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https://www.readbyqxmd.com/read/28110136/impaired-muscle-force-production-and-higher-fatigability-in-a-mouse-model-of-sickle-cell-disease
#1
Benjamin Chatel, Christophe Hourdé, Julien Gondin, Alexandre Fouré, Yann Le Fur, Christophe Vilmen, Monique Bernard, Laurent A Messonnier, David Bendahan
Skeletal muscle function has been scarcely investigated in sickle cell disease (SCD) so that the corresponding impact of sickle hemoglobin is still a matter of debate. The purpose of this study was to investigate muscle force production and fatigability in SCD and to identify whether exercise intensity could have a modulatory effect. Ten homozygous sickle cell (HbSS), ten control (HbAA) and ten heterozygous (HbAS) mice were submitted to two stimulation protocols (moderate and intense) to assess force production and fatigability...
January 11, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28110132/thromboelastographic-characterization-of-the-activated-clotting-system-in-children-with-sickle-cell-trait-or-sickle-cell-disease
#2
Shveta Gupta, Roxana Carmona, Jemily Malvar, Guy Young
BACKGROUND: Recent epidemiological evidence suggests sickle cell disease (SCD) and sickle cell trait (SCT) is a risk factor for venous thromboembolism. The increased in-vivo markers of thrombin generation support the notion that such patients are in a chronic hypercoagulable state. In an attempt to better understand the underlying mechanism, global hemostatic assays including thrombin generation assay (TGA) and thromboelastography (TEG) have been utilized by several groups, but thus far, have shown inconsistent results either due to small sample size or technical differences...
September 21, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/28109314/sickle-cell-disease-and-pregnancy-outcomes-a-study-of-the-community-based-hospital-in-a-tribal-block-of-gujarat-india
#3
Gayatri Desai, Ankit Anand, Pankaj Shah, Shobha Shah, Kapilkumar Dave, Hardik Bhatt, Shrey Desai, Dhiren Modi
BACKGROUND: Sickle cell disease (SCD) is a hereditary blood disorder prevalent in tribal regions of India. SCD can increase complications during pregnancy and in turn negatively influence pregnancy outcomes. This study reports the analysis of tribal maternal admissions in the community-based hospital of SEWA Rural (Kasturba Maternity Hospital) in Jhagadia block, Gujarat. The objective of the study is to compare the pregnancy outcomes among SCD, sickle cell trait and non-SCD admissions...
January 21, 2017: Journal of Health, Population, and Nutrition
https://www.readbyqxmd.com/read/28106560/face-name-associative-recognition-deficits-in-subjective-cognitive-decline-and-mild-cognitive-impairment
#4
Alexandra Polcher, Ingo Frommann, Alexander Koppara, Steffen Wolfsgruber, Frank Jessen, Michael Wagner
BACKGROUND: There is a need for more sensitive neuropsychological tests to detect subtle cognitive deficits emerging in the preclinical stage of Alzheimer's disease (AD). Associative memory is a cognitive function supported by the hippocampus and affected early in the process of AD. OBJECTIVE: We developed a short computerized face-name-associative-recognition test (FNART) and tested whether it would detect memory impairment in memory-clinic patients with mild cognitive impairment (MCI) and subjective cognitive decline (SCD)...
January 19, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28106552/meditation-and-music-improve-memory-and-cognitive-function-in-adults-with-subjective-cognitive-decline-a-pilot-randomized-controlled-trial
#5
Kim E Innes, Terry Kit Selfe, Dharma Singh Khalsa, Sahiti Kandati
BACKGROUND: While effective therapies for preventing or slowing cognitive decline in at-risk populations remain elusive, evidence suggests mind-body interventions may hold promise. OBJECTIVES: In this study, we assessed the effects of Kirtan Kriya meditation (KK) and music listening (ML) on cognitive outcomes in adults experiencing subjective cognitive decline (SCD), a strong predictor of Alzheimer's disease. METHODS: Sixty participants with SCD were randomized to a KK or ML program and asked to practice 12 minutes/day for 3 months, then at their discretion for the ensuing 3 months...
