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"Sickle Cell Anemia"

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https://www.readbyqxmd.com/read/28094443/validity-of-simple-clinical-and-biological-parameters-as-screening-tool-for-sickle-cell-anemia-for-referral-to-tertiary-center-in-highly-resource-constraints
#1
Bertin Tshimanga Kadima, Jean Lambert Gini-Ehungu, Fiston Ikwa Ndol Mbutiwi, John Tunda Bahati, Michel Ntetani Aloni
BACKGROUND: In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated around 40 000 neonates per year. However, it is notoriously difficult to perform conventional electrophoresis in all hospitals and laboratories, especially at peripheral levels and rural area. A panel of multiple clinical and laboratory features that would enhance sickle cell disease were assessed for the detection of the disease in highly resource-scarce settings. METHODS: A prospective study was conducted in Kinshasa...
January 17, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28091440/nocturnal-enuresis-in-school-aged-children-with-sickle-cell-anemia-any-relationship-with-hyposthenuria
#2
C I Eneh, A N Ikefuna, H U Okafor, S N Uwaezuoke
BACKGROUND: Reports show that children with sickle-cell anemia (SCA) have a tendency for nocturnal enuresis when compared with their counterparts with normal hemoglobin. Although nocturnal enuresis in SCA has been attributed to several factors including tubular and even bladder dysfunction, its relationship with hyposthenuria has been questioned in some studies. AIM: The study aims to determine the relationship of hyposthenuria with nocturnal enuresis seen in school-aged children with SCA...
February 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28090285/pulmonary-vascular-and-ventricular-dysfunction-in-the-susceptible-patient-2015-grover-conference-series
#3
REVIEW
Bradley A Maron, Roberto F Machado, Larissa Shimoda
Pulmonary blood vessel structure and tone are maintained by a complex interplay between endogenous vasoactive factors and oxygen-sensing intermediaries. Under physiological conditions, these signaling networks function as an adaptive interface between the pulmonary circulation and environmental or acquired perturbations to preserve oxygenation and maintain systemic delivery of oxygen-rich hemoglobin. Chronic exposure to hypoxia, however, triggers a range of pathogenetic mechanisms that include hypoxia-inducible factor 1α (HIF-1α)-dependent upregulation of the vasoconstrictor peptide endothelin 1 in pulmonary endothelial cells...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28067884/reversal-of-pre-capillary-pulmonary-hypertension-in-a-patient-with-sickle-cell-anemia-who-underwent-haploidentical-peripheral-blood-stem-cell-transplantation
#4
C Pittman, M M Hsieh, W Coles, J F Tisdale, N A Weir, C D Fitzhugh
No abstract text is available yet for this article.
January 9, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28052565/cerebral-hemodynamics-and-pseudo-continuous-arterial-spin-labeling-considerations-in-adults-with-sickle-cell-anemia
#5
Meher R Juttukonda, Lori C Jordan, Melissa C Gindville, Larry T Davis, Jennifer M Watchmaker, Sumit Pruthi, Manus J Donahue
Sickle cell anemia (SCA) is a genetic disorder resulting in reduced oxygen carrying capacity and elevated stroke risk. Pseudo-continuous arterial spin labeling (pCASL) measures of cerebral blood flow (CBF) may have relevance for stroke risk assessment; however, the effects of elevated flow velocity and reduced bolus arrival time (BAT) on CBF quantification in SCA patients have not been thoroughly characterized, and pCASL model parameters used in healthy adults are often applied to patients with SCA. Here, cervical arterial flow velocities and pCASL labeling efficiencies were computed in adults with SCA (n = 19) and age- and race-matched controls without sickle trait (n = 7) using pCASL in sequence with phase contrast MR angiography (MRA)...
