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Sickle cell anemia

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https://www.readbyqxmd.com/read/27919909/severe-anemia-early-in-life-as-a-risk-factor-for-sickle-cell-kidney-disease
#1
Inmaculada Aban, Sujatha Baddam, Lee M Hilliard, Thomas H Howard, Daniel Feig, Jeffrey D Lebensburger
No abstract text is available yet for this article.
December 5, 2016: Blood
https://www.readbyqxmd.com/read/27913539/prevention-of-central-nervous-system-sequelae-in-sickle-cell-disease-without-evidence-from-randomized-controlled-trials-the-case-for-a-team-based-learning-collaborative
#2
Michael R DeBaun, Allison A King
Since 1998, the National Institutes of Health has funded 5 randomized controlled trials (RCTs) for primary and secondary prevention of strokes in children with sickle cell anemia (SCA). In a systematic fashion, these trials have significantly advanced the care of children with SCA. In the absence of an RCT, clinicians are often compelled to make decisions at the bedside, based on experience, observational studies, and principles of hematology. We will provide an initial example that describes how a team-based, learning collaborative developed a multisite standard care protocol with a low budget (<$10 000 per year) to overcome the intrinsic limitations of advancing the care of neurologic complications in sickle cell disease (SCD)...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27912798/bilateral-anomalous-drainage-of-the-posterior-divisions-of-renal-veins-into-the-azygos-venous-system-in-a-20-year-old-woman-a-case-report
#3
Pedro Pallangyo, Frederick Lyimo, Paulina Nicholaus, Stephano Masatu, Mohamed Janabi
BACKGROUND: Renal vein anomalies are relatively infrequent and generally asymptomatic. Preoperative knowledge of such variants is, however, of paramount importance in several angiographic and surgical procedures including renal venography, renal vein sampling, spermatic embolization, and renal transplantation. Inadequate knowledge and failure to recognize such anatomic variations may lead to several operative hazards including hemorrhage, nephrectomy, and even death. CASE PRESENTATION: We report a case of bilateral anomalous drainage of the posterior divisions of renal veins into the azygos venous system in a 20-year-old woman of African descent from Tanzania who presented to us with a 12-year history of recurrent anemia...
December 3, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27895843/severe-anemia-in-malawian-children
#4
Job Cj Calis, Kamija S Phiri, E Brian Faragher, Bernard J Brabin, Imelda Bates, Luis E Cuevas, Rob J de Haan, Ajib I Phiri, Pelani Malange, Mirriam Khoka, Paul Jm Hulshof, Lisette van Lieshout, Marcel Ghm Beld, Yik Y Teo, Kirk A Rockett, Anna Richardson, Dominic P Kwiatkowski, Malcolm E Molyneux, Michaël Boele van Hensbroek
BACKGROUND: Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. METHODS: We conducted a case-control study of 381 preschool children with severe anemia (hemoglobin concentration, <5.0 g per deciliter) and 757 preschool children without severe anemia in urban and rural settings in Malawi. Causal factors previously associated with severe anemia were studied. The data were examined by multivariate analysis and structural equation modeling...
September 2016: Malawi Medical Journal: the Journal of Medical Association of Malawi
https://www.readbyqxmd.com/read/27876368/biomechanics-and-biorheology-of-red-blood-cells-in-sickle-cell-anemia
#5
Xuejin Li, Ming Dao, George Lykotrafitis, George Em Karniadakis
Sickle cell anemia (SCA) is an inherited blood disorder that causes painful crises due to vaso-occlusion of small blood vessels. The primary cause of the clinical phenotype of SCA is the intracellular polymerization of sickle hemoglobin resulting in sickling of red blood cells (RBCs) in deoxygenated conditions. In this review, we discuss the biomechanical and biorheological characteristics of sickle RBCs and sickle blood as well as their implications toward a better understanding of the pathophysiology and pathogenesis of SCA...
