keyword
MENU ▼
Read by QxMD icon Read
search

sickle

keyword
https://www.readbyqxmd.com/read/28544309/increased-complications-of-chronic-erythrocytapheresis-compared-with-manual-exchange-transfusions-in-children-and-adolescents-with-sickle-cell-disease
#1
Deborah Woods, Robert J Hayashi, Michael M Binkley, Gianna W Sparks, Monica L Hulbert
BACKGROUND: Children and adolescents with sickle cell disease (SCD) are at high risk of strokes and are frequently treated with red blood cell (RBC) transfusions. The goal is to suppress hemoglobin (Hb) S while minimizing transfusion-induced iron overload. RBCs may be given via simple transfusion, manual exchange transfusion (MET), or erythrocytapheresis (aRBCX). Chronic transfusion practices vary among institutions. METHODS: This single-institution, retrospective cohort study compares Hb S control and therapy complication rates between MET and aRBCX in a cohort of children and adolescents with SCD and stroke during a 5-year period from 2008 through 2012...
May 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28542880/suicidal-death-of-erythrocytes-in-cancer-and-its-chemotherapy-a-potential-target-in-the-treatment-of-tumor-associated-anemia
#2
REVIEW
Elisabeth Lang, Rosi Bissinger, Syed M Qadri, Florian Lang
In analogy to apoptosis of nucleated cells, erythrocytes may enter eryptosis characterized by cell shrinkage and cell membrane scrambling. Eryptotic erythrocytes are rapidly cleared from circulating blood and may adhere to the vascular wall. Stimulation of eryptosis thus impairs microcirculation and leads to anemia as soon as the loss of erythrocytes cannot be fully compensated by enhanced erythropoiesis. Signaling stimulating eryptosis includes increase of cytosolic Ca(2+) -activity, ceramide, caspases, calpain, p38-kinase, protein-kinase C, Janus-activated kinase 3, casein-kinase 1α, and cyclin-dependent kinase 4...
May 23, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28542469/biophysical-markers-of-the-peripheral-vasoconstriction-response-to-pain-in-sickle-cell-disease
#3
Patjanaporn Chalacheva, Maha Khaleel, John Sunwoo, Payal Shah, Jon A Detterich, Roberta M Kato, Wanwara Thuptimdang, Herbert J Meiselman, Richard Sposto, Jennie Tsao, John C Wood, Lonnie Zeltzer, Thomas D Coates, Michael C K Khoo
Painful vaso-occlusive crisis (VOC), a complication of sickle cell disease (SCD), occurs when sickled red blood cells obstruct flow in the microvasculature. We postulated that exaggerated sympathetically mediated vasoconstriction, endothelial dysfunction and the synergistic interaction between these two factors act together to reduce microvascular flow, promoting regional vaso-occlusions, setting the stage for VOC. We previously found that SCD subjects had stronger vasoconstriction response to pulses of heat-induced pain compared to controls but the relative degrees to which autonomic dysregulation, peripheral vascular dysfunction and their interaction are present in SCD remain unknown...
