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Sickle cell anemia

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https://www.readbyqxmd.com/read/29781568/diastolic-dysfunction-is-associated-with-exercise-impairment-in-patients-with-sickle-cell-anemia
#1
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
#2
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
#3
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/29756358/echocardiographic-parameters-to-identify-sickle-cell-patients-with-cardio-pathology
#4
Simbo Chiadika, Mary Lim-Fung, Fiorella Llanos-Chea, Astrid Serauto Canache, Wei Yang, Christina Paruthi, Xu Zhang, David D McPherson, Modupe Idowu
BACKGROUND: Sickle cell disease (SCD) affects millions of people and causes chronic hemolytic anemia leading to vasculopathies such as pulmonary hypertension and abnormalities in cardiac function that increase complications and mortality. It is therefore crucial to identify cardiac abnormalities in SCD. We aimed to assess the prevalence of echocardiographic parameters in SCD to help identify cardiopulmonary risk. METHODS: Ninety-one patients (53% male), median age of 30, body surface area (BSA) of 1...
May 14, 2018: Echocardiography
https://www.readbyqxmd.com/read/29752305/white-matter-integrity-and-processing-speed-in-sickle-cell-anemia
#5
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
#6
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
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/29724144/arrested-pneumatization-of-the-sphenoid-sinus-mimicking-skull-base-tumors-mri-prevalence-in-children-with-haematologic-diseases
#10
Taner Arpaci
OBJECTIVE: Arrested pneumatization of the sphenoid sinus (APSS) is a developmental anatomic variation but may be confused with serious diseases of the skull base. The purpose of this study was to investigate the prevalence of APSS in pediatric patients with haemotologic diseases like sickle cell anemia (SCA), thalassemia and leukemia. MATERIALS AND METHODS: One hundred and eight pediatric patients (43 girl, 65 boy; age range, 4-18 years; median age, 13 years) with haemotologic diseases who underwent at least one magnetic resonance imaging (MRI) of the head and neck between 2010-2017 in a single instution were included...
May 3, 2018: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29721517/automated-erythrocyte-detection-and-classification-from-whole-slide-images
#11
Darshana Govind, Brendon Lutnick, John E Tomaszewski, Pinaki Sarder
Blood smear is a crucial diagnostic aid. Quantification of both solitary and overlapping erythrocytes within these smears, directly from their whole slide images (WSIs), remains a challenge. Existing software designed to accomplish the computationally extensive task of hematological WSI analysis is too expensive and is widely unavailable. We have thereby developed a fully automated software targeted for erythrocyte detection and quantification from WSIs. We define an optimal region within the smear, which contains cells that are neither too scarce/damaged nor too crowded...
April 2018: Journal of Medical Imaging
https://www.readbyqxmd.com/read/29693782/access-to-hematopoietic-stem-cell-transplant-for-patients-with-sickle-cell-anemia
#12
REVIEW
Emily Riehm Meier, Theodore Johnson, Kerice Pinkney, Maria C Velez, Naynesh Kamani, Isaac Odame
Hematopoietic stem cell transplantation (HSCT) is a curative therapy for patients with phenotypically severe sickle cell anemia, and survival rates following matched-sibling HSCT are very high. However, despite cure rates much higher than HSCT for malignant diseases, the field has been slow to adopt this treatment modality for sickle cell anemia. This article explores some of the social forces that may contribute to this dichotomy.
April 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29676678/spontaneous-epidural-hematoma-secondary-to-bone-infarction-in-sickle-cell-anemia-case-report
#13
Rohini Komarla, Bruno P Soares, Joshua J Chern, Sarah S Milla
Spontaneous epidural hematoma (EDH) is a rare occurrence in patients with sickle cell disease, with a small number of cases reported. Appropriate diagnosis is critical, because rapid neurosurgical intervention may be required. This unique case illustrates clinical and MRI features of an 18-year-old woman presenting with a headache and subsequent progression to severe focal neurological symptoms. Imaging demonstrated a large EDH of mixed signal characteristics and underlying calvarial infarction, requiring emergency decompression and evacuation...
April 20, 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29675595/involvement-of-cytosolic-and-mitochondrial-iron-in-iron-overload-cardiomyopathy-an-update
#14
REVIEW
Richard Gordan, Suwakon Wongjaikam, Judith K Gwathmey, Nipon Chattipakorn, Siriporn C Chattipakorn, Lai-Hua Xie
Iron overload cardiomyopathy (IOC) is a major cause of death in patients with diseases associated with chronic anemia such as thalassemia or sickle cell disease after chronic blood transfusions. Associated with iron overload conditions, there is excess free iron that enters cardiomyocytes through both L- and T-type calcium channels thereby resulting in increased reactive oxygen species being generated via Haber-Weiss and Fenton reactions. It is thought that an increase in reactive oxygen species contributes to high morbidity and mortality rates...