January 18, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28106307/normal-saline-is-associated-with-increased-sickle-red-cell-stiffness-and-prolonged-transit-times-in-a-microfluidic-model-of-the-capillary-system
#6
Marcus A Carden, Meredith Fay, Yumiko Sakurai, Brynn McFarland, Sydney Blanche, Caleb Diprete, Clinton H Joiner, Todd Sulchek, Wilbur A Lam
Vaso-occlusive crisis (VOC) is a complex process that occurs in patients with sickle cell disease (SCD) and is often associated with pain and urgent hospitalization. A major instigator of VOC is microvascular obstruction by pathologically stiffened sickle red blood cells (RBCs), and thus therapy relies heavily on optimizing intravenous fluid (IVF) hydration to increase RBC deformability. However, no evidence-based guidelines regarding the choice of IVF currently exist. We therefore analyzed alterations in biomechanical properties of sickle RBCs isolated from patients with homozygous SCD (hemoglobin SS) after exposure to different osmolarities of clinical IVF formulations...
January 20, 2017: Microcirculation: the Official Journal of the Microcirculatory Society, Inc
https://www.readbyqxmd.com/read/28106161/cold-spells-and-ischaemic-sudden-cardiac-death-effect-modification-by-prior-diagnosis-of-ischaemic-heart-disease-and-cardioprotective-medication
#7
Niilo R I Ryti, Elina M S Mäkikyrö, Harri Antikainen, M Juhani Junttila, Eeva Hookana, Tiina M Ikäheimo, Marja-Leena Kortelainen, Heikki V Huikuri, Jouni J K Jaakkola
Sudden cardiac death (SCD) is the leading cause of death. The current paradigm in SCD requires the presence of an abnormal myocardial substrate and an internal or external transient factor that triggers cardiac arrest. Based on prior mechanistic evidence, we hypothesized that an unusually cold weather event (a cold spell) could act as an external factor triggering SCD. We tested potential effect modification of prior diagnoses and select pharmacological agents disrupting pathological pathways between cold exposure and death...
January 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28104156/long-term-outcomes-in-kidney-transplantation-from-expanded-criteria-donors-after-circulatory-death
#8
Y Tomita, T Tojimbara, K Iwadoh, I Nakajima, S Fuchinoue
The number of recipients waiting for a transplant is increasing. In Japan, there is more frequent use of organs from expanded-criteria donors (ECDs) after circulatory death. We retrospectively analyzed long-term outcomes of kidney transplantation (KT) from expanded-criteria donation after circulatory death (DCD). From 1995 to 2013, 97 cases of KT from DCD donors were performed in our department. Death-censored graft survival rates of ECD kidneys (n = 50) versus standard-criteria deceased-donor (SCD) kidneys (n = 47) for 1, 5, and 10 years after transplantation were 84...
January 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28099557/patients-with-sickle-cell-disease-are-frequently-excluded-from-the-benefits-of-transcranial-doppler-screening-for-the-risk-of-stroke-despite-extensive-and-compelling-evidence
#9
Daniela Laranja Gomes Rodrigues, Samuel Ademola Adegoke, Rejane de Souza Macedo Campos, Josefina Aparecida Pellegrini Braga, Maria Stella Figueiredo, Gisele Sampaio Silva
Methods: Transcranial doppler was performed in accordance with the Stroke Prevention Trial in Sickle Cell Anemia Protocol. Results: Of the 396 patients, 69.5% had homozygous SS hemoglobin. The TCD result was abnormal in 4.8%, conditional in 12.6%, inadequate in 4.3% and abnormally low in 1% of patients. The highest mean flow velocities were 121±23.83cm/s and 124±27.21cm/s in the left and right middle cerebral artery respectively. A total of 28.8% patients (mean age 9...
January 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28099399/the-association-of-serum-25-hydroxyvitamin-d-with-biomarkers-of-hemolysis-in-pediatric-patients-with-sickle-cell-disease
#10
Samuel A Adegoke, Josefina A P Braga, Adekunle D Adekile, Maria S Figueiredo
Although vitamin D deficiency (VDD) has been linked to anemia among sickle cell disease (SCD), its relationship with hemolysis is unclear. Serum 25-hydroxyvitamin D and biomarkers of hemolysis (hemoglobin [Hb]/hematocrit, reticulocyte percentage, absolute reticulocyte, and lactate dehydrogenase [LDH] levels) in 36 hydroxyurea-naive SCD children were quantified. Correlations were significantly positive with Hb/hematocrit (r=0.40, P=0.017; r=0.45, P=0.006, respectively); inverse with reticulocyte percentage, absolute reticulocyte, and LDH (r=-0...
