28,446 results match your criteria Sickle Cell Anemia


Impact of erythrocytapheresis on natural anticoagulant levels in children with sickle cell disease: A pilot study.

Pediatr Blood Cancer 2018 Dec 13:e27588. Epub 2018 Dec 13.

Department of Pediatrics, The Ohio State University, Division of Hematology/Oncology, Nationwide Children's Hospital, Columbus, Ohio.

Venous thromboembolism (VTE) is being increasingly recognized in children with sickle cell disease (SCD). In a retrospective cohort study, we identified bilateral central venous catheter (CVC) placement as an independent risk factor for VTE. At our institution, the only indication for bilateral CVC placement in children with SCD is erythrocytapheresis. Read More

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December 2018

Gene therapy targets sickle-cell disease.

Authors:
Anna Nowogrodzki

Nature 2018 Dec;564(7735):S12-S13

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December 2018
2 Reads

Safety and effectiveness of antimalarial therapy in sickle cell disease: a systematic review and network meta-analysis.

BMC Infect Dis 2018 Dec 12;18(1):650. Epub 2018 Dec 12.

Centre for Tropical Clinical Pharmacology and Therapeutics, School of Medicine and Dentistry, University of Ghana, Accra, Ghana.

Background: About 80% of all reported sickle cell disease (SCD) cases in children anually are recorded in Africa. Although malaria is considered a major cause of death in SCD children, there is limited data on the safety and effectiveness of the available antimalarial drugs used for prophylaxis. Also, previous systematic reviews have not provided quantitative measures of preventive effectiveness. Read More

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December 2018
2 Reads

Is chronic use of hydroxyurea safe for patients with sickle cell anemia? An account of genotoxicity and mutagenicity.

Environ Mol Mutagen 2018 Dec 7. Epub 2018 Dec 7.

Faculty of Pharmacy, Federal University of Ceará, Fortaleza, Brazil.

Sickle cell anemia (SCA) is a hereditary hematological disease that is characterized by a point mutation in the beta globin S gene and has no specific treatment; hydroxyurea (HU) is the only therapeutic agent used in clinical practice. In the present study, we evaluated the deoxyribonucleic acid (DNA) damage index (DI) and chromosomal damage in leukocytes of adult patients with SCA with and without HU. The DI was assessed by the comet assay and chromosomal damage by the leukocyte micronucleus test of adult patients treated with HU (SCA-HU) and without the use of HU (SCA-NoHU). Read More

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December 2018

Subclinical Cardiac Dysfunction Is Associated With Extracardiac Organ Damages.

Front Med (Lausanne) 2018 20;5:323. Epub 2018 Nov 20.

Cardiology Department, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris and UMR_S970, Université Paris Descartes, Inserm, Paris, France.

Several studies conducted in America or Europe have described major cardiac remodeling and diastolic dysfunction in patients with sickle cell disease (SCD). We aimed at assessing cardiac involvement in SCD in sub-Saharan Africa where SCD is the most prevalent. In Cameroon, Mali and Senegal, SCD patients and healthy controls of the CADRE study underwent transthoracic echocardiography if aged ≥10 years. Read More

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November 2018

Impact of a digital health intervention on asthma resource utilization.

World Allergy Organ J 2018 3;11(1):28. Epub 2018 Dec 3.

Propeller Health, 47 Maiden Lane, San Francisco, CA 94108 USA.

Digital health interventions have been associated with reduced rescue inhaler use and improved controller medication adherence. This quality improvement project assessed the benefit of these interventions on asthma-related healthcare utilizations, including hospitalizations, emergency department (ED) utilization and outpatient visits. The intervention consisted of electronic medication monitors (EMMs) that tracked rescue and controller inhaler medication use, and a digital health platform that presented medication use information and asthma control status to patients and providers. Read More

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December 2018

The spatial epidemiology of sickle-cell anaemia in India.

Sci Rep 2018 Dec 6;8(1):17685. Epub 2018 Dec 6.

Evolutionary Ecology of Infectious Disease Group, Peter Medawar Building for Pathogen Research, Department of Zoology, University of Oxford, Oxford, UK.

