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sickle cell anaemia

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https://www.readbyqxmd.com/read/29749368/effect-of-inherited-red-cell-defects-on-growth-of-plasmodium-falciparum-an-in-vitro-study
#1
Vrushali Pathak, Roshan Colah, Kanjaksha Ghosh
Background & objectives: High prevalence of certain polymorphic alleles of erythrocytes in malaria endemic area has been linked to the resistance provided by these alleles against parasitic infestations. Numerous studies undertaken to demonstrate this correlation have generated conflicting results. This study was undertaken to investigate the abilities of various polymorphic erythrocytes to support in vitro growth of Plasmodium falciparum parasites. Methods: In this study under in vitro condition the ability of P...
January 2018: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/29697214/sickle-cell-anaemia-and-the-experiences-of-young-people-living-with-the-condition
#2
Nicole Foster, Michelle Ellis
Sickle cell anaemia (SCA) is a life-threatening haemoglobin disorder acknowledged for its unpredictability and painful episodes. The aim of this qualitative literature review was to explore the experiences of young people living with SCA and its effect on their lives. The objective was to critically review selected primary research and make recommendations for practice, education and research. After reviewing potential articles using EBSCOhost, inclusion and exclusion criteria were devised and six appropriate studies were found with most participants in the 10-25 years age range...
April 26, 2018: Nursing Children and Young People
https://www.readbyqxmd.com/read/29676452/cardiac-manifestations-in-sickle-cell-disease-varies-with-patient-genotype
#3
Paul Guedeney, François Lionnet, Alexandre Ceccaldi, Katia Stankovic Stojanovic, Ariel Cohen, Sarah Mattioni, Gilles Montalescot, Claude Bachmeyer, Richard Isnard, Jean-Philippe Haymann, Nadjib Hammoudi
Cardiac involvement is well characterized in sickle cell anaemia (SCA) but cardiac features associated with Haemoglobin SC (HbSC) disease are mostly unknown. We compared 60 patients with HbSC disease (median age 31 years, 25 men) to 60 SCA patients and 60 controls matched for age and gender. Left ventricular ejection fraction (LVEF), left ventricle (LV) mass index (LVMi), cardiac index and peak tricuspid regurgitation velocity (TRV) were measured using echocardiography. LV filling pressures were assessed using the ratio of early diastolic transmitral velocity to tissue velocity (E/e' ratio)...
April 20, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29620730/molecular-nucleation-mechanisms-and-control-strategies-for-crystal-polymorph-selection
#4
Alexander E S Van Driessche, Nani Van Gerven, Paul H H Bomans, Rick R M Joosten, Heiner Friedrich, David Gil-Carton, Nico A J M Sommerdijk, Mike Sleutel
The formation of condensed (compacted) protein phases is associated with a wide range of human disorders, such as eye cataracts, amyotrophic lateral sclerosis, sickle cell anaemia and Alzheimer's disease. However, condensed protein phases have their uses: as crystals, they are harnessed by structural biologists to elucidate protein structures, or are used as delivery vehicles for pharmaceutical applications. The physiochemical properties of crystals can vary substantially between different forms or structures ('polymorphs') of the same macromolecule, and dictate their usability in a scientific or industrial context...
April 4, 2018: Nature
https://www.readbyqxmd.com/read/29610772/improving-routine-outpatient-monitoring-for-patients-with-sickle-cell-disease-on-hydroxyurea
#5
Joel Ward, Natasha Lewis, Dimitris A Tsitsikas
Hydroxyurea is the gold standard treatment for prevention of vaso-occlusive crises in patients with sickle-cell anaemia. It has a narrow therapeutic index and dangerous side effects including cytopenias. There is high variation in dose-response across the population. Therefore, a robust outpatient monitoring programme is crucial to ensure efficacy and safety of treatment. However, there has historically been difficulty engaging the target population in regular laboratory test monitoring programmes. This project aimed to ensure that all patients on hydroxyurea had routine blood tests at least once every 2 months which were reviewed and acted upon within the 3-year project life cycle...
