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sickle cell anaemia

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https://www.readbyqxmd.com/read/28005272/prophylactic-versus-selective-blood-transfusion-for-sickle-cell-disease-in-pregnancy
#1
REVIEW
Babasola O Okusanya, Olufemi T Oladapo
BACKGROUND: Pregnant women with sickle cell disease (HbSS, HbSC and HbSβThal) may require blood transfusion to prevent severe anaemia or to manage potential medical complications. Preventive blood transfusion in the absence of complications starting from the early weeks of pregnancy or blood transfusion only for medical or obstetric indications have been used as management policies. There is currently no consensus on the blood transfusion policy that guarantees optimal clinical benefits with minimal risks for such women and their babies...
22, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27994894/ischemic-monomeric-neuropathy-in-a-woman-with-sickle-cell-anaemia
#2
Alexandra Agapidou, Laura Aiken, Lisa Linpower, Dimitris A Tsitsikas
Sickle cell disease is an inherited haemoglobinopathy that can affect multiple organs and systems. The most common neurological complication in sickle cell disease is stroke and silent cerebral infarcts. Peripheral nervous system involvement has been described but is exceedingly rare. Herein, we describe the case of a young woman who presented with acute flaccid paralysis and sensory loss of the left lower extremity in the context of a painful vasoocclusive crisis which resolved rapidly after receiving an emergency automated red cell exchange transfusion...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27984639/exacerbation-of-oxidative-stress-during-sickle-vaso-occlusive-crisis-is-associated-with-decreased-anti-band-3-autoantibodies-rate-and-increased-red-blood-cell-derived-microparticle-level-a-prospective-study
#3
Régine Hierso, Nathalie Lemonne, Rinaldo Villaescusa, Marie-Laure Lalanne-Mistrih, Keyne Charlot, Maryse Etienne-Julan, Benoit Tressières, Yann Lamarre, Vanessa Tarer, Yohann Garnier, Ada Arce Hernandez, Serge Ferracci, Philippe Connes, Marc Romana, Marie-Dominique Hardy-Dessources
Painful vaso-occlusive crisis, a hallmark of sickle cell anaemia, results from complex, incompletely understood mechanisms. Red blood cell (RBC) damage caused by continuous endogenous and exogenous oxidative stress may precipitate the occurrence of vaso-occlusive crises. In order to gain insight into the relevance of oxidative stress in vaso-occlusive crisis occurrence, we prospectively compared the expression levels of various oxidative markers in 32 adults with sickle cell anaemia during vaso-occlusive crisis and steady-state conditions...
December 16, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27984631/interim-assessment-of-liver-damage-in-patients-with-sickle-cell-disease-using-new-non-invasive-techniques
#4
Emma Drasar, Emer Fitzpatrick, Kate Gardner, Moji Awogbade, Anil Dhawan, Adrian Bomford, Abid Suddle, Swee L Thein
We explored transient elastography (TE) and enhanced liver fibrosis (ELF(™) ) score with standard markers of liver function to assess liver damage in 193 well patients with sickle cell disease (SCD). Patients with HbSS or HbSβ(0) thalassaemia (sickle cell anaemia, SCA; N = 134), had significantly higher TE results and ELF scores than those with HbSC (N = 49) disease (TE, 6·8 vs. 5·3, P < 0·0001 and ELF, 9·2 vs. 8·6 P < 0·0001). In SCA patients, TE and ELF correlated significantly with age and all serum liver function tests (LFTs)...
December 16, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27929203/real-time-dose-adjustment-using-point-of-care-platelet-reactivity-testing-in-a-double-blind-study-of-prasugrel-in-children-with-sickle-cell-anaemia
#5
Joseph A Jakubowski, Carolyn C Hoppe, Chunmei Zhou, Brendan E Smith, Patricia B Brown, Lori E Heath, Baba Inusa, David C Rees, David S Small, Neehar Gupta, Suqin Yao, Matthew Heeney, Julie Kanter
Patients with sickle cell anaemia (SCA) have vaso-occlusive crises resulting from occlusive hypoxic-ischaemic injury. Prasugrel inhibits platelet activation and aggregation involved in SCA pathophysiology. Determining Effects of Platelet Inhibition on Vaso-Occlusive Events (DOVE) was a phase 3, double-blind, randomised, placebo-controlled trial assessing prasugrel efficacy. DOVE sought to bring patients' P2Y12 reaction unit (PRU) value within a targeted range via prasugrel dose adjustments using encrypted VerifyNow P2Y12(®) (VN-P2Y12) point-of-care testing and an interactive voice-response system (IVRS)...
