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https://www.readbyqxmd.com/read/28437457/tissue-factor-dependent-coagulation-activation-by-heme-a-thromboelastometry-study
#1
Gleice Regina de Souza, Bidossessi Wilfried Hounkpe, Maiara Marx Luz Fiusa, Marina Pereira Colella, Joyce M Annichino-Bizzacchi, Fabiola Traina, Fernando Ferreira Costa, Erich Vinicius De Paula
Heme has been characterized as potent trigger of inflammation. In hemostasis, although heme has been shown to both induce and inhibit different compartments of hemostasis, its net effect on the hemostatic balance, and the biological relevance of these effects remain to be determined. Herein we evaluated the effect of heme on hemostasis using a global assay able to generate clinically relevant data in several other complex hemostatic diseases. Citrated whole blood samples from healthy participants were stimulated by heme or vehicle and incubated for 4h at 37°C...
2017: PloS One
https://www.readbyqxmd.com/read/28436975/haploidentical-stem-cell-transplantation-with-cd3-cd19-depleted-peripheral-stem-cells-for-patients-with-advanced-stage-sickle-cell-disease-and-no-alternative-donor-results-of-a-pilot-study
#2
J Foell, B Pfirstinger, K Rehe, D Wolff, E Holler, S Corbacioglu
No abstract text is available yet for this article.
April 24, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28436274/depletion-of-hdl3-high-density-lipoprotein-and-altered-functionality-of-hdl2-in-blood-from-sickle-cell-patients
#3
Eric Soupene, Sandra K Larkin, Frans A Kuypers
In sickle cell disease (SCD), alterations of cholesterol metabolism is in part related to abnormal levels and activity of plasma proteins such as lecithin cholesterol acyltransferase (LCAT), and apolipoprotein A-I (ApoA-I). In addition, the size distribution of ApoA-I high density lipoproteins (HDL) differs from normal blood. The ratio of the amount of HDL2 particle relative to the smaller higher density pre-β HDL (HDL3) particle was shifted toward HDL2. This lipoprotein imbalance is exacerbated during acute vaso-occlusive episodes (VOE) as the relative levels of HDL3 decrease...
January 1, 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28435481/a-patient-centered-emergency-department-management-strategy-for-sickle-cell-disease-super-utilizers
#4
Grant G Simpson, Hallie R Hahn, Alex A Powel, Robert R Leverence, Linda A Morris, Lara G Thompson, Marc S Zumberg, Deepa J Borde, Joseph A Tyndall, Jonathan J Shuster, Donald M Yealy, Brandon R Allen
INTRODUCTION: A subpopulation of sickle-cell disease patients, termed super-utilizers, presents frequently to emergency departments (EDs) for vaso-occlusive events and may consume disproportionate resources without broader health benefit. To address the healthcare needs of this vulnerable patient population, we piloted a multidisciplinary intervention seeking to create and use individualized patient care plans that alter utilization through coordinated care. Our goals were to assess feasibility primarily, and to assess resource use secondarily...
April 2017: Western Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28435037/therapeutic-strategies-in-sickle-cell-anemia-the-past-present-and-future
#5
REVIEW
Queenie Fernandes
Sickle Cell Anemia (SCA) was one of the first hemoglobinopathies to be discovered. It is distinguished by the mutation-induced expression of a sickle cell variant of hemoglobin (HbS) that triggers erythrocytes to take a characteristic sickled conformation. The complex physiopathology of the disease and its associated clinical complications has initiated multi-disciplinary research within its field. This review attempts to lay emphasis on the evolution, current standpoint and future scope of therapeutic strategies in SCA...
April 20, 2017: Life Sciences
https://www.readbyqxmd.com/read/28432164/use-of-gabapentin-in-the-treatment-of-chronic-pain-in-an-adolescent-with-sickle-cell-disease
#6
Cátia R Correia, Ana Teresa Soares, Laura Azurara, Maria João Palaré
Vaso-occlusive crises are the most common manifestation of sickle cell disease (SCD) and the main cause of hospital admission in these patients. There is emerging evidence that vaso-occlusive pain has both nociceptive and neuropathic components. However, the treatment of SCD-related pain with neuropathic drugs has not yet been systematically studied, particularly in children. We describe a 14-year-old girl with SCD and multiple hospital admissions for pain management for severe acute vaso-occlusive pain episodes...
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28428653/doppler-velocimetry-of-the-orbital-arteries-in-patients-with-sickle-cell-anemia-relationship-with-biomarkers-of-hemolysis
#7
Thiago de Oliveira Ferrão, Paulo Ricardo Martins-Filho, Cleverton Aragão, Marlyson Santana, Allan Nascimento, Thayane Cardoso, Rosana Cipolotti
OBJECTIVE: To investigate orbital vascular resistance by Doppler velocimetry in patients with steady-state sickle cell anemia, as well as to characterize its relationship with biomarkers of hemolysis. MATERIALS AND METHODS: This was a cross-sectional study of two groups: 71 outpatients with sickle cell anemia; and 32 age- and gendermatched, healthy subjects (control group). All participants underwent Doppler velocimetry of the orbital arteries and laboratory tests...
