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https://www.readbyqxmd.com/read/29468808/prevalence-of-left-ventricular-hypertrabeculation-noncompaction-among-children-with-sickle-cell-disease
#1
M Louise Morrison, Corrina McMahon, Riona Tully, Noelle Enright, Ricardo Pignatelli, Jeffrey A Towbin, Colin J McMahon
OBJECTIVES: Incidence of sickle cell disease (SCD) in Ireland has dramatically increased. Disease survival has also steadily improved however cardiovascular manifestations remain important causes of morbidity. These include reports of left ventricular hypertrabeculation (LVHT)/noncompaction. We sought to investigate the prevalence of LVHT among a large cohort of children with SCD. METHODS: We retrospectively reviewed the records of all patients with a diagnosis of SCD who had undergone surveillance echocardiography at Our Lady's Children's Hospital Crumlin (OLCHC) from 1998 to 2015...
February 21, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29468088/neck-circumference-is-independently-associated-with-relative-systemic-hypertension-in-young-adults-with-sickle-cell-anaemia
#2
Lawrence A Olatunji, Olatunde P Olabode, Olawale M Akinlade, Abiola S Babatunde, Victoria A Olatunji, Ayodele O Soladoye
Background: A seemingly interesting observation in patients with sickle cell anaemia (SCA) is that they usually have lower systemic blood pressures (BP) and insulin resistance than persons in the general population in spite of chronic inflammation and vasculopathy. However, relative systemic hypertension (rHTN) has been linked to pulmonary hypertension, increased blood viscosity and renal insufficiency, which could indicate a risk of developing cardiometabolic disorder (CMD) in SCA.We therefore hypothesized that neck circumference (NC) and CMD marker; triglyceride glucose (TyG) index would independently predict rHTN in young adults with SCA in steady state...
2018: Clinical Hypertension
https://www.readbyqxmd.com/read/29465435/clinical-manifestations-of-sickle-cell-disease-in-india-misconceptions-and-reality
#3
Dipty Jain, Dipika Mohanty
PURPOSE OF REVIEW: In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India. RECENT FINDINGS: The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the other and also among different communities of India...
February 19, 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29461260/hemolysis-and-immune-regulation
#4
Hui Zhong, Karina Yazdanbakhsh
PURPOSE OF REVIEW: Hemolytic anemias caused by premature destruction of red blood cells occur in many disorders including hemoglobinopathies, autoimmune conditions, during infection or following reaction to drugs or transfusions. Recent studies which will be reviewed here have uncovered several novel mechanisms by which hemolysis can alter immunological functions and increase the risk of severe complications in hemolytic disorders. RECENT FINDINGS: Plasma-free heme can induce the formation of neutrophil extracellular traps (NETs) through reactive oxygen species signaling...
February 15, 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29460151/sphingosine-kinases-as-druggable-targets
#5
Susan Pyne, David R Adams, Nigel J Pyne
There is substantial evidence that the enzymes, sphingosine kinase 1 and 2, which catalyse the formation of the bioactive lipid sphingosine 1-phosphate, are involved in pathophysiological processes. In this chapter, we appraise the evidence that both enzymes are druggable and describe how isoform-specific inhibitors can be developed based on the plasticity of the sphingosine-binding site. This is contextualised with the effect of sphingosine kinase inhibitors in cancer, pulmonary hypertension, neurodegeneration, inflammation and sickling...
February 20, 2018: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/29459905/perception-of-treatment-needs-and-use-of-dental-services-for-children-and-adolescents-with-sickle-cell-disease
#6
Ana Luna, Monalisa Gomes, Ana Granville-Garcia, Valdenice Menezes
PURPOSE: To evaluate the perception of treatment needs and the use of dental services for children/adolescents with sickle cell disease. MATERIALS AND METHODS: A cross-sectional study was conducted with 250 children/adolescents with sickle cell disease at a reference center in the city of Recife, Brazil. Data on the use of dental services were collected using a 13-item questionnaire administered to parents/guardians. The examiner in charge of determining l caries and periodontal status had previously undergone a training and calibration exercise...
2018: Oral Health & Preventive Dentistry
https://www.readbyqxmd.com/read/29459113/sickle-cell-trait-and-diagnosis-of-type-2-diabetes
#7
Sarah Skinner, Vincent Pialoux, Bérengère Fromy, Dominique Sigaudo-Roussel, Philippe Connes
No abstract text is available yet for this article.
