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https://www.readbyqxmd.com/read/29339944/building-a-body-of-knowledge-sickle-cell-and-libraries
#1
Richard H Nollan
No abstract text is available yet for this article.
January 2018: Journal of the Medical Library Association: JMLA
https://www.readbyqxmd.com/read/29339270/proceedings-from-the-fourth-haploidentical-stem-cell-transplantation-symposium-haplo2016-san-diego-california-december-1-2016
#2
REVIEW
Monzr Al Malki, Richard Jones, Qing Ma, Dean Lee, Yair Reisner, Jeffrey S Miller, Peter Lang, Suradej Hongeng, Parameswaran Hari, Samuel Strober, Jianhua Yu, Richard Maziarz, Domenico Mavilio, Denis-Claude Roy, Chiara Bonini, Richard E Champlin, Ephraim J Fuchs, Stefan O Ciurea
The resurgence of haploidentical stem cell transplantation (HaploSCT) over the last decade is one of the most important advances in the field of hematopoietic stem cell transplantation (HSCT). The modified platforms of T cell depletion either ex vivo (CD34+ selection, 'mega-dose' of purified CD34+ cells, or selective depletion of T-cells) or newer platforms of in vivo depletion of T cells, with either post-transplant high-dose cyclophosphamide (PTCy) or intensified immune suppression, have contributed to better outcomes, with survival that is similar to HLA-match donor transplantation...
January 12, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29338872/sickle-cell-disease-an-overview-of-orofacial-and-dental-manifestations
#3
REVIEW
Nadia Kawar, Sahar Alrayyes, Haider Aljewari
No abstract text is available yet for this article.
January 12, 2018: Disease-a-month: DM
https://www.readbyqxmd.com/read/29337222/blockade-of-placental-growth-factor-reduces-vaso-occlusive-complications-in-murine-models-of-sickle-cell-disease
#4
Jian-Ming Gu, Shujun Yuan, Derek Sim, Keith Abe, Perry Liu, Martin Rosenbruch, Peter Bringmann, Katalin Kauser
Vaso-occlusive crisis (VOC) is the most common and debilitating complication of sickle cell disease (SCD); recurrent episodes cause organ damage and contribute to early mortality. Plasma placental growth factor (PlGF) levels are elevated in SCD and can further increase under hypoxic conditions in SCD mice. Treatment with a PlGF-neutralizing antibody (anti-PlGF Ab) in SCD mice reduced levels of monocyte chemoattractant protein-3, eotaxin, macrophage colony-stimulating factor, plasminogen activator inhibitor-1 significantly, and macrophage-derived chemokine and macrophage inflammatory protein-3β moderately; this may contribute to inhibition of leukocyte recruitment, activation, and thrombosis...
January 11, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29336892/gene-therapy-and-gene-editing-strategies-for-hemoglobinopathies
#5
REVIEW
Maria Rosa Lidonnici, Giuliana Ferrari
Gene therapy for hemoglobinopathies is currently based on transplantation of autologous hematopoietic stem cells genetically modified with an integrating lentiviral vector expressing a globin gene under the control of globin transcriptional regulatory elements. Studies and safety works demonstrated the potential therapeutic efficacy and safety of this approach, providing the rationale for clinical translation. The outcomes of early clinical trials, although showing promising results, have highlighted the current limitations to a more general application...
January 3, 2018: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29335234/a-patient-held-smartcard-with-a-unique-identifier-and-an-mhealth-platform-to-improve-the-availability-of-prenatal-test-results-in-rural-nigeria-demonstration-study
#6
Semiu Olatunde Gbadamosi, Chuka Eze, John Olajide Olawepo, Juliet Iwelunmor, Daniel F Sarpong, Amaka Grace Ogidi, Dina Patel, John Okpanachi Oko, Chima Onoka, Echezona Edozie Ezeanolue
BACKGROUND: Community-based strategies to test for HIV, hepatitis B virus (HBV), and sickle cell disease (SCD) have expanded opportunities to increase the proportion of pregnant women who are aware of their diagnosis. In order to use this information to implement evidence-based interventions, these results have to be available to skilled health providers at the point of delivery. Most electronic health platforms are dependent on the availability of reliable Internet connectivity and, thus, have limited use in many rural and resource-limited settings...
January 15, 2018: Journal of Medical Internet Research
https://www.readbyqxmd.com/read/29334538/sickle-cell-trait-testing-should-not-be-a-player-in-ncaa-athletics-examining-the-media-s-role-in-disseminating-awareness-and-information
#7
Anil P George, Antonio Logan, Mindy Simpson, Paul M Kent
No abstract text is available yet for this article.
