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"Sickle Cell Anemia"

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https://www.readbyqxmd.com/read/29905397/pneumococcal-vaccination-coverage-among-children-with-sickle-cell-anemia-sickle-cell-trait-and-normal-hemoglobin
#1
Sarah L Reeves, Hannah K Jary, Jennifer P Gondhi, Mary Kleyn, Abram L Wagner, Kevin J Dombkowski
BACKGROUND: Children with sickle cell anemia and sickle cell trait are at an increased risk of invasive pneumococcal disease compared to children with normal hemoglobin. We assessed and compared pneumococcal vaccination status among these three groups. PROCEDURE: Children with sickle cell anemia and sickle cell trait were identified using Michigan newborn screening records (1997-2014); each child was matched to four children with normal hemoglobin based on age, Medicaid enrollment (at least 1 year from 2012-2014), race, and census tract...
June 15, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29898152/aortic-valve-replacement-combined-with-ascending-aortic-aneurysmectomy-in-a-patient-with-sickle-cell-disease-a-case-report
#2
Lucas Molinari Veloso da Silveira, Ana Paula Tagliari, Ronaldo David da Costa, Cristiano Blaya Martins, Orlando Wender
Sickle cell anemia is a haematological disorder characterized by multiple vaso-occlusive complications, resulting in a reduced life expectancy. These patients are exposed to several triggering factors for sickle cell crises when they are submitted to cardiovascular surgeries with extracorporeal circulation. Therefore, meticulous care and perioperative management are required. This paper reports a successful case of combined cardiovascular surgery - aortic valve replacement and ascending aortic aneurysmectomy - with no serious post-operative complications...
March 2018: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29895661/l-glutamine-for-sickle-cell-anemia-more-questions-than-answers
#3
Charles T Quinn
In 2017, the Food and Drug Administration (FDA) approved two medications for sickle cell anemia (SCA): hydroxyurea for children (≥2 years of age) and L-glutamine for children and adults (≥5 years). The approval of hydroxyurea for children was long overdue, having been authorized by the FDA for adults in 1998 and by the European Medicines Agency for adults and children in 2007, but the approval of L-glutamine was a surprise to many in the field. There are few published studies of L-glutamine as a treatment for SCA, so all can be reviewed in this brief manuscript...
June 12, 2018: Blood
https://www.readbyqxmd.com/read/29887387/timing-and-causes-of-common-pediatric-readmissions
#4
Emily M Bucholz, James C Gay, Matthew Hall, Mitch Harris, Jay G Berry
OBJECTIVE: To evaluate and compare readmission causes and timing within the first 30 days after hospitalization for 3 acute and 3 chronic common pediatric conditions. STUDY DESIGN: Data from the 2013 to 2014 Nationwide Readmissions Database were used to examine the daily percentage of readmissions occurring on days 1-30 and the leading causes of readmission after hospitalization for 3 acute (appendicitis, bronchiolitis/croup, and gastroenteritis) and 3 chronic (asthma, epilepsy, and sickle cell) conditions for patients aged 1-17 years (n = 2 753 488)...
June 7, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29882519/emphysematous-osteomyelitis-caused-by-salmonella-typhi-in-beta-thalassemia-major
#5
P N Doctor, M Verma, A Varaiya, R H Merchant
There have been various cases of salmonella osteomyelitis reported in sickle cell anemia. We present a case of emphysematous osteomyelitis caused by Salmonella typhi in a 29-year-old beta thalassemia major patient. Diagnosis of emphysematous osteomyelitis was confirmed by computed tomography and magnetic resonance imaging, and culture of pus drained during surgical debridement confirmed the causative microorganism, Salmonella typhi. Antimicrobials were given according to microbiological sensitivity for a period of 8 weeks...
