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https://www.readbyqxmd.com/read/29163203/cyclic-stretch-alters-vascular-reactivity-of-mouse-aortic-segments
#1
Arthur Leloup, Sofie De Moudt, Cor Van Hove, Paul Fransen
Large, elastic arteries buffer the pressure wave originating in the left ventricle and are constantly exposed to higher amplitudes of cyclic stretch (10%) than muscular arteries (2%). As a crucial factor for endothelial and smooth muscle cell function, cyclic stretch has, however, never been studied in ex vivo aortic segments of mice. To investigate the effects of cyclic stretch on vaso-reactivity of mouse aortic segments, we used the Rodent Oscillatory Tension Set-up to study Arterial Compliance (ROTSAC). The aortic segments were clamped at frequencies of 6-600 bpm between two variable preloads, thereby mimicking dilation as upon left ventricular systole and recoiling as during diastole...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/29159269/pulmonary-artery-dysfunction-in-chronic-thromboembolic-pulmonary-hypertension
#2
Hidetoshi Chibana, Nobuhiro Tahara, Naoki Itaya, Takashi Ishimatsu, Masahiro Sasaki, Motoki Sasaki, Takaharu Nakayoshi, Masanori Ohtsuka, Shinji Yokoyama, Ken-Ichiro Sasaki, Takafumi Ueno, Yoshihiro Fukumoto
Background: Unresolved thromboemboli in the pulmonary arteries (PA) is known to cause chronic thromboembolic pulmonary hypertension (CTEPH). However, it remains unknown if vascular dysfunction in pulmonary arteries exists in patients with CTEPH. Methods and results: We enrolled 7 female patients with CTEPH in this study, who have stable pulmonary hemodynamics after balloon pulmonary angioplasty (age; 73.6 ± 3.0 years old, mean right atrial pressure; 4.1 ± 0...
December 2017: IJC Heart & Vasculature
https://www.readbyqxmd.com/read/29152307/coagulation-profile-of-sudanese-children-with-homozygous-sickle-cell-disease-and-the-effect-of-treatment-with-omega-3-fatty-acid-on-the-coagulation-parameters
#3
Shiekh Awoda, Ahmed A Daak, Nazik Elmalaika Husain, Kebreab Ghebremeskel, Mustafa I Elbashir
Background: It has been reported that patients with SCD do have an abnormal coagulation profile. Coagulopathy is thought to be one of the key factors that contribute to the vaso-occlusive crisis that characterises sickle cell disease (SCD). In this study, we investigated whether Sudanese sickle cell patients have an abnormal coagulation profile. In addition, the effect of treatment with either omega-3 fatty acids or hydroxyurea on coagulation profile was assessed. Methods: Homozygous SCD patients untreated (n = 52), omega-3 treated (n = 44), hydroxyurea (HU) treated (n = 8) and healthy (HbAA) controls (n = 52) matched for age (4-20 years), gender and socioeconomic status were enrolled...
2017: BMC Hematology
https://www.readbyqxmd.com/read/29149854/cardiac-arrest-in-a-toddler-treated-with-propranolol-for-infantile-hemangioma-a-case-report
#4
Alvise Tosoni, Mario Cutrone, Maurizio Dalle Carbonare, Andrea Pettenazzo, Giorgio Perilongo, Stefano Sartori
BACKGROUND: Propranolol has become the first-line treatment for complicated Infantile Hemangioma (IH), showing so far a good risk-benefit profile. CASE PRESENTATION: We report the case of a toddler, on propranolol, who suffered cardiac arrest during an acute viral infection. She had a neurally-mediated syncope that progressed to asystole, probably because of concurrent factors as dehydration, beta-blocking and probably individual susceptibility to vaso-vagal phenomena...
November 17, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29134776/acute-chest-syndrome-among-children-hospitalized-with-vaso-occlusive-crisis-a-nationwide-study-in-the-united-states
#5
Takuto Takahashi, Yusuke Okubo, Atsuhiko Handa
PURPOSE: Acute chest syndrome (ACS) is a common complication among pediatric inpatients with sickle cell disease and vaso-occlusive crisis (VOC). However, little is known about the factors associated with ACS complication. The present study assessed the epidemiological features of children hospitalized with VOC and ascertained factors associated with ACS complication. METHODS: Hospital discharge records of patients with VOC aged <20 years were obtained for the years 2003, 2006, 2009, and 2012 from the Kids' Inpatient Database...