April 19, 2018: Heart Failure Reviews
https://www.readbyqxmd.com/read/29673144/serum-hepcidin-concentration-in-individuals-with-sickle-cell-anemia-basis-for-the-dietary-recommendation-of-iron
#15
Juliana Omena, Cláudia Dos Santos Cople-Rodrigues, Jessyca Dias do Amaral Cardoso, Andrea Ribeiro Soares, Marcos Kneip Fleury, Flávia Dos Santos Barbosa Brito, Josely Correa Koury, Marta Citelli
Dietary iron requirements in patients with sickle cell disease (SCD) remain unclear. SCD is a neglected hemoglobinopathy characterized by intense erythropoietic activity and anemia. Hepcidin is the hormone mainly responsible for iron homeostasis and intestinal absorption. Intense erythropoietic activity and anemia may reduce hepcidin transcription. By contrast, iron overload and inflammation may induce it. Studies on SCD have not evaluated the role of hepcidin in the presence and absence of iron overload. We aimed to compare serum hepcidin concentrations among individuals with sickle cell anemia, with or without iron overload, and those without the disease...
April 17, 2018: Nutrients
https://www.readbyqxmd.com/read/29668544/splenectomy-to-optimize-hemoglobin-s-control-in-children-with-sickle-cell-anemia-on-chronic-transfusion-therapy-for-stroke-prevention
#16
L Vandy Black, Jolie S Ramesar, Catherine H Boston
Chronic transfusion therapy with the goal of maintaining a hemoglobin (Hb) S <30% is the primary recommended treatment for children with sickle cell anemia and a history of overt stroke or abnormal transcranial Doppler examination. We report chronic hypersplenism as a cause of poor HbS% control in 3 children on chronic transfusion therapy for stroke prevention. Splenectomy resulted in a 39.77% (95% confidence interval, 34.3-45.3, P<0.0001) mean reduction in HbS% with no perioperative or infectious complications suggesting the need for additional research into splenectomy as a therapeutic option for select high-risk children to optimize transfusion therapy for stroke prevention...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29667731/hemoglobin-s-monitoring-on-tosoh-g8-in-hemoglobin-a1c-mode-in-case-of-urgent-red-blood-cell-exchange
#17
Sophie Van Aelst, Helena Claerhout, Elke Nackers, Koen Desmet, Davy Kieffer
BACKGROUND: Pre- and post-transfusion hemoglobin S (HbS) levels are used to document the efficacy of red blood cell exchange (RCE) in patients with sickle cell disease (SCD). In case of urgent RCE a 24/7 short turn-around time (STAT) analysis, with the ability to identify and quantify HbS, is warranted. The use of TOSOH G8 (Tosoh Europe) is evaluated for this purpose, using the variant HbA1c mode. METHODS: Analytical performance of the HbS analysis on TOSOH G8 in variant HbA1c mode was evaluated, including assessment of imprecision and linearity for HbS...
April 18, 2018: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/29666400/the-corticosteroid-compounds-prednisolone-and-vamorolone-do-not-alter-the-nociception-phenotype-and-exacerbate-liver-injury-in-sickle-cell-mice
#18
Luis E F Almeida, Jesse M Damsker, Sarah Albani, Nina Afsar, Sayuri Kamimura, Drew Pratt, David E Kleiner, Martha Quezado, Heather Gordish-Dressman, Zenaide M N Quezado
Clinicians often hesitate prescribing corticosteroids to treat corticosteroid-responsive conditions in sickle cell disease (SCD) patients because their use can be associated with complications (increased hospital readmission, rebound pain, strokes, avascular necrosis, acute chest syndrome). Consequently, SCD patients may receive suboptimal treatment for corticosteroid-responsive conditions. We conducted a preclinical trial of dissociative (vamorolone) and conventional (prednisolone) corticosteroid compounds to evaluate their effects on nociception phenotype, inflammation, and organ dysfunction in SCD mice...
April 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29664198/hemoglobin-a-clearance-in-children-with-sickle-cell-anemia-on-chronic-transfusion-therapy
#19
Marianne E M Yee, Cassandra D Josephson, Anne M Winkler, Jennifer Webb, Naomi L C Luban, Traci Leong, Sean R Stowell, John D Roback, Ross M Fasano
BACKGROUND: Chronic transfusion therapy for sickle cell anemia reduces disease complications by diluting sickle-erythrocytes with hemoglobin A (HbA)-containing erythrocytes and suppressing erythropoiesis. Minor antigen mismatches may result in alloimmunization, but it is unknown if antigen mismatches or recipient characteristics influence HbA clearance posttransfusion. STUDY DESIGN AND METHODS: Children with sickle cell anemia on chronic transfusion therapy were followed prospectively for 12 months...
April 17, 2018: Transfusion
https://www.readbyqxmd.com/read/29663858/assessment-of-liver-and-cardiac-iron-overload-using-mri-in-patients-with-chronic-anemias-in-latin-american-countries-results-from-asimila-study
#20
Rodolfo Cancado, Nora P Watman, Clarisse Lobo, Zulay Chona, Fernando Manzur, Fabiola Traina, Miriam Park, Guillermo Drelichman, Juan Pablo Zarate, Luis Marfil
OBJECTIVES: A multicenter, noninterventional, observational study was conducted in the Latin American countries including Argentina, Brazil, Colombia, Mexico, and Venezuela to assess the prevalence of liver and cardiac iron overload using magnetic resonance imaging (MRI) in patients with chronic anemias except thalassemia. METHODS: Patients aged >10 years with transfusion-dependent anemias, except thalassemia, either with <20 units of red blood cell (RBC) transfusions with serum ferritin (SF) levels >2000 ng/mL or with ≥20 units of RBC transfusions regardless of SF level in their lifetime, were enrolled...
April 17, 2018: Hematology (Amsterdam, Netherlands)
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