23,364 results match your criteria Sickle Cell Anemia


Disruption of the MBD2-NuRD complex but not MBD3-NuRD induces high level HbF expression in human erythroid cells.

Haematologica 2019 Apr 19. Epub 2019 Apr 19.

Massey Cancer Center, Virginia Commonwealth University, Richmond, USA;

As high fetal hemoglobin levels ameliorate the underlying pathophysiologic defects in sickle cell anemia and β-thalassemia, understanding the mechanisms that enforce silencing of fetal hemoglobin postnatally offers the promise of effective molecular therapy. Depletion of the Nucleosome Remodeling and Deacetylase complex member MBD2 causes a 10-20 fold increase in γ-globin gene expression in adult β-globin locus yeast artificial chromosome transgenic mice. To determine the effect of MBD2 depletion in human erythroid cells, genome editing technology was utilized to knockout MBD2 in Human Umbilical cord Derived Erythroid Progenitor-2 cells resulting in γ/γ+β mRNA levels of ~50% and ~40% fetal hemoglobin by high performance liquid chromatography. Read More

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http://dx.doi.org/10.3324/haematol.2018.210963DOI Listing

[Decreased Heart Rate Variability in Sickle Cell Anemia as Effect of Pulmonary Arterial Hypertension].

Kardiologiia 2019 Apr 17;59(4):39-44. Epub 2019 Apr 17.

Central State Medical Academy, President Management Department RF.

Decreased heart rate variability (HRV) is associated with increased mortality risk in various diseases. The objective of this investigation:to study HRV in patients with sickle cell anemia (SCA) and to assess the effect of pulmonary arterial hypertension (PAH) on HRV in these patients. Materials and methods. Read More

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http://dx.doi.org/10.18087/cardio.2019.4.10237DOI Listing

Upper limb peripheral neuropathy in sickle cell anemia: MR neurography appearances.

Indian J Radiol Imaging 2019 Jan-Mar;29(1):67-71

Department of Radiology, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390.

Sickle cell anemia is an inherited disorder with many systemic complications. Peripheral neuropathy related to this disorder has been sparsely reported. We report an interesting case of upper limb peripheral neuropathy from sickle cell disease with emphasis on MR neurography appearances and electrophysiology correlation. Read More

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http://dx.doi.org/10.4103/ijri.IJRI_364_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467032PMC

Angiopoietin-2 as a Marker of Retinopathy in Children and Adolescents With Sickle Cell Disease: Relation to Subclinical Atherosclerosis.

J Pediatr Hematol Oncol 2019 Apr 15. Epub 2019 Apr 15.

Departments of Pediatric.

Objectives: Angiopoietin-2 (Ang-2) is a multifaceted cytokine that functions in both angiogenesis and inflammation. A proangiogenic state has been found in adults with sickle cell disease (SCD), mainly because of elevated Ang-2 levels. We determined Ang-2 level in 40 children and adolescents with SCD compared with 40 healthy controls and assessed its relation to retinopathy as well as carotid intimamedia thickness (CIMT). Read More

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http://dx.doi.org/10.1097/MPH.0000000000001486DOI Listing
April 2019
2 Reads

Isolated intermittent neurogenic priapism: an unusual presentation in degenerative lumbar spinal stenosis.

BMJ Case Rep 2019 Apr 14;12(4). Epub 2019 Apr 14.

Division of Neurology, Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

Priapism is a relatively uncommon clinical presentation. The well-recognised causes are sickle cell anaemia and the use of medications, such as vasoactive erectile agents. Infrequently, it could be the result of lumbar spinal stenosis. Read More

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http://dx.doi.org/10.1136/bcr-2018-228107DOI Listing
April 2019
5 Reads

The impact of sickle cell anemia on the quality of life of sicklers at school age.

J Family Med Prim Care 2019 Feb;8(2):468-471

Department of Pediatrics, College of Medicine, University of Bahri, Khatoum, Sudan.

Background: Sickle cell anemia (SCA) is prevalent in Sudan in general, and in particular in the western part of the country among Misseria tribe. School absence, depressive symptoms, embarrassment, social disturbances, and emotional disturbance present negative impact for sicklers. Therefore, an urgent call for improving the quality of life of sicklers is needed. Read More

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http://www.jfmpc.com/text.asp?2019/8/2/468/253056
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http://dx.doi.org/10.4103/jfmpc.jfmpc_444_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6436282PMC
February 2019
4 Reads

Gamna-Gandy bodies of the spleen in sickle cell disease.

Autops Case Rep 2019 Apr-Jun;9(2):e2018076. Epub 2019 Mar 22.

Universidade de São Paulo (USP), Faculty of Medicine, Department of Pathology. São Paulo, SP, Brazil.