June 7, 2018: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/29875912/relative-adrenal-insufficiency-in-adults-with-sickle-cell-disease
#6
Eugene Sobngwi, Noel Désirée Mbango, Eric Vounsia Balti, Francoise Ngo Sack, Vicky Ama Moor, Jean-Claude Mbanya
Rheological modifications observed in sickle cell anemia are associated with ischemic complications that can cause target organ functional impairment. The objective was to investigate adrenal function of adult patients with sickle cell disease. In this cross-sectional study conducted in a tertiary referral hospital of the capital city of Cameroon, we enrolled ten crisis-free adult patients with sickle cell disease (SCD) and ten age- and sex-matched healthy individuals. We assessed adrenal function by testing basal cortisol levels and 60 min after tetracosactide (Synacthen® ) injection using immuno-chemiluminescence method...
2018: Pan African Medical Journal
https://www.readbyqxmd.com/read/29870301/impact-of-zinc-on-sexual-maturation-of-female-sickle-cell-anemia-sca-children-in-enugu-southeast-nigeria
#7
Vivian Ozoemena Onukwuli, Josephat Chinawa, Christopher Bismarck Eke, Ada Rosemary Nwokocha, Ifeoma Josephine Emodi, Anthony Nnaemeka Ikefuna
Adolescence is an important developmental period of childhood. Good health and adequate nutrition consisting major food constituents and trace elements like zinc are fundamental for optimal sexual maturation. To determine the relationship between zinc levels and pattern of breast and pubic hair development, as well as menarcheal age of female SCA children aged 6-18 years and their matched controls with hemoglobin genotype AA. Cross sectional, case-control study. Information on biodata, age at menarche, medical and drug history as well as 24-hour dietary recall was documented using interviewer administered questionnaire...
June 5, 2018: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29865045/proliferative-retinopathy-and-maculopathy-are-two-independent-conditions-in-sickle-cell-disease-is-there-a-role-of-blood-rheology-1
#8
Laurence Beral, Nathalie Lemonne, Marc Romana, Keyne Charlot, Marie Billaud, Malik Acomat, Coralie Zorobabel, Elie Nader, Maryse Etienne-Julan, Thierry David, Philippe Connes
OBJECTIVE: Our study investigated the prevalence of retinopathy and maculopathy in sickle cell patients and tested the association between these two conditions. In addition, we tested whether hematological and hemorheological parameters, as well as genotype, were involved in the development of these two conditions. METHODS: Seventy sickle cell adult patients were recruited: 37 with sickle cell anemia (SCA) and 33 with sickle cell hemoglobin C disease (SCC). All patients underwent retinal examination and macular ocular coherence tomography...
May 25, 2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29858275/molecular-insights-of-inhibition-in-sickle-hemoglobin-polymerization-upon-glutathionylation-hydrogen-deuterium-exchange-mass-spectrometry-and-molecular-dynamics-simulation-based-approach
#9
Rajdeep Das, Amrita Mitra, Gopa Mitra, Dibyajyoti Maity, Vijay Bhat, Debnath Pal, Cecil Ross, Anura V Kurpad, Amit Kumar Mandal
In sickle cell anemia, polymerization of hemoglobin in its deoxy state leads to the formation of insoluble fibres that result in sickling of red blood cells. Stereo-specific binding of isopropyl group of βVal6, the mutated amino acid residue of a tetrameric sickle hemoglobin molecule (HbS), with hydrophobic groove of another HbS tetramer initiates the polymerization. Glutathionylation of βCys93 in HbS was reported to inhibit the polymerization. However, the mechanism of inhibition in polymerization is unknown to date...
June 1, 2018: Biochemical Journal
https://www.readbyqxmd.com/read/29851151/a-case-for-community-health-programs-for-sickle-cell-anemia-in-low-to-middle-income-countries-an-integrative-review
#10
REVIEW
Stephanie Ibemere
OBJECTIVE: The purpose of this review is to determine the factors associated with the outcomes of community health programs with particular focus on low- to middle-income countries. In addition, this review aims to suggest how these factors can affect the creation of a Sickle Cell Anemia health program in sub-Saharan Africa. DESIGN AND SAMPLE: A literature review spanning the last decade (March 2007 to February 2017) was conducted using the Whittemore and Knafl integrative review framework...