Sickle-cell anaemia (SCA) is a neglected chronic disorder of increasing global health importance, with India estimated to have the second highest burden of the disease. In the country, SCA is particularly prevalent in scheduled populations, which comprise the most socioeconomically disadvantaged communities. We compiled a geodatabase of a substantial number of SCA surveys carried out in India over the last decade. Read More

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December 2018
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Hemodynamic provocation with acetazolamide shows impaired cerebrovascular reserve in adults with sickle cell disease.

Haematologica 2018 Dec 6. Epub 2018 Dec 6.

Hematology, Internal Medicine, Amsterdam UMC, University of Amsterdam.

Sickle cell disease is characterized by chronic hemolytic anemia and vascular inflammation, which can diminish the vasodilatory capacity of the small resistance arteries, making them less adept at regulating cerebral blood flow. Autoregulation maintains adequate oxygen delivery, but when vasodilation is maximized, the low arterial oxygen content can lead to ischemia and silent cerebral infarcts. We used magnetic resonance imaging of cerebral blood flow to quantify whole-brain cerebrovascular reserve in 36 adult patients with sickle cell disease (mean age, 31. Read More

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December 2018

Current, Emerging, and Anticipated Therapies for Sickle Cell Disease.

Mayo Clin Proc 2018 Dec;93(12):1703-1706

Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN.

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December 2018

Men's position in family care on situations of chronic illness.

Rev Esc Enferm USP 2018 Dec 10;52:e03398. Epub 2018 Dec 10.

Universidade Federal de Mato Grosso, Programa de Pós-Graduação em Saúde Coletiva, Cuiabá, MT, Brasil.

Objective: To understand the care by men in situations of chronic illness of one or more of his children, based on the dimensions of care.

Method: It was based on a comprehensive approach and on the (re)view of the database of the matrix research to which the study is linked, with emphasis on three experiences of illness, in which the men effectively participated in the family care: two children with sickle cell anemia; son with adrenoleukodystrophy and son with concomitant diseases (cancer and kidney disease).

Results: The analysis diagram of each family demonstrated different ways of caring, explaining the relationship between the dimensions of care by men: social; affective/relational and physical/circulation, as well as the reverberations between these dimensions in the care. Read More

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December 2018

SOCIOECONOMIC AND NUTRITIONAL CHARACTERISTICS OF CHILDREN AND ADOLESCENTS WITH SICKLE CELL ANEMIA: A SYSTEMATIC REVIEW.

Rev Paul Pediatr 2018 Oct-Dec;36(4):491-499

Departamento de Pediatria, Universidade Federal de São Paulo, São Paulo, SP, Brasil.

Objective: To describe the socioeconomic and nutritional characteristics of children and adolescents with sickle cell anemia.

Data Sources: The present study is a systematic literature review based on published scientific articles. The searches were carried out using the electronic database of the National Library of Medicine, National Institutes of Health- PubMed. Read More

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To test or not: occurrence of sickle cell trait and assessment of the awareness toward its screening among patients attending Magale Health Center IV, Namisindwa District, Eastern Uganda.

J Blood Med 2018 21;9:219-225. Epub 2018 Nov 21.

Institute of Allied Health Sciences, Clarke International University, Kampala, Uganda,

Purpose: To evaluate the occurrence of sickle cell trait (SCT), assess patient awareness and evaluate the performance of a sickle cell hemoglobin-S (dithionate-qualitative solubility) point-of-care test among patients seeking care at Magale Health Center IV, Namisindwa District, Eastern Uganda.

Materials And Methods: We conducted a cross sectional study, in which we consecutively enrolled participants aged ≥18 years at Magale Health Center IV. Four milliliters of EDTA blood were collected by venipuncture and screened for SCT using solubility testing, and confirmed with hemoglobin (Hb) electrophoresis at Central Public Health Laboratory (CPHL), Kampala, Uganda. Read More

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November 2018
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Sickle cell disease with left ventricular non-compaction: A rare association.

J Cardiol Cases 2011 Apr 12;3(2):e75-e77. Epub 2011 Jan 12.

Department of Cardiology, Royal Hospital, Muscat, Oman.

