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Sickle cell disease

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https://www.readbyqxmd.com/read/28214498/vasculitic-and-autoimmune-wounds
#1
REVIEW
Victoria K Shanmugam, Divya Angra, Hamza Rahimi, Sean McNish
OBJECTIVE: Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions...
March 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28212878/evaluation-of-macular-vascular-abnormalities-identified-by-optical-coherence-tomography-angiography-in-sickle-cell-disease
#2
Ian C Han, Mongkol Tadarati, Katia D Pacheco, Adrienne W Scott
PURPOSE: To evaluate macular vascular flow abnormalities identified by optical coherence tomography angiography (OCT-A) in patients with various sickle cell genotypes. DESIGN: Prospective, observational case series. METHODS: This is a single institution case series of adult patients with various sickle cell genotypes. All patients underwent macular OCT-A (Avanti RTVue XR, Optovue Inc, Fremont, CA). Images were analyzed qualitatively for areas of flow loss and quantitatively for measures of foveal avascular area, parafoveal flow, and vascular density...
February 14, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28210918/nutcracker-syndrome-and-sickle-cell-trait-a-perfect-storm-for-hematuria
#3
Amier Ahmad, Samuel K McElwee, Ryan R Kraemer
We describe the case of a 27-year-old woman with a history of sickle cell trait (SCT) who presented with several months of hematuria and was found to have nutcracker syndrome (NCS). While SCT is a common cause of hematuria resulting from renal papillary necrosis, our patient had concomitant abdominal pain and anemia, prompting further evaluation and the subsequent diagnosis of NCS. Interestingly, the anoxia in the left renal vein from NCS predisposes patients with SCT to sickling. Our case highlights key clinical features of both NCS and SCT and the relationship between the two disease processes...
February 16, 2017: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/28210360/management-of-sickle-cell-disease-super-utilizers
#4
EDITORIAL
Gary A Johnson
No abstract text is available yet for this article.
February 2017: Western Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28209642/orbital-infarction-with-haematoma-in-sickle-cell-disease
#5
Matthew R Edmunds, Lucilla Butler
No abstract text is available yet for this article.
February 16, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/28209096/increased-oxidative-stress-alters-nucleosides-metabolite-levels-in-sickle-cell-anemia
#6
Lívia Gelain Castilhos, Juliana Sorraila de Oliveira, Stephen Adeniyi Adefegha, Luana Pereira Magni, Pedro Henrique Doleski, Fatima Husein Abdalla, Cínthia Melazzo de Andrade, Daniela Bitencourt Rosa Leal
OBJECTIVES: This study was conducted to assess the markers of oxidative stress, myeloperoxidase (MPO), acetylcholinesterase (AChE) and xanthine oxidase (XO) activities as well as the levels of nucleotide metabolites in sickle cell anemia (SCA) patients. METHODS: Fifteen SCA treated patients and 30 health subjects (control group) were selected. The markers of oxidative stress (levels of reactive oxygen species (ROS), plasma proteins, carbonyl content, lipid peroxidation (TBARS), total thiols (T-SH), glutathione and catalase activity), MPO, AChE and XO activities as well as the levels of nucleotide metabolites were measured in SCA patients...
February 16, 2017: Redox Report: Communications in Free Radical Research
https://www.readbyqxmd.com/read/28207871/correction-inflammation-in-sickle-cell-disease-differential-and-down-expressed-plasma-levels-of-annexin-a1-protein
#7
Lidiane S Torres, Jéssika V Okumura, Danilo G H Silva, Kallyne K O Mimura, Édis Belini-Júnior, Renan G Oliveira, Clarisse L C Lobo, Sonia M Oliani, Claudia R Bonini-Domingos
[This corrects the article DOI: 10.1371/journal.pone.0165833.].
2017: PloS One
https://www.readbyqxmd.com/read/28203649/increased-iron-export-by-ferroportin-induces-restriction-of-hiv-1-infection-in-sickle-cell-disease
#8
Namita Kumari, Tatiana Ammosova, Sharmin Diaz, Xionghao Lin, Xiaomei Niu, Andrey Ivanov, Marina Jerebtsova, Subhash Dhawan, Patricia Oneal, Sergei Nekhai
The low incidence of HIV-1 infection in patients with sickle cell disease (SCD) and inhibition of HIV-1 replication in vitro under the conditions of low intracellular iron or heme treatment suggests a potential restriction of HIV-1 infection in SCD. We investigated HIV-1 ex vivo infection of SCD peripheral blood mononuclear cells (PBMCs) and found that HIV-1 replication was inhibited at the level of reverse transcription (RT) and transcription. We observed increased expression of heme and iron-regulated genes, previously shown to inhibit HIV-1, including ferroportin, IKBα, HO-1, p21, and SAM domain and HD domain-containing protein 1 (SAMHD1)...