2018: BMJ Open Quality
https://www.readbyqxmd.com/read/29607473/emergency-blood-transfusion-in-children-in-a-tertiary-hospital-in-nigeria-indications-frequency-and-outcome
#6
B I Abhulimhen-Iyoha, Y T Israel-Aina
BACKGROUND: Blood transfusion is a life-saving procedure in paediatric practice. It is important in replacing blood volume in cases of haemorrhage or providing specific blood components as required. However, the procedure carries some risks and complications. The decision to transfuse, frequency of transfusion and the availability of safe blood and blood products are essential determinants of the success of the procedure. Hence, knowledge of the indications and rate of transfusion is important to ensure that blood for transfusion is safe and made available as at when due...
January 2018: West African Journal of Medicine
https://www.readbyqxmd.com/read/29601112/cerebral-perfusion-characteristics-show-differences-in-younger-versus-older-children-with-sickle-cell-anaemia-results-from-a-multiple-inflow-time-arterial-spin-labelling-study
#7
Jamie M Kawadler, Patrick W Hales, Simon Barker, Timothy C S Cox, Fenella J Kirkham, Chris A Clark
Sickle cell anaemia (SCA) is associated with chronic anaemia and oxygen desaturation, which elevate cerebral blood flow (CBF) and increase the risk of neurocognitive complications. Arterial spin labelling (ASL) provides a methodology for measuring CBF non-invasively; however, ASL techniques using only a single inflow time are not sufficient to fully characterize abnormal haemodynamic behaviour in SCA. This study investigated haemodynamic parameters from a multi-inflow-time ASL acquisition in younger (8-12 years) and older (13-18 years) children with SCA with and without silent cerebral infarction (SCI+/-) (n = 20 and 19 respectively, 6 and 4 SCI+ respectively) and healthy controls (n = 9 and 7 respectively)...
March 30, 2018: NMR in Biomedicine
https://www.readbyqxmd.com/read/29557482/shear-dependent-red-blood-cell-adhesion-in-microscale-flow
#8
Erdem Kucukal, Jane A Little, Umut A Gurkan
Non-adherence and deformability are the key intrinsic biomechanical features of the red blood cell (RBC), which allow it to tightly squeeze and pass through even the narrowest of microcirculatory networks. Blockage of microcirculatory flow, also known as vaso-occlusion, is a consequence of abnormal cellular adhesion to the vascular endothelium. In sickle cell disease (SCD), an inherited anaemia, even though RBCs have been shown to be heterogeneous in adhesiveness and deformability, this has not been studied in the context of physiologically relevant dynamic shear gradients at the microscale...
March 20, 2018: Integrative Biology: Quantitative Biosciences From Nano to Macro
https://www.readbyqxmd.com/read/29553439/red-blood-cell-alloantibodies-in-multiply-transfused-patients-in-the-occupied-palestinian-territory-a-pilot-study
#9
Ahmad Yaseen, Sa'd Suleiman, Omar Abu Zenah, Adham Abu Taha
BACKGROUND: Red blood-cell transfusion has greatly reduced the mortality and morbidity in multiply transfused patients with thalassaemia and sickle cell disease. However, this can result in red blood-cell isoimmunisation with autoantibodies and alloantibodies, which can lead to serious complications such as delayed haemolytic transfusion reaction. The aim of this study was to assess the frequency and types of alloantibodies in multiply transfused patients living in the north of the West Bank...
February 21, 2018: Lancet
https://www.readbyqxmd.com/read/29548624/sickle-cell-anaemia-needs-more-food
#10
Hyacinth I Hyacinth
No abstract text is available yet for this article.
