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sickle cell anaemia

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https://www.readbyqxmd.com/read/28627939/off-label-prescribing-for-children-with-chronic-diseases-in-nigeria-findings-and-implications
#1
Kazeem Adeola Oshikoya, Ibrahim Adekunle Oreagba, Brian Godman, Joseph Fadare, Samuel Orubu, Amos Massele, Idowu Odunayo Senbanjo
BACKGROUND AND AIMS: Prescribing medicines in an off-label manner for children with chronic conditions is sparsely documented, even more so among developing countries. This needs addressing. The objective was to investigate the extent of this prescribing among children with epilepsy, asthma, and sickle cell anaemia in Nigeria. METHODS: Prescriptions for children ≤16 years documented in their case files that attended paediatric clinics in Lagos, Nigeria, for these three conditions between January and October 2015, were reviewed retrospectively to extract data on the medicines prescribed...
June 19, 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/28616936/encephaloduroateriosynangiosis-edas-in-the-management-of-moyamoya-syndrome-in-children-with-sickle-cell-disease
#2
Alexander Alamri, Pennylouise Hever, Jebet Cheserem, Catia Gradil, Sanj Bassi, Christos M Tolias
BACKGROUND: Encephalo-duro-arterio-synangiosis (EDAS) in Moyamoya syndrome (MMS) treatment has been well described in the literature, however in MMS caused by sickle cell anaemia (SCA), EDAS use remains controversial with poor long-term follow-up. We present a case-series of SCA patients who have undergone EDAS for SCA-related MMS and describe their post-operative course as well as provide a literature review of the role of EDAS in the treatment of sickle cell anaemia. METHODS: A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015...
June 15, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/28549163/factors-influencing-the-academic-performance-of-children-with-sickle-cell-anaemia-in-ekiti-south-west-nigeria
#3
Oladele Simeon Olatunya, Oluwasola Julius Oke, Bankole Peter Kuti, Iyiade Adeseye Ajayi, Oyebanji Olajuyin, Olubunmi Omotosho-Olagoke, Adekunle Bamidele Taiwo, Opeyemi Ayodeji Faboya, Ayodeji Ajibola
Background: There is a paucity of information on factors that influence the school performance of children with sickle cell anaemia (SCA) in Nigeria, despite her huge burden of the disease. Methods: In total, 101 children with SCA were recruited at a paediatric clinic in Nigeria. Their socio-demographic-matched classmates were the controls. Academic performance and cognitive functioning were obtained from school reports and Ziler's Draw-a-Person Test, respectively...
May 26, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28518049/red-blood-cell-storage-time-and-transfusion-current-practice-concerns-and-future-perspectives
#4
REVIEW
María García-Roa, María Del Carmen Vicente-Ayuso, Alejandro M Bobes, Alexandra C Pedraza, Ataúlfo González-Fernández, María Paz Martín, Isabel Sáez, Jerard Seghatchian, Laura Gutiérrez
Red blood cells (RBCs) units are the most requested transfusion product worldwide. Indications for transfusion include symptomatic anaemia, acute sickle cell crisis, and acute blood loss of more than 30% of the blood volume, with the aim of restoring tissue oxygen delivery. However, stored RBCs from donors are not a qualitative equal product, and, in many ways, this is a matter of concern in the transfusion practice. Besides donor-to-donor variation, the storage time influences the RBC unit at the qualitative level, as RBCs age in the storage bag and are exposed to the so-called storage lesion...