November 12, 2016: Journal of Biomechanics
https://www.readbyqxmd.com/read/27872769/mantle-cell-hyperplasia-of-peripheral-lymph-nodes-as-initial-manifestation-of-sickle-cell-disease
#6
Ahmad Monabbati, Sadat Noori, Akbar Safaei, Mani Ramzi, Seyedsajjad Eghbali, Ali Adib
Sickle cell disease (SCD) is a well known hemoglobinopathy with usual manifestations including anemia, hyperbilirubinemia, and vasoocclusive complications. Despite presence of mild splenomegaly in early phase of the disease, lymphadenopathy is not an often finding of SCD. We introduce an undiagnosed case of SCD who presented in third decade of his life with multiple cervical lymphadenopathies and mild splenomegaly persistent for about five years. Histopathologic examination of the resected lymph nodes showed expansion of the mantle cell layers of secondary follicles as well as several monomorphic mantle cell nodules...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27872735/fetal-hemoglobin-modifies-the-disease-manifestation-of-severe-plasmodium-falciparum-malaria-in-adult-patients-with-sickle-cell-anemia
#7
Prasanta Purohit, Siris Patel, Pradeep Kumar Mohanty, Padmalaya Das, Jogeswar Panigrahi
No abstract text is available yet for this article.
2016: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/27871907/prognostic-value-of-t786c-and-g894t-enos-polymorphisms-in-sickle-cell-disease
#8
Iakovos Armenis, Vassiliki Kalotychou, Revekka Tzanetea, Panagoula Kollia, Zoi Kontogeorgiou, Dimitra Anastasopoulou, Marina Mantzourani, Michael Samarkos, Konstantinos Pantos, Kostas Konstantopoulos, Ioannis Rombos
Endothelial Nitric Oxide Synthase (eNOS) is crucial for vascular homeostasis. Polymorphisms T786C and G894T are reportedly affecting eNOS regulation and have been related to various diseases. Sickle Cell Disease (SCD), a clinically diverse chronic hemolytic anemia, implies impaired nitric oxide bioavailability. Aim of this study was to determine eNOS genotype for T786C and G894T polymorphisms in Greek patients with SCD and to elucidate its functional consequences and possible effects on clinical phenotype. Seventy nine steady state cases, most of them compound heterozygous for Sickle cell anemia/beta thalassemia and 48 controls were enrolled...
November 18, 2016: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/27869662/the-cell-killing-mechanisms-of-hydroxyurea
#9
REVIEW
Amanpreet Singh, Yong-Jie Xu
Hydroxyurea is a well-established inhibitor of ribonucleotide reductase that has a long history of scientific interest and clinical use for the treatment of neoplastic and non-neoplastic diseases. It is currently the staple drug for the management of sickle cell anemia and chronic myeloproliferative disorders. Due to its reversible inhibitory effect on DNA replication in various organisms, hydroxyurea is also commonly used in laboratories for cell cycle synchronization or generating replication stress. However, incubation with high concentrations or prolonged treatment with low doses of hydroxyurea can result in cell death and the DNA damage generated at arrested replication forks is generally believed to be the direct cause...
November 17, 2016: Genes
https://www.readbyqxmd.com/read/27860202/parent-reported-sleep-problems-in-preschool-children-with-sickle-cell-anemia-and-controls-in-east-london
#10
Michelle Downes, Michelle de Haan, Fenella J Kirkham, Paul T Telfer
Snoring and poor sleep may affect cognition, particularly in young children with chronic conditions. Parents of London preschoolers with sickle cell anemia (SCA; n = 22), matched controls (n = 24), and unselected typically developing (n = 142) preschoolers completed sleep questionnaires. Preschoolers with SCA had significantly more sleep problems when compared to matched controls and the larger population. Snoring occurred at least one to two nights a week for 79% of the SCA group. This is compared with 25% of matched controls and 33% of larger population...