Cardiac abnormalities described in sickle cell disease are pulmonary hypertension, dilated left or right atrium/ventricle, valvular abnormalities, hyperdynamic left ventricle with hypertrophy, and left or right ventriclular dysfunction. However, features consistent with left ventricular non-compaction have not been described previously in patients with sickle cell disease. We describe the case of a 21-year-old male with sickle cell disease and left ventricular non-compaction, which is a rare association and discuss the possible mechanisms for such an association. Read More

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Classic pseudoxanthoma elasticum in a girl with sickle cell disease.

Pediatr Dermatol 2018 Dec 9. Epub 2018 Dec 9.

Department of Dermatology and Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.

A pseudoxanthoma elasticum (PXE)-like phenotype develops in a subset of patients with inherited hemoglobinopathies. Although PXE tissue changes are thought to develop in the absence of ABCC6 mutations in patients with beta-thalassemia, ABCC6 mutations have not been well evaluated among sickle cell disease patients with PXE-like disease. To our knowledge, we describe the first patient with sickle cell disease, PXE skin findings, and two confirmed pathogenic ABCC6 mutations. Read More

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December 2018
1 Read

Lung function impairment in pediatric patients with sickle cell anemia from Nigeria is associated with low steady state hemoglobin.

Am J Hematol 2018 Dec 8. Epub 2018 Dec 8.

Department of Pediatric Haematology, Evelina Children's Hospital, Guy's and St. Thomas NHS Trust, London, United Kingdom.

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December 2018

The sickle cell trait affects contact dynamics and endothelial cell activation in -infected erythrocytes.

Commun Biol 2018 30;1:211. Epub 2018 Nov 30.

1Department of Infectious Diseases, Parasitology, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 324, 69120 Heidelberg, Germany.

Sickle cell trait, a common hereditary blood disorder, protects carriers from severe disease in infections with the human malaria parasite . Protection is associated with a reduced capacity of parasitized erythrocytes to cytoadhere to the microvascular endothelium and cause vaso-occlusive events. However, the underpinning cellular and biomechanical processes are only partly understood and the impact on endothelial cell activation is unclear. Read More

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November 2018
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Pre-clinical Development of a Lentiviral Vector Expressing the Anti-sickling βAS3 Globin for Gene Therapy for Sickle Cell Disease.

Mol Ther Methods Clin Dev 2018 Dec 1;11:167-179. Epub 2018 Nov 1.

Department of Life Sciences, University of Modena and Reggio Emilia, Modena, Italy.

Sickle cell disease (SCD) is caused by a mutation (E6V) in the hemoglobin (Hb) β-chain that induces polymerization of Hb tetramers, red blood cell deformation, ischemia, anemia, and multiple organ damage. Gene therapy is a potential alternative to human leukocyte antigen (HLA)-matched allogeneic hematopoietic stem cell transplantation, available to a minority of patients. We developed a lentiviral vector expressing a β-globin carrying three anti-sickling mutations (T87Q, G16D, and E22A) inhibiting axial and lateral contacts in the HbS polymer, under the control of the β-globin promoter and a reduced version of the β-globin locus-control region. Read More

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December 2018

Microvasculature-on-a-chip for the long-term study of endothelial barrier dysfunction and microvascular obstruction in disease.

Nat Biomed Eng 2018 23;2:453-463. Epub 2018 Apr 23.

Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, GA, USA.

Alterations in the mechanical properties of erythrocytes occurring in inflammatory and hematologic disorders such as sickle cell disease (SCD) and malaria often lead to increased endothelial permeability, haemolysis, and microvascular obstruction. However, the associations among these pathological phenomena remain unknown. Here, we report a perfusable, endothelialized microvasculature-on-a-chip featuring an interpenetrating-polymer-network hydrogel that recapitulates the stiffness of blood-vessel intima, basement membrane self-deposition and self-healing endothelial barrier function for longer than 1 month. Read More

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April 2018
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Multifactorial Origin of Exertional Rhabdomyolysis, Recurrent Hematuria, and Episodic Pain in a Service Member with Sickle Cell Trait.

Case Rep Genet 2018 7;2018:6898546. Epub 2018 Nov 7.