February 16, 2018: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/29456754/management-of-osteonecrosis-of-the-femoral-head-in-children-with-sickle-cell-disease-results-of-conservative-and-operative-treatments-at-skeletal-maturity
#8
C Mallet, A Abitan, C Vidal, L Holvoet, K Mazda, A-L Simon, B Ilharreborde
Purpose: Sickle cell disease (SCD) is the most common cause of femoral head osteonecrosis (ONFH) during childhood with an overall prevalence of 10%. In children, spontaneous revascularization can occur, as in Legg-Calve-Perthes disease. Consequently, the aim of treatment is to restore proper hip containment to prevent joint arthritis. This is the first study reporting long-term results at skeletal maturity of non-operative and surgical treatments for ONFH in SCD children. Methods: All children with ONFH due to SCD were retrospectively reviewed...
February 1, 2018: Journal of Children's Orthopaedics
https://www.readbyqxmd.com/read/29453648/supplemental-diagnosis-of-gyrodactylus-fairporti-van-cleave-1921-monogenea-from-1-month-old-black-bullhead-ameiurus-melas-in-riverside-pools-in-wisconsin
#9
Eric Leis, Russell Easy, David Cone
Samples of Gyrodactylus fairporti Van Cleave, 1921 from young-of-the-year black bullhead (Ameiurus melas) stranded in riverside pools of the Black River (La Crosse County,) WI, USA, are used to supplement the species diagnosis, including new details on the marginal hook sickles, the male copulatory organ (MCO), and 18S rRNA gene sequence data. The anchors of G. fairporti are relatively long and thin, 58.2 ± 1.2 μm in length; roots 15.5 ± 1.0 μm; shaft 38.1 ± 1.5 μm; point 31.3 ± 1.5 μm. The ventral bar is 19...
February 17, 2018: Parasitology Research
https://www.readbyqxmd.com/read/29453624/hydroxyurea-responses-in-clinically-varied-beta-hbe-beta-thalassaemia-and-sickle-cell-anaemia-patients-of-eastern-india
#10
Tridip Chatterjee, Amit Chakravarty, Sudipa Chakravarty
The haematological and clinical response to hydroxyurea was estimated in HbE-beta, beta thalassaemia and sickle cell anaemia patients of Eastern India, with variable clinical severity and transfusion requirement to determine whether hydroxyurea can help these patients to maintain their steady haemoglobin level without blood transfusions. Three hundred patients (189 HbE-beta thalassaemia, 95 beta thalassaemia and 16 other haemoglobinopathies including sickle cell anaemia) were selected for hydroxyurea therapy and were followed up for 48-60 months...
February 17, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29451227/statural-growth-and-prevalence-of-endocrinopathies-in-relation-to-liver-iron-content-lic-in-adult-patients-with-beta-thalassemia-major-btm-and-sickle-cell-disease-scd
#11
Mohamed A Yassin, Ashraf T Soliman, Vincenzo De Sanctis, Mohammad Aj Abdula, Lubna M Riaz, Firdous F Ghori, Anil Yousaf, Abdulqadir J Nashwan, Sandara Abusamaan, Abbas Moustafa, Samah Kohla, Dina S Soliman
Despite regular blood transfusion and iron chelation therapy, growth impairment and pubertal delay are commonly seen in children and adolescents with transfusion-dependent Beta thalassaemia major (BTM) and sickle cell disease (SCD). We evaluated growth parameters and endocrine disorders in relation to the liver iron concentration (LIC) assessed by the Ferriscan® method in a cohort of adults with SCD (n =40) and BTM (n = 52) receiving blood transfusions and iron chelation therapy since early childhood. Before transfusion, hemoglobin concentration had not been less than 9 g/dl in the past 12 years; subcutaneous daily desferrioxamine was administered for all of them since early childhood (2- 5 years of age)...
February 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29450676/comorbid-obstructive-sleep-apnea-and-increased-risk-for-sickle-cell-disease-morbidity
#12
Tal Katz, Jeffrey Schatz, Carla W Roberts
PURPOSE: Sickle cell disease (SCD) imparts an increased risk for obstructive sleep apnea (OSA) in childhood. Studies of pediatric SCD have identified an increased risk for pain and neurologic complications with comorbid OSA. We determined the rate of a broad range of SCD-related medical complications to better characterize the spectrum of SCD complications related to OSA. METHODS: Retrospective chart review at a single hematology clinic identified 641 youth with SCD who received consistent screenings for OSA as part of routine hematological health maintenance visits over an 11-year period...