December 8, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27917209/left-ventricular-systolic-function-in-sickle-cell-anaemia-an-echocardiographic-evaluation-in-adult-nigerian-patients
#6
Emmanuel Ejim, Nelson Oguanobi
BACKGROUND: Reliable diagnostic measures for the evaluation of left ventricular systolic performance in the setting of altered myocardial loading characteristics in sickle cell anaemia remains unresolved. OBJECTIVE: The study was designed to assess left ventricular systolic function in adult sickle cell patients using non-invasive endsystolic stress - end-systolic volume index ratio. METHODS: A descriptive cross sectional comparative study was done using 52 patients recruited at the adult sickle cell anaemia clinic of the University of Nigeria Teaching Hospital Enugu...
September 2016: African Health Sciences
https://www.readbyqxmd.com/read/27914684/sickle-cell-disease-in-the-older-adult
#7
REVIEW
Mya S Thein, Norris E Igbineweka, Swee Lay Thein
Sickle cell disease (SCD) is an inherited haemoglobin disorder, associated with recurrent painful episodes, ongoing haemolytic anaemia and progressive multi-organ damage. Until the early 1990s, survival beyond the fourth decade for a patient with SCD was considered unusual and prompted case reports. Nowadays, in countries with developed health care systems, more than 90 percent of newborns with SCD survive into adulthood. Nevertheless, their life expectancy is still shortened by more than two decades compared to the general population...
January 2017: Pathology
https://www.readbyqxmd.com/read/27911152/non-tuberculous-mycobacterial-bloodstream-infections-in-patients-with-indwelling-vascular-catheters-the-role-of-sickle-cell-anaemia
#8
Babatunde Edun, Ansal Shah, Martin Durkin, Melanie Whitmire, Shanetta Patterson Williams, Helmut Albrecht, Majdi Al-Hasan, Sharon Weissman
INTRODUCTION: Few studies have examined risk factors for nontuberculous mycobacteria (NTM) bloodstream infections (BSI) involving indwelling vascular catheters (IDVC). Sickle cell anaemia (HbSS/SC) is known to affect several aspects of the immune system leading to relative immune deficiency. The purpose of this retrospective nested case-control study was to determine if HbSS/SC is a risk factor for NTM BSI among individuals with IDVCs. METHODS: All NTM IDVC infections (cases) at two tertiary hospitals from 2008 to 2014 were reviewed...
December 2, 2016: Infectious Diseases
https://www.readbyqxmd.com/read/27905100/the-severity-of-anaemia-depletes-cerebrovascular-dilatory-reserve-in-children-with-sickle-cell-disease-a-quantitative-magnetic-resonance-imaging-study
#9
Przemyslaw D Kosinski, Paula L Croal, Jackie Leung, Suzan Williams, Isaac Odame, Gregory M T Hare, Manohar Shroff, Andrea Kassner
Overt ischaemic stroke is one of the most devastating complications in children with sickle cell disease (SCD). The compensatory response to anaemia in SCD includes an increase in cerebral blood flow (CBF) by accessing cerebrovascular dilatory reserve. Exhaustion of dilatory reserve secondary to anaemic stress may lead to cerebral ischaemia. The purpose of this study was to investigate CBF and cerebrovascular reactivity (CVR) using magnetic resonance imaging (MRI) in children with SCD and to correlate these with haematological markers of anaemia...
December 1, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27858986/stroke-in-sickle-cell-anaemia-is-more-than-stenosis-and-thrombosis-the-role-of-anaemia-and-hyperemia-in-ischaemia
#10
EDITORIAL
Michael M Dowling, Fenella J Kirkham
No abstract text is available yet for this article.