Consortium for Health and Military Performance, Department of Military and Emergency Medicine, Hébert School of Medicine, Uniformed Services University, 4301 Jones Bridge Rd., Bethesda, MD 20184, USA.

Individuals with Sickle Cell Trait (SCT), generally considered a benign carrier state of hemoglobin S (HbAS), are thought to be at risk for exertional rhabdomyolysis and hematuria, conditions that can also be caused by various other acquired and inherited factors. We report an SCT positive service member with an exertional rhabdomyolysis event, recurrent hematuria with transient proteinuria, and episodic burning pain in the lower extremities. Clinical and genetic studies revealed the multifactorial nature of his complex phenotype. Read More

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November 2018

Decreased median survival of adults with sickle cell disease after adjusting for left truncation bias: a pooled analysis.

Blood 2018 Dec 10. Epub 2018 Dec 10.

Center for Sickle Cell Disease, University of Tennessee Health Science Center, Memphis, TN, United States.

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December 2018
10.452 Impact Factor

Differential role of Kruppel like factor 1 (KLF1) gene in red blood cell disorders.

Genomics 2018 Dec 4. Epub 2018 Dec 4.

National Institute of Immunohematology (ICMR), 13th Floor, New Multi-storeyed Building, K.E.M. Hospital Campus, Parel, Mumbai 400012, India. Electronic address:

The master erythroid regulator KLF1,plays a pivotal role during erythroid lineage development by regulating the expression of many erythroid genes. Variations in the KLF1 gene are found to be associated with varied erythroid phenotypes. With the aim of determining the role of KLF1 gene variations in HbF induction and their genotype phenotype relationship, in this study, we screened 370 individuals with different hemoglobinopathy condition. Read More

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December 2018

Vaccines for preventing invasive salmonella infections in people with sickle cell disease.

Cochrane Database Syst Rev 2018 Dec 5;12:CD006975. Epub 2018 Dec 5.

Department of Paediatrics, University of Calabar Teaching Hospital, PMB 1115, Calabar, Cross River State, Nigeria, 540261.

Background: Salmonella infections are a common bacterial cause of invasive disease in people with sickle cell disease especially children, and are associated with high morbidity and mortality rates. Although available in some centres, people with sickle cell anaemia are not routinely immunized with salmonella vaccines. This is an update of a previously published Cochrane Review. Read More

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December 2018
7 Reads

Objectively measured physical activity levels and sedentary time in children and adolescents with sickle cell anemia.

PLoS One 2018 6;13(12):e0208916. Epub 2018 Dec 6.

Federal University of Sergipe, Aracaju, Brazil.

The aim of this study was to identify the levels of physical activity and sedentary behaviour of children and adolescents with sickle cell disease (SCA) compared to healthy individuals. A cross-sectional study with a quantitative approach was performed at a reference center for the treatment of patients with hemoglobinopathies in northeastern Brazil. Patients were recruited between October 2015 and January 2017. Read More

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December 2018
2 Reads

Childhood Hearing Loss in Patients With Sickle Cell Disease in the United States.

J Pediatr Hematol Oncol 2018 Dec 4. Epub 2018 Dec 4.

Departments of Otolaryngology-Head and Neck Surgery.

This study sought to examine if modern medical evaluations including newborn screening and early diagnosis along with better methods of disease control have improved rates of hearing loss in children with sickle cell disease (SCD). Audiometric and medical data for patients with SCD was obtained from the AudGen Database and analyzed for the presence of hearing loss, type of hearing loss, severity of hearing loss, and correlation with comorbid conditions. Children with sickle cell trait (SCT) were used as a comparison group. Read More

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December 2018
1 Read

Thrombospondin-1 and Vitamin D in Children With Sickle Cell Anemia.

J Pediatr Hematol Oncol 2018 Dec 4. Epub 2018 Dec 4.

Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait.

Background: Thrombospondin-1 (TSP-1) and 25-hydroxyvitamin D (25-OHD) play significant roles in the pathogenesis of sickle cell anemia (SCA). TSP-1 enhances cellular adhesion/inflammation, hence contributing to vaso-occlusive crisis (VOC); vitamin D, in contrast, retards inflammation and may lower rate of pain episodes. We determined serum levels of TSP-1 and 25-OHD in Nigerian children with SCA and their matched hemoglobin AA controls; and assess the relationship between the 2 biomarkers. Read More

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December 2018
3 Reads

New variants in beta globin gene among the Palestinian refugees with sickle cell disease in Lebanon.