December 27, 2016: Blood Adv
https://www.readbyqxmd.com/read/28195097/phenotypic-expression-of-hbo-indonesia-in-two-indian-families-and-its-interaction-with-sickle-hemoglobin
#9
Amar Das Gupta, Anita Nadkarni, Pallavi Mehta, Manju Goriwale, Manisha Ramani, Pradnya Chaudhary, Vishal Mehrotra, Roshan Colah
BACKGROUND: Alpha globin chain variants are clinically significant since they directly influence the structure and function of the hemoglobin (Hb) molecules they constitute, either in combination with normal beta globin chains or with variant beta chains, thereby altering the morbidity and mortality associated with the resultant hemoglobinopathies. We describe here two unrelated families from Madhya Pradesh who had a nondeletional alpha-chain variant, HbO Indonesia (CD116 G → A). Members of one of the two families also had coinheritance of sickle hemoglobin (HbS)...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28192225/a-randomized-trial-of-yoga-for-children-hospitalized-with-sickle-cell-vaso-occlusive-crisis
#10
Karen Moody, Bess Abrahams, Rebecca Baker, Ruth Santizo, Deepa Manwani, Veronica Carullo, Doris Eugenio, Aaron Carroll
CONTEXT: Sickle cell disease (SCD) vaso-occlusive crisis (VOC) remains an important cause of acute pain in pediatrics and the most common SCD complication. Pain management recommendations in SCD include nonpharmacological interventions. Yoga is one nonpharmacological intervention that has been shown to reduce pain in some populations, however, evidence is lacking in children with VOC. OBJECTIVES: The primary objective of this study was to compare the effect of yoga versus an attention control on pain in children with VOC...
February 9, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28190778/genome-editing-for-sickle-cell-disease-a-little-bcl11a-goes-a-long-way
#11
EDITORIAL
Mir A Hossain, Jörg Bungert
No abstract text is available yet for this article.
February 9, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28186661/brain-neurochemical-and-hemodynamic-findings-in-the-ny1dd-mouse-model-of-mild-sickle-cell-disease
#12
Min-Hui Cui, Sandra M Suzuka, Nicholas A Branch, Kamalakar Ambadipudi, Sangeetha Thangaswamy, Seetharama A Acharya, Henny H Billett, Craig A Branch
To characterize the cerebral profile associated with sickle cell disease (SCD), we used in vivo proton MRI and MRS to quantify hemodynamics and neurochemicals in the thalamus of NY1DD mice, a mild model of SCD, and compared them with wild-type (WT) control mice. Compared with WT mice, NY1DD mice at steady state had elevated cerebral blood flow (CBF) and concentrations of N-acetylaspartate (NAA), glutamate (Glu), alanine, total creatine and N-acetylaspartylglutamate. Concentrations of glutathione (GSH) at steady state showed a negative correlation with BOLD signal change in response to 100% oxygen, a marker for oxidative stress, and mean diffusivity assessed using diffusion-tensor imaging, a marker for edematous inflammation...
February 10, 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/28185828/the-role-of-molecular-typing-and-perfect-match-transfusion-in-sickle-cell-disease-and-thalassaemia-an-innovative-transfusion-strategy
#13
Rossana Putzulu, Nicola Piccirillo, Nicoletta Orlando, Giuseppina Massini, Maddalena Maresca, Fernando Scavone, Bianca Maria Ricerca, Gina Zini
Chronic red blood cell transfusions remain an essential part of supportive treatment in patients with thalassaemia and sickle cell disease (SCD). Red blood cell (RBC) transfusions expose patients to the risk of developing antibodies: RBC alloimmunization occurs when the immune system meets foreign antigens. We created a register of extensively genotyped donors to achieve a better matched transfusion in order to reduce transfusion alloimmunization. Extended RBC antigen typing was determined and confirmed by molecular biology techniques using Human Erythrocyte Antigen (HEA) BeadChip (BioArray Solutions Ltd...