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http://www.autopsyandcasereports.org/article/doi/10.4322/acr
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http://dx.doi.org/10.4322/acr.2018.076DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6433140PMC
March 2019
3 Reads

Causes of hospitalization in sickle cell diseased children in western region of Saudi Arabia. A single center study.

Saudi Med J 2019 Apr;40(4):401-404

Pediatric Department, Taibah University, Al-Madinah Al-Munawarah, Kingdom of Saudi Arabia. E-mail.

Objectives: To highlight the causes of hospitalization among sickle cell diseased (SCD) children in Al-Madinah Al-Munawarah, Saudi Arabia.

Methods: A retrospective study conducted at the Maternity and Children's Hospital, Al-Madinah Al-Munawarah, Saudi Arabia. A data of 739 SCD children admitted to the hematology/oncology unit between October 2010 and September 2015 were collected. Read More

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http://dx.doi.org/10.15537/smj.2019.4.24049DOI Listing
April 2019
1 Read

Microcytosis in children and adolescents with the sickle cell trait in Basra, Iraq.

Blood Res 2019 Mar 21;54(1):38-44. Epub 2019 Mar 21.

Center for Hereditary Blood Diseases, Basrah Heath Directorate, Basrah, Iraq.

Background: Microcytic anemia, the most common form of anemia in children and adolescents, is a heterogeneous group of diseases that is acquired or inherited. We assessed the frequency and causes of microcytosis in children and adolescents with the sickle cell trait (SCT).

Methods: This descriptive study included 95 subjects (49 males and 46 females) with SCT who attended Basra Center for Hereditary Blood Diseases for evaluation. Read More

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http://dx.doi.org/10.5045/br.2019.54.1.38DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439302PMC
March 2019
1 Read

Biochemical effects and safety of Gum arabic () supplementation in patients with sickle cell anemia.

Blood Res 2019 Mar 21;54(1):31-37. Epub 2019 Mar 21.

Department of Physiology, Faculty of Medicine, University of Khartoum, Khartoum, Sudan.

Background: Sickle cell anemia (SCA) is a hereditary chronic hemolytic anemia with several clinical consequences. Intravascular sickling of red blood cells leads to multi-organ dysfunction. Moreover, several biochemical abnormalities have been associated with SCA. Read More

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http://dx.doi.org/10.5045/br.2019.54.1.31DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439290PMC
March 2019
2 Reads

Stroke Recurrence in Nigerian Children With Sickle Cell Disease: Evidence for a Secondary Stroke Prevention Trial.

Pediatr Neurol 2019 Jan 17. Epub 2019 Jan 17.

Department of Hematology and Blood Transfusion, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria. Electronic address:

Background: To improve the quality of care for children with sickle cell anemia in Kano, Nigeria, we initiated a standard care protocol in 2014 to manage children with strokes at Aminu Kano Teaching Hospital.

Methods: The standard care protocol requires that children with acute strokes be treated with hydroxyurea at a fixed dose of 20 mg/kg/day within two months of the stroke.

Results: Twenty-nine children with sickle cell anemia and initial stroke were identified based on clinical World Health Organization criteria from 2014 to 2017. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183089
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.008DOI Listing
January 2019
4 Reads

Effect of Sickle Cell Anemia Therapies on the Natural History of Growth and Puberty Patterns.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, University of Alabama at Birmingham, Alabama.

As pediatric patients with sickle cell anemia (SCA) have impaired growth and puberty patterns, we studied the effect of disease-modifying therapies on growth and puberty patterns for patients with SCA receiving hydroxyurea (HU), transfusions, or no therapy. We performed a retrospective study of children with SCA in whom anthropometric measurements and therapy type were recorded. Penalized smoothing splines were fitted to estimate growth curves and growth velocity, and linear mixed models were used to examine differences across treatment groups. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001477DOI Listing
April 2019
3 Reads

Sickle Cell Disease and Stroke.

Pediatr Neurol 2019 Feb 27. Epub 2019 Feb 27.

Developmental Neurosciences Section and Biomedical Research Unit, Clinical and Experimental Sciences, University of Southampton, UCL Great Ormond Street Institute of Child Health, London, UK. Electronic address:

Cerebral infarction is a common complication of sickle cell disease and may manifest as overt stroke or cognitive impairment associated with "silent" cerebral infarction on magnetic resonance imaging. Vasculopathy may be diagnosed on transcranial Doppler or magnetic resonance angiography. The risk factors in sickle cell disease for cognitive impairment, overt ischemic stroke, silent cerebral infarction, overt hemorrhagic stroke, and vasculopathy defined by transcranial Doppler or magnetic resonance angiography overlap, with severe acute and chronic anemia, acute chest crisis, reticulocytosis, and low oxygen saturation reported with the majority. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183130
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.018DOI Listing
February 2019
2 Reads

Sickle red blood cells are more susceptible to in vitro haemolysis when exposed to normal saline versus Plasma-Lyte A.

