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"Sickle Cell Anemia"

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https://www.readbyqxmd.com/read/29236859/mentha-pulegium-crude-extracts-induce-thiol-oxidation-and-potentiate-hemolysis-when-associated-to-t-butyl-hydroperoxide-in-human-s-erythrocytes
#1
Matheus C Bianchini, Dennyura O Galvão, Tatiana Tamborena, Claudia O Alves, Robson L Puntel
Mentha pulegium (Lamiaceae) tea has been used as a traditional medicine; however, the modulatory effect of M. pulegium extracts on damage to human erythrocytes associated to t-butyl hydroperoxide (t-BHP) exposure remains to be investigated. Accordingly, we perform this study in order to test the hypothesis that aqueous and ethanolic extracts of M. pulegium could modulate the hemolysis associated to t-BHP exposure, non-protein thiol (NPSH) oxidation and lipid peroxidation (measured as thiobarbituric acid reactive substances - TBARS) in human erythrocytes...
December 11, 2017: Anais da Academia Brasileira de Ciências
https://www.readbyqxmd.com/read/29226507/age-is-the-only-predictor-of-small-decrease-in-lung-function-in-children-with-sickle-cell-anemia
#2
Shaina M Willen, Robyn Cohen, Mark Rodeghier, Fenella Kirkham, Susan S Redline, Carol Rosen, Jane Kirkby, Michael R DeBaun
The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV1 % predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: 1) FEV1 % predicted declines over time; and 2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ0 thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study...
December 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29221667/interleukin-27-and-interleukin-37-are-elevated-in-sickle-cell-anemia-patients-and-inhibit-in-vitro-secretion-of-interleukin-8-in-neutrophils-and-monocytes
#3
A E Alagbe, A S Justo Junior, L P Ruas, W V Tonassé, R M Santana, T H C Batista, I F Domingos, A S Araujo, M A C Bezerra, M N N Santos, M H S L Blotta
BACKGROUND AND OBJECTIVE: Inflammation is implicated in the pathogenesis of most complications seen in sickle cell anemia (SCA) patients. We aimed to evaluate serum levels of two newly discovered anti-inflammatory cytokines (IL-27 and IL-37), and pro-inflammatory cytokines among Brazilian SCA patients that are not on hydroxyurea therapy (HbSS), compared with hydroxyurea-treated patients (HbSSHU) and healthy controls (HbAA). Furthermore, we demonstrated the effect of IL-27, IL-37, and heme on in vitro secretions of IL-8 in human neutrophils and monocytes...
December 5, 2017: Cytokine
https://www.readbyqxmd.com/read/29220407/respiratory-resistance-and-reactance-in-adults-with-sickle-cell-anemia-correlation-with-functional-exercise-capacity-and-diagnostic-use
#4
Cirlene de Lima Marinho, Maria Christina Paixão Maioli, Jorge Luis Machado do Amaral, Agnaldo José Lopes, Pedro Lopes de Melo
BACKGROUND: The improvement in sickle cell anemia (SCA) care resulted in the emergence of a large population of adults living with this disease. The mechanisms of lung injury in this new population are largely unknown. The forced oscillation technique (FOT) represents the current state-of-the-art in the assessment of lung function. The present work uses the FOT to improve our knowledge about the respiratory abnormalities in SCA, evaluates the associations of FOT with the functional exercise capacity and investigates the early detection of respiratory abnormalities...
