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Sickle cell anemia

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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
#1
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/28067730/observations-on-iron-anemia-and-sickle-cell-trait
#2
E Randy Eichner
No abstract text is available yet for this article.
January 2017: Current Sports Medicine Reports
https://www.readbyqxmd.com/read/28052565/cerebral-hemodynamics-and-pseudo-continuous-arterial-spin-labeling-considerations-in-adults-with-sickle-cell-anemia
#3
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
#4
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
#5
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/28026839/mj%C3%A3-ltsekvestrering-hos-barn-med-sicklecellanemi-akut-livshotande-komplikation-snabb-behandling-%C3%A3-r-avg%C3%A3-rande
#6
Fredrik Larsson, Anders Åhlin, Mats Marshall Heyman, Jonas Abrahamsson
Acute splenic sequestration in children with sickle cell disease - an overview Acute splenic sequestration (ASS) is a life-threatening complication of sickle cell disease (SCD). The condition is important to recognize due to the fact that it can occur with previously unknown disease. ASS is one of the most common causes of death in children with SCD and is the result of blood suddenly getting congested in the spleen, resulting in splenomegaly, acute anemia, and hypovolemic shock. Timely and appropriate treatment is essential in preventing death...
December 20, 2016: Läkartidningen
https://www.readbyqxmd.com/read/28008293/hormonal-and-echocardiographic-abnormalities-in-adult-patients-with-sickle-cell-anemia-in-bahrain
#7
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
#8
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
#9
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
#10
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/27959846/-sickle-cell-syndrome-association-between-hemoglobin-s-and-%C3%AE-thalassemia
#11
Nehuen P Gasparini, Evangelina E Agriello, M J Lorena Zanella, María P Iommi, Juan Maradei, Marisa J Sandoval
Sickle cell syndrome HbS/β thalassemia is an inheritable mendelian type disease where two affected alleles are simultaneously present, one from HbS (βS) and the other from β thalassemia. That situation is mainly linked to individuals who share African and Mediterranean ancestors. The mutation responsible for HbS is a point mutation, whereas for β thalassemia, there are more than 200 mutations that cause different degrees of deficiency synthesis of β globin chain, which justifies the clinical and genetic heterogeneity of this syndrome...
2016: Medicina
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/27919909/severe-anemia-early-in-life-as-a-risk-factor-for-sickle-cell-kidney-disease
#15
Inmaculada Aban, Sujatha Baddam, Lee M Hilliard, Thomas H Howard, Daniel Feig, Jeffrey D Lebensburger
No abstract text is available yet for this article.
December 5, 2016: Blood
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
#16
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/27912798/bilateral-anomalous-drainage-of-the-posterior-divisions-of-renal-veins-into-the-azygos-venous-system-in-a-20-year-old-woman-a-case-report
#17
Pedro Pallangyo, Frederick Lyimo, Paulina Nicholaus, Stephano Masatu, Mohamed Janabi
BACKGROUND: Renal vein anomalies are relatively infrequent and generally asymptomatic. Preoperative knowledge of such variants is, however, of paramount importance in several angiographic and surgical procedures including renal venography, renal vein sampling, spermatic embolization, and renal transplantation. Inadequate knowledge and failure to recognize such anatomic variations may lead to several operative hazards including hemorrhage, nephrectomy, and even death. CASE PRESENTATION: We report a case of bilateral anomalous drainage of the posterior divisions of renal veins into the azygos venous system in a 20-year-old woman of African descent from Tanzania who presented to us with a 12-year history of recurrent anemia...
December 3, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27895843/severe-anemia-in-malawian-children
#18
Job Cj Calis, Kamija S Phiri, E Brian Faragher, Bernard J Brabin, Imelda Bates, Luis E Cuevas, Rob J de Haan, Ajib I Phiri, Pelani Malange, Mirriam Khoka, Paul Jm Hulshof, Lisette van Lieshout, Marcel Ghm Beld, Yik Y Teo, Kirk A Rockett, Anna Richardson, Dominic P Kwiatkowski, Malcolm E Molyneux, Michaël Boele van Hensbroek
BACKGROUND: Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. METHODS: We conducted a case-control study of 381 preschool children with severe anemia (hemoglobin concentration, <5.0 g per deciliter) and 757 preschool children without severe anemia in urban and rural settings in Malawi. Causal factors previously associated with severe anemia were studied. The data were examined by multivariate analysis and structural equation modeling...
September 2016: Malawi Medical Journal: the Journal of Medical Association of Malawi
https://www.readbyqxmd.com/read/27876368/biomechanics-and-biorheology-of-red-blood-cells-in-sickle-cell-anemia
#19
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/27872769/mantle-cell-hyperplasia-of-peripheral-lymph-nodes-as-initial-manifestation-of-sickle-cell-disease
#20
Ahmad Monabbati, Sadat Noori, Akbar Safaei, Mani Ramzi, Seyedsajjad Eghbali, Ali Adib
Sickle cell disease (SCD) is a well known hemoglobinopathy with usual manifestations including anemia, hyperbilirubinemia, and vasoocclusive complications. Despite presence of mild splenomegaly in early phase of the disease, lymphadenopathy is not an often finding of SCD. We introduce an undiagnosed case of SCD who presented in third decade of his life with multiple cervical lymphadenopathies and mild splenomegaly persistent for about five years. Histopathologic examination of the resected lymph nodes showed expansion of the mantle cell layers of secondary follicles as well as several monomorphic mantle cell nodules...
2016: Case Reports in Hematology
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