March 13, 2018: Lancet Haematology
https://www.readbyqxmd.com/read/29546732/folate-supplementation-in-people-with-sickle-cell-disease
#11
REVIEW
Ruchita Dixit, Sowmya Nettem, Simerjit S Madan, Htoo Htoo Kyaw Soe, Adinegara Bl Abas, Leah D Vance, Patrick J Stover
BACKGROUND: Sickle cell disease (SCD) is a group of disorders that affects haemoglobin, which causes distorted sickle- or crescent-shaped red blood cells. It is characterized by anaemia, increased susceptibility to infections and episodes of pain. The disease is acquired by inheriting abnormal genes from both parents, the combination giving rise to different forms of the disease. Due to increased erythropoiesis in people with SCD, it is hypothesized that they are at an increased risk for folate deficiency...
March 16, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29541675/simulated-datasets-for-population-dynamics-of-sickle-cell-anaemia
#12
S O Edeki, O O Akanbi
The datasets contained in this article are simulated data with respect to Sickle Cell Anaemia (SCA) in order to examine the mathematical inheritance formation of the SCA disease. The simulation is done using Monte Carlos Simulation (MCS) Technique to complement the Physical Simulation Smith's Statistical (PSSS) package used as random number generator for birth simulation. One hundred and fifty-six (156) births for seven (7) generations were considered in the simulation alongside non-gestating reproductive females with fertile male adults while immigration and emigration are not permitted...
February 2018: Data in Brief
https://www.readbyqxmd.com/read/29531654/adult-sickle-cell-anaemia-patients-in-bone-pain-crisis-have-elevated-pro-inflammatory-cytokines
#13
Adekunle Emmanuel Alagbe, John Ayodele Olaniyi, Oladapo Wale Aworanti
Background and Objectives: Inflammatory markers that influence bone pain crisis (BPC) and other complications of sickle cell anaemia (SCA) are numerous and play various roles. This study determined the plasma levels of tumour necrosis factor (TNF) - α, interleukin - 8 (IL-8), and endothelin - 1 (ET-1) in adult SCA patients during BPC and in steady state. In addition, the plasma levels of these cytokines were correlated with the severity of BPC of the patients. Methods and Materials: Sixty adult SCA patients (30 during BPC and 30 during steady state) and 30 haemoglobin A controls were enrolled for this cross-sectional study...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29504121/transcranial-doppler-velocity-among-jamaican-children-with-sickle-cell-anaemia-determining-the-significance-of-haematological-values-and-nutrition
#14
Angela E Rankine-Mullings, Nadine Morrison-Levy, Deanne Soares, Karen Aldred, Lesley King, Susanna Ali, Jennifer M Knight-Madden, Margaret Wisdom-Phipps, Robert J Adams, Russell E Ware, Marvin Reid
This study investigated the association of nutritional and haematological variables with maximum time-averaged mean velocity (TAMV) measured by transcranial Doppler (TCD) velocity and the agreement of classification between two protocols. TCD categories included: normal (<170 cm/s), conditional (170-199 cm/s) and abnormal (≥200 cm/s) based on TAMV in distal internal carotid artery (dICA), middle cerebral artery (MCA), internal carotid bifurcation, anterior and posterior cerebral arteries. Of 358 children with sickle cell anaemia (SCA) examined, the mean age (±standard deviation) was 7·4 ± 2·7 years; 13·1% and 6·7% had conditional and abnormal velocities, respectively...
April 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29472357/plerixafor-enables-safe-rapid-efficient-mobilization-of-hematopoietic-stem-cells-in-sickle-cell-disease-patients-after-exchange-transfusion
#15
Chantal Lagresle-Peyrou, François Lefrère, Elisa Magrin, Jean-Antoine Ribeil, Oriana Romano, Leslie Weber, Alessandra Magnani, Hanem Sadek, Clémence Plantier, Aurélie Gabrion, Brigitte Ternaux, Tristan Félix, Chloé Couzin, Aurélie Stanislas, Jean-Marc Tréluyer, Lionel Lamhaut, Laure Joseph, Marianne Delville, Annarita Miccio, Isabelle André-Schmutz, Marina Cavazzana
Sickle cell disease is characterized by chronic anemia and vaso-occlusive crises, which eventually lead to multi-organ damage and premature death. Hematopoietic stem cell transplantation is the only curative treatment but it is limited by toxicity and poor availability of HLA-compatible donors. A gene therapy approach based on the autologous transplantation of lentiviral-corrected hematopoietic stem and progenitor cells was shown to be efficacious in one patient. However, alterations of the bone marrow environment and properties of the red blood cells hamper the harvesting and immunoselection of patients' stem cells from bone marrow...