November 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29130518/automated-red-blood-cell-exchange-in-preparation-for-filgrastim-mobilization-of-autologous-peripheral-blood-hematopoietic-progenitor-cells-in-a-patient-with-sickle-cell-anemia
#6
Yong Zhao, Jeffrey A Bailey, Jeanne Linden, Patricia St Pierre, Jan Cerny, Michelle Vauthrin, Mindy Greene, Robert Weinstein
Increasing survival of patients with sickle cell anemia (SCA) well into adulthood results in a rising likelihood of developing hematological malignancy. High-dose chemotherapy with autologous hematopoietic progenitor cell (HPC) rescue is standard of care for several hematological malignancies, but the risk of severe or life-threatening vaso-occlusive phenomena during filgrastim mobilization of HPC for collection poses a potential barrier to this approach. We report the use of automated red cell exchange in preparation for filgrastim mobilization in a patient with homozygous SCA...
November 11, 2017: Journal of Clinical Apheresis
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
#7
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/29104118/clinical-trial-conduct-in-special-populations-and-developing-regions-an-overview-of-the-dove-study-in-pediatric-patients-with-sickle-cell-disease
#8
Stephen G Reams, Linda Messana, Patricia B Brown, Kevin Nanry, Alexandria E Gunnell
Clinical trials conducted in unique patient populations or individuals with rare diseases are typically hampered by limitations in availability of qualified patients, requiring sponsors to broaden their global outreach to achieve enrollment. Engaging clinical study centers in developing regions may offer access to a substantially larger patient pool. However, they provide a unique set of challenges based on local cultures and requirements. The DOVE study (Determining effects Of platelet inhibition on Vaso-occlusive Events) was a clinical trial of prasugrel hydrochloride (prasugrel) in pediatric patients (aged 2 to <18years) with sickle cell anemia...
November 2, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/29099347/the-morbidity-pattern-of-children-with-sickle-cell-disorders-admitted-to-the-queen-elizabeth-hospital-barbados-2009-2013
#9
D Grannum, Paula M Lashley
A retrospective study was conducted by examining all the medical files of all the children with sickle cell anaemia (Hb SS) admitted from 1 January 2009 to 31 December 2013. A total of 220 admission notes (59 patients aged 5 months-16 years) were analysed. Of these, 53.2% were boys; 85.8% of the patients had Hb SS. The most common reason for admission was vaso-occlusive crisis. Blood transfusions were used in 39.7% of admissions There were no deaths during the study period. We note the number of sickle cell-related admissions and morbidity has increased, along with an increase in the number of blood transfusions...
January 1, 2017: Tropical Doctor
https://www.readbyqxmd.com/read/29096686/vaso-vitamin-d-and-arthroplasty-surgery-outcomes-study-supplementation-of-vitamin-d-deficiency-to-improve-outcomes-after-total-hip-or-knee-replacement-study-protocol-for-a-randomised-controlled-feasibility-trial
#10
Rory J M Morrison, Deborah Bunn, William K Gray, Paul N Baker, Craig White, Amar Rangan, Kenneth S Rankin, Mike R Reed
BACKGROUND: Vitamin D deficiency has been linked to poor outcomes after total hip replacement (THR) or total knee replacement (TKR), including lower patient-reported outcome measures (PROMs), peri-prosthetic infection and longer hospital stay. We present a randomised feasibility trial protocol designed to prospectively investigate the influence of vitamin D testing, and subsequent supplementation for deficiency, prior to THR/TKR. METHODS/DESIGN: One hundred adult patients undergoing primary THR/TKR for osteoarthritis at two NHS hospital trusts in North East England will be recruited...
November 2, 2017: Trials
https://www.readbyqxmd.com/read/29082316/the-use-of-cannabis-by-patients-with-sickle-cell-disease-increased-the-frequency-of-hospitalization-due-to-vaso-occlusive-crises
#11
Samir K Ballas
Introduction: The objective of this study was to determine if patients with sickle cell disease using cannabis had decreased frequency of acute vaso-occlusive crises (VOCs) that required hospitalization. Method: This was a retrospective study in which 270 urine drug screen tests were done on 72 patients: 40 males and 32 females. Results: Cannabinoids were found in 144 urine tests from 37 patients and were negative in 126 tests from 35 patients. Males who used cannabis were significantly younger (p<0.001) than males who did not...