November 11, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27855617/eryptosis-ally-or-enemy
#11
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/27800099/prevalence-of-anaemia-among-quranic-school-khalawi-students-heiran-in-wad-el-magboul-village-rural-rufaa-gezira-state-central-sudan-a-cross-sectional-study
#12
Mohammed Saeed Elsamani Eltayeb, Awad Eseed Elsaeed, Ahmed Abdalla Mohamedani, Abbas Abdalrahman Assayed
INTRODUCTION: This is a cross sectional descriptive community-based study. The aim was to assess the prevalence of anaemia among quranic schoolchildren in khalawi Wad EL Magboul village, rural Rufaa, Gezira State, central Sudan. METHODS: A sample of 180 male participants were included in the study. Informed consent was obtained. Venous blood samples were obtained to measure the hematological parameters and blood films for malaria parasites. Urine and stool analyses were also done...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27774320/awareness-knowledge-and-acceptance-of-haematopoietic-stem-cell-transplantation-for-sickle-cell-anaemia-in-nigeria
#13
Adewumi Adediran, Modu Baba Kagu, Tamunomieibi Wakama, Aliyu Ahmadu Babadoko, Dapus Obadiah Damulak, Sunday Ocheni, Marcus Inyama Asuquo
Background. Sickle cell anaemia (SCA) is an inherited condition whose clinical manifestations arise from the tendency of haemoglobin to polymerize and deform red blood cells into characteristic sickle shape. Allogeneic bone marrow transplantation offers a cure. The aim of this study was to determine the level of awareness, knowledge, and acceptance of this beneficial procedure in Nigeria. Materials and Methods. This multicentre cross-sectional study was conducted in 7 tertiary hospitals in Nigeria in 2015. Approval was obtained from each institution's research and ethics committee...
2016: Bone Marrow Research
https://www.readbyqxmd.com/read/27766637/increased-prevalence-of-potential-right-to-left-shunting-in-children-with-sickle-cell-anaemia-and-stroke
#14
Michael M Dowling, Charles T Quinn, Claudio Ramaciotti, Julie Kanter, Ifeyinwa Osunkwo, Baba Inusa, Rathi Iyer, Janet L Kwiatkowski, Clarissa Johnson, Melissa Rhodes, William Owen, John J Strouse, Julie A Panepinto, Lynne Neumayr, Sharada Sarnaik, Patricia A Plumb, Nomazulu Dlamini, Fenella Kirkham, Linda S Hynan
'Paradoxical' embolization via intracardiac or intrapulmonary right-to-left shunts (RLS) is an established cause of stroke. Hypercoagulable states and increased right heart pressure, which both occur in sickle cell anaemia (SCA), predispose to paradoxical embolization. We hypothesized that children with SCA and overt stroke (SCA + stroke) have an increased prevalence of potential RLS. We performed contrasted transthoracic echocardiograms on 147 children (aged 2-19 years) with SCA + stroke) mean age 12·7 ± 4·8 years, 54·4% male) and a control group without SCA or stroke (n = 123; mean age 12·1 ± 4·9 years, 53·3% male)...
January 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/27738439/electrocardiographic-study-in-adult-homozygous-sickle-cell-disease-patients-in-lagos-nigeria
#15
Adedoyin Dosunmu, Akinsegun Akinbami, Ebele Uche, Adewumi Adediran, Sarah John-Olabode
Background. This study sought to identify the pattern of electrocardiographic changes in steady state adult sickle cell anaemia. Methods. A case-control, cross-sectional study was conducted amongst sickle cell patients attending the sickle cell clinic of Lagos State University Teaching Hospital, Ikeja, and HbAA controls. All consenting participants had haemoglobin electrophoresis done and were subjected to electrocardiography (ECG). The descriptive data were given as means ± standard deviation (SD). The differences were considered to be statistically significant when the p value obtained was <0...
