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"Sickle Cell Anemia"

Farahnaz Golriz, Lane F Donnelly, Sridevi Devaraj, Raj Krishnamurthy
BACKGROUND: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism. OBJECTIVE: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations...
October 24, 2016: Pediatric Radiology
Benjamin Roche, Virginie Rougeron, Lluis Quintana-Murci, François Renaud, Jessica Lee Abbate, Franck Prugnolle
Malarial infections have long been recognized as a driver of human evolution, as demonstrated by the influence of Plasmodium falciparum on sickle-cell anemia persistence. Duffy-negativity is another blood disorder thought to have been selected because it confers nearly complete resistance against Plasmodium vivax infection. Recent evidence suggests that the benefits of being Duffy-negative cannot be expected to play a strong selective pressure on humans, whereas its costs cannot be considered as negligible...
October 17, 2016: Trends in Parasitology
Susanna A Curtis, Neeraja Danda, Zipora Etzion, Hillel W Cohen, Henny H Billett
INTRODUCTION: White Blood Cell (WBC) count, %HbF, and serum creatinine (Cr), have been identified as markers for increased mortality in sickle cell anemia (SCA) but no studies have examined the significance of longitudinal rate of change in these or other biomarkers for SCA individuals. METHODS: Clinical, demographic and laboratory data from SCA patients seen in 2002 by our hospital system were obtained. Those who were still followed in 2012 (survival cohort) were compared to those who had died in the interim (mortality cohort)...
2016: PloS One
Maa-Ohui Quarmyne, Wei Dong, Rodney Theodore, Sonia Anand, Vaughn Barry, Olufolake Adisa, Iris D Buchanan, James Bost, Robert C Brown, Clinton H Joiner, Peter A Lane
The clinical efficacy of hydroxyurea in patients with sickle cell anemia (SCA) has been well established. However, data about its clinical effectiveness in practice is limited. We evaluated the clinical effectiveness of hydroxyurea in a large pediatric population using a retrospective cohort, pre-post treatment study design to control for disease severity selection bias. The cohort included children with SCA (SS, Sβ(0) thalassemia) who received care at Children's Healthcare of Atlanta (CHOA) and who initiated hydroxyurea in 2009-2011...
October 19, 2016: American Journal of Hematology
Abel Makubi, Philip Sasi, Mariam Ngaeje, Enrico M Novelli, Bruno P Mmbando, Mark T Gladwin, Julie Makani
BACKGROUND: Hydroxyurea (HU) has been demonstrated to be efficacious in reducing complications in individuals with sickle cell anemia (SCA) but poor adherence is a barrier. Directly Observed Therapy (DOT) has been shown to improve adherence in various chronic diseases but there is limited data in adults with SCA. METHODS AND DESIGN: To examine the effect of mobile-directly observed therapy (mDOT) on adherence to HU (mDOT-HuA) in adults with SCA at Muhimbili National Hospital in Tanzania...
October 18, 2016: BMC Medical Research Methodology
Georgios Tsivgoulis, Andrei V Alexandrov
PURPOSE OF REVIEW: Low cost, avoidance of irradiation, and high temporal resolution are inherent advantages of ultrasound imaging that translate into multiple clinical uses in many domains of neurology. This article presents clinical uses of ultrasound examination in cerebrovascular, neurodegenerative, and peripheral nervous system diseases. RECENT FINDINGS: Modern treatment and prevention of ischemic stroke rely on prompt diagnosis. Ultrasonography has found a place as a noninvasive screening test and bedside technique that provides estimates of the degree of stenosis as well as hemodynamic and structural information about intracranial and extracranial vessels in real time...
October 2016: Continuum: Lifelong Learning in Neurology
N Quraishy, S Sapatnekar
The clinical importance of blood group antigens relates to their ability to evoke immune antibodies that are capable of causing hemolysis. The most important antigens for safe transfusion are ABO and D (Rh), and typing for these antigens is routinely performed for patients awaiting transfusion, prenatal patients, and blood donors. Typing for other blood group antigens, typically of the Kell, Duffy, Kidd, and MNS blood groups, is sometimes necessary, for patients who have, or are likely to develop antibodies to these antigens...