January 4, 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/28035747/secondary-benefit-of-maintaining-normal-transcranial-doppler-velocities-when-using-hydroxyurea-for-prevention-of-severe-sickle-cell-anemia
#6
Djamila Labib Ghafuri, Shruti Chaturvedi, Mark Rodeghier, Sarah-Jo Stimpson, Brandi McClain, Jeannie Byrd, Michael R DeBaun
In a retrospective cohort study, we tested the hypothesis that when prescribing hydroxyurea (HU) to children with sickle cell anemia (SCA) to prevent vaso-occlusive events, there will be a secondary benefit of maintaining low transcranial Doppler (TCD) velocity, measured by imaging technique (TCDi). HU was prescribed for 90.9% (110 of 120) of children with SCA ≥5 years of age and followed for a median of 4.4 years, with 70% (n = 77) receiving at least one TCDi evaluation after starting HU. No child prescribed HU had a conditional or abnormal TCDi measurement...
December 30, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28029271/hemodynamic-mechanisms-underlying-elevated-oxygen-extraction-fraction-oef-in-moyamoya-and-sickle-cell-anemia-patients
#7
Jennifer M Watchmaker, Meher R Juttukonda, Larry T Davis, Allison O Scott, Carlos C Faraco, Melissa C Gindville, Lori C Jordan, Petrice M Cogswell, Angela L Jefferson, Howard S Kirshner, Manus J Donahue
Moyamoya is a bilateral, complex cerebrovascular condition characterized by progressive non-atherosclerotic intracranial stenosis and collateral vessel formation. Moyamoya treatment focuses on restoring cerebral blood flow (CBF) through surgical revascularization, however stratifying patients for revascularization requires abilities to quantify how well parenchyma is compensating for arterial steno-occlusion. Globally elevated oxygen extraction fraction (OEF) secondary to CBF reduction may serve as a biomarker for tissue health in moyamoya patients, as suggested in patients with sickle cell anemia (SCA) and reduced oxygen carrying capacity...
January 1, 2016: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28008293/hormonal-and-echocardiographic-abnormalities-in-adult-patients-with-sickle-cell-anemia-in-bahrain
#8
Taysir S Garadah, Ahmed A Jaradat, Mohammed E Alalawi, Adla B Hassan
BACKGROUND: Adrenal, thyroid, and parathyroid gland hormonal changes are recognized in children with homozygous (HbSS) sickle-cell anemia (SCA), but are not clear in adult patients with SCA. AIM: To assess the metabolic and endocrine abnormalities in adult patients with SCA and evaluate left ventricular (LV) systolic and diastolic functions compared with patients with no SCA and further study the relationship between serum levels of cortisol, free thyroxine (T4), and testosterone with serum ferritin...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27994488/an-unfortunate-case-of-acquired-hemochromatosis-a-case-report-review-of-the-clinical-presentation-diagnosis-management-and-prognosis
#9
Anam Tariq, Kevin Westra, Arben Santo
BACKGROUND: While blood transfusions are commonly used for prophylaxis and treatment for acute chest syndromes and strokes in sickle cell patients, accumulation of excess iron resulting in secondary hemochromatosis remains a rare disease. Chelation is the mainstay for preventing and treating iron overload to deter potential end-organ damages; it is rare when therapy fails. CASE REPORT: A 52-year-old African American woman with chronic anemia secondary to sickle cell anemia and history of multiple blood transfusions presented with elevated serum ferritin (8000 ng/mL) and bilirubin (16...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27981798/frequencies-and-phenotypic-consequences-of-association-of-%C3%AE-and-%C3%AE-thalassemia-alleles-with-sickle-cell-disease-in-bahrain
#10
S Abuamer, D K Shome, A Jaradat, A Radhi, J P Bapat, K A Sharif, J Al-Touq, A Al-Asheeri, A Al-Ajami
INTRODUCTION: Bahrain has high prevalence rates of sickle cell and thalassemia in the population. This study reports the frequencies and phenotypic characteristics of α- and/or β-thalassemia associated with sickle-cell disease (SCD) in a tertiary care hospital. METHODS: Adult SCD patients (n = 200) were screened for the common α- and β-thalassemia alleles prevalent in the region using molecular techniques. Results of CBC, hemoglobin analysis, and average annual frequencies of severe pain episodes and numbers of transfused red cell units were documented...