2017: PloS One
https://www.readbyqxmd.com/read/28540888/assessment-of-renal-function-in-indian-patients-with-sickle-cell-disease
#4
Bhaskar V K S Lakkakula, Henu Kumar Verma, Mona Choubey, Suneeta Patra, Prafukka Kumar Khodiar, Pradeep Kumar Patra
Sickle cell disease (SCD) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin. Renal disease is one of the most frequent complications, and kidney damage starts very early and progresses throughout life causing severe complications. The present study is aimed to analyze creatinine-based estimated glomerular filtration rate (eGFR) in 616 SCD patients (507 HbSS and 109 HbSB+), receiving medical care at outpatient wing of Sickle Cell Institute, Chhattisgarh. Glomerular filtration rate (GFR) estimated using the Modification of Diet in Renal Disease (MDRD), Cockcroft-Gault, chronic kidney disease epidemiology collaboration (CKD-EPI) (<17 years analyzed with Schwartz), and SCD specific Jamaica Sickle Cell Cohort Study (JSCCS)-GFR equations were compared...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28540180/hemoglobin-and-mean-platelet-volume-predicts-diffuse-t1-mri-white-matter-volume-decrease-in-sickle-cell-disease-patients
#5
Soyoung Choi, Adam M Bush, Matthew T Borzage, Anand A Joshi, William J Mack, Thomas D Coates, Richard M Leahy, John C Wood
Sickle cell disease (SCD) is a life-threatening genetic condition. Patients suffer from chronic systemic and cerebral vascular disease that leads to early and cumulative neurological damage. Few studies have quantified the effects of this disease on brain morphometry and even fewer efforts have been devoted to older patients despite the progressive nature of the disease. This study quantifies global and regional brain volumes in adolescent and young adult patients with SCD and racially matched controls with the aim of distinguishing between age related changes associated with normal brain maturation and damage from sickle cell disease...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28538513/attention-deficit-hyperactivity-disorder-in-children-with-sickle-cell-disease-referred-for-an-evaluation
#6
Melissa A Acquazzino, Meghan Miller, Matthew Myrvik, Robert Newby, John Paul Scott
Neuropsychological deficits, including difficulties with attention, are well described in children with sickle cell disease (SCD). Very little is known about attention deficit hyperactivity disorder (ADHD) in children with SCD. The objective of this study was to determine the proportion of ADHD in children with SCD referred for neuropsychological evaluation. This prospective, cross-sectional study included patients (age, 4 to 18 y) with SCD and completion of a neuropsychological evaluation between December 2013 and March 2016...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538505/thalassemia-major-and-priapism-a-case-report-of-an-adolescent
#7
Sibel Öz, Serhan Küpeli, Gülay Sezgin, İbrahim Bayram
Priapism is defined as a prolonged pathologic penile erection without sexual stimulation. In children, priapism secondary to sickle cell disease or hematological malignancy is a frequent condition. Appropriate treatment of priapism varies; the treatment is primarily etiological, conservative management. In the present report, we aimed to present a case of asplenic thalassemia major who developed priapism, improved with hydration and ibuprofen treatment. Clinicians should take into account that priapism can be encountered in patients with thalassemia major...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538501/invasive-pneumococcal-disease-in-patients-with-sickle-cell-disease
#8
Pournima Navalkele, Bülent Özgönenel, Eric McGrath, Paul Lephart, Sharada Sarnaik
BACKGROUND: Patients with sickle cell disease (SCD) are at risk of fatal sepsis with encapsulated bacteria, such as Streptococcus pneumoniae, because of the inherent autosplenectomy that occurs in SCD. This risk is thwarted with oral penicillin prophylaxis during the first 5 years of life, and with stringent vaccination against S. pneumoniae alongside routine childhood immunization. But compared with the general African American pediatric population, the rate of invasive pneumococcal disease (IPD) in patients with SCD still remains high, resulting in hospitalization and fatality...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538131/gene-therapy-in-a-patient-with-sickle-cell-disease
#9
LETTER
Philippe Leboulch, Marina Cavazzana
No abstract text is available yet for this article.
May 25, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28538130/gene-therapy-in-a-patient-with-sickle-cell-disease
#10
LETTER
(no author information available yet)
New England Journal of Medicine, Volume 376, Issue 21, Page 2093-2094, May 2017.
May 25, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28536723/improving-medication-adherence-with-two-way-short-message-service-reminders-in-sickle-cell-disease-and-asthma-a-feasibility-randomized-controlled-trial
#11
B M Pernell, Michael R DeBaun, Kathleen Becker, Mark Rodeghier, Valencia Bryant, Robert M Cronin
INTRODUCTION: Sickle cell disease (SCD) is a childhood and adult disease that primarily affects African Americans, characterized by life threatening sequelae mitigated by medications. One-way and two-way short message service (SMS) medication reminders have differing efficacy in chronic diseases. There is limited literature about SMS medication reminders in SCD. OBJECTIVE: The goal of this study was to test the feasibility, defined by recruitment/acceptance, retention/attrition, and technology utilization, of two-way SMS medication reminders in individuals with SCD with and without asthma...