Vox Sang 2019 Apr 2. Epub 2019 Apr 2.

Department of Pathology and Laboratory Medicine (Transfusion Medicine), University of Rochester Medical Center, Rochester, NY, USA.

Background: Normal saline has been the fluid of choice for resuscitation, rehydration and fluid replacement during plasma or red cell exchange/cytapheresis. There are increased concerns about its clinical effects and data showing it causes more haemolysis in vitro than buffered solutions such as Plasma-Lyte A.

Methods: We investigated whether normal saline or Plasma-Lyte A was associated with greater haemolysis during hours of in vitro incubation with both normal red cells and samples from patients with sickle cell anaemia. Read More

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http://dx.doi.org/10.1111/vox.12777DOI Listing
April 2019
2 Reads

High prevalence of altered sudomotor function in homozygous sickle cell patients: influence of age and anaemia.

Br J Haematol 2019 Apr 1. Epub 2019 Apr 1.

Service d'Explorations Fonctionnelles Multidisciplinaires, Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Tenon, Paris, France.

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http://dx.doi.org/10.1111/bjh.15892DOI Listing
April 2019
3 Reads

Sickle-cell anaemia-keeping alive Wethers' legacy.

Lancet Haematol 2019 04;6(4):e167

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http://dx.doi.org/10.1016/S2352-3026(19)30055-9DOI Listing
April 2019
3 Reads

Altered parasite life-cycle processes characterize Babesia divergens infection in human sickle cell anemia.

Haematologica 2019 Mar 28. Epub 2019 Mar 28.

Department of Blood-Borne Parasites, New York Blood Center, New York, USA;

Babesia divergens is an intra-erythrocytic parasite that causes malaria-like symptoms in infected people. As the erythrocyte provides the parasite with the infra-structure to grow and multiply, any perturbation to the cell should impact parasite viability. Support for this comes from the multitude of studies that have shown that the sickle trait has in fact been selected for because of the protection in provides against a related Apicomplexan parasite, Plasmodium that causes malaria. Read More

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http://dx.doi.org/10.3324/haematol.2018.214304DOI Listing
March 2019
1 Read

Autologous adipose-derived stem cell for painful leg ulcers in patients with sickle cell disease. A preliminary study.

Br J Haematol 2019 Mar 28. Epub 2019 Mar 28.

Division of Plastic Surgery, Department of Surgery and Anatomy, Hemocentro and Ribeirão Preto Medical School of University of São Paulo, Ribeirão Preto, São Paulo, Brazil.

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http://dx.doi.org/10.1111/bjh.15891DOI Listing
March 2019
1 Read

Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.

Am J Hematol 2019 Mar 27. Epub 2019 Mar 27.

Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.

Vaso-occlusive pain events (VOE) are the leading cause of emergency department (ED) visits in sickle cell anemia (SCA). This study assessed the variability in use of intravenous fluids (IVFs) and the association of normal saline bolus (NSB) on pain and other clinical outcomes in children with SCA presenting to pediatric emergency departments (PED) with VOE. Four-hundred charts of children age 3-21 years with SCA/VOE receiving parenteral opioids at 20 high-volume PEDs were evaluated in a retrospective study. Read More

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http://dx.doi.org/10.1002/ajh.25471DOI Listing
March 2019
3.798 Impact Factor

The effect of the antisickling compound GBT1118 on the permeability of red blood cells from patients with sickle cell anemia.

Physiol Rep 2019 Mar;7(6):e14027

Department of Veterinary Medicine, University of Cambridge, Cambridge, United Kingdom.

Sickle cell anemia (SCA) is one of the commonest severe inherited disorders. Nevertheless, effective treatments remain inadequate and novel ones are avidly sought. A promising advance has been the design of novel compounds which react with hemoglobin S (HbS) to increase oxygen (O ) affinity and reduce sickling. Read More

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http://dx.doi.org/10.14814/phy2.14027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6436144PMC
March 2019
1 Read

Assessment of iron status and interplay between lipid peroxidation and antioxidant capacity in common hemoglobin variants in Osun State, southwestern Nigeria.

Kaohsiung J Med Sci 2019 Mar 26. Epub 2019 Mar 26.

Department of Medical Laboratory, Reddington Multispecialist Hospital, Victoria Island, Lagos, Nigeria.