December 16, 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27979584/impact-of-chronic-conditions-on-emergency-department-visits-of-children-using-medicaid
#11
Jay G Berry, Jonathan Rodean, Matthew Hall, Elizabeth R Alpern, Paul L Aronson, Stephen B Freedman, David C Brousseau, Samir S Shah, Harold K Simon, Eyal Cohen, Jennifer R Marin, Rustin B Morse, Margaret O'Neill, Mark I Neuman
OBJECTIVE: To assess the impact of chronic conditions on children's emergency department (ED) use. STUDY DESIGN: Retrospective analysis of 1 850 027 ED visits in 2010 by 3 250 383 children ages 1-21 years continuously enrolled in Medicaid from 10 states included in the Truven Marketscan Medicaid Database. The main outcome was the annual ED visit rate not resulting in hospitalization per 1000 enrollees. We compared rates by enrollees' characteristics, including type and number of chronic conditions, and medical technology (eg, gastrostomy, tracheostomy), using Poisson regression...
December 13, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27957790/transcranial-doppler-in-hemoglobin-sc-disease
#12
Camilo Vieira, Carolina Nogueira Costa de Oliveira, Ludmila Araújo Borges de Figueiredo, Rayra Pereira Santiago, Corynne Stephanie Ahouefa Adanho, Sanzio Silva Santana, Caroline Lang Burak, Isa Menezes Lyra, Marilda Souza Goncalves
BACKGROUND: Stroke is a severe clinical disorder in sickle cell disease (SCD), and few studies have evaluated transcranial Doppler (TCD) flow velocities in hemoglobin SC disease (HbSC). The guidelines for stroke risk are based on evaluations in sickle cell anemia (SCA) or HbS/β thalassemia. PROCEDURE: In this study, we compare cerebral blood flow in patients with SCD stratified by genotypes. A total of 1,664 pediatric patients with SCD underwent TCD velocity screening, and the time-averaged maximum mean velocity (TAMM) was determined in the middle cerebral artery (MCA), anterior cerebral artery (ACA), and distal intracranial internal carotid artery (ICA) ...
December 13, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27951596/expression-pattern-of-hif-1%C3%AE-and-vegf-supports-circumferential-application-of-scatter-laser-for-proliferative-sickle-retinopathy
#13
Murilo Rodrigues, Fabiana Kashiwabuchi, Monika Deshpande, Kathleen Jee, Morton F Goldberg, Gerard Lutty, Gregg L Semenza, Silvia Montaner, Akrit Sodhi
Purpose: Retinal vascular occlusions in sickle cell anemia patients cause tissue ischemia and the release of angiogenic mediators that promote the development of retinal neovascularization, initiating proliferative sickle retinopathy (PSR). Laser photocoagulation (LPC) has emerged as the most common treatment for PSR. Nonetheless, only two randomized controlled clinical trials have evaluated the use of LPC for PSR, and both failed to definitively demonstrate efficacy of this approach...
December 1, 2016: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/27930540/invasive-bacterial-infections-in-gambians-with-sickle-cell-anemia-in-an-era-of-widespread-pneumococcal-and-hemophilus-influenzae-type-b-vaccination
#14
Germander Soothill, Saffiatou Darboe, Gibril Bah, Lawal Bolarinde, Aubrey Cunnington, Suzanne T Anderson
There is relatively little data on the etiology of bacterial infections in patients with sickle cell anemia (SCA) in West Africa, and no data from countries that have implemented conjugate vaccines against both Streptococcus pneumoniae and Haemophilus influenzae type b (Hib).We conducted a retrospective analysis of SCA patients admitted to the Medical Research Council Unit, The Gambia, during a 5-year period when there was high coverage of Hib and Pneumococcal conjugate vaccination. We evaluated 161 admissions of 126 patients between April 2010 and April 2015...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27913539/prevention-of-central-nervous-system-sequelae-in-sickle-cell-disease-without-evidence-from-randomized-controlled-trials-the-case-for-a-team-based-learning-collaborative
#15
Michael R DeBaun, Allison A King
Since 1998, the National Institutes of Health has funded 5 randomized controlled trials (RCTs) for primary and secondary prevention of strokes in children with sickle cell anemia (SCA). In a systematic fashion, these trials have significantly advanced the care of children with SCA. In the absence of an RCT, clinicians are often compelled to make decisions at the bedside, based on experience, observational studies, and principles of hematology. We will provide an initial example that describes how a team-based, learning collaborative developed a multisite standard care protocol with a low budget (<$10 000 per year) to overcome the intrinsic limitations of advancing the care of neurologic complications in sickle cell disease (SCD)...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27876368/biomechanics-and-biorheology-of-red-blood-cells-in-sickle-cell-anemia
#16
Xuejin Li, Ming Dao, George Lykotrafitis, George Em Karniadakis
Sickle cell anemia (SCA) is an inherited blood disorder that causes painful crises due to vaso-occlusion of small blood vessels. The primary cause of the clinical phenotype of SCA is the intracellular polymerization of sickle hemoglobin resulting in sickling of red blood cells (RBCs) in deoxygenated conditions. In this review, we discuss the biomechanical and biorheological characteristics of sickle RBCs and sickle blood as well as their implications toward a better understanding of the pathophysiology and pathogenesis of SCA...