2017: Cannabis and Cannabinoid Research
https://www.readbyqxmd.com/read/29078924/sickle-cell-disease-in-the-emergency-department
#12
REVIEW
Paris B Lovett, Harsh P Sule, Bernard L Lopez
Acute painful episodes are the most common reason for emergency department visits among patients with sickle cell disease (SCD). Early and aggressive pain management is a priority. Emergency providers (EPs) must also diagnose other emergent diagnoses in patients with SCD and differentiate them from vaso-occlusive crisis. EPs should be aware of cognitive biases that may misdirect the diagnostic process. Administration of intravenous fluids should be used judiciously. Blood transfusion may be considered. Coordination of care with hematology is an important part of the effective emergency department and long-term management of patients with SCD...
December 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29051184/novel-use-of-hydroxyurea-in-an-african-region-with-malaria-noharm-a-trial-for-children-with-sickle-cell-anemia
#13
Robert O Opoka, Christopher M Ndugwa, Teresa S Latham, Adam Lane, Heather A Hume, Phillip Kasirye, James S Hodges, Russell E Ware, Chandy C John
Hydroxyurea treatment is recommended for children with sickle cell anemia (SCA) living in high-resource malaria-free regions, but its safety and efficacy in malaria-endemic sub-Saharan Africa, where the greatest sickle cell burden exists, remain unknown. In vitro studies suggest hydroxyurea could increase malaria severity, and hydroxyurea-associated neutropenia could worsen infections. NOHARM was a randomized, double-blinded, placebo-controlled trial conducted in malaria-endemic Uganda, comparing hydroxyurea to placebo at 20 ± 2...
October 19, 2017: Blood
https://www.readbyqxmd.com/read/29047145/a-randomized-controlled-trial-comparing-two-vaso-occlusive-episode-voe-protocols-in-sickle-cell-disease-scd
#14
Paula Tanabe, Susan Silva, Hayden B Bosworth, Regina Crawford, Judith A Paice, Lynne D Richardson, Christopher N Miller, Jeffrey Glassberg
Limited evidence guides opioid dosing strategies for acute Sickle Cell (SCD) pain. We compared two National Heart, Lung and Blood (NHBLI) recommended opioid dosing strategies (weight-based vs. patient-specific) for ED treatment of acute vaso-occlusive episodes (VOE). A prospective randomized controlled trial (RCT) was conducted in two ED's. Adults ≥ 21 years of age with SCD disease were eligible. Among the 155 eligible patients, 106 consented and 52 had eligible visits. Patients were pre-enrolled in the outpatient setting and randomized to one of two opioid dosing strategies for a future ED visit...
October 19, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29046485/dimethyl-fumarate-increases-fetal-hemoglobin-provides-heme-detoxification-and-corrects-anemia-in-sickle-cell-disease
#15
Sriram Krishnamoorthy, Betty Pace, Dipti Gupta, Sarah Sturtevant, Biaoru Li, Levi Makala, Julia Brittain, Nancy Moore, Benjamin F Vieira, Timothy Thullen, Ivan Stone, Huo Li, William E Hobbs, David R Light
Sickle cell disease (SCD) results from a point mutation in the β-globin gene forming hemoglobin S (HbS), which polymerizes in deoxygenated erythrocytes, triggering recurrent painful vaso-occlusive crises and chronic hemolytic anemia. Reactivation of fetal Hb (HbF) expression ameliorates these symptoms of SCD. Nuclear factor (erythroid derived-2)-like 2 (Nrf2) is a transcription factor that triggers cytoprotective and antioxidant pathways to limit oxidative damage and inflammation and increases HbF synthesis in CD34+ stem cell-derived erythroid progenitors...
October 19, 2017: JCI Insight
https://www.readbyqxmd.com/read/29039727/identification-of-aptamers-that-bind-to-sickle-hemoglobin-and-inhibit-its-polymerization
#16
Shirley H Purvis, Jeffrey R Keefer, Yolanda M Fortenberry, Emily A Barron-Casella, James F Casella
The pathophysiology of sickle cell disease (SCD) is dependent on the polymerization of deoxygenated sickle hemoglobin (HbS), leading to erythrocyte deformation (sickling) and vaso-occlusion within the microvasculature. Following deoxygenation, there is a delay time before polymerization is initiated, during which nucleation of HbS monomers occurs. An agent with the ability to extend this delay time or slow polymerization would therefore hold a therapeutic, possibly curative, potential. We used the Systematic Evolution of Ligands by Exponential Enrichment (SELEX) method to screen for HbS-binding RNA aptamers modified with nuclease-resistant 2'-fluoropyrimidines...