2016: Journal of Tropical Medicine
https://www.readbyqxmd.com/read/27718361/the-regulation-of-human-globin-promoters-by-ccaat-box-elements-and-the-recruitment-of-nf-y
#16
Gabriella E Martyn, Kate G R Quinlan, Merlin Crossley
CCAAT boxes are motifs found within the proximal promoter of many genes, including the human globin genes. The highly conserved nature of CCAAT box motifs within the promoter region of both α-like and β-like globin genes emphasises the functional importance of the CCAAT sequence in globin gene regulation. Mutations within the β-globin CCAAT box result in β-thalassaemia, while mutations within the distal γ-globin CCAAT box cause the Hereditary Persistence of Foetal Haemoglobin, a benign condition which results in continued γ-globin expression during adult life...
October 5, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27704520/the-emergent-use-of-cardiopulmonary-bypass-and-extracorporeal-membrane-oxygenator-in-a-child-with-sickle-cell-disease
#17
Wassim Ben Ameur, Sonia Ouerghi, Amira Dridi, Mouna Bousnina, Atef Ben Youssef, Tarek Kilani, Tahar Mestiri
The use of Cardiopulmonary bypass (CPB) and extracorporeal membrane oxygenator (ECMO) in patients suffering from Sickle cell disease (SCD) needs specific precautions. Whereas, no consensual protocols have been established to clarify therapeutic management. CASE REPORT A 7-year-old boy was admitted to the hospital for surgery of advanced endocarditis.  Major dyspnea, hemodynamic distress and fever were noted on physical examination. Biological tests exploring anaemia revealed Haemoglobin (Hb) S levels of 39...
April 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27703935/major-surgery-in-a-jehovah-witness-with-sickle-cell-disease-case-presentation
#18
Udo Ego Anyaehie, Cajetan Uwatoronye Nwadinigwe, Arinze Duke Nwosu, Valentine Ogochukwu Ogbui
INTRODUCTION: A Jehovah's Witness belongs to the religious group that does not accept blood transfusion in any form, while a sickle cell disease patient has abnormal haemoglobins that do not last in circulation predisposing one to anaemia and other systemic complications. Performing a major surgery in a Jehovah's Witness who has sickle cell disease is tasking for a surgeon. CASE PRESENTATION: This case reports a 28-year-old African female with sickle cell disease who outrightly refused any form of blood transfusion as being a Jehovah's Witness and having a complex primary hip that required total hip replacement...
April 2016: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/27703480/glucose-6-phosphate-dehydrogenase-deficiency-and-sickle-cell-trait-among-prospective-blood-donors-a-cross-sectional-study-in-berekum-ghana
#19
Patrick Adu, David Larbi Simpong, Godfred Takyi, Richard K D Ephraim
Background. Blood transfusion is a therapeutic procedure usually undertaken in patients with severe anaemia. In Ghana, severe anaemia is mostly due to malaria caused by severe Plasmodium falciparum infection, road traffic accidents, and haemoglobinopathy-induced acute haemolysis. Method. This cross-sectional study evaluated coinheritance of sickle cell haemoglobin variant and G6PD enzymopathy among individuals that donated blood at the Holy Trinity Hospital, Berekum, in the Brong-Ahafo Region, Ghana. Demographic data and other pertinent information were captured using questionnaire...
2016: Advances in Hematology
https://www.readbyqxmd.com/read/27692991/chronic-brain-damage-in-sickle-cell-disease-and-its-relation-with-quality-of-life
#20
Elena Cela, Ana G Vélez, Alejandra Aguado, Gabriela Medín, José M Bellón, Cristina Beléndez
BACKGROUND AND OBJECTIVE: Sickle cell anaemia causes progressive organ damage. The objective is to describe school performance of patients with sickle cell anaemia and their clinical parameters and quality of life that may have an influence. The hypothesis is that if school alterations occur without other objective data, additional factors must be present besides the disease itself. PATIENTS AND METHODS: Transversal study performed in November 2015 considering analytical variables, complications and neuroradiological images of children with sickle cell anaemia, and family survey on school performance and quality of life...
December 16, 2016: Medicina Clínica
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