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"Sickle Cell Anemia"

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https://www.readbyqxmd.com/read/27876368/biomechanics-and-biorheology-of-red-blood-cells-in-sickle-cell-anemia
#1
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/27872735/fetal-hemoglobin-modifies-the-disease-manifestation-of-severe-plasmodium-falciparum-malaria-in-adult-patients-with-sickle-cell-anemia
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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/27847659/interferon-induced-focal-segmental-glomerulosclerosis
#8
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/27843377/pain-frequency-severity-and-qt-dispersion-in-adult-patients-with-sickle-cell-anemia-correlation-with-inflammatory-markers
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/27814430/computational-biomechanics-of-human-red-blood-cells-in-hematological-disorders
#13
Xuejin Li, He Li, Hung-Yu Chang, George Lykotrafitis, George Em Karniadakis
We review recent advances in multiscale modeling of the biomechanical characteristics of red blood cells (RBCs) in hematological diseases, and their relevance to the structure and dynamics of defective RBCs. We highlight examples of successful simulations of blood disorders including malaria and other hereditary disorders, such as sickle-cell anemia, spherocytosis and elliptocytosis.
November 4, 2016: Journal of Biomechanical Engineering
https://www.readbyqxmd.com/read/27814292/hemorheological-alterations-in-sickle-cell-anemia-and-their-clinical-consequences-the-role-of-genetic-modulators
#14
Marisa Silva, Sofia Vargas, Andreia Coelho, Alexandra Dias, Teresa Ferreira, Anabela Morais, Raquel Maia, Paula Kjöllerström, João Lavinha, Paula Faustino
Sickle cell anemia (SCA) is an autosomal recessive disease caused by the HBB:c.20A>T mutation that leads to hemoglobin S synthesis. The disease presents with high clinical heterogeneity characterized by chronic hemolysis, recurrent episodes of vaso-oclusion and infection. This work aimed to characterize by in silico studies some genetic modulators of severe hemolysis and stroke risk in children with SCA, and understand their consequences at the hemorheological level.Association studies were performed between hemolysis biomarkers as well as the degree of cerebral vasculopathy and the inheritance of several polymorphic regions in genes related with vascular cell adhesion and vascular tonus in pediatric SCA patients...
November 4, 2016: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/27813033/-evaluation-of-the-effectiveness-of-patient-controlled-analgesia-in-children-with-sickle-cell-anemia-from-the-perspective-of-healthcare-professionals-and-parents
#15
Ayşegül Turaç, Şebnem Rumeli Atıcı
OBJECTIVES: This study evaluated the efficacy of patient-controlled analgesia (PCA) used by children with sickle cell anemia (SCA) based on the attitudes of parents and healthcare professionals. METHODS: A total of 86 individuals were involved in the study: 54 parents of children with SCA who were receiving treatment and 32 healthcare providers (doctors, nurses). To evaluate the effectiveness of the PCA method, a questionnaire was prepared to determine the level of knowledge of the participants about the PCA method and their perception of its advantages and disadvantages...
July 2016: Aǧrı: Ağrı (Algoloji) Derneği'nin Yayın Organıdır, the Journal of the Turkish Society of Algology
https://www.readbyqxmd.com/read/27802215/sickle-cell-anemia-nitric-oxide-related-genetic-modifiers-of-hematological-and-biochemical-parameters
#16
Laura Aguiar, Andreia Matos, Ângela Gil, Conceiçã Afonso, Lígia Braga, Lavinha João, Paula Kjollerstrom, Paula Faustino, Manuel Bicho, Ângela Inácio
BACKGROUND: Sickle cell anemia (SCA) is an inherited blood disorder. SCA patients present clinical and hematologic variability that cannot be only explained by the single mutation in the beta-globin gene. Others genetic modifiers and environmental effects are important for the clinical phenotype. SCA patients present arginine deficiency that contributes to a lower nitric oxide (NO) bioactivity. OBJECTIVE: The aim of this work is to determine the association between hematological and biochemical parameters and genetic variants from eNOS gene, in pediatric SCA patients...
October 14, 2016: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/27787922/bone-health-in-patients-with-hematopoietic-disorders-of-bone-marrow-origin-systematic-review-and-meta-analysis
#17
Kieran Steer, Mariya Stavnichuk, Martin Morris, Svetlana V Komarova
Blood cell production and bone homeostasis are physically interlinked systems that exhibit active cross-talk. We examined how bone health is affected in patients with hematopoietic disorders due to abnormal proliferation of bone marrow cells. The electronic databases Medline, Embase, PubMed, BIOSIS Previews, Web of Science, and Cochrane were searched for studies presenting numerical values for trabecular bone volume or bone mineral density in control and patients with hematopoietic disorders. We identified 5 studies for beta-thalassemia, 6 for sickle cell anemia, 2 for polycythemia vera and essential thrombocythemia, 3 for chronic myelogenous leukemia, 6 for myelofibrosis, 5 for multiple myeloma, and 4 studies each for systemic mastocytosis, lymphocytic leukemia, and hemochromatosis...
October 27, 2016: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/27778040/modern-american-scurvy-experience-with-vitamin-c-deficiency-at-a-large-children-s-hospital
#18
Farahnaz Golriz, Lane F Donnelly, Sridevi Devaraj, Raj Krishnamurthy
BACKGROUND: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism. OBJECTIVE: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations...
October 24, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27765439/might-interspecific-interactions-between-pathogens-drive-host-evolution-the-case-of-plasmodium-species-and-duffy-negativity-in-human-populations
#19
Benjamin Roche, Virginie Rougeron, Lluis Quintana-Murci, François Renaud, Jessica Lee Abbate, Franck Prugnolle
Malarial infections have long been recognized as a driver of human evolution, as demonstrated by the influence of Plasmodium falciparum on sickle-cell anemia persistence. Duffy-negativity is another blood disorder thought to have been selected because it confers nearly complete resistance against Plasmodium vivax infection. Recent evidence suggests that the benefits of being Duffy-negative cannot be expected to play a strong selective pressure on humans, whereas its costs cannot be considered as negligible...
October 17, 2016: Trends in Parasitology
https://www.readbyqxmd.com/read/27764159/longitudinal-analysis-of-patient-specific-predictors-for-mortality-in-sickle-cell-disease
#20
Susanna A Curtis, Neeraja Danda, Zipora Etzion, Hillel W Cohen, Henny H Billett
INTRODUCTION: White Blood Cell (WBC) count, %HbF, and serum creatinine (Cr), have been identified as markers for increased mortality in sickle cell anemia (SCA) but no studies have examined the significance of longitudinal rate of change in these or other biomarkers for SCA individuals. METHODS: Clinical, demographic and laboratory data from SCA patients seen in 2002 by our hospital system were obtained. Those who were still followed in 2012 (survival cohort) were compared to those who had died in the interim (mortality cohort)...
2016: PloS One
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