent to a reference laboratory. The patient was last transfused with red blood cells (RBCs) 2 years earlier, but had no history of transfusion reactions...
June 2017: Immunohematology
https://www.readbyqxmd.com/read/28638723/low-dose-methotrexate-in-sickle-cell-disease-a-pilot-study-with-rationale-borrowed-from-rheumatoid-arthritis
#19
Silvia R Brandalise, Rosemary Assis, Angelo B A Laranjeira, José Andrés Yunes, Pedro O de Campos-Lima
BACKGROUND: Inflammation is a major feature of sickle cell disease (SCD). Low-dose methotrexate (MTX) has long been used in chronic inflammatory diseases. This pilot study examined the MTX effect on acute vaso-occlusive pain crises (VOC) in SCD patients. METHODS: Fourteen adults on hydroxyurea with severe and refractory VOC received one intramuscular injection of 10 mg of MTX per week for 12 weeks. A single weekly dose of 5 mg of leucovorin was administered orally 48 h after each MTX injection...
2017: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/28606962/endothelin-1-promotes-vascular-smooth-muscle-cell-migration-across-the-artery-wall-a-mechanism-contributing-to-vascular-remodelling-and-intimal-hyperplasia-in-giant-cell-arteritis
#20
Ester Planas-Rigol, Nekane Terrades-Garcia, Marc Corbera-Bellalta, Ester Lozano, Marco A Alba, Marta Segarra, Georgina Espígol-Frigolé, Sergio Prieto-González, José Hernández-Rodríguez, Sara Preciado, Rodolfo Lavilla, Maria C Cid
BACKGROUND: Giant-cell arteritis (GCA) is an inflammatory disease of large/medium-sized arteries, frequently involving the temporal arteries (TA). Inflammation-induced vascular remodelling leads to vaso-occlusive events. Circulating endothelin-1 (ET-1) is increased in patients with GCA with ischaemic complications suggesting a role for ET-1 in vascular occlusion beyond its vasoactive function. OBJECTIVE: To investigate whether ET-1 induces a migratory myofibroblastic phenotype in human TA-derived vascular smooth muscle cells (VSMC) leading to intimal hyperplasia and vascular occlusion in GCA...
June 12, 2017: Annals of the Rheumatic Diseases
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