January 4, 2017: Journal of Biomechanics
https://www.readbyqxmd.com/read/27872735/fetal-hemoglobin-modifies-the-disease-manifestation-of-severe-plasmodium-falciparum-malaria-in-adult-patients-with-sickle-cell-anemia
#17
Prasanta Purohit, Siris Patel, Pradeep Kumar Mohanty, Padmalaya Das, Jogeswar Panigrahi
No abstract text is available yet for this article.
2016: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/27871907/prognostic-value-of-t786c-and-g894t-enos-polymorphisms-in-sickle-cell-disease
#18
Iakovos Armenis, Vassiliki Kalotychou, Revekka Tzanetea, Panagoula Kollia, Zoi Kontogeorgiou, Dimitra Anastasopoulou, Marina Mantzourani, Michael Samarkos, Konstantinos Pantos, Kostas Konstantopoulos, Ioannis Rombos
Endothelial Nitric Oxide Synthase (eNOS) is crucial for vascular homeostasis. Polymorphisms T786C and G894T affect eNOS regulation and have been related to various diseases. Sickle Cell Disease (SCD), a clinically diverse chronic hemolytic anemia, implies impaired nitric oxide bioavailability. Our aim was to determine eNOS genotype for T786C and G894T polymorphisms in Greek patients with SCD and to elucidate its consequences and effects if any on clinical phenotype. Seventy nine steady state cases, mostly compound heterozygous for Sickle Cell anemia/beta thalassemia and 48 controls were measured...
January 30, 2017: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/27869662/the-cell-killing-mechanisms-of-hydroxyurea
#19
REVIEW
Amanpreet Singh, Yong-Jie Xu
Hydroxyurea is a well-established inhibitor of ribonucleotide reductase that has a long history of scientific interest and clinical use for the treatment of neoplastic and non-neoplastic diseases. It is currently the staple drug for the management of sickle cell anemia and chronic myeloproliferative disorders. Due to its reversible inhibitory effect on DNA replication in various organisms, hydroxyurea is also commonly used in laboratories for cell cycle synchronization or generating replication stress. However, incubation with high concentrations or prolonged treatment with low doses of hydroxyurea can result in cell death and the DNA damage generated at arrested replication forks is generally believed to be the direct cause...
November 17, 2016: Genes
https://www.readbyqxmd.com/read/27860202/parent-reported-sleep-problems-in-preschool-children-with-sickle-cell-anemia-and-controls-in-east-london
#20
Michelle Downes, Michelle de Haan, Fenella J Kirkham, Paul T Telfer
Snoring and poor sleep may affect cognition, particularly in young children with chronic conditions. Parents of London preschoolers with sickle cell anemia (SCA; n = 22), matched controls (n = 24), and unselected typically developing (n = 142) preschoolers completed sleep questionnaires. Preschoolers with SCA had significantly more sleep problems when compared to matched controls and the larger population. Snoring occurred at least one to two nights a week for 79% of the SCA group. This is compared with 25% of matched controls and 33% of larger population...
November 15, 2016: Pediatric Blood & Cancer
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