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https://www.readbyqxmd.com/read/28221266/body-mass-index-and-the-association-with-vaso-occlusive-crises-in-pediatric-sickle-cell-disease
#1
Andrea Zivot, Nataly Apollonsky, Edward Gracely, Deepti Raybagkar
BACKGROUND: Children with sickle cell disease (SCD) historically have been underweight and have poor overall growth. Recent studies have demonstrated a trend toward obesity in pediatric SCD populations. MATERIALS AND METHODS: Through retrospective chart review of patients with SCD followed at our center, we collected patient's data, including body mass index (BMI), weight percentiles, sickle cell genotype, baseline hemoglobin, medical and psychiatric comorbidities, 25-hydroxy vitamin D level, treatment with hydroxyurea, and chronic transfusions...
February 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28192225/a-randomized-trial-of-yoga-for-children-hospitalized-with-sickle-cell-vaso-occlusive-crisis
#2
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/28159390/sickle-cell-disease
#3
REVIEW
Russell E Ware, Mariane de Montalembert, Léon Tshilolo, Miguel R Abboud
Sickle cell disease is a common and life-threatening haematological disorder that affects millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in small vessels, and this vaso-occlusion leads to distal tissue ischaemia and inflammation, with symptoms defining the acute painful sickle-cell crisis. Repeated sickling and ongoing haemolytic anaemia, even when subclinical, lead to parenchymal injury and chronic organ damage, causing substantial morbidity and early mortality. Currently available treatments are limited to transfusions and hydroxycarbamide, although stem cell transplantation might be a potentially curative therapy...
January 31, 2017: Lancet
https://www.readbyqxmd.com/read/28152086/nnktt120-an-anti-inkt-cell-monoclonal-antibody-produces-rapid-and-sustained-inkt-cell-depletion-in-adults-with-sickle-cell-disease
#4
Joshua J Field, Elaine Majerus, Kenneth I Ataga, Elliot P Vichinsky, Robert Schaub, Robert Mashal, David G Nathan
: Invariant NKT (iNKT) cells can be activated to stimulate a broad inflammatory response. In murine models of sickle cell disease (SCD), interruption of iNKT cell activity prevents tissue injury from vaso-occlusion. NKTT120 is an anti-iNKT cell monoclonal antibody that has the potential to rapidly and specifically deplete iNKT cells and, potentially, prevent vaso-occlusion. We conducted an open-label, multi-center, single-ascending-dose study of NKTT120 to determine its pharmacokinetics, pharmacodynamics and safety in steady-state patients with SCD...
2017: PloS One
https://www.readbyqxmd.com/read/28138561/cd44-expression-in-endothelial-colony-forming-cells-regulates-neurovascular-trophic-effect
#5
Susumu Sakimoto, Valentina Marchetti, Edith Aguilar, Kelsey Lee, Yoshihiko Usui, Salome Murinello, Felicitas Bucher, Jennifer K Trombley, Regis Fallon, Ravenska Wagey, Carrie Peters, Elizabeth L Scheppke, Peter D Westenskow, Martin Friedlander
Vascular abnormalities are a common component of eye diseases that often lead to vision loss. Vaso-obliteration is associated with inherited retinal degenerations, since photoreceptor atrophy lowers local metabolic demands and vascular support to those regions is no longer required. Given the degree of neurovascular crosstalk in the retina, it may be possible to use one cell type to rescue another cell type in the face of severe stress, such as hypoxia or genetically encoded cell-specific degenerations. Here, we show that intravitreally injected human endothelial colony-forming cells (ECFCs) that can be isolated and differentiated from cord blood in xeno-free media collect in the vitreous cavity and rescue vaso-obliteration and neurodegeneration in animal models of retinal disease...
January 26, 2017: JCI Insight
https://www.readbyqxmd.com/read/28133359/myonecrosis-in-sickle-cell-anemia-case-study
#6
Lalita Prabha Turaga, Prajwal Boddu, Steve Kipferl, Anupam Basu, Martin Yorath
BACKGROUND Myonecrosis is one of the more poorly studied, painful manifestations of sickle cell crisis. Medical literature is sparse detailing the manifestations and management of such symptoms. In myonecrosis, red cells containing sickle hemoglobin become rigid, resulting in reduced blood flow and myonecrosis. CASE REPORT We present a case study of a patient in sickle cell crisis with an episode of acute pain and swelling to the intrinsic muscles of the foot as a prominent feature of the crises. Although muscle biopsy is considered the gold standard for the diagnosis of myositis or myonecrosis, a low intensity signal on T1 and high intensity signal on T2 at the affected muscle belly can be as conclusive as imaging studies...