March 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/28426137/hydroxyurea-hydroxycarbamide-for-sickle-cell-disease
#8
REVIEW
Sarah J Nevitt, Ashley P Jones, Jo Howard
BACKGROUND: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea (hydroxycarbamide), an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising fetal haemoglobin. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the effects of hydroxyurea therapy in people with SCD (all genotypes), of any age, regardless of setting...
April 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28425750/modeling-alloantibody-formation-to-high-incidence-red-blood-cell-antigens-in-immune-responders-using-genotypic-data
#9
Patricia A R Brunker, Keerthana Ravindran, R S Shirey
Alloimmunization to red blood cell antigens is unpredictable and poorly understood. Patients who are negative for high-incidence antigens (HIAs) are at risk for developing the corresponding antibodies. Molecular methods can easily predict the lack of an antigen and thus, the risk of an individual to become immunized. We examined the prevalence and risk factors for HIA alloimmunization in patients at risk based on genotyping results. Genotyping using a molecular method (HEA BeadChip™, Immucor, Warren, NJ) was performed on all patient specimens referred for molecular testing over 45 months; serologic and clinical data were analyzed...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28425749/hemolytic-transfusion-reaction-attributable-to-anti-dia
#10
Arthur J Joyce, Kelli M Quantock, Ray Banh, Yew W Liew
In situations when a patient's antibody detection test is negative, many institutions have moved from an indirect antiglobulin test (IAT) crossmatch to an electronic crossmatch system. Here we report a case of an acute hemolytic transfusion reaction attributable to anti-Dia in a patient with a negative antibody detection test. A 22-year-old female patient with a diagnosis of β thalassemia and sickle cell anemia commenced a routine exchange transfusion of 5 units of red blood cells (RBCs) in the apheresis unit as part of her regular treatment...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28424766/the-need-for-hematology-nurse-education-in-low-and-middle-income-countries-a-community-case-study-in-tanzania
#11
Julie M Buser
Hematology-related diseases, such as anemia, malaria, sickle cell disease (SCD), and blood cancers, have differing rates of survival between high-income and low- and middle-income countries (LMICs). Nurses in LMICs have an unmet need for specialty training and education to address hematology and hemato-oncology disorders. A gap in the literature exists about hematology nurse education and clinical service demands in LMICs. This community case study documents a collaborative hematology and basic hemato-oncology education program to sustainably strengthen nurse capacity at a national referral hospital and university in Tanzania...
2017: Frontiers in Public Health
https://www.readbyqxmd.com/read/28423290/sickle-cell-disease
#12
Frédéric B Piel, Martin H Steinberg, David C Rees
No abstract text is available yet for this article.
April 20, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28422403/blood-chimerism-in-dizygotic-monochorionic-twins-during-five-years-observation
#13
Morten Hanefeld Dziegiel, Morten Høgh Hansen, Sofie Haedersdal, Angela Natalie Barrett, Klaus Rieneck, Katharina Maria Main, Anne Todsen Hansen, Frederik Banch Clausen
Dizygotic monochorionic twin pregnancies can result in blood chimerism due to in utero twin-to-twin exchange of stem cells. In this case, we examined the proportion of allogeneic red blood cells (RBCs) by flow cytometry and the proportion of allogeneic nucleated cells by digital PCR at seven months and again at five years. We found an increase in the proportion of allogeneic cells from 63% to 89% in one twin, and a similar increase in autologous cells in the other twin from 57% to 84%. A paradigm for stem cell therapy could be modelled on this case: induction of tolerance and chimerism by antenatal transfusion of donor stem cells...
April 19, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28421813/hemin-causes-lung-microvascular-endothelial-barrier-dysfunction-by-necroptotic-cell-death
#14
Sunit Singla, Justin R Sysol, Benjamin Dille, Nicole Jones, Jiwang Chen, Roberto F Machado
Hemin, the oxidized prosthetic moiety of hemoglobin, has been implicated in the pathogenesis of acute chest syndrome (ACS) in sickle cell patients by virtue of its endothelial-activating properties. In this study, we examined whether hemin can cause lung microvascular endothelial barrier dysfunction. By assessing transendothelial resistance using electrical cell impedance sensing, and by directly measuring trans-monolayer FITC-dextran flux, we found that hemin does cause endothelial barrier dysfunction in a concentration-dependent manner...