January 12, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29334305/twelve-tips-for-teaching-a-comprehensive-disease-focused-course-with-a-global-perspective-a-sickle-cell-disease-example
#8
Dominique Bulgin, Paula Tanabe, Monika Asnani, Charmaine D M Royal
A disease-focused course entitled "Understanding Sickle Cell Disease: A Biopsychosocial Approach" addressed the complex nature of SCD using patient-centered, global and interdisciplinary approaches. Sickle cell disease (SCD) is a rare inherited blood disorder that requires multidisciplinary care. Worldwide 20-25 million individuals have SCD, which is associated with a shortened lifespan due to many medical complications and social and behavioral health challenges. Health care professionals often have limited knowledge of SCD as they typically learn about it within the context of their own disciplines...
January 15, 2018: Medical Teacher
https://www.readbyqxmd.com/read/29331393/oral-health-considerations-for-pediatric-patients-with-sickle-cell-disease
#9
REVIEW
Sahar Alrayyes, Anne-Ashley Compton, Nadia Kawar
No abstract text is available yet for this article.
January 10, 2018: Disease-a-month: DM
https://www.readbyqxmd.com/read/29331296/a-case-of-autosplenectomy-in-sickle-cell-trait-following-an-exposure-to-high-altitude
#10
Uday Yanamandra, Reena Das, Pankaj Malhotra, Subhash Varma
A 24-year-old man presented with acute abdominal pain upon ascent to moderate altitude (3500 m). An immediate evaluation revealed a splenic infarct, and he was evacuated to sea level. Upon recovery, he was sent back to 3500 m without detailed etiological evaluation, whereupon he experienced recurrent episodes of left-side subcostal pain. Imaging suggested autosplenectomy, and workup revealed a negative thrombophilia profile but was positive for sickle cell trait (SCT). Individuals with SCT can be asymptomatic until exposure to severe hypoxia, upon which they can manifest clinically as sickle cell syndrome...
January 10, 2018: Wilderness & Environmental Medicine
https://www.readbyqxmd.com/read/29330014/frequency-of-red-blood-cell-genotypes-in-multi-transfused-patients-and-blood-donors-from-minas-gerais-southeast-brazil
#11
Vitor Mendonça Alves, Fernanda Bernadelli De Vito, Paulo Roberto Juliano Martins, Sheila Soares Silva, Lilian Castilho, Helio Moraes-Souza
BACKGROUND AND OBJECTIVES: The frequency of red blood cell (RBC) antigens in Brazil varies due to differences in the ethnic groups in different regions; however, these studies have not been performed in Minas Gerais, where African admixture is more prevalent in comparison with other states. Due to these facts, this study aimed to determine the frequency of RBC genotypes on Rh, Kell, Duffy and Kidd systems in blood donors and multi-transfused patients from Minas Gerais, Southeast Brazil...
December 16, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29326800/sterile-abscess-of-the-spleen-and-the-sickle-cell-trait
#12
COMMENT
Lucio Luzzatto
No abstract text is available yet for this article.
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29325430/rapid-and-sensitive-assessment-of-globin-chains-for-gene-and-cell-therapy-of-haemoglobinopathies
#13
Constantinos Christos Loucari, Petros Patsali, Thamar B van Dijk, Coralea Stephanou, Panayiota Papasavva, Maria Zanti, Ryo Kurita, Yukio Nakamura, Soteroulla Christou, Maria Sitarou, Sjaak Philipsen, Carsten Werner Lederer, Marina Kleanthous
The β-haemoglobinopathies sickle cell anaemia and β-thalassaemia are the focus of many gene-therapy studies. A key disease parameter is the abundance of globin chains, because it indicates the level of anaemia, likely toxicity of excess or aberrant globins, and therapeutic potential of induced or exogenous β-like globins. Reversed-phase high-performance liquid chromatography (HPLC) allows versatile and inexpensive globin quantification, but commonly applied protocols suffer either from long run times, high sample requirements or inability to separate murine from human β-globin chains...
January 12, 2018: Human Gene Therapy Methods
https://www.readbyqxmd.com/read/29324967/theoretical-saturation-in-qualitative-research-an-experience-report-in-interview-with-schoolchildren
#14
Luciana de Cassia Nunes Nascimento, Tania Vignuda de Souza, Isabel Cristina Dos Santos Oliveira, Juliana Rezende Montenegro Medeiros de Moraes, Rosane Cordeiro Burla de Aguiar, Liliane Faria da Silva
OBJECTIVE: report the experience of applying the theoretical data saturation technique in qualitative research with schoolchildren. METHOD: critical reading of primary sources and compilation of raw data, followed by thematic grouping through colorimetric codification and allocation of themes/types of statements in charts to find theoretical saturation for each grouping. RESULTS: colorimetric codification occurred according to previously established themes: bodily hydration; physical activities and play; handling of sickle-cell disease; feeding and clothing...
