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https://www.readbyqxmd.com/read/28816916/developmental-screening-in-pediatric-sickle-cell-disease-disease-related-risk-and-screening-outcomes-in-4-year-olds
#1
Jeffrey Schatz, Alyssa Schlenz, Laura Reinman, Kelsey Smith, Carla W Roberts
OBJECTIVE: Studies of early child development in sickle cell disease (SCD) have found modest associations between disease-related risks and developmental status in infants and toddlers, but such associations are evident by early elementary school. We screened 4-year-old children with SCD using 2 screening strategies to assess if biomedical risk factors for neurologic disease are related to developmental screening outcomes at this intermediate age. METHODS: Seventy-seven 4-year-old children with SCD (M = 4...
August 4, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28816800/acute-sickle-cell-orbitopathy-masquerading-as-orbital-cellulitis
#2
Christopher M Stewart, Zuzana Sipkova, Goran D Hildebrand, Jonathan H Norris
Sickle cell orbitopathy is a rare manifestation of a sickle cell crisis and can closely mimic orbital cellulitis, both clinically and radiologically. We present a case where a child presented with what was thought to be orbital cellulitis and was treated accordingly. Subtle radiologic features of sickle cell orbitopathy were initially overlooked, resulting in an incorrect diagnosis and a treatment delay for the patient. Correctly treated most cases resolve with no adverse affects, highlighting how important it is to consider acute sickle cell orbitopathy in the differential diagnosis of acute periorbital swelling in children with sickle disease...
August 14, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28815969/-318c-t-polymorphism-of-the-ctla-4-gene-is-an-independent-risk-factor-for-rbc-alloimmunization-among-sickle-cell-disease-patients
#3
V B Oliveira, M R Dezan, F C A Gomes, S F Menosi Gualandro, J E Krieger, A C Pereira, J D Marsiglia, J E Levi, V Rocha, A Mendrone-Junior, E C Sabino, C L Dinardo
Cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) molecule is expressed on T-lymphocyte membrane and negatively influences the antigen-presenting process. Reduced expression of CTLA-4 due to gene polymorphisms is associated with increased risk of autoimmune disorders, whose physiopathology is similar to that of post-transfusion red blood cell (RBC) alloimmunization. Our goal was to evaluate if polymorphisms of CTLA-4 gene that affect protein expression are associated with RBC alloimmunization. This was a case-control study in which 134 sickle cell disease (SCD) patients and 253 non-SCD patients were included...
August 17, 2017: International Journal of Immunogenetics
https://www.readbyqxmd.com/read/28815799/characterization-of-opioid-use-in-sickle-cell-disease
#4
Jin Han, Jifang Zhou, Santosh L Saraf, Victor R Gordeuk, Gregory S Calip
PURPOSE: Opioid analgesics are commonly used to treat vaso-occlusive pain episodes in sickle cell disease (SCD), but comprehensive evidence characterizing opioid use in this patient population is limited. Our objective was to characterize opioid use patterns among SCD patients using a large nationwide database. METHODS: A large, US medical claims database was utilized to identify a cohort of 3882 SCD patients, and characteristics of opioid use were analyzed. Clinical variables including age, gender, medication use, health care utilization, and medical history were evaluated for correlations with opioid use...
August 16, 2017: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/28815042/predictive-role-of-pd-l1-expression-in-the-response-of-renal-medullary-carcinoma-to-pd-1-inhibition
#5
Quaovi Sodji, Kandy Klein, Kavuri Sravan, Jigarkumar Parikh
BACKGROUND: Renal medullary carcinoma is one of the rarest malignancies arising from the kidney. Despite various aggressive therapeutic regimens, mortality remains significantly high (95%) with a median overall survival of 5 months. Furthermore, the scarcity of this malignancy renders randomized clinical trials impossible. We examined the expression of programmed death ligand 1 (PD-L1) in two new renal medullary carcinoma cases, investigated their responses to the PD-L1 inhibitor nivolumab and explored the predictive role of the rate of PD-L1 expression in such response...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28814957/induced-pluripotent-stem-cell-for-the-study-and-treatment-of-sickle-cell-anemia
#6
Luiza Cunha Junqueira Reis, Virgínia Picanço-Castro, Bárbara Cristina Martins Fernandes Paes, Olívia Ambrozini Pereira, Isabela Gerdes Gyuricza, Fabiano Tófoli de Araújo, Mariana Morato-Marques, Lílian Figueiredo Moreira, Everton de Brito Oliveira Costa, Tálita Pollyanna Moreira Dos Santos, Dimas Tadeu Covas, Lygia da Veiga Pereira Carramaschi, Elisa Maria de Sousa Russo
Sickle cell anemia (SCA) is a monogenic disease of high mortality, affecting millions of people worldwide. There is no broad, effective, and safe definitive treatment for SCA, so the palliative treatments are the most used. The establishment of an in vitro model allows better understanding of how the disease occurs, besides allowing the development of more effective tests and treatments. In this context, iPSC technology is a powerful tool for basic research and disease modeling, and a promise for finding and screening more effective and safe drugs, besides the possibility of use in regenerative medicine...
