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sickle cell anaemia

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https://www.readbyqxmd.com/read/27911152/non-tuberculous-mycobacterial-bloodstream-infections-in-patients-with-indwelling-vascular-catheters-the-role-of-sickle-cell-anaemia
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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)...
October 21, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27738439/electrocardiographic-study-in-adult-homozygous-sickle-cell-disease-patients-in-lagos-nigeria
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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...
September 29, 2016: Medicina Clínica
https://www.readbyqxmd.com/read/27692921/-patient-with-homozygous-sickle-cell-disease-and-free-flap-surgery-ensuring-the-success-of-the-procedure
#14
S Deneuve, L Maire, V Bachelot, M-A Dammacco, P Zrounba, E Delay
Sickle cell anaemia is rare in France but frequent in Africa, leading to rigid, sickle-like shape red blood cells which bind together blocking microcirculation under certain circumstances. The vaso-occlusive crisis is the most frequent clinical manifestation especially in case of homozygous disease. Sickle cells disease is therefore usually considerated as a contraindication to microsurgery, however sometimes, a free flap procedure is mandatory. We here report the case of a 47-year-old man suffering with homozygous sickle cell anaemia and needing an antebrachial free flap procedure for a tongue reconstruction...
September 28, 2016: Annales de Chirurgie Plastique et Esthétique
https://www.readbyqxmd.com/read/27650269/rates-and-risk-factors-of-hypertension-in-adolescents-and-adults-with-sickle-cell-anaemia-in-tanzania-10%C3%A2-years-experience
#15
Abel Makubi, Bruno P Mmbando, Enrico M Novelli, Johnson Lwakatare, Deogratius Soka, Harvest Marik, Kemi Tibarazwa, Mariam Ngaeje, Charles R Newton, Mark T Gladwin, Julie Makani
Data on the magnitude and risk factors for hypertension in sickle cell anaemia (SCA) are limited. A retrospective analysis of individuals with SCA aged ≥15 years enrolled from 2004-2014 at Muhimbili National Hospital, Tanzania was conducted to determine the prevalence, incidence and risk factors for hypertension. A total of 1013 individuals with SCA were analysed, of whom 571(56%) were females. The median age [interquartile range] was 17 [15-22] years. Four hundred and forty-one (44%) of the patients had relative hypertension [systolic blood pressure (SBP) 120-139 mmHg or diastolic blood pressure (DBP) 70-89 mmHg], and 79 (8%) had hypertension (SBP ≥ 140 mmHg or DBP ≥ 90 mmHg)...
September 21, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27639979/prevalence-of-stroke-in-children-admitted-with-sickle-cell-anaemia-to-mulago-hospital
#16
Deogratias Munube, Elly Katabira, Grace Ndeezi, Moses Joloba, Samden Lhatoo, Martha Sajatovic, James K Tumwine
BACKGROUND: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. METHODS: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in Kampala, Uganda...
September 17, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27614462/angioid-streaks-in-aagenaes-syndrome
#17
Viara Shoumnalieva-Ivanova, Ivan Tanev, Yani Zdravkov, Simeon Monov, Russka Shumnalieva
Aagenaes syndrome, also called lymphoedema cholestasis syndrome 1 (LSC1), is characterized by neonatal intrahepatic cholestasis, often lessening and becoming intermittent with age and severe chronic lymphoedema, mainly affecting the lower extremities. The condition is autosomal recessively inherited, and the gene is located on chromosome 15q. The locus, LCS1, was mapped to a 6.6 cM region on chromosome 15. Angioid streaks are visible irregular crack-like dehiscences in bruch's membrane that are associated with atrophic degeneration of the overlying retinal pigment epithelium...
September 10, 2016: International Ophthalmology
https://www.readbyqxmd.com/read/27604981/hydroxycarbamide-treatment-and-brain-mri-mra-findings-in-children-with-sickle-cell-anaemia
#18
Kerri A Nottage, Russell E Ware, Banu Aygun, Matthew Smeltzer, Guolian Kang, Joseph Moen, Winfred C Wang, Jane S Hankins, Kathleen J Helton
Silent cerebral infarction (SCI) is the most common neurological abnormality among children with sickle cell anaemia (SCA). The effect of hydroxycarbamide (also termed hydroxyurea) on the development and progression of SCI is unclear. We evaluated brain magnetic resonance imaging/angiography (MRI/MRA) in children with SCA receiving long-term hydroxycarbamide therapy. Fifty participants (median 9·4 years, range 1·1-17·3) enrolled in the Hydroxyurea Study of Long-Term Effects (HUSTLE; NCT00305175) underwent brain MRI/MRA and laboratory evaluations before hydroxycarbamide initiation and after 3 and 6 years of treatment to maximum tolerated dose...
October 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27587381/nocturnal-enuresis-and-k-transport-in-red-blood-cells-from-patients-with-sickle-cell-anemia
#19
LETTER
Sanjay Tewari, David C Rees, Anke Hannemann, Oluwabukola T Gbotosho, Halima W M Al Balushi, John S Gibson
No abstract text is available yet for this article.
December 2016: Haematologica
https://www.readbyqxmd.com/read/27504413/buccal-micronucleus-cytome-assay-in-sickle-cell-disease
#20
Mallika Bokka Sri Satya Naga, Shreya Gour, Nalini Nallagutta, Kranti Kiran Reddy Ealla, Surekha Velidandla, Sangameshwar Manikya
INTRODUCTION: Sickle Cell Anaemia (SCA) is a commonly inherited blood disorder preceded by episodes of pain, chronic haemolytic anaemia and severe infections. The underlying phenomenon which causes this disease is the point mutation in the haemoglobin beta gene (Hbβ) found on chromosome 11 p. Increased oxidative stress leads to DNA damage. DNA damage occurring in such conditions can be studied by the buccal micronucleus cytome assay, which is a minimally invasive method for studying chromosomal instability, cell death and regenerative potential of human buccal tissue...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
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