2016: Advances in Clinical Chemistry
Beverly A Schaefer, Jonathan M Flanagan, Ofelia A Alvarez, Stephen C Nelson, Banu Aygun, Kerri A Nottage, Alex George, Carla W Roberts, Connie M Piccone, Thad A Howard, Barry R Davis, Russell E Ware
Discovery and validation of genetic variants that influence disease severity in children with sickle cell anemia (SCA) could lead to early identification of high-risk patients, better screening strategies, and intervention with targeted and preventive therapy. We hypothesized that newly identified genetic risk factors for the general African American population could also impact laboratory biomarkers known to contribute to the clinical disease expression of SCA, including variants influencing the white blood cell count and the development of albuminuria and abnormal glomerular filtration rate...
2016: PloS One
Caitlin S Garwood, Paul J Kim, Vinay Matai, John S Steinberg, Karen K Evans, Carol Deane B Mitnick, Christopher E Attinger
The primary purpose of this study was to evaluate the use of bovine collagen-glycosaminoglycan matrix on atypical lower extremity ulcers. A retrospective chart review was performed on patients who underwent application of bovine collagen matrix to a lower extremity ulcer with an atypical etiology including autoimmune disease, sickle cell anemia, radiation therapy, connective tissue disease, vasculitis, or coagulopathy from January 2009 to October 2014. The following outcomes were evaluated: rate of ulcer healing and closure, number of ulcers that received a split-thickness skin graft, improvement in pain, and complications related to the ulcer...
September 2016: Wounds: a Compendium of Clinical Research and Practice
Anu Marahatta, Vandana Megaraj, Patrick T McGann, Russell E Ware, Kenneth D R Setchell
BACKGROUND: Sickle cell anemia (SCA) is a life-threatening blood disorder characterized by the presence of sickle-shaped erythrocytes. Hydroxyurea is currently the only US Food and Drug Administration-approved treatment and there is a need for a convenient method to monitor compliance and hydroxyurea concentrations, especially in pediatric SCA patients. METHODS: We describe a novel approach to the determination of hydroxyurea concentrations in dried whole blood collected on DMPK-C cards or volumetric absorptive microsampling (VAMS) devices...
September 30, 2016: Clinical Chemistry
Dana R Jorgensen, Caterina Rosano, Enrico M Novelli
Adults with homozygous sickle cell anemia have, on average, lower cognitive function than unaffected controls. The mechanisms underlying cognitive deterioration in this population are poorly understood, but cerebral small vessel disease (CSVD) is likely to be implicated. We conducted a systematic review using the Prisma Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines of articles that included both measures of cognitive function and magnetic resonance imaging (MRI) neuroimaging markers of small vessel disease...
September 30, 2016: Hemoglobin
Ömer Barış Yücel, Emre Salabaş, Bahadır Ermeç, Ateş Kadıoğlu
INTRODUCTION: Priapism, taking its name from God Priapus, is total or partial erection lasting longer than 4 hours independent of sexual stimulus and can result in erectile dysfunction. There are three subtypes of priapism. AIM: To review the three subtypes of priapism, their pathophysiology, current treatment options, and complications. METHODS: The literature including priapism guidelines, review articles, and current trial studies was reviewed and the priapism type of God Priapus was investigated according to the mythology...
September 26, 2016: Sexual Medicine Reviews
Kenneth I Ataga, Vimal K Derebail, Melissa Caughey, Laila Elsherif, Jessica H Shen, Susan K Jones, Poulami Maitra, David M Pollock, Jianwen Cai, David R Archer, Alan L Hinderliter
BACKGROUND: The pathogenesis of albuminuria in SCD remains incompletely understood. We evaluated the association of albuminuria with measures of endothelial function, and explored associations of both albuminuria and measures of endothelial function with selected biological variables (vascular endothelial growth factor [VEGF], endothelin-1 [ET-1], soluble fms-like tyrosine kinase-1 [sFLT-1], soluble vascular cell adhesion molecule-1 [soluble VCAM-1] and plasma hemoglobin). METHODS: Spot urine measurements for albumin-creatinine ratio (UACR) and 24-hour urine protein were obtained...
2016: PloS One
Roberta D'Ambrosio, Marco Maggioni, Giovanna Graziadei
No abstract text is available yet for this article.
September 8, 2016: Digestive and Liver Disease
Rodney D Averett, David G Norton, Natalie K Fan, Manu O Platt
Sickle cell disease is a single point mutation disease that is known to alter the coagulation system, leading to hypercoagulable plasma conditions. These hypercoagulable conditions can lead to complications in the vasculature, caused by fibrin clots that form undesirably. There is a need to understand the morphology and structure of fibrin clots from patients with sickle cell disease, as this could lead to further discovery of treatments and life-saving therapies. In this work, a computational imaging analysis method is presented to evaluate fibrin agglomeration in the presence of erythrocytes (RBCs) homozygous for the sickle cell mutation (SS)...