May 24, 2017: Applied Clinical Informatics
https://www.readbyqxmd.com/read/28534764/traveling-to-angola-to-validate-a-paper-based-sickle-cell-disease-test
#12
Nathaniel Z Piety
When I started graduate school, I had no idea that it would take me halfway around the world to a small clinic in Cabinda, Angola, but I'm glad that it did. Since 2011, I have been a part of the team of engineers and clinicians, led by Dr. Sergey Shevkoplyas, working to develop a simple, low-cost, paper-based test for sickle cell disease-a common inherited blood disorder that is most prevalent in sub-Saharan Africa (Figure S1).
May 2017: IEEE Pulse
https://www.readbyqxmd.com/read/28534763/paper-based-diagnostics-rethinking-conventional-sickle-cell-screening-to-improve-access-to-high-quality-health-care-in-resource-limited-settings
#13
Nathaniel Z Piety, Sergey S Shevkoplyas
Every year, hundreds of thousands of children worldwide are born with sickle cell disease, a genetic disorder that impacts the hemoglobin molecules in blood. If left undiagnosed and untreated, most affected children will die before reaching the age of five. However, highly accurate diagnostic methods and effective treatment regimens for sickle cell disease have been known for many years, and children who receive early diagnosis and subsequent comprehensive care survive well into adulthood-as evidenced by the tremendous success of universal newborn screening programs in North America and Europe...
May 2017: IEEE Pulse
https://www.readbyqxmd.com/read/28532265/encephaloduroarteriosynangiosis-edas-in-young-patients-with-cerebrovascular-complications-of-sickle-cell-disease-single-institution-experience
#14
Michael Winstead, Peter P Sun, Kenneth Martin, Janice Earl, Lynne Neumayr, Carolyn Hoppe, Elliott Vichinsky
Moyamoya syndrome occurs in sickle cell disease (SCD) as a secondary complication of large-artery stenosis. Moyamoya increases the risk of stroke, but its optimal management in SCD is not established.  Encephaloduroarteriosynangiosis (EDAS) is a neurosurgical revascularization procedure for moyamoya whose use has been reported in SCD patients. We report the outcomes of 11 patients with SCD systematically evaluated for EDAS by a multidisciplinary team and compare the rate of stroke in patients who received EDAS to those who did not...
May 22, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28530652/cd34-cells-from-dental-pulp-stem-cells-with-a-zfn-mediated-and-homology-driven-repair-mediated-locus-specific-knock-in-of-an-artificial-%C3%AE-globin-gene
#15
S Chattong, O Ruangwattanasuk, W Yindeedej, A Setpakdee, K Manotham
In humans, mutations in the β-globin gene (HBB) have two important clinical manifestations: β-thalassemia and sickle cell disease. The progress in genome editing and stem cell research may be relevant to the treatment of β-globin-related diseases. In this work, we employed zinc finger nuclease (ZFN)-mediated gene integration of synthetic β-globin cDNA into HBB loci, thus correcting almost all β-globin mutations. The integration was achieved in both HEK 293 cells and isolated dental pulp stem cell (DPSCs)...
May 22, 2017: Gene Therapy
https://www.readbyqxmd.com/read/28530510/self-reported-physical-activity-and-exercise-patterns-in-children-with-sickle-cell-disease
#16
Osarhiemen A Omwanghe, Devin S Muntz, Soyang Kwon, Simone Montgomery, Opeyemi Kemiki, Lewis L Hsu, Alexis A Thompson, Robert I Liem
PURPOSE: Sickle cell disease (SCD) significantly affects physical functioning. We examined physical activity (PA) patterns in children with SCD versus a national sample and factors associated with PA and participation in physical education and organized sports. METHOD: One hundred children with SCD completed a 58-item survey with questions from the 2009-2010 National Health and Nutrition Examination Survey (NHANES) Physical Activity Questionnaire and others on physical education and sports, disease impact, and physical functioning...