May 31, 2018: Public Health Nursing
https://www.readbyqxmd.com/read/29843828/assessment-of-executive-functions-in-preschool-children-with-sickle-cell-anemia
#11
Michelle Downes, Fenella J Kirkham, Paul T Telfer, Michelle de Haan
OBJECTIVES: Children with sickle cell anemia (SCA) are commonly reported to experience executive dysfunction. However, the development of executive function (EF) in preschool-age children without stroke in this patient population has not been investigated so it is unclear when and how these deficits emerge. METHODS: This case-control study examines the feasibility of assessing the early development of executive functioning in 22 preschool children years with SCA in the domains of processing speed, working memory, attention, inhibitory control, and cognitive flexibility, as well as everyday function, in comparison to matched control children...
May 30, 2018: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/29806800/sensorineural-hearing-loss-in-children-with-sickle-cell-anemia-and-its-association-with-endothelial-dysfunction
#12
Mara Renata Rissatto Lago, Luciene da Cruz Fernandes, Isa Menezes Lyra, Regina Terse Ramos, Rozana Teixeira, Cristina Salles, Ana Marice Teixeira Ladeia
OBJECTIVES: To investigate the prevalence of sensorineural hearing loss (SNHL) in children and adolescents with sickle cell anemia (SCA) and its association with endothelial dysfunction (ED). METHODS: Fifty-two participants with stable SCA and 44 apparently healthy (AA genotype) participants aged 6-18 years were evaluated for pure tone audiometry and endothelial function using ultrasonographic imaging of the brachial artery to assess flow-mediated dilation (FMD)...
May 28, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29781568/diastolic-dysfunction-is-associated-with-exercise-impairment-in-patients-with-sickle-cell-anemia
#13
Tarek Alsaied, Omar Niss, Adam W Powell, Robert J Fleck, James F Cnota, Clifford Chin, Punam Malik, Charles T Quinn, Michael D Taylor
BACKGROUND: Left ventricular diastolic dysfunction (DD) is an independent risk factor for mortality in sickle cell anemia (SCA) and is associated with increased extracellular volume (ECV) on cardiac MRI (CMR). Exercise impairment is common in SCA, but its causes and prognostic value are not well understood. OBJECTIVE: To study the effects of DD and ECV on cardiopulmonary exercise test (CPET) in patients with SCA. METHODS AND RESULTS: As part of a prospective study to characterize the cardiomyopathy of SCA (NCT02410811), 20 children and adults with SCA underwent CMR, echocardiography, and cycle ergometer CPET (age range 8-43 years)...
May 21, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29773497/indications-for-red-cell-transfusions-in-pediatric-patients
#14
REVIEW
Jill M Cholette, Suzie A Noronha, Jerard Seghatchian, Neil Blumberg
Red cell transfusions are amongst the most common therapeutic procedures in seriously ill children, particularly in the inpatient setting. This is despite the fact that there is no evidence base for most clinical settings, with the exception of patients with hemoglobinopathies, particularly thalassemia and sickle cell anemia. Obviously exsanguinating hemorrhage and life threatening anemia are urgent indications for which no other therapeutic approach is currently available. Most transfusions are, however, given prophylactically to prevent the complications of hypoxia or hemodynamic stability, based upon expert opinion and a faith in the oxygen carrying capacity and beneficial hemodynamic properties of transfused red cells...
May 10, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29765433/left-ventricular-structural-and-functional-changes-evaluated-by-echocardiography-and-two-dimensional-strain-in-patients-with-sickle-cell-disease
#15
Ricardo Bedirian, Andrea Ribeiro Soares, Maria Christina Maioli, Jussara Fonseca Fernandes de Medeiros, Agnaldo José Lopes, Marcia Bueno Castier
Introduction: Patients with sickle cell disease have increased left ventricular size, which is not usually accompanied by changes in systolic function indexes. We assessed echocardiographic abnormalities present in patients with sickle cell anemia (SCA) and compared echocardiographic parameters to other sickle cell diseases (OSCD). Material and methods: A blind cross-sectional study with 60 patients with SCA and 16 patients with OSCD who underwent transthoracic echocardiography was performed...