Saudi Med J 2018 Dec;39(12):1253-1258

Department of Biological Sciences, Beirut Arab University, Beirut, Lebanon. E-mail.

Objectives: To examine the association between beta-globin sequence variations and phenotypes of sickle-cell disease (SCD) complications among Palestinian refugees in Lebanon correlating them with chromatographic readings and co-inheritance with β-thalassemia traits.   Methods: This cross-sectional study included 47 Palestinian refugees aged 4 to 54 living in different regions in Lebanon during the year 2015. Participant filled a well-designed questionnaire. Read More

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December 2018
3 Reads

One single bone marrow harvesting from donors under 3 years of age: assessing safety and efficacy of the procedure.

Bone Marrow Transplant 2018 Dec 5. Epub 2018 Dec 5.

International Centre for Transplantation in Thalassaemia and Sickle Cell Anaemia, Mediterranean Institute of Haematology, Rome, Italy.

To candidate children as bone marrow donors raises two main concerns: donor safety and adequate marrow cell dose. Data in the field are limited and guidelines for child donor care management are lacking. In this context, we herein report the experience collected in our center by comparing very-young donors (defined as age ≤ 3 years) with young donors (defined as age > 3 years) who donated bone marrow (BM) for patients affected by beta-globin disorders. Read More

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December 2018
2 Reads

Robust adaptive immune response against infection marked by low parasitemia in a murine model of sickle cell disease.

Blood Adv 2018 Dec;2(23):3462-3478

Laboratory of Complement Biology and.

The intraerythrocytic parasite is the number 1 cause of transfusion-transmitted infection and can induce serious, often life-threatening complications in immunocompromised individuals including transfusion-dependent patients with sickle cell disease (SCD). Despite the existence of strong long-lasting immunological protection against a second infection in mouse models, little is known about the cell types or the kinetics of protective adaptive immunity mounted following infection, especially in infection-prone SCD that are thought to have an impaired immune system. Here, we show, using a mouse infection model, that infected wild-type (WT) mice mount a very strong adaptive immune response, characterized by (1) coordinated induction of a robust germinal center (GC) reaction; (2) development of follicular helper T (T) cells that comprise ∼30% of splenic CD4 T cells at peak expansion by 10 days postinfection; and (3) high levels of effector T-cell cytokines, including interleukin 21 and interferon γ, with an increase in the secretion of antigen (Ag)-specific antibodies (Abs). Read More

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December 2018
1 Read

Circularly Polarized Broadband Printed Antenna for Wireless Applications.

Sensors (Basel) 2018 Dec 4;18(12). Epub 2018 Dec 4.

Centre of Advanced Electronic and Communication Engineering, Universiti Kebangsaan Malaysia, Bangi 43600, Malaysia.

A simple, compact sickle-shaped printed antenna with a slotted ground plane is designed and developed for broadband circularly polarized (CP) radiation. The sickle-shaped radiator with a tapered feed line and circular slotted square ground plane are utilized to realize the wideband CP radiation feature. With optimized dimensions of 0. Read More

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December 2018
1 Read

Neuronal transient receptor potential (TRP) channels and noxious sensory detection in sickle cell disease.

Neurosci Lett 2018 Nov 30;694:184-191. Epub 2018 Nov 30.

Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, United States.

Pain is the leading cause for hospitalization in patients with sickle cell disease (SCD). While the characteristics of SCD pain can vary widely between patients and between phases of the disease (e.g. Read More

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November 2018
1 Read

Feasibility of implementing mobile technology-delivered mental health treatment in routine adult sickle cell disease care.

Transl Behav Med 2018 Dec 3. Epub 2018 Dec 3.

UCSF Center for Vulnerable Populations, Zuckerberg San Francisco General Hospital, University of California, San Francisco, CA, USA.