May 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28511389/variability-of-iron-load-in-patients-of-sickle-cell-anaemia-hbss-a-study-from-eastern-india
#5
Pranati Mohanty, Rabindra Kumar Jena, Sudha Sethy
INTRODUCTION: Sickle Cell Anaemia (SCA) is one of the commonest haemoglobinopathies due to a point mutation (A→T) of the β-globin gene. Out of five haplotypes, the Arab-Indian haplotype present in India is one of the least severe phenotype and least studied also. It is characterized by lifelong haemolytic anaemia requiring red cell transfusion leading to iron overload. In contrast, there is very high incidence of deficiency of iron, folic acid and vitamin B12. AIM: Our objective was to access the Iron status of SCA patients and to find its correlation with various parameters like red cell transfusion, haemolysis and serum hepcidin...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28466968/clinical-and-genetic-predictors-of-renal-dysfunctions-in-sickle-cell-anaemia-in-cameroon
#6
Amy Geard, Gift D Pule, Bernard Chetcha Chemegni, Valentina J Ngo Bitoungui, Andre P Kengne, Emile R Chimusa, Ambroise Wonkam
Micro-albuminuria and glomerular hyperfiltration are primary indicators of renal dysfunctions in Sickle Cell Disease (SCD), with more severe manifestations previously associated with variants in APOL1 and HMOX1 among African Americans. We have investigated 413 SCD patients from Cameroon. Anthropometric variables, haematological indices, crude albuminuria, albumin-to-creatinine ratio (ACR) and estimated glomerular filtration rate (eGFR) were measured. Patients were genotyped for 3·7 kb alpha-globin gene (HBA1/HBA2) deletion, and for variants in APOL1 (G1/G2; rs60910145, rs73885319, rs71785313) and HMOX1 (rs3074372, rs743811)...
May 3, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28466542/association-between-oxidative-stress-and-vascular-reactivity-in-children-with-sickle-cell-anaemia-and-sickle-haemoglobin-c-disease
#7
Berenike Möckesch, Philippe Connes, Keyne Charlot, Sarah Skinner, Marie-Dominique Hardy-Dessources, Marc Romana, Stéphane Jumet, Marie Petras, Lydia Divialle-Doumdo, Cyril Martin, Benoît Tressières, Vanessa Tarer, Olivier Hue, Maryse Etienne-Julan, Sophie Antoine, Vincent Pialoux
Oxidative stress and haemolysis-associated nitric oxide (NO) depletion plays a crucial role in the development of vasculopathy in sickle cell anaemia (SS). However it remains unknown whether oxidative stress and haemolysis levels influence vascular function in patients with sickle haemoglobin C disease (SC). Microvascular response to heat (using Laser Doppler flowmetry on finger), oxidative stress biomarkers, NO metabolites, endothelin-1 and haematological parameters were compared between patients with SS and SC...
May 3, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28450766/coinheritance-of-b-thalassemia-and-sickle-cell-anaemia-in-southwestern-nigeria
#8
Osunkalu Vincent, Bamisaye Oluwaseyi, Babatunde James, Lawal Saidat
BACKGROUND: Genes for haemoglobin S are found in high frequencies in Nigeria. However, there is little information on beta thalassemia in sickle cell anaemia in this population. The clinical presentation of HbS- β thalassemia is enormously variable, ranging from an asymptomatic state to a severe disorder similar to homozygous sickle cell disease. MATERIALS AND METHODS: Haemoglobin A2 and HbF were determined in sickle cell anaemia patients attending LAUTECH Teaching Hospital, Osogbo, by elution after electrophoresis and alkaline denaturation methods respectively...
November 2016: Ethiopian Journal of Health Sciences
https://www.readbyqxmd.com/read/28426137/hydroxyurea-hydroxycarbamide-for-sickle-cell-disease
#9
REVIEW
Sarah J Nevitt, Ashley P Jones, Jo Howard
BACKGROUND: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea (hydroxycarbamide), an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising fetal haemoglobin. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the effects of hydroxyurea therapy in people with SCD (all genotypes), of any age, regardless of setting...
April 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28417035/jaw-osteomyelitis-as-a-complication-of-sickle-cell-anaemia-in-three-omani-patients-case-reports-and-literature-review
#10
Hilal Al-Ismaili, Omar Nasim, Abdulaziz Bakathir
Sickle cell anaemia (SCA) is a common haemoglobinopathy among people from the Middle East, the Afro-Caribbean region, the Mediterranean and East India. While osteomyelitis of the long bones is a well-documented complication of SCA, there are few documented cases of SCA patients presenting with jaw osteomyelitis. We report three SCA patients with chronic jaw osteomyelitis who presented to the Department of Oral Health, Sultan Qaboos University Hospital, Muscat, Oman, between 2009 and 2013. Two of the patients had osteomyelitis of the mandible and the third had osteomyelitis of the maxilla...
