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https://www.readbyqxmd.com/read/29791238/low-dose-ketamine-infusion-for-adjunct-management-during-vaso-occlusive-episodes-in-adults-with-sickle-cell-disease-a-case-series
#1
Nicole Palm, Catherine Floroff, Tanna B Hassig, Alice Boylan, Julie Kanter
The optimal management of recurrent painful episodes in individuals living with sickle cell disease (SCD) remains unclear. Currently, the primary treatment for these episodes remains supportive, using fluids and intravenous opioid and anti-inflammatory medications. Few reports have described the use of adjunct subanesthetic doses of ketamine to opioids for treatment of refractory pain in SCD. This article reports a retrospective case series of five patients admitted to the intensive care unit (ICU) with prolonged vaso-occlusive episodes (VOEs)...
May 23, 2018: Journal of Pain & Palliative Care Pharmacotherapy
https://www.readbyqxmd.com/read/29784054/systematic-literature-review-and-assessment-of-patient-reported-outcome-instruments-in-sickle-cell-disease
#2
Grammati Sarri, Menaka Bhor, Seye Abogunrin, Caroline Farmer, Savita Nandal, Rashid Halloway, Dennis A Revicki
BACKGROUND: Sickle cell disease (SCD) is a chronic condition associated with high mortality and morbidity. It is characterized by acute clinical symptoms such as painful vaso-occlusive crises, which can impair health-related quality of life (HRQL). This study was conducted to identify validated patient-reported outcome (PRO) instruments for use in future trials of potential treatments for SCD. METHODS: A systematic literature review (SLR) was performed using MEDLINE and EMBASE to identify United States (US)-based studies published in English between 1997 and 2017 that reported on validated PRO instruments used in randomized controlled trials and real-world settings...
May 21, 2018: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/29778648/impact-of-insulin-on-the-intestinal-microcirculation-in-a-model-of-sepsis-related-hyperglycemia
#3
Alexa Caldwell, Jan Niklas Morick, Anne-Marie Jentsch, Annette Wegener, Dragan Pavlovic, Nadia Al-Banna, Christian Lehmann
BACKGROUND: Sepsis involves dysfunctional glucose metabolism. Among patients with sepsis, hyperglycemia is frequent and insulin administration has been evaluated for glycemic control to improve patient outcomes. Only few studies have examined the hyperglycemic microcirculation and the impact of insulin on the microvasculature in sepsis. OBJECTIVE: To study the functional capillary density (FCD) and leukocyte activation within the intestinal microcirculation in endotoxin-induced experimental sepsis...
May 17, 2018: Microvascular Research
https://www.readbyqxmd.com/read/29755709/perioperative-management-of-sickle-cell-disease
#4
REVIEW
Kwame Ofori Adjepong, Folashade Otegbeye, Yaw Amoateng Adjepong
Over 30 million people worldwide have sickle cell disease (SCD). Emergent and non-emergent surgical procedures in SCD have been associated with relatively increased risks of peri-operative mortality, vaso-occlusive (painful) crisis, acute chest syndrome, post-operative infections, congestive heart failure, cerebrovascular accident and acute kidney injury. Pre-operative assessment must include a careful review of the patient's known crisis triggers, baseline hematologic profile, usual transfusion requirements, pre-existing organ dysfunction and opioid use...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29730854/renal-involvement-in-antiphospholipid-syndrome
#5
REVIEW
Francisco Vileimar Andrade de Azevedo, Diego Germano Maia, Jozelio Freire de Carvalho, Carlos Ewerton Maia Rodrigues
This is a review of scientific publications on renal involvement in antiphospholipid syndrome (APS), with focus on clinical and histopathological findings and treatment. A search for English-language articles on renal involvement in APS covering the period 1980-2017 was conducted in Medline/PubMed and Scopus databases using the MeSH terms "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis" and "thrombotic microangiopathy" (TMA). APS nephropathy is primarily the result of thromboses in renal arteries or veins, intraparenchymatous arteries and glomerular capillaries...
May 5, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29724898/how-i-safely-transfuse-patients-with-sickle-cell-disease-and-manage-delayed-hemolytic-transfusion-reactions
#6
France Pirenne, Karina Yazdanbakhsh
Transfusions can be a life-saving treatment for patients with sickle cell disease (SCD). However, availability of matched units can be limiting due to distinctive blood group polymorphisms in patients of African descent. Development of antibodies against the transfused red blood cells (RBCs), resulting in delayed hemolytic transfusion reactions (DHTRs), can be life-threatening and pose unique challenges for this population with regard to treatment strategies and transfusion management protocols. In cases where the transfused cells as well as patient's own RBCs are destroyed, diagnosis of DHTR can be difficult because symptoms may mimic vaso-occlusive crisis, and frequently antibodies are undetectable...