Sickle cell disease (SCD) is a severe hemoglobinopathy characterized by acute and chronic pain. Sufferers of the disease, most of whom are underrepresented minorities, are at increased risk for mental health disorders. The purpose of this study is to test the acceptability and implementation of a computerized cognitive behavioral therapy (cCBT) intervention, Beating the Blues, to improve depression, anxiety, and pain in patients with SCD. Read More

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December 2018
1 Read

Rapid Detection of Fetal Mendelian Disorders: Thalassemia and Sickle Cell Syndromes.

Methods Mol Biol 2019 ;1885:207-219

Department of Medical Genetics, St. Sophia's Children's Hospital, National and Kapodistrian University of Athens, Athens, Greece.

The inherited disorders of hemoglobin synthesis constitute the most common monogenic diseases worldwide. The clinical severity of β-thalassemia major and the sickle cell syndromes targets them as priority genetic diseases for prevention programs, which incorporates population screening to identify heterozygotes, with the option of prenatal diagnosis for carrier couples. Rapid genotype characterization is fundamental in the diagnostic laboratory, especially when offering prenatal diagnosis. Read More

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January 2019
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White Paper: Pathways to Progress in Newborn Screening for Sickle Cell Disease in Sub-Saharan Africa.

J Trop Dis Public Health 2018 10;6(2):260. Epub 2018 Jul 10.

Department of Public Health Sciences, Loyola University Chicago Stritch School of Medicine, Maywood, IL, USA.

Sickle Cell Disease (SCD) is among the most common single-gene diseases in the world but evidence-based comprehensive health care has not been implemented where the highest prevalence of SCD occurs, in sub-Saharan Africa (SSA). It represents an urgent health burden, both in terms of mortality and morbidity with an estimated mortality of 8-16% in children under 5 years in SSA. Addressing the high mortality of SCD in SSA and for effective management of SCD, newborn screening (NBS) should be incorporated with prevention of infections (including pneumococcal septicaemia and malaria), parental education and support at all levels of healthcare provision to enable timely recognition. Read More

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July 2018
1 Read

New insights into the pathophysiology and development of novel therapies for sickle cell disease.

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):493-506

Children's Hospital, Montefiore Medical Center, Bronx, NY; and.

Although the seminal event in sickle cell disease is the polymerization of abnormal hemoglobin, the downstream pathophysiology of vasoocclusion results from heterotypic interactions between the altered, adhesive sickle cell red blood cells, neutrophils, endothelium, and platelets. Ischemia reperfusion injury, hemolysis, and oxidant damage all contribute to heightened inflammation and activation of the hemostatic system. These various pathways are the focus of emerging treatments with potential to ameliorate disease manifestations. Read More

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November 2018
1 Read

Measuring success: utility of biomarkers in sickle cell disease clinical trials and care.

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):482-492

Division of Hematology/Oncology and UPMC Heart, Lung and Blood Vascular Medicine Institute, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA.

Progress in the care of sickle cell disease (SCD) has been hampered by the extreme complexity of the SCD phenotype despite its monogenic inheritance. While epidemiological studies have identified clinical biomarkers of disease severity, with a few exceptions, these have not been routinely incorporated in clinical care algorithms. Furthermore, existing biomarkers have been poorly apt at providing objective parameters to diagnose sickle cell crisis, the hallmark, acute complication of SCD. Read More

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November 2018
4 Reads

The current state of sickle cell trait: implications for reproductive and genetic counseling.

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):474-481

Division of Hematology, Department of Medicine, Johns Hopkins University, Baltimore, MD.

Sickle cell trait (SCT) is unique among the carrier states that are identified during newborn screening. Unlike other heterozygous states for rare recessive diseases, SCT is exceedingly prevalent throughout regions of the world, making sickle cell disease one of the most common monogenetic diseases worldwide. Because of this high frequency, reproductive counseling is of paramount importance. Read More

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November 2018
1 Read

Balancing exercise risk and benefits: lessons learned from sickle cell trait and sickle cell anemia.

Authors:
Robert I Liem

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):418-425

Hematology, Oncology and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL; and Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL.