April 2018: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/29752305/white-matter-integrity-and-processing-speed-in-sickle-cell-anemia
#16
Hanne Stotesbury, Fenella J Kirkham, Melanie Kölbel, Philippa Balfour, Jonathan D Clayden, Sati Sahota, Simrat Sakaria, Dawn E Saunders, Jo Howard, Rachel Kesse-Adu, Baba Inusa, Maria Pelidis, Subarna Chakravorty, David C Rees, Moji Awogbade, Olu Wilkey, Mark Layton, Christopher A Clark, Jamie M Kawadler
OBJECTIVE: The purpose of this retrospective cross-sectional study was to investigate whether changes in white matter integrity are related to slower processing speed in sickle cell anemia. METHODS: Thirty-seven patients with silent cerebral infarction, 46 patients with normal MRI, and 32 sibling controls (age range 8-37 years) underwent cognitive assessment using the Wechsler scales and 3-tesla MRI. Tract-based spatial statistics analyses of diffusion tensor imaging (DTI) and neurite orientation dispersion and density imaging (NODDI) parameters were performed...
May 11, 2018: Neurology
https://www.readbyqxmd.com/read/29745276/prevalence-and-characteristics-of-priapism-in-sickle-cell-disease
#17
Giovanna A O Arduini, Alessandra B Trovó de Marqui
Priapism is a pathological condition of persistent penile erection in the absence of sexual arousal or desire. It is an urological emergency and its identification is important as lack of prompt treatment can result in erectile dysfunction. The aim of this study was to estimate and describe the characteristics (number of episodes, duration, time of occurrence and evolution) of priapism in patients with sickle cell disease. A bibliographical research was carried out in PubMed, searching for papers published in the last 5 years...
May 10, 2018: Hemoglobin
https://www.readbyqxmd.com/read/29744323/insulin-sensitivity-inflammation-and-basal-metabolic-rate-in-adults-with-sickle-cell-anemia
#18
Kehinde Sola Akinlade, Ayodeji Samuel Kumuyi, Sheu Kadiri Rahamon, John Ayodele Olaniyi
Background: Chronic inflammation and elevated basal metabolic rate (BMR) are established features of sickle-cell anemia (SCA). However, there is little information on the possible impacts of these afore-mentioned features on glycemia and insulin sensitivity status of this group of people. Aim: This study aims to determine the insulin sensitivity status as well the effect of BMR on glycemia in adults with SCA in steady state. Materials and Methods: Fifty participants comprising 30 adults with SCA in steady state and 20 age- and gender-matched apparently healthy adults with hemoglobin genotype AA (HbAA) genotype that served as controls...
April 2018: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/29744315/oxidative-stress-in-diabetic-patients-with-sickle-cell-anemia-a-warning-call-for-endemic-areas
#19
EDITORIAL
Rajiv Mahajan
No abstract text is available yet for this article.
April 2018: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/29732592/transcranial-doppler-ultrasound-in-peninsular-arab-patients-with-sickle-cell-disease
#20
Adekunle Adekile, Meaad Hassan, Akram Asbeutah, Mohamed Al-Hinai, Omar Trad, Nayef Farhan
OBJECTIVES: Transcranial Doppler ultrasound is used to identify patients with sickle cell disease (SCD) at risk for stroke. We performed transcranial Doppler studies in patients from 4 countries in the Arabian Peninsula (Kuwait, Oman, Iraq, and United Arab Emirates) to document the prevalence of abnormal transcranial Doppler findings. METHODS: The patients were recruited from outpatient clinics and studied in a steady state. Transcranial Doppler examinations were performed with standard equipment by experienced operators...
May 6, 2018: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
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