February 2017: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/28406128/variations-in-the-%C3%AE-globin-genes-of-sickle-cell-anaemia-patients-in-zaria-northwestern-nigeria
#11
S Awwalu, A I Mamman, A Hassan, L G Dogara, A D Waziri, S M Aminu, A U Musa, H Bello-Manga
CONTEXT: Sickle Cell Anaemia (SCA) is a genetic disorder with a life-long disability, which is of public health importance. The diversity in its clinico-pathologic and laboratory presentations may be due to the interplay between additional genetic differences and environmental factors. The genetic factors may be within the β-globin gene itself, the β-globin gene cluster or elsewhere in the genome. AIM: To characterize the β-globin gene for variations associated with the Sickle Cell mutation...
April 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28401342/tuberculosis-in-children-with-sickle-cell-anaemia-a-retrospective-study-in-french-tertiary-care-centres
#12
Nina Droz, Agathe De Lauzanne, Laurent Holvoet, Florence Missud, Malika Benkerrou, Valentine Brousse, Marie-Hélène Odièvre, Albert Faye, Berengere Koehl
Tuberculosis (TB) and sickle cell anaemia (SCA) may affect the same population of patients, particularly in Africa but also in high-TB incidence areas in developed countries. However, few data are available from children with SCA who develop TB. The aim of this study was to describe the clinical features and outcome of TB diagnosed in children with SCA. We conducted a retrospective, descriptive study in three referral centre of Sickle Cell Disease in Paris, France. We included 11 patients with SCA who develop TB...
April 11, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28399358/care-for-haemoglobinopathy-patients-in-slovakia
#13
Viera Fábryová, Peter Božek, Monika Drakulová, Andrea Kollárová, Zuzana Laluhová Striežencová, Michaela Macichová, Adriena Sakalová
BACKGROUND: The paper presents the results od 22-year study of screening and follow-up of haemoglobinopathies in Slovakia, an overview of genetic mutations, the coincidence with hereditary haemochromatosis mutations, and the procedure in genetic councelling. METHODS: Between 1993-2015, in three centres in Bratislava and in one centre in Kosice, carriers of beta-thalassaemic genes or other haemoglobinopathies were searched for. Diagnosis was performed by haematologists, whereby the family history was evaluated, together with the overall clinical condition, blood count and blood smear, iron and haemolysis parameters, mutations of hereditary haemochromatosis, and haemoglobin electrophoresis testing...
March 2017: Central European Journal of Public Health
https://www.readbyqxmd.com/read/28390266/relationship-between-serum-25-hydroxyvitamin-d-and-inflammatory-cytokines-in-paediatric-sickle-cell-disease
#14
Samuel Ademola Adegoke, Olufemi Samuel Smith, Adekunle D Adekile, Maria Stella Figueiredo
BACKGROUND: Alteration in the concentration of inflammatory cytokines may contribute to pathogenesis in sickle cell anaemia (SCA). Vitamin D may suppress pro-inflammatory cytokines and enhance anti-inflammatory cytokines. OBJECTIVE: To compare steady state levels of pro-and anti-inflammatory cytokines of Nigerian SCA children with age- and sex-matched healthy controls, and determine the relationship with 25-hydroxyvitamin-D (25-OHD). Effects of three months of vitamin D supplementation on cytokines of SCA children with suboptimal 25-OHD were also evaluated...
April 5, 2017: Cytokine
https://www.readbyqxmd.com/read/28388354/haemoglobin-oxygen-saturation-leucocyte-count-and-lactate-dehydrogenase-are-predictors-of-elevated-cerebral-blood-flow-velocity-in-nigerian-children-with-sickle-cell-anaemia
#15
Oyesola Oyewole Ojewunmi, Titilope Adenike Adeyemo, Akinniyi Adediran Osuntoki, Ngozi Awa Imaga, Ajoke Idayat Oyetunji
BACKGROUND: Transcranial Doppler ultrasound (TCD) scan, which measures blood flow velocity through the time-averaged mean of maximum velocities (TAMMVs) in the internal carotid arteries and middle cerebral arteries, is a useful screening tool for predicting stroke risk in children with sickle cell anaemia (SCA). AIM: To investigate which clinical and laboratory indices predict abnormal TCD velocity in children with SCA. METHODS: Fifty-four SCA patients with normal TCD (TAMMV < 170 cm/s), classified as negative TCD (NTCD), and 93 patients with conditional and abnormal TCD velocities (TAMMV ≥ 170 cm/s) classified as positive TCD were recruited...