Development of exercise guidelines for individuals with sickle cell trait (SCT) and sickle cell anemia (SCA) is hampered by the need to weigh the benefits against risks of exercise in these populations. In SCT, concern for exercise collapse associated with sickle cell trait has resulted in controversial screening of student athletes for SCT. In SCA, there exists unsubstantiated concerns that high-intensity exercise may result in pain and other complications. Read More

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November 2018
1 Read

Neuroimaging in patients with sickle cell anemia: capacity building in Africa.

Blood Adv 2018 Nov;2(Suppl 1):26-29

Developmental Neurosciences, Section and Biomedical Research Centre, University College London, Great Ormond Street Institute of Child Health, London, United Kingdom.

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November 2018
1 Read

Establishing a national sickle cell disease program in the Republic of Congo.

Blood Adv 2018 Nov;2(Suppl 1):17-18

Center for Sickle Cell Disease, Howard University, Washington, DC.

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November 2018
1 Read

Hydroxyurea for Children with Sickle Cell Anemia in Sub-Saharan Africa.

N Engl J Med 2018 Dec 1. Epub 2018 Dec 1.

From Centre Hospitalier Monkole, Kinshasa, Democratic Republic of Congo (L.T.); the Department of Medicine, University Health Network and Mt. Sinai Hospital, and the University of Toronto, Toronto (G.T.); the Kenya Medical Research Institute (KEMRI)-Wellcome Trust Research Program, Kilifi, Kenya (T.N.W.); the Department of Medicine, Imperial College London, London (T.N.W.); Hospital Pediátrico David Bernardino, Luanda, Angola (B.S.); Mbale Clinical Research Institute and Mbale Regional Referral and Teaching Hospital-Busitema University, Mbale, Uganda (P.O.-O.); the Division of Hematology, Department of Pediatrics, Cincinnati Children's Hospital (A.L., S.E.S., T.S.L., P.T.M., R.E.W.), University of Cincinnati College of Medicine (A.L., P.T.M., R.E.W.), and the Global Health Center, Cincinnati Children's Hospital Medical Center (S.E.S., P.T.M., R.E.W.), Cincinnati; and Cohen Children's Medical Center, New Hyde Park, and the Zucker School of Medicine at Hofstra/Northwell, Hempstead - both in New York (B.A.).

Background: Hydroxyurea is an effective treatment for sickle cell anemia, but few studies have been conducted in sub-Saharan Africa, where the burden is greatest. Coexisting conditions such as malnutrition and malaria may affect the feasibility, safety, and benefits of hydroxyurea in low-resource settings.

Methods: We enrolled children 1 to 10 years of age with sickle cell anemia in four sub-Saharan countries. Read More

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December 2018
2 Reads

Efficacy of the National Thalassaemia and Sickle Cell Disease Prevention Programme in Northern Greece: 15-Year Experience, Practice and Policy Gaps for Natives and Migrants.

Hemoglobin 2018 Dec 3:1-6. Epub 2018 Dec 3.

c Thalassaemia National Centre of Greece , Laiko General Hospital of Athens , Athens , Greece.

Hemoglobinopathies constitute the most frequent monogenic disorders worldwide and in Greece. In Greece, carrier frequency is estimated at about 8.0%, resulting in a heavy disease burden in the past. Read More

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December 2018
1 Read
0.955 Impact Factor

Sickle Cell Nephropathy in the Pediatric Population.

Blood Purif 2018 Dec 5:1-9. Epub 2018 Dec 5.

Division of Nephrology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts,

Background: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Read More

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December 2018
4 Reads

Sickle Cell and α+-Thalassemia Traits Influence the Association between Ferritin and Hepcidin in Rural Kenyan Children Aged 14-26 Months.

J Nutr 2018 Dec;148(12):1903-1910

Department of Nutrition, University of California, Davis, Davis, CA.

Background: The relation between subclinical hemoglobinopathies and concentrations of the iron-regulatory hormone hepcidin is not well characterized.

Objective: We investigated the relation of hepcidin concentration with hemoglobinopathies among young children in Kenya.

Methods: We quantified serum hepcidin and ferritin in 435 Kenyan children aged 14-20 mo in a subsample of the Water, Sanitation, and Handwashing (WASH) Benefits Trial. Read More

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December 2018
2 Reads