February 15, 2018: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/29449005/association-between-oxidative-stress-genetic-factors-and-clinical-severity-in-children-with-sickle-cell-anemia
#13
Céline Renoux, Philippe Joly, Camille Faes, Pauline Mury, Buse Eglenen, Mine Turkay, Gokce Yavas, Ozlem Yalcin, Yves Bertrand, Nathalie Garnier, Daniela Cuzzubbo, Alexandra Gauthier, Marc Romana, Berenike Möckesch, Giovanna Cannas, Sophie Antoine-Jonville, Vincent Pialoux, Philippe Connes
OBJECTIVES: To investigate the associations between several sickle cell disease genetic modifiers (beta-globin haplotypes, alpha-thalassemia, and glucose-6-phosphate dehydrogenase deficiency) and the level of oxidative stress and to evaluate the association between oxidative stress and the rates of vaso-occlusive events. STUDY DESIGN: Steady-state oxidative and nitrosative stress markers, biological variables, genetic modulators, and vaso-occlusive crisis events requiring emergency admissions were measured during a 2-year period in 62 children with sickle cell anemia (58 SS and 4 Sβ 0 )...
February 13, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29447985/selected-terpenes-from-leaves-of-ocimum-basilicum-l-induce-hemoglobin-accumulation-in-human-k562-cells
#14
Giordana Feriotto, Nicola Marchetti, Valentina Costa, Piera Torricelli, Simone Beninati, Federico Tagliati, Carlo Mischiati
Re-expression of fetal hemoglobin (HbF) was proposed as a possible therapeutic strategy for β-haemoglobinopathies. Although several inducers of HbF were tested in clinical trials, only hydroxyurea (HU) received FDA approval. Despite it produced adequate HbF levels only in half of HU-treated SCD patients, and was ineffective at all in β-thalassemia patients, beneficial effects of this approach suggested to continue in this direction identifying further molecules capable of inducing HbF. We tested the potential of essential oil isolated from Ocimum basilicum L...
February 13, 2018: Fitoterapia
https://www.readbyqxmd.com/read/29447888/pathogenesis-of-chronic-rhinosinusitis-in-patients-affected-by-%C3%AE-thalassemia-major-and-sickle-cell-anaemia-post-allogenic-bone-marrow-transplant
#15
F Martino, R Di Mauro, K Paciaroni, J Gaziev, C Alfieri, L Greco, R Floris, S Di Girolamo, M Di Girolamo
OBJECTIVES: Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29446825/phytomedicines-medicines-derived-from-plants-for-sickle-cell-disease
#16
REVIEW
Oluseyi Oniyangi, Damian H Cohall
BACKGROUND: Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. Laboratory research and limited clinical trials have suggested positive effects of phytomedicines both in vivo and in vitro...
February 15, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29445298/presence-of-pain-on-three-or-more-days-of-the-week-is-associated-with-worse-patient-reported-outcomes-in-adults-with-sickle-cell-disease
#17
Nitya Bakshi, Diana Ross, Lakshmanan Krishnamurti
While acute episodic pain is the hallmark of sickle cell disease (SCD), transition to chronic pain is a major cause of morbidity and impaired quality of life. One of the core diagnostic criteria used by Analgesic, Anesthetic, and Addiction Clinical Trial Translations Innovations Opportunities and Networks-American Pain Society Pain Taxonomy (AAPT) to define chronic SCD pain is the presence of pain on a "majority of days" in the past 6 months in one or more locations. The frequency characteristic of "majority of days" is adapted from the criteria of 15 days or more per month, used to define chronic migraine, but there are inadequate data to support this cutoff in SCD...
2018: Journal of Pain Research
https://www.readbyqxmd.com/read/29444869/effective-screening-leads-to-better-outcomes-in-sickle-cell-disease
#18
EDITORIAL
Lisa M Shook, Russell E Ware
No abstract text is available yet for this article.
February 14, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29443408/vascular-access-for-red-blood-cell-exchange
#19
REVIEW
Zaher K Otrock, Suzanne R Thibodeaux, Ronald Jackups
Red blood cell exchange is the process of removing red blood cells from a patient and replacing them with donated blood using either automated or manual techniques. Red blood cell exchange is a well-recognized and effective therapy for many red blood cell-related diseases, especially sickle cell disease. However, decisions regarding the best methods for vascular access are not intuitive and must account for the patient's clinical condition, complication risks, and lifestyle, especially in the context of long-term vascular access...
February 2018: Transfusion
https://www.readbyqxmd.com/read/29440190/a-model-linking-sickle-cell-hemoglobinopathies-and-smarcb1-loss-in-renal-medullary-carcinoma
#20
Pavlos Msaouel, Nizar M Tannir, Cheryl Lyn Walker
Renal medullary carcinoma (RMC) is a highly aggressive malignancy that predominantly afflicts young adults and adolescents with sickle hemoglobinopathies. It is characterized by complete loss of expression of the chromatin remodeler and tumor suppressor SMARCB1. Despite therapy, the outcomes of patients with RMC remain very poor, highlighting the need to understand the etiology of this cancer, and develop new diagnostic, preventive, and therapeutic strategies. A key knowledge gap in RMC biology is why sickle hemoglobinopathies predispose to the development of this cancer...
February 12, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
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