November 15, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27857895/angiotensin-converting-enzyme-insertion-deletion-polymorphism-is-not-associated-with-vasoocclusive-complications-of-sickle-cell-anemia
#11
Sana Abass Mahjoub, Enaam Abdelrhman, Mohammed Elfatih Mohy El-Deen, Mustafa Sharf Eldin Mustafa, Elshazali Widaa Ali
CONTEXT: Sickle cell anemia (SCA) is a group of hemoglobin disorders in which the sickle β-globin gene is inherited. It is associated with many complications; most of them are related to thrombotic events. AIM: This study aimed to investigate the association between angiotensin converting enzyme (ACE) insertion/deletion polymorphism and complications of SCA. SETTINGS AND DESIGN: A case-control study was conducted in Khartoum state. SUBJECTS AND METHODS: A total of 50 patients with SCA and 40 healthy volunteers as a control group were enrolled in this study...
October 2016: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/27855647/albuminuria-serum-antioxidant-enzyme-levels-and-markers-of-hemolysis-and-inflammation-in-steady-state-children-with-sickle-cell-anemia
#12
Karen E Itokua, Jean Robert Makulo, François B Lepira, Michel N Aloni, Pépé M Ekulu, Ernest K Sumaili, Justine B Bukabau, Vieux M Mokoli, Augustin L Longo, François M Kajingulu, Chantal V Zinga, Yannick M Nlandu, Yannick M Engole, Pierre Z Akilimali, René M Ngiyulu, Jean Lambert Gini, Nazaire M Nseka
BACKGROUND: Oxidative stress is thought to be involved in the pathogenesis of microalbuminuria in Sickle cell anemia (SCA). Antioxidant enzymes such as glutathione peroxidase (GPx) and Cu-Zn superoxide dismutase (SOD) may play an important protective role. This study aimed to evaluate the association between albuminuria and these two antioxidant enzymes. METHODS: We consecutively recruited Steady state children aged between 2 and 18 years old with established diagnosis of homozygous SCA in two hospitals of Kinshasa/DR Congo...
November 17, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27855617/eryptosis-ally-or-enemy
#13
Marilena Briglia, Maria Antonia Rossi, Caterina Faggio
Prior to senescence, erythrocytes may, experience injury which compromises their integrity and thus triggers suicidal erythrocyte death or eryptosis. This mechanism is characterised by cell shrinkage, cell membrane blebbing, and cell membrane phospholipid scrambling after phosphatidylserine exposure on the cell surface that is identified by macrophages which engulf and degrade the eryptotic cells. The term eryptosis also includes typical mechanisms, which contribute to the triggering of this process. Among them: oxidative stress, Ca2+ entry with an increase in cytosolic Ca2+ activity ([Ca ]i) and the activation of p38 kinase, which is a kinase expressed in human erythrocytes and activated after hyperosmotic shock...
November 18, 2016: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/27852523/anemia-offers-stronger-protection-than-sickle-cell-trait-against-the-erythrocytic-stage-of-falciparum-malaria-and-this-protection-is-reversed-by-iron-supplementation
#14
M M Goheen, R Wegmüller, A Bah, B Darboe, E Danso, M Affara, D Gardner, J C Patel, A M Prentice, C Cerami
BACKGROUND: Iron deficiency causes long-term adverse consequences for children and is the most common nutritional deficiency worldwide. Observational studies suggest that iron deficiency anemia protects against Plasmodiumfalciparum malaria and several intervention trials have indicated that iron supplementation increases malaria risk through unknown mechanism(s). This poses a major challenge for health policy. We investigated how anemia inhibits blood stage malaria infection and how iron supplementation abrogates this protection...