2017: Stem Cells International
https://www.readbyqxmd.com/read/28811769/significance-of-electronic-health-records-a-comparative-study-of-vaccination-rates-in-patients-with-sickle-cell-disease
#7
Asli Korur, Süheyl Asma, Cigdem Gereklioglu, Soner Solmaz, Can Boga, Akatlı Kürsat Ozsahin, Altug Kut
OBJECTIVE: In this study, we investigated the influence of electronic health records (EHR) and electronic vaccination schedule applications on the vaccination status of patients who were admitted to our Center for the treatment of sickle cell disease (SCD). METHODS: The vaccination status against influenza and pneumococcus infection was determined in 93 patients who were admitted to the hematology outpatient clinic, Baskent University Adana Hospital from April 2004 to March 2009...
May 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28811740/multiple-testing-in-the-context-of-gene-discovery-in-sickle-cell-disease-using-genome-wide-association-studies
#8
Kevin H M Kuo
The issue of multiple testing, also termed multiplicity, is ubiquitous in studies where multiple hypotheses are tested simultaneously. Genome-wide association study (GWAS), a type of genetic association study that has gained popularity in the past decade, is most susceptible to the issue of multiple testing. Different methodologies have been employed to address the issue of multiple testing in GWAS. The purpose of the review is to examine the methodologies employed in dealing with multiple testing in the context of gene discovery using GWAS in sickle cell disease complications...
2017: Genomics Insights
https://www.readbyqxmd.com/read/28811305/red-blood-cells-in-thrombosis
#9
James R Byrnes, Alisa S Wolberg
Red blood cells (RBCs) have historically been considered passive bystanders in thrombosis. However, clinical and epidemiological studies have associated quantitative and qualitative abnormalities in RBCs, including altered hematocrit, sickle cell disease, thalassemia, hemolytic anemias, and malaria, with both arterial and venous thrombosis. A growing body of mechanistic studies suggests RBCs can promote thrombus formation and enhance thrombus stability. These findings suggest RBCs may contribute to thrombosis pathophysiology and reveal potential strategies for therapeutically targeting RBCs to reduce thrombosis...
August 15, 2017: Blood
https://www.readbyqxmd.com/read/28810839/relationships-between-sickle-cell-trait-malaria-and-educational-outcomes-in-tanzania
#10
Kevin Croke, Deus S Ishengoma, Filbert Francis, Julie Makani, Mathias L Kamugisha, John Lusingu, Martha Lemnge, Horacio Larreguy, Günther Fink, Bruno P Mmbando
BACKGROUND: Sickle Cell Trait (SCT) has been shown to be protective against malaria. A growing literature suggests that malaria exposure can reduce educational attainment. This study assessed the relationship and interactions between malaria, SCT and educational attainment in north-eastern Tanzania. METHODS: Seven hundred sixty seven children were selected from a list of individuals screened for SCT. Febrile illness and malaria incidence were monitored from January 2006 to December 2013 by community health workers...
August 15, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28810008/sickle-cell-therapy-drought-ends
#11
Rebecca Voelker
No abstract text is available yet for this article.
August 15, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/28808516/double-whammy-acute-splenic-sequestration-crisis-in-patient-with-aplastic-crisis-due-to-acute-parvovirus-infection
#12
Parminder S Minhas, Jaspreet K Virdi, Rajeshkumar Patel
Splenic dysfunction is a major feature of sickle cell disease (SCD) and can manifest as acute splenic sequestration crisis (ASSC), which is the earliest life-threatening complication seen in patients with SCD. Aplastic crisis is another potentially deadly complication of sickle cell disease that develops when erythrocyte production temporarily drops. Infection with parvovirus B-19 frequently causes aplastic crises. These two complications are known to be mutually exclusive due to their classic presentation signs and symptoms but there have been few cases where a patient can have concomitant presentation of both phenomena, which can result in a fatal outcome...
July 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28807879/apigenin-induced-abcc1-mediated-efflux-of-glutathione-from-mature-erythrocytes-inhibits-the-proliferation-of-plasmodium-falciparum
#13
Ohud Fallatah, Elias Georges
Plasmodium falciparum infection of mature normal erythrocytes leads to heightened oxidative stress that is tolerated in normal but not in erythrocytes from sickle-cell, β-thalassemia, and glucose-6-phosphate dehydrogenase (G6PD) deficiency hosts. In this report, it was of interest to perturb the redox homeostasis of normal erythrocytes through drug-induced active efflux of glutathione via erythrocyte ABCC1 (member of the C- subfamily of the human ATP-binding cassette (ABC) transporters). To achieve this objective, we made use of apigenin, shown previously to activate ABCC1 glutathione efflux in mature erythrocytes...
August 11, 2017: International Journal of Antimicrobial Agents
https://www.readbyqxmd.com/read/28807656/opportunities-for-model-based-precision-dosing-in-the-treatment-of-sickle-cell-anemia
#14
Min Dong, Tomoyuki Mizuno, Alexander A Vinks
Hydroxyurea is the primary pharmacotherapy to prevent complications of sickle cell anemia (SCA). Accumulated clinical experience across multiple age ranges has suggested that the use of an individualized maximum tolerated dose (MTD) will achieve optimal benefit of hydroxyurea treatment. However, the current empirical and trial-and-error approach for dose escalation often results in a lengthy titration process and is not strictly implemented in many clinics. Opportunities exist for pharmacokinetics model-based precision dosing of hydroxyurea to quickly achieve individual MTD...