April 19, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28420988/heme-as-a-target-for-therapeutic-interventions
#15
REVIEW
Stephan Immenschuh, Vijith Vijayan, Sabina Janciauskiene, Faikah Gueler
Heme is a complex of iron and the tetrapyrrole protoporphyrin IX with essential functions in aerobic organisms. Heme is the prosthetic group of hemoproteins such as hemoglobin and myoglobin, which are crucial for reversible oxygen binding and transport. By contrast, high levels of free heme, which may occur in various pathophysiological conditions, are toxic via pro-oxidant, pro-inflammatory and cytotoxic effects. The toxicity of heme plays a major role for the pathogenesis of prototypical hemolytic disorders including sickle cell disease and malaria...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28417646/cerebral-hemodynamic-assessment-and-neuroimaging-across-the-lifespan-in-sickle-cell-disease
#16
Lori C Jordan, Michael R DeBaun
Children and adults with sickle cell anemia (SCA) have a higher risk of strokes compared to age- and race-matched peers. Velocity in the middle cerebral or distal internal carotid artery as measured by transcranial Doppler ultrasound is a recognized method to identify children but not adults with SCA at high-risk for first stroke. For both children and adults with SCA that have had a stroke, no methods clearly identify individuals at highest risk of recurrent strokes or an initial silent stroke, the most common neurological injury...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28417347/-serological-markers-of-viral-hepatitis-b-and-c-in-children-with-sickle-cell-disease-monitored-in-the-pediatrics-department-at-the-university-hospital-of-bobo-dioulasso-burkina-faso
#17
S A Kissou, M Koura, A Sawadogo, A-S Ouédraogo, H Traoré, E Kamboulé, W W F Zogona, B Nacro
Viral hepatitis B and C are universal public health problems. Burkina Faso is a high endemic area for hepatitis B. Patients with sickle cell disease are at risk. The aim of this study was to investigate and quantify the serological markers of viral hepatitis B and C among pediatric patients with sickle cell disease. This was a descriptive cross-sectional study, which took place from July to November 2014 at the Department of Pediatrics, University Hospital of Bobo- Dioulasso. The study included 44 SS and 26 SC sickle cell patients...
April 17, 2017: Bulletin de la Société de Pathologie Exotique
https://www.readbyqxmd.com/read/28417035/jaw-osteomyelitis-as-a-complication-of-sickle-cell-anaemia-in-three-omani-patients-case-reports-and-literature-review
#18
Hilal Al-Ismaili, Omar Nasim, Abdulaziz Bakathir
Sickle cell anaemia (SCA) is a common haemoglobinopathy among people from the Middle East, the Afro-Caribbean region, the Mediterranean and East India. While osteomyelitis of the long bones is a well-documented complication of SCA, there are few documented cases of SCA patients presenting with jaw osteomyelitis. We report three SCA patients with chronic jaw osteomyelitis who presented to the Department of Oral Health, Sultan Qaboos University Hospital, Muscat, Oman, between 2009 and 2013. Two of the patients had osteomyelitis of the mandible and the third had osteomyelitis of the maxilla...
February 2017: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/28413532/spontaneous-extradural-and-subgaleal-hematoma-a-rare-neurosurgical-crisis-of-sickle-cell-disease
#19
Sudhansu S Mishra, Satya B Senapati, Amiya K Gouda, Sanjay K Behera, Ashis Patnaik
Extradural hematoma (EDH) in absence of trauma is a rare entity with only few cases reported in literature. The various causes reported include: Vascular malformation of dura, coagulopathies, sinus infection, middle ear or orbital infection, and tumor. Occurrence of spontaneous EDH as a complication of sickle cell disease is even much rarer. We report a case with sickle cell disease who presented with spontaneous extradural and subgaleal hematomas following an episode of vaso-oclusive crisis. He was managed successfully with surgery...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28412236/are-dental-and-jaw-changes-more-prevalent-in-a-brazilian-population-with-sickle-cell-anemia
#20
Halinna Larissa Cruz Correia de Carvalho, José Yagoh Saraiva Rolim, Érika Bárbara Abreu Fonseca Thomaz, Soraia de Fátima Carvalho Souza
OBJECTIVE: The aim of this study was to analyze the prevalence of dental and jaw changes in individuals with sickle cell anemia (SCA) compared with a control group from a Brazilian population. STUDY DESIGN: This was a retrospective cross-sectional study. Participants were divided into 2 groups: SCA and control (123 patients per group). Intraoral periapical radiographs were taken. Changes in bone pattern and dental changes were investigated by using the following radiographic parameters: (1) trabecular bone (step-ladder or spider web); (2) integrity of the lamina dura (loss of sharpness and continuity, partial or complete absence, and increased thickness); and (3) dental changes (pulp chamber, root surface, periapex, dental form, number, and position)...
March 9, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
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