May 3, 2018: Blood
https://www.readbyqxmd.com/read/29694434/haptoglobin-and-hemopexin-inhibit-vaso-occlusion-and-inflammation-in-murine-sickle-cell-disease-role-of-heme-oxygenase-1-induction
#7
John D Belcher, Chunsheng Chen, Julia Nguyen, Fuad Abdulla, Ping Zhang, Hao Nguyen, Phong Nguyen, Trevor Killeen, Sylvia M Miescher, Nathan Brinkman, Karl A Nath, Clifford J Steer, Gregory M Vercellotti
During hemolysis, hemoglobin and heme released from red blood cells promote oxidative stress, inflammation and thrombosis. Plasma haptoglobin and hemopexin scavenge free hemoglobin and heme, respectively, but can be depleted in hemolytic states. Haptoglobin and hemopexin supplementation protect tissues, including the vasculature, liver and kidneys. It is widely assumed that these protective effects are due primarily to hemoglobin and heme clearance from the vasculature. However, this simple assumption does not account for the consequent cytoprotective adaptation seen in cells and organs...
2018: PloS One
https://www.readbyqxmd.com/read/29690499/levels-of-soluble-endothelium-adhesion-molecules-and-complications-among-sickle-cell-disease-patients-in-ghana
#8
Charles Antwi-Boasiako, Eric S Donkor, Fredericka Sey, Bartholomew Dzudzor, Gifty B Dankwah, Kate H Otu, Alfred Doku, Campbell A Dale, Ivy Ekem
BACKGROUND: Soluble adhesion molecules are involved in the gathering and joining of inflammatory cells to vascular endothelium. Therefore, they serve as potential markers of endothelial dysfunction in vascular diseases including sickle cell disease (SCD). In Ghana, there are scarcely any report on the levels of adhesion molecules among SCD patients. The current study aimed to determine plasma levels of ICAM-1, VCAM-1 and E-Selectin as markers of endothelial dysfunction in SCD patients in steady state, complications and controls...
April 21, 2018: Diseases (Basel)
https://www.readbyqxmd.com/read/29675069/moyamoya-vasculopathy-in-indian-children-our-experience
#9
Varsha Anant Patil, Shilpa Dattaprasanna Kulkarni, Chandrashekhar E Deopujari, Naresh K Biyani, Anaita H Udwadia-Hegde, Krishnakumar N Shah
Background: Moyamoya vasculopathy is a chronic progressive vaso-occlusive disease affecting the distal intracranial carotid arteries and their proximal branches. It is an important cause of recurrent strokes in children. Surgical revascularization procedures are now considered as the treatment option for moyamoya vasculopathy. The data from Indian children with moyamoya vasculopathy are limited to a very few studies. Study Design: We analyzed the records of children with moyamoya vasculopathy treated at our tertiary care center from 2000 to 2014...
October 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29668477/clinical-significance-of-the-cerebral-perforating-arteries
#10
Vaso Antunović, Aleksandar Mirčić, Slobodan Marinković, Luciano Brigante, Miloš Mališ, Biljana Georgievski, Miljana Aksić
There is scarce data in the contemporary literature regarding the correlation of the microanatomy of the perforating arteries, their atherosclerosis, and the ischemia in their territory. In order to examine, at least partially, those parameters, the perforating arteries of 12 brains were microdissected or their vascular casts were obtained. In addition, 30 specimens of the perforators were used for a histological and immunohistochemical study. Finally, radiological images of 14 patients with deep cerebral infarcts were examined following a selection among 62 subjects...
December 1, 2017: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/29663878/role-of-angiogenenic-growth-factors-in-psoriasis-a-review
#11
Tahseena Akhtar, Willayat Yousuf Wani, Mohammad A Kamal, Rajinder Kaur
BACKGROUND: Psoriasis is a chronic inflammatory disease, involving autoimmune mechanism wherein immune system, autoantigens and various environmental triggers all interact to create the systemically diseased state. It is a multifactorial skin disease of complex nature characterized by infiltration of multiple immune cells, kerationcyte proliferation, and increased dermal vascularity. Among all factors, vascular alterations present a significant feature of the disease and angiogenesis seems to have an important role in giving rise to psoriasis phenotype...
April 16, 2018: Current Drug Metabolism
https://www.readbyqxmd.com/read/29661609/assessing-the-effect-of-an-educational-intervention-on-nurses-and-patient-care-assistants-comprehension-and-documentation-of-functional-ability-in-pediatric-patients-with-sickle-cell-disease
#12
Katherine M Bernier, Megan Strobel, Ruth Lucas
PURPOSE: In 2014, the Youth Acute Pain Functional Ability Questionnaire (YAPFAQ) was developed to investigate patient's self-rated functional ability during times of acute pain in the inpatient clinical setting. Although it has great potential, the application of this tool has not been made a standard of care. The purpose of this multiple methods study was to determine if, through an educational intervention, hospital staff could consistently document the YAPFAQ in children with sickle cell disease (SCD) during a vaso-occlusive episode...
April 13, 2018: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/29651930/molecular-insights-into-the-irreversible-mechanical-behavior-of-sickle-hemoglobin
#13
Sumith Yesudasan, Simone A Douglas, Manu O Platt, Xianqiao Wang, Rodney D Averett
Sickle cell disease is caused by the amino acid substitution of glutamic acid to valine, which leads to the polymerization of deoxygenated sickle hemoglobin (HbS) into long strands. These strands are responsible for the sickling of red blood cells (RBCs), making blood hyper-coagulable leading to an increased chance of vaso-occlusive crisis. The conformational changes in sickled RBCs traveling through narrow blood vessels in a highly viscous fluid are critical in understanding; however, there are few studies that investigate the origins of the molecular mechanical behavior of sickled RBCs...