2017: PloS One
https://www.readbyqxmd.com/read/29214337/sleep-disordered-breathing-in-patients-with-sickle-cell-disease
#5
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
https://www.readbyqxmd.com/read/29212341/scavenging-reactive-oxygen-species-production-normalizes-ferroportin-expression-and-ameliorates-cellular-and-systemic-iron-disbalances-in-hemolytic-mouse-model
#6
Naveen Kumar Tangudu, Betuel Alan, Francesca Vinchi, Katharina Woerle, Dilay Lai, Sabine Vettorazzi, Kerstin Leopold, Maja Vujic Spasic
AIMS: The release of large amounts of free heme into the circulation, overproduction of reactive oxygen species (ROS) and the activation of toll-like receptor (Tlr)-4 dependent responses are considered critical for the ability of heme to promote oxidative stress and to initiate pro-inflammatory responses, posing a serious threat to the body. A deep understanding of the consequences of heme overload on the regulation of cellular and systemic iron homeostasis is however, still lacking. RESULTS: The effects of heme on iron metabolism were studied in primary macrophages and in mouse models of acute and chronic hemolysis...
December 6, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29211984/promiscuous-protein-binding-as-a-function-of-protein-stability
#7
Ruth Cohen-Khait, Orly Dym, Shelly Hamer-Rogotner, Gideon Schreiber
Proteins have evolved to balance efficient binding of desired partners with rejection of unwanted interactions. To investigate the evolution of protein-protein interactions, we selected a random library of pre-stabilized TEM1 β-lactamase against wild-type TEM1 using yeast surface display. Three mutations were sufficient to achieve micromolar affinity binding between the two. The X-ray structure emphasized that the main contribution of the selected mutations was to modify the protein fold, specifically removing the N'-terminal helix, which consequently allowed protein coupling via a β-sheet-mediated interaction resembling amyloid interaction mode...
December 5, 2017: Structure
https://www.readbyqxmd.com/read/29211182/-prevalence-of-hemoglobin-variants-in-quilombola-communities-in-the-state-of-piau%C3%A3-brazil
#8
Leonardo Ferreira Soares, Eleonidas Moura Lima, José Alexsandro da Silva, Suenia Soares Fernandes, Keyla Malba da Costa Silva, Sarah Pereira Lins, Bolivar Ponciano Goulart de Lima Damasceno, Roseane Mara Cardoso Lima Verde, Marilda de Souza Gonçalves
Hemoglobin variants (Hb) result from mutations in globin genes, with amino acid substitution in the polypeptide chain. Among the most common structural variants are HbS, HbC, HbD and HbE. The S hemoglobin gene is a high frequency gene across America and Brazil, where it is more frequent in the Southeast and Northeast. The scope of this article is to investigate the presence of hemoglobin variants in 15 quilombos (fugitive slave communities) of Piaui. The sample was of 1,239 people and hemoglobin was screened by high-performance liquid chromatography (HPLC)...
November 2017: Ciência & Saúde Coletiva
https://www.readbyqxmd.com/read/29202133/the-accuracy-of-hospital-icd-9-cm-codes-for-determining-sickle-cell-disease-genotype
#9
Angela B Snyder, Peter A Lane, Mei Zhou, Susan T Paulukonis, Mary M Hulihan
Sickle cell disease affects more than 100,000 individuals in the United States, among whom disease severity varies considerably. One factor that influences disease severity is the sickle cell disease genotype. For this reason, clinical prevention and treatment guidelines tend to differentiate between genotypes. However, previous research suggests caution when using a claimsbased determination of sickle cell disease genotype in healthcare quality studies. The objective of this study was to describe the extent of miscoding for the major sickle cell disease genotypes in hospital discharge data...
2017: Journal of Rare Diseases Research & Treatment
https://www.readbyqxmd.com/read/29197110/macrophage-activation-syndrome-and-post-transplant-microangiopathy-following-haploidentical-bone-marrow-transplantation-for-sickle-cell-anemia
#10
James O J Davies, Alice C Hart, Josu De La Fuente, Barbara J Bain
No abstract text is available yet for this article.
December 2, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29182088/perinatal-maternal-mortality-in-sickle-cell-anemia-two-case-reports-and-review-of-the-literature
#11
Sanaa Rizk, Elizabeth D Pulte, David Axelrod, Samir K Ballas
As outcomes of patients with sickle cell anemia improve and survival into adulthood with good quality of life and expectation of long-term survival becomes more common, challenges have developed, including issues related to reproduction. Pregnancy is frequently complicated in patients with sickle cell anemia with mortality up to 4.0%. Here we report maternal perinatal mortality in two women with sickle cell anemia who died post-partum due to acute chest syndrome (ACS), caused by bone marrow fat embolism and review the literature pertinent to this subject...