September 23, 2016: Journal of Thrombosis and Thrombolysis
Santosh L Saraf, Binal N Shah, Xu Zhang, Jin Han, Bamidele O Tayo, Taimur Abbasi, Adam Ostrower, Elizabeth Guzman, Robert E Molokie, Michel Gowhari, Johara Hassan, Shivi Jain, Richard S Cooper, Roberto F Machado, James P Lash, Victor R Gordeuk
No abstract text is available yet for this article.
September 22, 2016: Haematologica
Charles T Quinn, Eric P Smith, Shahriar Arbabi, Paramjit K Khera, Christopher J Lindsell, Omar Niss, Clinton H Joiner, Robert S Franco, Robert M Cohen
Hemolysis is a key feature of sickle cell anemia (HbSS). Direct quantitation of hemolysis could be used as an objective outcome in clinical trials of new therapeutics for HbSS and would also enable better human studies of the pathogenesis of complications of HbSS that are ostensibly hemolysis-related, such as pulmonary hypertension. However, contemporary human studies in HbSS have used only surrogate markers of hemolysis rather than direct measurements of RBC survival. We directly quantified hemolysis in HbSS by measuring survival of an age cohort of RBCs labeled with a stable isotope, administered orally as (15) N-glycine, a metabolic precursor of heme...
September 20, 2016: American Journal of Hematology
J Strouse
Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a mutation in the sixth amino acid of the β-globin gene (HBB). It is the most common serious genetic diseases in childhood, affecting approximately 1 in 2500 births and 100 000 individuals in the USA, in addition to 300 000 new cases globally each year. Central nervous system injury is the most debilitating frequent complication of SCD and includes stroke, silent cerebral infarct (SCI), and cognitive impairment. Among children with sickle cell anemia (HbSS), 11% had a stroke by age 18 years before the implementation of transcranial Doppler screening...
2016: Handbook of Clinical Neurology
Angela E Rankine-Mullings, Courtney R Little, Marvin E Reid, Deanne P Soares, Carolyn Taylor-Bryan, Jennifer M Knight-Madden, Susan E Stuber, Asha V Badaloo, Karen Aldred, Margaret E Wisdom-Phipps, Teresa Latham, Russell E Ware
BACKGROUND: Cerebral vasculopathy in sickle cell anemia (SCA) begins in childhood and features intracranial arterial stenosis with high risk of ischemic stroke. Stroke risk can be reduced by transcranial doppler (TCD) screening and chronic transfusion therapy; however, this approach is impractical in many developing countries. Accumulating evidence supports the use of hydroxyurea for the prevention and treatment of cerebrovascular disease in children with SCA. Recently we reported that hydroxyurea significantly reduced the conversion from conditional TCD velocities to abnormal velocities; whether hydroxyurea can be used for children with newly diagnosed severe cerebrovascular disease in place of starting transfusion therapy remains unknown...
September 12, 2016: JMIR Research Protocols
Taciana Furtado de Mendonça Belmont, Kleyton Palmeira do Ó, Andreia Soares da Silva, Kamila de Melo Vilar, Fernanda Silva Medeiros, Luydson Richardson Silva Vasconcelos, Ana Claudia Mendonça Dos Anjos, Betânia Lucena Domingues Hatzlhofer, Maíra Galdino da Rocha Pitta, Marcos André Cavalcanti Bezerra, Aderson da Silva Araújo, Moacyr Jesus Barreto de Melo Rego, Patrícia Moura, Maria do Socorro Mendonça Cavalcanti
INTRODUCTION: Patients with sickle cell anemia (SCA) may present chronic hemolytic anemia, vaso-occlusion and respiratory tract infection (RTI) episodes. Galectin-3 (GAL-3) is a multifunctional protein involved in inflammation, apoptosis, adhesion and resistance to reactive oxygen species. Studies point to a dual role for GAL-3 as both a circulation damage-associated molecular pattern and a cell membrane associated pattern recognition receptor. OBJECTIVE: To investigate associations between the SNPs of GAL-3 gene (LGALS3) and serum levels with RTI and vaso-occlusive crisis (VOC) in children with SCA...
2016: PloS One
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