November 9, 2016: EBioMedicine
https://www.readbyqxmd.com/read/27847659/interferon-induced-focal-segmental-glomerulosclerosis
#15
Yusuf Kayar, Nuket Bayram Kayar, Nadir Alpay, Jamshid Hamdard, Iskender Ekinci, Sebnem Emegil, Rabia Bag Soydas, Birol Baysal
Behçet's disease is an inflammatory disease of unknown etiology which involves recurring oral and genital aphthous ulcers and ocular lesions as well as articular, vascular, and nervous system involvement. Focal segmental glomerulosclerosis (FSGS) is usually seen in viral infections, immune deficiency syndrome, sickle cell anemia, and hyperfiltration and secondary to interferon therapy. Here, we present a case of FSGS identified with kidney biopsy in a patient who had been diagnosed with Behçet's disease and received interferon-alpha treatment for uveitis and presented with acute renal failure and nephrotic syndrome associated with interferon...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27845754/secondhand-smoke-is-an-important-modifiable-risk-factor-in-sickle-cell-disease-a-review-of-the-current-literature-and-areas-for-future-research
#16
REVIEW
S Christy Sadreameli, Benjamin T Kopp, Susan E Creary, Michelle N Eakin, Sharon McGrath-Morrow, John J Strouse
Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that causes significant morbidity and mortality related to chronic hemolytic anemia, vaso-occlusion, and resultant end-organ damage. Tobacco smoke exposure (TSE) through secondhand smoke exposure in people with SCD of all ages and through primary smoking in adolescents and adults is associated with significantly increased morbidity, with increased rates of emergency department visits and hospitalizations for painful vaso-occlusive crises and acute chest syndrome (ACS)...
November 12, 2016: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/27843377/pain-frequency-severity-and-qt-dispersion-in-adult-patients-with-sickle-cell-anemia-correlation-with-inflammatory-markers
#17
Taysir S Garadah, Ahmed A Jaradat, Mohammed E AlAlawi, Adla B Hassan, Reginald P Sequeira
BACKGROUND: Inflammatory markers are increased during vaso-occlusive crisis (VOC) in adult patients with sickle cell anemia (SCA), but this is not clear in clinical steady state. AIM: The present study aims to establish the frequency and intensity of bone pain episodes in adult patients with SCA in clinical steady state and to determine the correlation between different inflammatory markers, other variables including QT dispersion (QTd) and pain frequency and intensity in SCA...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27821508/sickle-cell-anemia-in-sub-saharan-africa-advancing-the-clinical-paradigm-through-partnerships-and-research
#18
Patrick T McGann, Arielle G Hernandez, Russell E Ware
No abstract text is available yet for this article.
November 7, 2016: Blood
https://www.readbyqxmd.com/read/27815018/-against-medical-advice-discharges-among-hiv-infected-patients-health-and-health-services-outcomes
#19
David Alfandre, Jingyan Yang, Katherine Harwood, Peter Gordon, Helen-Maria Lekas, Steven J Chang, Michael T Yin
Nurses are often first to identify and manage a patient leaving against medical advice (AMA), and so they are critical contributors to the development of strategies to address this problem. We studied AMA discharge in order to help develop useful interventions. We performed a cross-sectional analysis of 55,938 discharges from a single urban hospital for the years 2002-2003 and 2012-2013. AMA discharge rates were higher for HIV-infected patients than for patients with alcohol-related disorders or sickle cell anemia in both time periods, even after adjustment for age, race, sex, insurance status, and household income...
October 21, 2016: Journal of the Association of Nurses in AIDS Care: JANAC
https://www.readbyqxmd.com/read/27814863/predictive-models-of-six-minute-walking-distance-in-adults-with-sickle-cell-anemia-implications-for-rehabilitation
#20
Cirlene de Lima Marinho, Maria Christina Paixão Maioli, Andrea Ribeiro Soares, Ricardo Bedirian, Pedro Lopes de Melo, Fernando Silva Guimarães, Arthur de Sá Ferreira, Agnaldo José Lopes
BACKGROUND: Sickle cell anemia (SCA) is characterized by a broad spectrum of abnormalities that affect most body organs and systems. To date, there is few data on the influence of these patients' clinical characteristics on the functional exercise capacity. AIM: To investigate the effect of the clinical complications on the functional exercise capacity of adult SCA patients. METHOD: Cross-sectional study, where 45 SCA patients underwent clinical evaluations, echocardiography, pulmonary function testing, and determination of six-minute walking distance (6MWD)...
October 2016: Journal of Bodywork and Movement Therapies
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