November 28, 2017: Hemoglobin
https://www.readbyqxmd.com/read/29168218/the-clinical-epidemiology-of-sickle-cell-anemia-in-africa
#12
Alex W Macharia, George Mochamah, Sophie Uyoga, Carolyne M Ndila, Gideon Nyutu, Johnstone Makale, Metrine Tendwa, Emily Nyatichi, John Ojal, Mohammed Shebe, Kennedy O Awuondo, Neema Mturi, Norbert Peshu, Benjamin Tsofa, J Anthony G Scott, Kathryn Maitland, Thomas N Williams
Sickle cell anemia (SCA) is the commonest severe monogenic disorders of humans. The disease has been highly characterized in high-income countries but not in sub-Saharan Africa where SCA is most prevalent. We conducted a retrospective cohort study of all children 0-13 years admitted from within a defined study area to Kilifi County Hospital in Kenya over a five-year period. Children were genotyped for SCA retrospectively and incidence rates calculated with reference to population data. Overall, 576 of 18,873 (3...
November 23, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29166365/cdc-grand-rounds-improving-the-lives-of-persons-with-sickle-cell-disease
#13
Mary Hulihan, Kathryn L Hassell, Jean L Raphael, Kim Smith-Whitley, Phoebe Thorpe
Approximately 100,000 Americans have sickle cell disease (SCD), a group of recessively inherited red blood cell disorders characterized by abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in the red blood cells. Persons with hemoglobin SS or hemoglobin Sß0 thalassemia, also known as sickle cell anemia (SCA), have the most severe form of SCD. Hemoglobin SC disease and hemoglobin Sß+ thalassemia are other common forms of SCD. Red blood cells that contain sickle hemoglobin are inflexible and can stick to vessel walls, causing a blockage that slows or stops blood flow...
November 24, 2017: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/29159826/pharmacological-and-molecular-approaches-for-the-treatment-of-%C3%AE-hemoglobin-disorders
#14
REVIEW
Neelam Lohani, Nupur Bhargava, Anjana Munshi, Sivaprakash Ramalingam
β-hemoglobin disorders, such as β-thalassemia and sickle cell anemia are among the most prevalent inherited genetic disorders worldwide. These disorders are caused by mutations in the gene encoding hemoglobin-β (HBB), a vital protein found in red blood cells (RBCs) that carries oxygen from lungs to all parts of the human body. As a consequence, there has been an enduring interest in this field in formulating therapeutic strategies for the treatment of these diseases. Currently, there is no cure available for hemoglobin disorders, although, some patients have been treated with bone marrow transplantation, whose scope is limited because of the difficulty in finding a histocompatible donor and also due to transplant-associated clinical complications that can arise during the treatment...
November 20, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29146708/inflammatory-molecule-reduction-with-hydroxyurea-therapy-in-children-with-sickle-cell-anemia
#15
Rhiannon R Penkert, Julia L Hurwitz, Paul Thomas, Jason Rosch, Jola Dowdy, Yilun Sun, Li Tang, Jane S Hankins
No abstract text is available yet for this article.
