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https://www.readbyqxmd.com/read/29337222/blockade-of-placental-growth-factor-reduces-vaso-occlusive-complications-in-murine-models-of-sickle-cell-disease
#1
Jian-Ming Gu, Shujun Yuan, Derek Sim, Keith Abe, Perry Liu, Martin Rosenbruch, Peter Bringmann, Katalin Kauser
Vaso-occlusive crisis (VOC) is the most common and debilitating complication of sickle cell disease (SCD); recurrent episodes cause organ damage and contribute to early mortality. Plasma placental growth factor (PlGF) levels are elevated in SCD and can further increase under hypoxic conditions in SCD mice. Treatment with a PlGF-neutralizing antibody (anti-PlGF Ab) in SCD mice reduced levels of monocyte chemoattractant protein-3, eotaxin, macrophage colony-stimulating factor, plasminogen activator inhibitor-1 significantly, and macrophage-derived chemokine and macrophage inflammatory protein-3β moderately; this may contribute to inhibition of leukocyte recruitment, activation, and thrombosis...
January 11, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29318647/realizing-effectiveness-across-continents-with-hydroxyurea-enrollment-and-baseline-characteristics-of-the-multicenter-reach-study-in-sub-saharan-africa
#2
Patrick T McGann, Thomas N Williams, Peter Olupot-Olupot, George A Tomlinson, Adam Lane, José Luís Reis da Fonseca, Robert Kitenge, George Mochamah, Ham Wabwire, Susan Stuber, Thad A Howard, Kathryn McElhinney, Banu Aygun, Teresa Latham, Brígida Santos, Léon Tshilolo, Russell E Ware
Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries...
January 10, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29311011/-screening-for-nephropathy-in-major-sickle-cell-syndromes-in-patients-monitored-at-the-national-reference-center-for-sickle-cell-disease-in-niamey-niger
#3
Moumouni Garba, Zeinabou Maiga Moussa Tondi, Hassan Diongoule, Samaila Aboubacar, Abdou Ide, Nadège Ruddy Biyao-Nelson, Illiassou Soumaila, Soumana Alido
BACKGROUND: Sickle cell anemia is the most common hereditary hemopathy in the world. It is a disease that attacks all the systems of the organism. The kidneys are among the most sensitive organs of this disease. The main objective of this study is to detect sickle cell nephropathy in patients followed at the National Reference Center for Sickle Cell Disease in Niamey. METHODS: It is a prospective study carried out over a period of one year (January to December 2016)...
January 5, 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29297515/erratum-field-jj-majerus-e-gordeuk-vr-et-al-randomized-phase-2-trial-of-regadenoson-for-treatment-of-acute-vaso-occlusive-crises-in-sickle-cell-disease-blood-adv-2017-1-20-1645-1649
#4
(no author information available yet)
[This corrects the article DOI: 10.1182/bloodadvances.2017009613.].
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296811/randomized-phase-2-trial-of-regadenoson-for-treatment-of-acute-vaso-occlusive-crises-in-sickle-cell-disease
#5
Joshua J Field, Elaine Majerus, Victor R Gordeuk, Michel Gowhari, Carolyn Hoppe, Matthew M Heeney, Maureen Achebe, Alex George, Hillary Chu, Brian Sheehan, Maneka Puligandla, Donna Neuberg, Gene Lin, Joel Linden, David G Nathan
Adenosine A2A receptor (A2AR) agonists have been shown to decrease tissue inflammation induced by hypoxia/reoxygenation in mice with sickle cell disease (SCD). The key mediator of the A2AR agonist's anti-inflammatory effects is a minor lymphocyte subset, invariant natural killer T (iNKT) cells. We tested the hypothesis that administration of an A2AR agonist in patients with SCD would decrease iNKT cell activation and dampen the severity of vaso-occlusive (VO) crises. In a phase 2, randomized, placebo-controlled trial, we administered a 48-hour infusion of the A2AR agonist regadenoson (1...
September 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296801/pharmacotherapeutical-strategies-in-the-prevention-of-acute-vaso-occlusive-pain-in-sickle-cell-disease-a-systematic-review
#6
REVIEW
Joep W R Sins, David J Mager, Shyrin C A T Davis, Bart J Biemond, Karin Fijnvandraat
Sickle-cell disease (SCD) is characterized by frequent and painful vaso-occlusive crises (VOCs). Various treatments have been evaluated over the years. However, a clear overview is lacking. The objective of this study was to systematically review all pharmacotherapeutical strategies in the prevention of VOCs beyond hydroxyurea. We performed a systematic literature search (MEDLINE, Embase, CENTRAL). Eligible studies were controlled clinical trials evaluating pharmacotherapeutical interventions targeting the reduction of VOCs in patients with SCD...