January 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28097661/estimating-the-skull-to-camera-distance-from-facial-photographs-for-craniofacial-superimposition
#11
Carl N Stephan
The overlay of a skull and a face image for identification purposes requires similar subject-to-camera distances (SCD) to be used at both photographic sessions so that differences in perspective do not compromise the anatomical comparisons. As the facial photograph is the reference standard, it is crucial to determine its SCD first and apply this value to photography of the skull. So far, such a method for estimating the SCD has been elusive (some say impossible), compromising the technical validity of the superimposition procedure...
January 18, 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/28097236/lung-vaso-occlusion-in-sickle-cell-disease-mediated-by-arteriolar-neutrophil-platelet-microemboli
#12
Margaret F Bennewitz, Maritza A Jimenez, Ravi Vats, Egemen Tutuncuoglu, Jude Jonassaint, Gregory J Kato, Mark T Gladwin, Prithu Sundd
In patients with sickle cell disease (SCD), the polymerization of intraerythrocytic hemoglobin S promotes downstream vaso-occlusive events in the microvasculature. While vaso-occlusion is known to occur in the lung, often in the context of systemic vaso-occlusive crisis and the acute chest syndrome, the pathophysiological mechanisms that incite lung injury are unknown. We used intravital microscopy of the lung in transgenic humanized SCD mice to monitor acute vaso-occlusive events following an acute dose of systemic lipopolysaccharide sufficient to trigger events in SCD but not control mice...
January 12, 2017: JCI Insight
https://www.readbyqxmd.com/read/28096988/successful-outcome-of-three-patients-with-sickle-cell-disease-and-fat-embolism-syndrome-treated-with-intensive-exchange-transfusion
#13
Paul Greaves, Vivek Mathew, Catherine Peters, Susan Rowe, Roger J Amos, Dimitris A Tsitsikas
Fat embolism syndrome (FES) is a rare complication of sickle-cell disease (SCD) associated with extremely high mortality rates. It affects predominantly non-SS patients and those with previously mild disease. Rapid institution of exchange transfusion with an aim to reduce HbS to very low levels as soon as FES is suspected can be life-saving.
January 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28095750/effects-of-pre-germinated-brown-rice-treatment-high-fat-diet-induced-metabolic-syndrome-in-c57bl-6j-mice
#14
Hsueh-Wei Yen, Hui-Li Lin, Chi-Long Hao, Fu-Chih Chen, Chun-Yun Chen, Jia-Hao Chen, Kuo-Ping Shen
To investigate using pre-germinated brown rice (PGBR) to treat metabolic syndrome, we fed one group of mice standard-regular-diet (SRD) for 20 weeks and another group of mice high-fat-diet (HFD) for 16 weeks. We subdivided them into HFD group and HFD + PGBR group whose dietary carbohydrate was replaced with PGBR for 4 weeks. The HFD group gained more weight, had higher blood pressure, heart rate, blood glucose and lipids, liver levels of TG, feces TG and bile acid, lower adipose levels of adipocytokine, lower skeletal muscle IR, IRS-1, IRS-2, PI3 K, Akt/PKB, GLUT-1, GLUT-4, GCK and PPAR-γ; higher liver SREBP-1, SCD-1, FAS, HMGCR, LDLR, CYP7α1 and PPAR-α, and higher adipose SREBP-1, SCD-1, FAS, and lower adipose PPAR-α and adiponectin...
January 18, 2017: Bioscience, Biotechnology, and Biochemistry
https://www.readbyqxmd.com/read/28091967/balancing-ethical-pros-and-cons-of-stem-cell-derived-gametes
#15
Seppe Segers, Heidi Mertes, Guido de Wert, Wybo Dondorp, Guido Pennings
In this review we aim to provide an overview of the most important ethical pros and cons of stem cell derived gametes (SCD-gametes), as a contribution to the debate about reproductive tissue engineering. Derivation of gametes from stem cells holds promising applications both for research and for clinical use in assisted reproduction. We explore the ethical issues connected to gametes derived from embryonic stem cells (both patient specific and non-patient specific) as well as those related to gametes derived from induced pluripotent stem cells...