May 22, 2017: Pediatric Exercise Science
https://www.readbyqxmd.com/read/28528302/nanomolar-anti-sickling-compounds-identified-by-ligand-based-pharmacophore-approach
#17
Odailson Santos Paz, Milena de Jesus Pinheiro, Renan Fernandes do Espirito Santo, Cristiane Flora Villarreal, Marcelo Santos Castilho
Adenosine receptors are considered as potential targets for drug development against several diseases. The discovery of subtype 2B adenosine receptors role in erythrocyte sickling process proved its importance to neglected diseases such as sickle cell anemia, which affects approximately 29.000 people around the world, but whose treatment is restricted to just one FDA approved drug (hydroxyurea). In order to widen the therapeutic arsenal available to treat sickle cell anemia patients, it is imperative to identify new lead compounds that modify the sickling course and not just its symptoms...
May 12, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28527641/identifying-social-behavioral-health-needs-of-adults-with-sickle-cell-disease-in-the-emergency-department
#18
Sophia K Smith, Julia Johnston, Carlton Rutherford, Rachel Hollowell, Paula Tanabe
INTRODUCTION: Sickle cell disease (SCD) is a complex illness with many social-behavioral co-morbidities. The aim of this project was to describe unmet social-behavioral health needs for adults with SCD who presented to the emergency department for treatment of vaso-occlusive episodes (VOEs). METHODS: A descriptive study using 1:1 interviews during an ED visit for a VOE was conducted; a brief social behavioral health screening interview guide was used. A convenience sample of adults with SCD treated in the emergency department for a VOE were eligible for inclusion...
May 17, 2017: Journal of Emergency Nursing: JEN: Official Publication of the Emergency Department Nurses Association
https://www.readbyqxmd.com/read/28520780/towards-a-point-of-care-strip-test-to-diagnose-sickle-cell-anemia
#19
Meaghan Bond, Brady Hunt, Bailey Flynn, Petri Huhtinen, Russell Ware, Rebecca Richards-Kortum
A rapid test to identify patients with sickle cell disease could have important benefits in low-resource settings. Sickle cell anemia (SCA) affects about 300,000 newborns each year, the majority of whom are born in sub-Saharan Africa. Low-cost therapies are available to treat SCA, but most countries in sub-Saharan Africa lack robust neonatal screening programs needed to identify patients in need of treatment. To address this need, we developed and evaluated a competitive lateral flow assay that identifies patients with SCA (genotype HbSS) in 15 minutes using undiluted whole blood...
2017: PloS One
https://www.readbyqxmd.com/read/28518049/red-blood-cell-storage-time-and-transfusion-current-practice-concerns-and-future-perspectives
#20
REVIEW
María García-Roa, María Del Carmen Vicente-Ayuso, Alejandro M Bobes, Alexandra C Pedraza, Ataúlfo González-Fernández, María Paz Martín, Isabel Sáez, Jerard Seghatchian, Laura Gutiérrez
Red blood cells (RBCs) units are the most requested transfusion product worldwide. Indications for transfusion include symptomatic anaemia, acute sickle cell crisis, and acute blood loss of more than 30% of the blood volume, with the aim of restoring tissue oxygen delivery. However, stored RBCs from donors are not a qualitative equal product, and, in many ways, this is a matter of concern in the transfusion practice. Besides donor-to-donor variation, the storage time influences the RBC unit at the qualitative level, as RBCs age in the storage bag and are exposed to the so-called storage lesion...
May 2017: Blood Transfusion, Trasfusione del Sangue
keyword
keyword
55991
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"