January 30, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28129479/a-microfluidic-platform-to-study-the-effects-of-vascular-architecture-and-oxygen-gradients-on-sickle-blood-flow
#7
Xinran Lu, Michelle M Galarneau, John M Higgins, David K Wood
OBJECTIVE: Our goal was to develop a model of the microvasculature that would allow us to quantify changes in the rheology of sickle blood as it traverses the varying vessel sizes and oxygen tensions in the microcirculation. METHODS: We designed and implemented a microfluidic model of the microcirculation that comprises a branching microvascular network and physiologic oxygen gradients. We used computational modeling to determine the parameters necessary to generate stable, linear gradients in our devices...
January 27, 2017: Microcirculation: the Official Journal of the Microcirculatory Society, Inc
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
#8
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/28105733/vitamin-d-supplementation-for-sickle-cell-disease
#9
REVIEW
Htoo Htoo Kyaw Soe, Adinegara Bl Abas, Nan Nitra Than, Han Ni, Jaspal Singh, Abdul Razzak Bin Mohd Said, Ifeyinwa Osunkwo
BACKGROUND: Sickle cell disease is a genetic chronic haemolytic and pro-inflammatory disorder. The clinical manifestations of sickle cell disease result from the presence of mutations on the beta globin genes that generate an abnormal haemoglobin product (called haemoglobin S) within the red blood cell. Sickle cell disease can lead to many complications such as acute chest syndrome, stroke, acute and chronic bone complications (including painful vaso-occlusive crisis, osteomyelitis, osteonecrosis and osteoporosis)...
January 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28099660/socioeconomic-hygienic-and-sanitation-factors-in-reducing-diarrhea-in-the-amazon
#10
Katiuscia Shirota Imada, Thiago Santos de Araújo, Pascoal Torres Muniz, Valter Lúcio de Pádua
OBJECTIVE: To analyze the contributions of the socioeconomic, hygienic, and sanitation improvements in reducing the prevalence of diarrhea in a city of the Amazon. METHODS: In this population-based cross-sectional study, we analyzed data from surveys conducted in the city of Jordão, Acre. In 2005 and 2012, these surveys evaluated, respectively, 466 and 826 children under five years old. Questionnaires were applied on the socioeconomic conditions, construction of houses, food and hygienic habits, and environmental sanitation...
December 22, 2016: Revista de Saúde Pública
https://www.readbyqxmd.com/read/28099589/which-coronary-lesions-are-more-prone-to-cause-acute-myocardial-infarction
#11
Taner Sen, Mehmet Ali Astarcioglu, Osman Beton, Lale Dinc Asarcikli, Celal Kilit
Background: According to common belief, most myocardial infarctions (MIs) are due to the rupture of nonsevere, vulnerable plaques with < 70% obstruction. Data from recent trials challenge this belief, suggesting that the risk of coronary occlusion is, in fact, much higher after severe stenosis. The aim of this study was to investigate whether or not acute ST-elevation MIs result from high-grade stenoses by evaluating the presence of coronary collateral circulation (CCC). Methods: We retrospectively included 207 consecutive patients who had undergone primary percutaneous coronary intervention for acute ST-elevation MI...
January 16, 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28099586/serotoninergic-modulation-of-basal-cardiovascular-responses-and-responses-induced-by-isotonic-extracellular-volume-expansion-in-rats
#12
Isadora Ferraz Semionatto, Adrieli Oliveira Raminelli, Angelica Cristina Alves, Caroline Santos Capitelli, Rosangela Soares Chriguer
Background: Isotonic blood volume expansion (BVE) induced alterations of sympathetic and parasympathetic activity in the heart and blood vessels, which can be modulated by serotonergic pathways. Objective: To evaluate the effect of saline or serotonergic agonist (DOI) administration in the hypothalamic paraventricular nucleus (PVN) on cardiovascular responses after BVE. Methods: We recorded pulsatile blood pressure through the femoral artery to obtain the mean arterial pressure (MAP), systolic (SBP) and diastolic blood pressure (DBP), heart rate (HR) and the sympathetic-vagal ratio (LF/HF) of Wistar rats before and after they received bilateral microinjections of saline or DOI into the PVN, followed by BVE...