April 7, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28369746/safety-and-benefits-of-interventions-to-increase-folate-status-in-malaria-endemic-areas
#16
REVIEW
Hans Verhoef, Jacobien Veenemans, Martin N Mwangi, Andrew M Prentice
For decades, folic acid has routinely been given to prevent or treat anaemia in children, pregnant women and people with sickle cell disease. However, there is no conclusive evidence that folate deficiency anaemia constitutes a public health problem in any of these groups. Industrial flour fortification is recommended and implemented in many countries to combat neural tube defects. Dietary folates or folic acid can antagonise the action of antifolate drugs that play a critical role in the prevention and treatment of malaria...
June 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28369718/simvastatin-reduces-vaso-occlusive-pain-in-sickle-cell-anaemia-a-pilot-efficacy-trial
#17
Carolyn Hoppe, Eufemia Jacob, Lori Styles, Frans Kuypers, Sandra Larkin, Elliott Vichinsky
Sickle cell anaemia (SCA) is a progressive vascular disease characterized by episodic vaso-occlusive pain. Despite the broad impact of inflammation on acute and chronic clinical manifestations of SCA, no directed anti-inflammatory therapies currently exist. Statins are cholesterol-lowering agents shown to confer protection from vascular injury by suppressing inflammation. We previously documented a reduction in soluble biomarkers of inflammation in patients with sickle cell disease treated with simvastatin...
May 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28348773/pleural-empyema-due-to-salmonella-enterica-serovar-enteritidis-in-an-immunocompetent-elderly-patient-a-case-report
#18
Panagiota Xaplanteri, Stelios F Assimakopoulos, Kostis Karachalios, Dimitrios Siagris, Alexandra Lekkou, Evangelos D Anastassiou, Iris Spiliopoulou, Charalambos Gogos, Fevronia Kolonitsiou
INTRODUCTION: Pleural empyema as a focal infection due to Salmonella enterica serovar Enteritidis is rare and most commonly described among immunosuppressed patients or patients who suffer from sickle cell anaemia and lung malignancies. CASE PRESENTATION: Here, we present an 81-year-old immunocompetent Greek woman with bacteraemia and pleural empyema due to Salmonella Enteritidis without any gastrointestinal symptoms. CONCLUSION: In our case, we suggest that patient's pleural effusion secondary to heart failure was complicated by empyema and that focal intravascular infection was the cause of bacteraemia...
August 2016: JMM Case Reports
https://www.readbyqxmd.com/read/28340403/micro-and-macrovascular-function-in-children-with-sickle-cell-anaemia-and-sickle-cell-haemoglobin-c-disease
#19
Berenike Möckesch, Keyne Charlot, Stéphane Jumet, Marc Romana, Lydia Divialle-Doumdo, Marie-Dominique Hardy-Dessources, Marie Petras, Benoît Tressieres, Vanessa Tarer, Olivier Hue, Maryse Etienne-Julan, Philippe Connes, Sophie Antoine-Jonville
It is unclear whether vascular function is affected similarly in children with sickle cell anaemia (SS) and children with sickle haemoglobin C (SC) disease. Therefore, we compared micro and macrovascular functions in healthy (AA) children, children with SS and SC disease, and assessed their association with physical activity. Participants (24 SS, 22 SC and 16 AA), were compared in terms of 1) thermal hyperaemic response (finger pad warming to 42°C) measured by Laser Doppler techniques, 2) arterial stiffness determined by pulse wave velocity, 3) daily energy expenditure related to moderate and intense physical activities estimated by questionnaire and 4) fitness level, evaluated by the six-minute walk test...
February 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28293401/alloimmunization-in-patients-with-sickle-cell-disease-and-thalassemia-experience-of-a-single-centre-in-oman
#20
Salam Alkindi, Saba AlMahrooqi, Sumaiya AlHinai, Ali AlMarhoobi, Saif Al-Hosni, Shahina Daar, Naglaa Fawaz, Anil Pathare
BACKGROUND: Blood transfusion is an integral part of the supportive care for patients with sickle cell disease (SCD) and thalassaemia. The hazard of red cell alloimmunization, however, is one of the main complications of this therapy. OBJECTIVES: The aim of this study was to evaluate the prevalence of red cell alloimmunization in Omani patients with sickle cell anaemia and thalassemia. METHODS: This study included 262 patients whose historical transfusion records were available...
2017: Mediterranean Journal of Hematology and Infectious Diseases
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