August 22, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296770/osteonecrosis-of-the-femoral-head-in-sickle-cell-disease-prevalence-comorbidities-and-surgical-outcomes-in-california
#7
Oyebimpe Adesina, Ann Brunson, Theresa H M Keegan, Ted Wun
Osteonecrosis of the femoral head (ONFH) is a prevalent complication of sickle cell disease (SCD) that has not been well described in population-based cohort studies. Using California's Office of Statewide Planning and Development discharge databases (1991-2013), we estimated the cumulative incidence of ONFH after accounting for the competing risk of death and used a multivariable Cox proportional hazards regression to identify factors associated with ONFH diagnosis. We also calculated rates of readmissions to the hospital or emergency department within 30 to 90 days of hip replacement surgery...
July 11, 2017: Blood Advances
https://www.readbyqxmd.com/read/29279787/acute-chest-syndrome-in-children-with-sickle-cell-disease
#8
REVIEW
Shilpa Jain, Nitya Bakshi, Lakshmanan Krishnamurti
Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray...
December 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/29260101/antiphosphospholipid-syndrome-presenting-with-amaurosis-fugax-and-cotton-wool-spots
#9
Kimberly D Tran, Sarah P Read, Nimesh A Patel, Harry W Flynn, Norman J Schatz
Purpose: To describe the importance of considering vaso-occlusive disease on the differential diagnosis of a patient presenting with amaurosis fugax (AF) and unilateral cotton wool spots (CWS). Observations: A 69-year-old female with history of obesity, hyperlipidemia and recent orthopedic surgery, presented with 3 days of worsening monocular AF and CWS in the right eye. She was diagnosed with antiphosphospholipid syndrome based on positive serologic testing for antiphosphatidylserine IgM, anticardiolipin IgM...
September 2017: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29259931/bone-marrow-necrosis-and-fat-embolism-syndrome-a-dreadful-complication-of-hemoglobin-sickle-cell-disease
#10
Eduardo Pelegrineti Targueta, André Carramenha de Góes Hirano, Fernando Peixoto Ferraz de Campos, João Augusto Dos Santos Martines, Silvana Maria Lovisolo, Aloisio Felipe-Silva
Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management...
October 2017: Autopsy & Case Reports
https://www.readbyqxmd.com/read/29258454/atypical-acute-retinal-necrosis-accompanied-by-terson-s-syndrome-a-case-report
#11
Jong Young Lee, Dong Yoon Kim, Hye Jin Lee, Jin Ho Jeong, Sung Pyo Park, Jin Young Kim
BACKGROUND: Acute retinal necrosis (ARN) has characterized by panuveitis, vitritis, severe vaso-occlusive vasculitis, and diffuse necrotizing retinitis. There are no case reports on atypical ARN combined with Terson's syndrome. Herein, we report a case of ARN with atypical clinical features combined with Terson's syndrome that we successfully treated by intravitreal ganciclovir injection. CASE PRESENTATION: A 64-year-old man visited our eye clinic with a complaint of decreased visual acuity in his right eye...
December 19, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/29251012/enhancing-effect-of-hydroxyurea-on-hb-f-in-sickle-cell-disease-ten-year-egyptian-experience
#12
Ilham Youssry, Amina Abdel-Salam, Rania Ismail, Rayan Bou-Fakhredin, Rania Mohamed Samy, Fatma Ezz El-Deen, Ali T Taher
Patients with sickle cell disease experience hemolytic anemia and vaso-occlusions that result in pain, organ injury, and premature mortality. Several prospective studies have verified the efficacy and tolerability of hydroxyurea (HU), and demonstrated its efficacy in reducing painful vaso-occlusive crises (VOCs) in addition to its ability to increase Hb F levels. We aimed to evaluate the long-term effects of HU therapy on Hb F and assess its long term efficacy and safety in sickle cell disease patients. A retrospective study on 60 sickle cell disease patients was conducted...
December 18, 2017: Hemoglobin
https://www.readbyqxmd.com/read/29243035/systemic-lupus-erythematosus-and-ocular-involvement-an-overview
#13
REVIEW
Rosanna Dammacco
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control...