January 13, 2017: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/28089760/enhanced-long-term-brain-mri-evaluation-of-children-with-sickle-cell-disease-following-hematopoietic-cell-transplantation
#16
Nancy S Green, Monica Bhatia, Erica Y Griffith, Mahvish Qureshi, Courtney Briamonte, Mirko Savone, Stephen Sands, Margaret T Lee, Angela Lignelli, Adam M Brickman
Progressive neurovasculopathy in children with sickle cell disease (SCD) results in decreased cognitive function and quality of life (QoL). Hematopoietic cell transplant (HCT) is believed to halt progression of neurovasculopathy. Quantitative analysis of T2-weighted fluid attenuated inversion recovery (FLAIR) MRI for white matter hyperintensity (WMH) burden provides a meaningful estimate of small vessel cerebrovascular disease. We asked if quantitative analysis of WMH could complement standardized clinical assessment of MRI/MRA for assessing SCD CNS vasculopathy pre- and post-HCT...
January 9, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28088643/molecular-mechanisms-of-bio-catalysis-of-heme-extraction-from-hemoglobin
#17
Serzhan Sakipov, Olga Rafikova, Maria G Kurnikova, Ruslan Rafikov
Red blood cell hemolysis in sickle cell disease (SCD) releases free hemoglobin. Extracellular hemoglobin and its degradation products, free heme and iron, are highly toxic due to oxidative stress induction and decrease in nitric oxide availability. We propose an approach that helps to eliminate extracellular hemoglobin toxicity in SCD by employing a bacterial protein system that evolved to extract heme from extracellular hemoglobin. NEAr heme Transporter (NEAT) domains from iron-regulated surface determinant proteins from Staphylococcus aureus specifically bind free heme as well as facilitate its extraction from hemoglobin...
January 7, 2017: Redox Biology
https://www.readbyqxmd.com/read/28087964/arrythmogenic-right-ventricular-dysplasia
#18
M K Anis Munirah, W K Lim, I M Sharifah Ainon
A 10-year-old well and asymptomatic female was referred for screening of acute right ventricular dilatation (ARVD) as she had an elder brother diagnosed with ARVD whom died of sudden cardiac death. Electrocardiography (ECG), transthoracic echocardiography (TTE) and cardiac magnetic resonance imaging (CMR) were performed. Results of these investigations were suggestive of ARVD. Despite being a rare cardiac disease and largely unrecognised in children and young adult population, ARVD is an important cause of ventricular arrhythmias in this group of patients and is one of the causes of sudden cardiac death (SCD) in this population...
December 2016: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28081534/enhanced-auditory-sensitivity-to-body-vibrations-in-superior-canal-dehiscence-syndrome
#19
Krister Brantberg, Luca Verrecchia, Magnus Westin
A key feature of superior canal dehiscence (SCD) syndrome is supranormal hearing of body sounds. The aim of the present study was to quantify this phenomenon and to ascertain whether auditory sensitivity to body vibrations can distinguish SCD patients. Hearing thresholds in response to vibration at the vertex, at the spinous process of the 7th cervical vertebra, and at the medial malleolus were tested in 10 SCD patients and 10 controls. Both patients and controls had insert earphones in both ears. The insert in the test ear was blocked while masking was presented to the other ear...
January 13, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28076805/regulation-of-active-icam-4-on-normal-and-sickle-cell-disease-rbcs-via-akaps-is-revealed-by-afm
#20
Jing Zhang, Krithika Abiraman, Sasia-Marie Jones, George Lykotrafitis, Biree Andemariam
Human healthy (wild-type (WT)) and homozygous sickle (SS) red blood cells (RBCs) express a large number of surface receptors that mediate cell adhesion between RBCs, and between RBCs and white blood cells, platelets, and the endothelium. In sickle cell disease (SCD), abnormal adhesion of RBCs to endothelial cells is mediated by the intercellular adhesion molecule-4 (ICAM-4), which appears on the RBC membrane and binds to the endothelial αvβ3 integrin. This is a key factor in the initiation of vaso-occlusive episodes, the hallmark of SCD...
January 10, 2017: Biophysical Journal
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