January 16, 2017: Arquivos Brasileiros de Cardiologia
https://www.readbyqxmd.com/read/28097791/alpha-thalassaemia-promotes-frequent-vaso-occlusive-crises-in-children-with-sickle-cell-anaemia-through-haemorheological-changes
#13
Céline Renoux, Philippe Connes, Elie Nader, Sarah Skinner, Camille Faes, Marie Petras, Yves Bertrand, Nathalie Garnier, Daniela Cuzzubbo, Lydia Divialle-Doumdo, Kamila Kebaïli, Cécile Renard, Alexandra Gauthier, Maryse Etienne-Julan, Giovanna Cannas, Cyril Martin, Marie-Dominique Hardy-Dessources, Vincent Pialoux, Marc Romana, Philippe Joly
BACKGROUND: Sickle cell anaemia (SCA) is a severe hereditary haemoglobinopathy characterised by haemorheological abnormalities, which play a role in the occurrence of several acute and chronic clinical complications. While β(S) -haplotypes and alpha-thalassaemia modulate SCA clinical severity, their effects on blood rheology have been incompletely described. The aim of this study was to test the effects of these genetic modifiers on the haemorheological properties and clinical complication of children with SCA...
January 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28097236/lung-vaso-occlusion-in-sickle-cell-disease-mediated-by-arteriolar-neutrophil-platelet-microemboli
#14
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/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#15
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28088737/the-hemostatic-effect-study-of-cirsium-setosum-on-regulating-%C3%AE-1-ars-via-mediating-norepinephrine-synthesis-by-enzyme-catalysis
#16
Nianwei Chang, Yanmei Li, Mengge Zhou, Jie Gao, Yuanyuan Hou, Min Jiang, Gang Bai
BACKGROUND: Cirsium setosum (CS) is the aboveground part of Cephalanoplos segetum Kitam. Although it has been used as a hemostatic treatment for thousands of years and is still in use today, the mechanism of CS on regulating ARs is still not clear. PURPOSE: In this study, we aimed to clarify the mechanism of CS on regulating ARs. METHODS: We developed a simple method based on UPLC/Q-TOF MS combined adrenergic receptor dual-luciferase reporter assay systems for the rapid determination of active constituents in CS...
March 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28088487/adenosine-receptors-and-caffeine-in-retinopathy-of-prematurity
#17
REVIEW
Jiang-Fan Chen, Shuya Zhang, Rong Zhou, Zhenlang Lin, Xiaohong Cai, Jing Lin, Yuqing Huo, Xiaoling Liu
Retinopathy of prematurity (ROP) is a major cause of childhood blindness in the world and is caused by oxygen-induced damage to the developing retinal vasculature, resulting in hyperoxia-induced vaso-obliteration and subsequent delayed retinal vascularization and hypoxia-induced pathological neovascularization driven by vascular endothelial growth factor (VEGF) signaling pathway in retina. Current anti-VEGF therapy has shown some effective in a clinical trial, but is associated with the unintended effects on delayed eye growth and retinal vasculature development of preterm infants...
January 11, 2017: Molecular Aspects of Medicine
https://www.readbyqxmd.com/read/28076855/microrna-126-reduces-blood-retina-barrier-breakdown-via-the-regulation-of-vcam-1-and-bcl2l11-in-ischemic-retinopathy
#18
Xia Bai, Junpeng Luo, Xiaofeng Zhang, Jingjing Han, Zhaoyue Wang, Jingcheng Miao, Yanyan Bai
To evaluate the role of microRNA-126 (miR-126) in maintaining the integrity of the blood-retina barrier (BRB), we established a mouse model of oxygen-induced retinopathy (OIR) and measured the retinal levels of miR-126 using recombinant plasmid pCMV-MIR or pCMV-MIR-126 intravitreal injections. We also detected VCAM-1 and BCL2L11 levels. Retinal vaso-obliteration, VCAM-1 localization on retinal endothelial cells, the blood-retina vascular permeability or albumin leakage in retinas, TUNEL histology, Evans blue assays, or Western blotting for detecting albumin or tight junction levels in the retina was performed...
January 12, 2017: Ophthalmic Research
https://www.readbyqxmd.com/read/28076805/regulation-of-active-icam-4-on-normal-and-sickle-cell-disease-rbcs-via-akaps-is-revealed-by-afm
#19
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
https://www.readbyqxmd.com/read/28065771/risk-factors-for-30-day-readmission-in-adults-with-sickle-cell-disease
#20
Max A Brodsky, Mark Rodeghier, Maureen Sanger, Jeannie Byrd, Brandi McClain, Brittany Covert, Dionna O Roberts, Karina Wilkerson, Michael R DeBaun, Adetola A Kassim
BACKGROUND: Readmission to the hospital within 30-days is a measure of quality care; however, only few modifiable risk factors for 30-day readmission in adults with sickle cell disease are known. METHODS: We performed a retrospective review of the medical records of adults with sickle cell disease at a tertiary care center, to identify potentially modifiable risk factors for 30-day re-admission due to vaso-occulsive pain episodes. A total of 88 patients > 18 years of age were followed for 3...
January 5, 2017: American Journal of Medicine
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