December 14, 2017: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/29224750/blood-transfusion-in-children-with-sickle-cell-disease-undergoing-tonsillectomy
#14
Carlyn M Atwood, Sharon H Gnagi, Ronald J Teufel, Shaun A Nguyen, David R White
INTRODUCTION: Tonsillectomy is the second most common surgery in children with sickle cell disease. These children are at an increased risk of perioperative complications due to vaso-occlusive events. Although controversial, preoperative blood transfusions are sometimes given in an effort to prevent such complications. The purpose of this study is to analyze trends in the use of blood transfusion for management of children with sickle cell disease (SCD) undergoing tonsillectomy in a national database...
December 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29222304/targeting-novel-mechanisms-of-pain-in-sickle-cell-disease
#15
REVIEW
Huy Tran, Mihir Gupta, Kalpna Gupta
Patients with sickle cell disease (SCD) suffer from intense pain that can start during infancy and increase in severity throughout life, leading to hospitalization and poor quality of life. A unique feature of SCD is vaso-occlusive crises (VOCs) characterized by episodic, recurrent, and unpredictable episodes of acute pain. Microvascular obstruction during a VOC leads to impaired oxygen supply to the periphery and ischemia reperfusion injury, inflammation, oxidative stress, and endothelial dysfunction, all of which may perpetuate a noxious microenvironment leading to pain...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222302/pain-measurement-tools-in-sickle-cell-disease-where-are-we-now
#16
REVIEW
Deepika S Darbari, Amanda M Brandow
Pain is a complex multidimensional experience and the most common morbidity in patients with sickle cell disease (SCD). Tools to assess pain can be of use not only to guide pain treatment but also to provide insight into underlying pain neurobiology. Mechanisms of pain in SCD are multifactorial and are not completely elucidated. Although vaso-occlusion of microcirculation by sickled red cells is believed to be the underlying mechanism of acute vaso-occlusive pain, mechanisms for chronic pain and the transition from acute to chronic pain are under investigation...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222291/evolving-treatment-paradigms-in-sickle-cell-disease
#17
REVIEW
Ramasamy Jagadeeswaran, Angela Rivers
Sickle cell disease (SCD) is an inheritable hemoglobinopathy characterized by polymerization of hemoglobin S in red blood cells resulting in chronic hemolytic anemia, vaso-occlusive painful crisis, and multiorgan damage. In SCD, an increased reactive oxygen species (ROS) generation occurs both inside the red blood cells and inside the vascular lumen, which augment hemolysis and cellular adhesion. This review discusses the evolving body of literature on the role of ROS in the pathophysiology of SCD as well as some emerging therapeutic approaches to SCD with a focus on the reduction of ROS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222289/what-is-the-role-of-screening-for-pulmonary-hypertension-in-adults-and-children-with-sickle-cell-disease
#18
REVIEW
Shaina M Willen, Mark T Gladwin
Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography. The patient's case is described in detail in Lancet1 He had been treated with regular transfusions since childhood for stroke, had rare episodes of vaso-occlusive pain episodes, and did not take narcotic pain medications. He presented with progressive severe dyspnea on exertion and lower extremity edema. His laboratory tests were notable for a total hemoglobin level of 11...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222288/cardiovascular-complications-in-patients-with-sickle-cell-disease
#19
REVIEW
Mark T Gladwin
Sickle cell disease (SCD) is an autosomal recessive disease in which homozygosity for a single point mutation in the gene encoding the β-globin chain produces hemoglobin S molecules that polymerize within the erythrocyte during deoxygenation; the result is sustained hemolytic anemia and vaso-occlusive events. As patients live to adulthood, the chronic impact of sustained hemolytic anemia and episodic vaso-occlusive episodes leads to progressive end-organ complications. This scenario culminates in the development of 1 or more major cardiovascular complications of SCD for which there are no approved or consensus therapies...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29214337/sleep-disordered-breathing-in-patients-with-sickle-cell-disease
#20
REVIEW
Vikram M Raghunathan, Peter L Whitesell, Seah H Lim
Sickle cell disease is one of the most common hereditary hemoglobinopathies worldwide, and its vaso-occlusive and hemolytic crises cause considerable patient morbidity. A growing body of evidence has shown that sleep-disordered breathing, and in particular, obstructive sleep apnea, occurs at high frequency in the sickle cell population, and that there is significant overlap in the underlying pathophysiology of these two conditions. Through a variety of mechanisms including nocturnal hypoxemia and increased oxidative stress, production of pro-inflammatory cytokines, and endothelial dysfunction, sickle cell anemia and sleep-disordered breathing potentiate each other's clinical effects and end-organ complications...
December 6, 2017: Annals of Hematology
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