January 2018: Revista Brasileira de Enfermagem
https://www.readbyqxmd.com/read/29324963/quality-of-life-in-adults-with-sickle-cell-disease-an-integrative-review-of-the-literature
#15
Sandra Luzinete Felix de Freitas, Maria Lucia Ivo, Maria Stella Figueiredo, Maria Auxiliadora de Souza Gerk, Cristina Brandt Nunes, Fernando de Freitas Monteiro
OBJECTIVE: To identify the available evidence in the literature on health-related quality of life in adults with sickle cell disease. METHOD: integrative review of MEDLINE, CUMED, LILACS and SciELO databases, from articles developed in this area, published between 2005 and 2015, in English, Portuguese or Spanish. RESULTS: 22 articles were included, six scales were used to evaluate health-related quality of life scores: three generic and three specific...
January 2018: Revista Brasileira de Enfermagem
https://www.readbyqxmd.com/read/29324574/sleep-problem-risk-for-adolescents-with-sickle-cell-disease-sociodemographic-physical-and-disease-related-correlates
#16
Cecelia R Valrie, Krystal L Trout, Kayzandra E Bond, Rebecca J Ladd, Nichelle L Huber, Kristen J Alston, Alicia M Sufrinko, Erik Everhart, Beng R Fuh
The aims of the current study were to investigate whether SCD incurs an additional risk for poor sleep over and above the influence of sociodemographic factors (ie, race and sex) during adolescence, and to explore the relationships between sociodemographic, physical (ie, age and pubertal status), and disease-related factors (ie, SCD genotype and hydroxyurea use) on sleep problem risk during adolescence. Black adolescents (age, 12 to 17 y) with SCD (n=53) were recruited from regional pediatric SCD clinics in the southeast and a sample of healthy black adolescents (n=160) were recruited from middle and high schools...
January 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29323695/lead-toxicity-in-the-pediatric-patient-with-sickle-cell-disease-unique-risks-and-management
#17
Josephine Misun Jung, Radhika Peddinti
Lead toxicity is the result of lead ingestion, one of the most common ingestions in the pediatric population. Nationwide and statewide efforts to recognize and curtail this epidemic have led to declining rates of toxicity. In patients with sickle cell disease (SCD), lead toxicity can be an elusive diagnosis due to overlapping symptom profiles, and inconsistent follow-up with a primary care physician can make the diagnosis even more difficult. In this article, two illustrative cases of lead toxicity in patients with SCD are described...
January 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29323687/pediatric-endocrinology-lead-toxicity-in-sickle-cell-disease-and-hiv-pre-exposure-prophylaxis
#18
EDITORIAL
Joseph R Hageman
No abstract text is available yet for this article.
January 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29321025/diagnostic-challenges-of-prolonged-post-treatment-clearance-of-plasmodium-nucleic-acids-in-a-pre-transplant-autosplenectomized-patient-with-sickle-cell-disease
#19
Paul M Luethy, Sean C Murphy, Annette M Seilie, Yingda L Xie, Chuen-Yen Lau, John F Tisdale, Matthew M Hsieh, Jessica L Reinhardt, Anna F Lau, Gary A Fahle
BACKGROUND: Autosplenectomy, as a result of sickle cell disease, is an important risk factor for severe malaria. While molecular methods are helpful in providing rapid and accurate infection detection and species identification, the effect of hyposplenism on result interpretation during the course of infection should be carefully considered. CASE PRESENTATION: A 32-year old autosplenectomized Nigerian male with severe sickle cell disease was referred to the National Institutes of Health for allogenic hematopoietic stem cell transplant...
January 10, 2018: Malaria Journal
https://www.readbyqxmd.com/read/29319041/successful-pulmonary-thromboendarterectomy-in-a-patient-with-sickle-cell-disease-and-associated-resolution-of-a-leg-ulcer
#20
Abhinav Agrawal, Rakesh Shah, Matthew D Bacchetta, Arunabh Talwar
Pulmonary hypertension (PH) is a relatively frequent and severe complication of sickle cell disease (SCD). PH associated with SCD is classified as Group 5 PH. The exact pathogenesis of PH in SCD in not known. There are also very limited treatment options available at this time for such patients with Group 5 PH. Patients with SCD are predisposed to a hypercoagulable state and thus can also suffer from chronic thromboembolism. These patients can have associated chronic thromboembolic pulmonary hypertension (CTEPH), thus being classified as Group 4 PH...
January 2018: Lung India: Official Organ of Indian Chest Society
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