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29138844/the-%C3%A2-%C3%AE-3-7-deletion-in-%C3%AE-%C3%A2-globin-genes-increases-the-concentration-of-fetal-hemoglobin-and-hemoglobin-a2-in-a-saudi-arabian-population
#16
J Francis Borgio, Sayed Abdulazeez, Noor B Almandil, Zaki A Naserullah, Sana Al-Jarrash, Ahmed M Al-Suliman, Huda Ismail Elfakharay, Fuad S Qaw, Fatimah I Alabdrabalnabi, Mohammed A Alkhalifah, Mohammed Shakil Akhtar, Hatem Qutub, Amein K Al-Ali
The regions of Al‑Qatif and Al‑Ahssa in the Eastern Province of Saudi Arabia are known for their high prevalence of hemoglobinopathies, including β‑thalassemia and sickle cell anemia. Previously, the α‑gene deletion has been demonstrated as highly prevalent among populations residing in these two regions. The present study was conducted in order to investigate the implications of the α‑globin gene deletion on fetal hemoglobin (HbF) and hemoglobin α2 (HbA2) concentrations in patients with transfusion‑dependent β‑thalassemia...
November 13, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29130518/automated-red-blood-cell-exchange-in-preparation-for-filgrastim-mobilization-of-autologous-peripheral-blood-hematopoietic-progenitor-cells-in-a-patient-with-sickle-cell-anemia
#17
Yong Zhao, Jeffrey A Bailey, Jeanne Linden, Patricia St Pierre, Jan Cerny, Michelle Vauthrin, Mindy Greene, Robert Weinstein
Increasing survival of patients with sickle cell anemia (SCA) well into adulthood results in a rising likelihood of developing hematological malignancy. High-dose chemotherapy with autologous hematopoietic progenitor cell (HPC) rescue is standard of care for several hematological malignancies, but the risk of severe or life-threatening vaso-occlusive phenomena during filgrastim mobilization of HPC for collection poses a potential barrier to this approach. We report the use of automated red cell exchange in preparation for filgrastim mobilization in a patient with homozygous SCA...
November 11, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/29127680/gene-addition-strategies-for-%C3%AE-thalassemia-and-sickle-cell-anemia
#18
Alisa C Dong, Stefano Rivella
Beta-thalassemia and sickle cell anemia are two of the most common diseases related to the hemoglobin protein. In these diseases, the beta-globin gene is mutated, causing severe anemia and ineffective erythropoiesis. Patients can additionally present with a number of life-threatening co-morbidities, such as stroke or spontaneous fractures. Current treatment involves transfusion and iron chelation; allogeneic bone marrow transplant is the only curative option, but is limited by the availability of matching donors and graft-versus-host disease...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29127678/allogeneic-matched-related-transplantation-for-%C3%AE-thalassemia-and-sickle-cell-anemia
#19
Françoise Bernaudin, Corinne Pondarré, Claire Galambrun, Isabelle Thuret
Allogeneic hematopoietic stem cell transplantation (HSCT) can cure single gene disorders such as thalassemia and sickle cell anemia (SCA). These non-malignant diseases have in common severe hemolytic anemia and high proliferative bone marrow, requiring frequent transfusions. The risk of rejection is high and graft-vs-host disease is not desirable. Important progress has been made in the management of these diseases, including leukocyte depletion of blood products, and chelation therapy, for both diseases, and erythrocytapheresis and hydroxycarbamide for SCA...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29127268/sickle-mice-are-sensitive-to-hypoxia-ischemia-induced-stroke-but-respond-to-tissue-type-plasminogen-activator-treatment
#20
Yu-Yo Sun, Jolly Lee, Henry Huang, Mary B Wagner, Clinton H Joiner, David R Archer, Chia-Yi Kuan
BACKGROUND AND PURPOSE: The effects of lytic stroke therapy in patients with sickle cell anemia are unknown, although a recent study suggested that coexistent sickle cell anemia does not increase the risk of cerebral hemorrhage. This finding calls for systemic analysis of the effects of thrombolytic stroke therapy, first in humanized sickle mice, and then in patients. There is also a need for additional predictive markers of sickle cell anemia-associated vasculopathy. METHODS: We used Doppler ultrasound to examine the carotid artery of Townes sickle mice tested their responses to repetitive mild hypoxia-ischemia- and transient hypoxia-ischemia-induced stroke at 3 or 6 months of age, respectively...
November 10, 2017: Stroke; a Journal of Cerebral Circulation
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