May 2018: Haematologica
https://www.readbyqxmd.com/read/29468088/neck-circumference-is-independently-associated-with-relative-systemic-hypertension-in-young-adults-with-sickle-cell-anaemia
#16
Lawrence A Olatunji, Olatunde P Olabode, Olawale M Akinlade, Abiola S Babatunde, Victoria A Olatunji, Ayodele O Soladoye
Background: A seemingly interesting observation in patients with sickle cell anaemia (SCA) is that they usually have lower systemic blood pressures (BP) and insulin resistance than persons in the general population in spite of chronic inflammation and vasculopathy. However, relative systemic hypertension (rHTN) has been linked to pulmonary hypertension, increased blood viscosity and renal insufficiency, which could indicate a risk of developing cardiometabolic disorder (CMD) in SCA.We therefore hypothesized that neck circumference (NC) and CMD marker; triglyceride glucose (TyG) index would independently predict rHTN in young adults with SCA in steady state...
2018: Clinical Hypertension
https://www.readbyqxmd.com/read/29453624/hydroxyurea-responses-in-clinically-varied-beta-hbe-beta-thalassaemia-and-sickle-cell-anaemia-patients-of-eastern-india
#17
Tridip Chatterjee, Amit Chakravarty, Sudipa Chakravarty
The haematological and clinical response to hydroxyurea was estimated in HbE-beta, beta thalassaemia and sickle cell anaemia patients of Eastern India, with variable clinical severity and transfusion requirement to determine whether hydroxyurea can help these patients to maintain their steady haemoglobin level without blood transfusions. Three hundred patients (189 HbE-beta thalassaemia, 95 beta thalassaemia and 16 other haemoglobinopathies including sickle cell anaemia) were selected for hydroxyurea therapy and were followed up for 48-60 months...
May 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29447888/pathogenesis-of-chronic-rhinosinusitis-in-patients-affected-by-%C3%AE-thalassemia-major-and-sickle-cell-anaemia-post-allogenic-bone-marrow-transplant
#18
F Martino, R Di Mauro, K Paciaroni, J Gaziev, C Alfieri, L Greco, R Floris, S Di Girolamo, M Di Girolamo
OBJECTIVES: Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29446825/phytomedicines-medicines-derived-from-plants-for-sickle-cell-disease
#19
REVIEW
Oluseyi Oniyangi, Damian H Cohall
BACKGROUND: Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. Laboratory research and limited clinical trials have suggested positive effects of phytomedicines both in vivo and in vitro...
February 15, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29438485/lower-than-expected-elevated-tricuspid-regurgitant-jet-velocity-in-adults-with-sickle-cell-disease-in-nigeria
#20
Baba Maiyaki Musa, Chisom N Odoh, Najibah A Galadanci, Hadiza Saidu, Muktar H Aliyu
Background: Cardiopulmonary disease is a major cause of morbidity and mortality in persons with sickle cell disease (SCD). Tricuspid regurgitant jet velocity (TRJV) and predicted forced expiratory volume in 1 s (FEV1%) predicted are independently associated with death in SCD. The goal of this study was to determine the prevalence of elevated TRJV and the association, if any, between TRJV and FEV1% predicted among persons with sickle cell anaemia (SCA) in Nigeria. Methods: Using a cross-sectional design, we enrolled 100 adult Nigerians (≥15 y) with SCA...
February 9, 2018: International Health
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