April 13, 2018: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/29622872/beneficial-effect-of-low-fixed-dose-of-hydroxyurea-in-vaso-occlusive-crisis-and-transfusion-requirements-in-adult-hbss-patients-a-prospective-study-in-a-tertiary-care-center
#14
Sudha Sethy, Tribikram Panda, Rabindra Kumar Jena
Significant reduction in morbidity and mortality have been documented in patients with sickle cell disease (HbSS) by most of the studies using hydroxyurea at a dose of 25-35 mg/kg/day or maximum tolerated dose. But toxicities, need for frequent monitoring, compliance and cost are important hurdles particularly in Indian set up. We undertook this study to find out the efficacy, safety compliance rate of low fixed dose of hydroxyurea (10 mg/kg/day) in patients presenting to our hospital and its impact on clinical profile and laboratory parameters...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29614637/inflammation-in-sickle-cell-disease
#15
Nicola Conran, John D Belcher
The primary β-globin gene mutation that causes sickle cell disease (SCD) has significant pathophysiological consequences that result in hemolytic events and the induction of the inflammatory processes that ultimately lead to vaso-occlusion. In addition to their role in the initiation of the acute painful vaso-occlusive episodes that are characteristic of SCD, inflammatory processes are also key components of many of the complications of the disease including autosplenectomy, acute chest syndrome, pulmonary hypertension, leg ulcers, nephropathy and stroke...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614636/autonomic-nervous-system-involvement-in-sickle-cell-disease
#16
Thomas D Coates, Patjanaporn Chalacheva, Lonnie Zeltzer, Michael C K Khoo
Sickle cell disease (SCD) is a genetic disorder of hemoglobin producing hemoglobin-S (HbS) and resulting in recurrent severe episodes of pain, organ damage and premature death due to vaso- occlusion. Deoxy HbS polymerizes, causing red cells to become rigid and lodge in the microvasculature if they do not escape into larger vessels before this transformation occurs. The mechanism that triggers this transition from steady state to vaso-occlusive crisis (VOC) is not known. Patients state that cold, emotional stress, and pain itself can trigger these events...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614635/oxidative-stress-in-sickle-cell-disease-more-than-a-damp-squib
#17
Eduard J van Beers, Richard van Wijk
Sickle cell disease (SCD) is a monogenetic disorder marked by hemolytic anemia and vaso-occlusive complications. The hallmark of SCD is the intracellular polymerization of sickle hemoglobin (HbS) after deoxygenation, and the subsequent characteristic shape change (sickling) of red cells. Vaso-occlusion occurs after endothelial activation, expression of adhesion molecules and subsequent adhesion of leucocytes and sickle erythrocytes to the vascular wall. Here we review how oxidative stress from various sources influences this process...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614630/blood-rheological-abnormalities-in-sickle-cell-anemia
#18
Philippe Connes, Céline Renoux, Marc Romana, Manouk Abkarian, Philippe Joly, Cyril Martin, Marie-Dominique Hardy-Dessources, Samir K Ballas
This review focuses on the contribution of abnormal blood rheology in the pathophysiology of sickle cell anemia (SCA). SCA is characterized by a reduction of red blood cell (RBC) deformability but this reduction is very heterogeneous among patients. Recent works have shown that patients with the lowest RBC deformability (measured by ektacytometry) have enhanced hemolysis and would be more prone to develop several complications such as priapism, leg ulcers and glomerulopathy. In contrast, patients with the highest deformability, and not under hydroxyurea therapy, seem to develop more frequently vaso-occlusive like events...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29614628/comorbidities-in-aging-patients-with-sickle-cell-disease
#19
Samir K Ballas
Sickle cell disease (SCD) in general and sickle cell anemia in particular is a highly complex disorder both at the molecular and clinical levels. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of the disease. Moreover, despite the fact that SCD is a chronic malady, its manifestations are both acute and chronic. The former include, among other things, the recurrent vaso-occlusive crises (its hallmark) and acute chest syndrome...
2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29610772/improving-routine-outpatient-monitoring-for-patients-with-sickle-cell-disease-on-hydroxyurea
#20
Joel Ward, Natasha Lewis, Dimitris A Tsitsikas
Hydroxyurea is the gold standard treatment for prevention of vaso-occlusive crises in patients with sickle-cell anaemia. It has a narrow therapeutic index and dangerous side effects including cytopenias. There is high variation in dose-response across the population. Therefore, a robust outpatient monitoring programme is crucial to ensure efficacy and safety of treatment. However, there has historically been difficulty engaging the target population in regular laboratory test monitoring programmes. This project aimed to ensure that all patients on hydroxyurea had routine blood tests at least once every 2 months which were reviewed and acted upon within the 3-year project life cycle...
2018: BMJ Open Quality
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