October 17, 2017: Nucleic Acid Therapeutics
https://www.readbyqxmd.com/read/29039292/an-innovative-short-stay-health-care-model-for-treatment-of-uncomplicated-vaso-occlusive-crisis-in-adult-sickle-cell-disease-patients-in-canada-to-reduce-emergency-department-utilization
#17
Andrew Binding, Richard Ward, Chai Phua, Veronique Naessens, Tara O'Brien, Sacha Bhatia, Hayley Baranek, Husayn Marani, Geetha Mukerji
OBJECTIVES: Patients with sickle cell disease (SCD) with vaso-occlusive crises (VOC) often visit the emergency department (ED) for management of painful episodes. The primary objective of this pilot study was to evaluate the acceptability of a short-stay model for treatment of VOC in SCD outside of the ED in Toronto, Canada. Secondary objectives were to assess patient satisfaction of this model, barriers to its use and comparison of clinical outcomes to a historical control. METHODS: Adult SCD patients with symptoms of an uncomplicated VOC between October 2014 to July 2016 were managed according to best practice recommendations in a short-stay unit as an alternative to the local emergency room...
October 17, 2017: CJEM
https://www.readbyqxmd.com/read/29033725/assessment-of-patient-controlled-analgesia-versus-intermittent-opioid-therapy-to-manage-sickle-cell-disease-vaso-occlusive-crisis-in-adult-patients
#18
Alaa Al-Anazi, Lowloa Al-Swaidan, Maha Al-Ammari, Tariq Al-Debasi, Abdulmalik M Alkatheri, Shmeylan Al-Harbi, Aiman A Obaidat, Abdulkareem M Al-Bekairy
BACKGROUND: Vaso-occlusive crisis (VOC) is one of the acute complications of sickle-cell disease (SCD). Treatment mainly relies on hydration and pain control by analgesics. The specific aim of this study was to assess potential health outcomes within the first 72 h of admission between intermittent and patient-controlled analgesia (PCA) by opioids among VOC patients. METHODS: A retrospective chart review study was conducted to determine SCD patients with VOC. Using the hospital electronic system, the following data were collected: patient's age, gender, blood pressure, heart rate, respiratory rate, oxygen saturation, and pain score on admission and daily for 3 days as well as the cumulative opioid analgesic dose for 72 h which is reported as morphine equivalent...
October 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/29022080/urinary-potassium-to-urinary-potassium-plus-sodium-ratio-can-accurately-identify-hypovolemia-in-nephrotic-syndrome-a-provisional-study
#19
Werner Keenswijk, Mohamad Ikram Ilias, Ann Raes, Raymond Donckerwolcke, Johan Vande Walle
There is evidence pointing to a decrease of the glomerular filtration rate (GFR) in a subgroup of nephrotic children, likely secondary to hypovolemia. The aim of this study is to validate the use of urinary potassium to the sum of potassium plus sodium ratio (UK/UK+UNa) as an indicator of hypovolemia in nephrotic syndrome, enabling detection of those patients who will benefit from albumin infusion. We prospectively studied 44 nephrotic children and compared different parameters to a control group (36 children)...
October 11, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28994869/acute-acoustic-trauma-how-to-manage-and-how-to-prevent
#20
Ali Mardassi, Senda Turki, Hajer Mbarek, Amani Hachicha, Sonia Benzarti, Maher Abouda
INTRODUCTION: The consequence of an exposure to intense sounds can be a temporary or permanent hearing loss and even with a rapid therapeutic management, severe sensorineural sequelae may persist. METHODS: the authors report a retrospective study about 64 patients followed for an acute acoustic trauma during a period of 8 years (2006 to 2013). For all the cases, a clinical examination associated to a pure-tone audiometry was conducted. Hearing levels were measured at the frequencies 500, 1000, 2000 and 3000 Hertz...
November 2016: La Tunisie Médicale
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