August 9, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28807004/predictive-role-of-pd-l1-expression-in-the-response-of-renal-medullary-carcinoma-to-pd-1-inhibition
#15
Quaovi Sodji, Kandy Klein, Kavuri Sravan, Jigarkumar Parikh
BACKGROUND: Renal medullary carcinoma is one of the rarest malignancies arising from the kidney. Despite various aggressive therapeutic regimens, mortality remains significantly high (95%) with a median overall survival of 5 months. Furthermore, the scarcity of this malignancy renders randomized clinical trials impossible. We examined the expression of programmed death ligand 1 (PD-L1) in two new renal medullary carcinoma cases, investigated their responses to the PD-L1 inhibitor nivolumab and explored the predictive role of the rate of PD-L1 expression in such response...
August 15, 2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28806176/evaluating-the-use-of-ketamine-for-pain-control-with-sickle-cell-crisis-in-pregnancy-a-report-of-2-cases
#16
Alexis C Gimovsky, Kate Fritton, Eugene Viscusi, Amanda Roman
Sickle cell crises occur frequently during pregnancy and are difficult to treat, even with high-dose opioids. Analgesia with ketamine has been suggested as an alternative, but its use during pregnancy is underreported. Two pregnant patients with uncontrolled sickle cell pain were treated with ketamine. Patient A reported no decrease in her pain, but her opioid requirements decreased. Patient B's pain resolved during ketamine administration. No serious maternal or neonatal adverse effects occurred. Ketamine may be considered as an adjunct analgesic in pregnant patients with sickle cell pain, although prospective clinical data are needed to fully assess its efficacy...
August 10, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28805282/autologous-breast-reconstruction-in-sickle-cell-trait-a-fine-line-between-success-failure
#17
Danny A Young-Afat, Marije J Hoornweg, Martine A van Huizum
Autologous breast reconstructions are increasingly being performed. While increased risks for flap loss after free tissue transfer (FTT) in sickle cell patients are known to most plastic surgeons, risks for patients with sickle cell trait (SCT) are less known. Unfortunately, recommendations to prevent complications after FTT in SCT are still scarce. We present additional and novel surgical measures that we took aiming to enhance probabilities for successful FTT in a SCT patient undergoing autologous breast reconstruction...
August 14, 2017: Breast Journal
https://www.readbyqxmd.com/read/28802541/opioid-doses-and-acute-care-utilization-outcomes-for-adults-with-sickle-cell-disease-emergency-department-versus-acute-care-unit
#18
Robert E Molokie, Chariz Montminy, Corissa Dionisio, Muhammad Ahmen Farooqui, Michel Gowhari, Yingwei Yao, Marie L Suarez, Miriam O Ezenwa, Judith M Schlaeger, Zaijie J Wang, Diana J Wilkie
BACKGROUND: Acute care units (ACUs) with focused sickle cell disease (SCD) care have been shown to effectively address pain and limit hospitalizations compared to emergency departments (ED), the reason for differences in admission rates is understudied. Our aim was compare effects of usual care for adult SCD pain in ACU and ED on opioid doses and discharge pain ratings, hospital admission rates and lengths of stay. METHODS: In a retrospective, comparative cohort, single academic tertiary center study, 148 adults with sickle cell pain received care in the ED, ACU or both...
July 13, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28801954/an-analysis-of-inpatient-pediatric-sickle-cell-disease-incidence-costs-and-outcomes
#19
Laura M Bou-Maroun, Fabien Meta, Curtis J Hanba, Andrew D Campbell, Gregory A Yanik
OBJECTIVE: To identify characteristics of pediatric sickle cell disease (SCD) hospitalizations and to examine admission demographics and medical expenditures. METHODS: Admissions with SCD were identified from the 2009 and 2012 releases of the Healthcare and Cost Utilization Project's Kids Inpatient Database. Disease-specific secondary diagnoses including acute chest syndrome (ACS), vaso-occlusive pain crisis (VOC), splenic sequestration, and stroke/transient ischemic attack were analyzed for patient and hospital demographics...
August 12, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28801805/pulmonary-cta-in-sickle-cell-patients-quantitative-assessment-of-enhancement-quality
#20
Jeff Jensen, Tony Lin, Elliot K Fishman, Pamela T Johnson
PURPOSE: The purpose of this study was to validate the observation that pulmonary artery (PA) enhancement is often decreased in sickle cell disease (SCD) patients imaged with MDCT for suspected pulmonary embolism and determine whether contrast infusion parameters are accountable for lower enhancement levels. MATERIALS AND METHODS: Retrospective comparison of 35 adult SCD patients imaged for suspected pulmonary embolism (PE) in our emergency department using 128-slice dual source MDCT scanner to 34 age and weight matched adult controls...
August 11, 2017: Emergency Radiology
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