January 23, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28183857/coexistent-sickle-cell-disease-has-no-impact-on-the-safety-or-outcome-of-lytic-therapy-in-acute-ischemic-stroke-findings-from-get-with-the-guidelines-stroke
#14
Robert J Adams, Margueritte Cox, Shelly D Ozark, Julie Kanter, Phillip J Schulte, Ying Xian, Gregg C Fonarow, Eric E Smith, Lee H Schwamm
BACKGROUND AND PURPOSE: The recommended treatment for ischemic stroke is tPA (tissue-type plasminogen activator). Although sickle cell disease (SCD) represents no known contraindication to tPA, National Heart Lung and Blood Institute of the National Institutes of Health recommended acute exchange transfusion for stroke in SCD, not tPA. Data on safety and outcomes of tPA in patients are needed to guide tPA use in SCD. METHODS: We matched patients from the American Heart Association and American Stroke Association Get With The Guidelines-Stroke registry with SCD to patients without SCD and compared usage, complications, and discharge outcomes after tPA...
February 9, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28175446/198%C3%A2-effectiveness-of-surgical-revascularization-for-stroke-prevention-in-pediatric-patients-with-sickle-cell-disease-and-moyamoya-syndrome
#15
Wuyang Yang, Jose Luis Porras, Risheng Xu, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander Lewis Coon, Rafael J Tamargo, Judy Huang, Edward Sanghoon Ahn
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
https://www.readbyqxmd.com/read/28174191/augmentation-of-muscle-blood-flow-by-ultrasound-cavitation-is-mediated-by-atp-and-purinergic-signaling
#16
J Todd Belcik, Brian P Davidson, Aris Xie, Melinda D Wu, Mrinal Yadava, Yue Qi, Sherry Liang, Chae Ryung Chon, Azzdine Y Ammi, Joshua Field, Leanne Harmann, William M Chilian, Joel Linden, Jonathan R Lindner
BACKGROUND: - Augmentation of tissue blood flow by therapeutic ultrasound is thought to rely on convective shear. Microbubble contrast agents that undergo ultrasound-mediated cavitation markedly amplify these effects. We hypothesized that purinergic signalling is responsible for shear-dependent increases in muscle perfusion during therapeutic cavitation. METHODS: -Unilateral exposure of the proximal hindlimb of mice (with or without ischemia produced by iliac ligation) to therapeutic ultrasound (1...
February 7, 2017: Circulation
https://www.readbyqxmd.com/read/28173069/a-genetic-variation-associated-with-plasma-erythropoietin-and-a-non-coding-transcript-of-prkar1a-in-sickle-cell-disease
#17
Xu Zhang, Binal N Shah, Wei Zhang, Santosh L Saraf, Galina Miasnikova, Adelina Sergueeva, Tatiana Ammosova, Xiaomei Niu, Mehdi Nouraie, Sergei Nekhai, Oswaldo Castro, Mark T Gladwin, Josef T Prchal, Joe G N Garcia, Roberto F Machado, Victor R Gordeuk
No abstract text is available yet for this article.
October 15, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28170418/impact-of-prematurity-and-immigration-on-neonatal-screening-for-sickle-cell-disease
#18
Ernesto Cortés-Castell, Antonio Palazón-Bru, Carolina Pla, Mercedes Goicoechea, María Mercedes Rizo-Baeza, Mercedes Juste, Vicente Francisco Gil-Guillén
BACKGROUND: Others have described a relationship between hemoglobin A levels and gestational age, gender and ethnicity. However, studies are needed to determine normal cut-off points considering these factors. To address this issue we designed a study to determine the percentiles of normality of neonatal hemoglobin A levels taking these factors into account. METHODS: This cross-sectional study involved 16,025 samples for sickle cell disease screening in the province of Alicante, Spain, which has a high immigration rate...
2017: PloS One
https://www.readbyqxmd.com/read/28163560/american-society-of-hematology-annual-meeting-and-exposition
#19
Walter Alexander
Key sessions on pharmacotherapy clinical trials across a wide span of disease states, including leukemia, graft-versus-host disease, myelofibrosis, multiple myeloma, and sickle cell disease, are reviewed.
February 2017: P & T: a Peer-reviewed Journal for Formulary Management
https://www.readbyqxmd.com/read/28162517/-283-beta2-adrenergic-receptor-gene-polymorphisms-and-haplotypes-associate-with-chronic-pain-in-sickle-cell-disease
#20
E Jhun, Y He, Y Yao, D Wilkie, R Molokie, J Wang
No abstract text is available yet for this article.
April 2016: Journal of Pain: Official Journal of the American Pain Society
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