Hemoglobin (Hb) and iron are prooxidants in nature and sources of free radicals in the biological system of all Hb phenotypes. Recent evidence linked abnormal hemoglobin S and C (HbSC) in sickle cell disease (SCD) to various complications in multiple oxidative processes. However, similar studies in relation to abnormal Hb traits are sparse. Read More

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http://dx.doi.org/10.1002/kjm2.12062DOI Listing
March 2019
1 Read

The Extended Use of Eculizumab in Pregnancy and Complement Activation⁻Associated Diseases Affecting Maternal, Fetal and Neonatal Kidneys-The Future Is Now?

J Clin Med 2019 Mar 24;8(3). Epub 2019 Mar 24.

Department of Obstetrics and Gynecology, Helsinki University and Helsinki University Hospital, Haartmaninkatu 2, 00290 Helsinki, Finland.

Excessive complement activation is involved in the pathogenesis of many diseases and the kidney is an organ with particular susceptibility to complement-mediated injury. Apart from paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), there are several other diseases with clear evidence of complement activation affecting both maternal and fetal kidneys during pregnancy and causing long-term adverse outcomes. Several novel drugs have been recently developed for blocking the complement cascade, including purified plasma proteins, new monoclonal antibodies, recombinant proteins, small molecules, and small interfering RNA agents. Read More

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https://www.mdpi.com/2077-0383/8/3/407
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http://dx.doi.org/10.3390/jcm8030407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6463259PMC
March 2019
7 Reads

Elevated tricuspid regurgitation velocity in congenital hemolytic anemias: Prevalence and laboratory correlates.

Pediatr Blood Cancer 2019 Mar 25:e27717. Epub 2019 Mar 25.

Department of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Elevated tricuspid valve regurgitation jet velocity (TRV ≥ 2.5 m/s) is associated with mortality among adults with sickle cell disease (SCD), but correlative biomarkers are not studied according to treatment exposure or genotypes. To investigate the associations between biomarkers and TRV elevation, we examined the relationship between TRV and hemolytic, inflammatory, and cardiac biomarkers, stratified by disease-modifying treatments and SCD genotype. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/pbc.27717
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http://dx.doi.org/10.1002/pbc.27717DOI Listing
March 2019
3 Reads

Novel X-ray sequences and crystal structures of Persian and Starry sturgeon methemoglobins: Highlighting the role of heme pocket waters in causing autoxidation.

Biochim Biophys Acta Proteins Proteom 2019 Mar 21;1867(6):586-594. Epub 2019 Mar 21.

Institute of Biochemistry and Biophysics, University of Tehran, P.O. Box 13145-1384, Tehran, Iran.

Although there is a high sequence similarity between mammalian and fish hemoglobin (Hb), the oxidation and heme loss rates can vary greatly between them such that fish Hbs oxidise much more rapidly than mammalian Hbs. There is to date no sequence or structural data for any sturgeon Hb to reveal the level of autoxidation in these fish. In this study, novel high resolution X-ray sequences and crystal structures of methemoglobin (Met-Hb) from two sturgeon fish including Persian sturgeon (Acipenser percisus) and Starry sturgeon (Acipenser stellatus) belonging to the Caspian sea has been determined. Read More

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http://dx.doi.org/10.1016/j.bbapap.2019.03.008DOI Listing
March 2019
4 Reads

Ischemia-Reperfusion Injury in Sickle Cell Disease: From Basics to Therapeutics.

Am J Pathol 2019 Apr;189(4):706-718

Department of Molecular & Cellular Physiology, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana. Electronic address:

Sickle cell disease (SCD) is one of the most common hereditary hemoglobinopathies worldwide, affecting almost 400,000 newborns globally each year. It is characterized by chronic hemolytic anemia and endothelial dysfunction, resulting in a constant state of disruption of the vascular system and leading to recurrent episodes of ischemia-reperfusion injury (I/RI) to multiple organ systems. I/RI is a fundamental vascular pathobiological paradigm and contributes to morbidity and mortality in a wide range of conditions, including myocardial infarction, stroke, acute kidney injury, and transplantation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00029440183062
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http://dx.doi.org/10.1016/j.ajpath.2018.12.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6446225PMC
April 2019
6 Reads

Hydroxyurea use among children with sickle cell anemia.

Pediatr Blood Cancer 2019 Jun 22;66(6):e27721. Epub 2019 Mar 22.

Susan B Meister Child Health Evaluation and Research Center, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan.

This study describes hydroxyurea use among children ages 1 to 17 with sickle cell anemia (SCA) enrolled in at least one year of Medicaid in six states from 2005 to 2012. Administrative claims were used to summarize the number of days' supply of hydroxyurea dispensed by state and year. A total of 7963 children with SCA contributed 22 424 person-years. Read More

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http://dx.doi.org/10.1002/pbc.27721DOI Listing

Nutritional Status, Nutrient Intake, and Food Diversity Among Children With Sickle Cell Anemia.

J Pediatr Hematol Oncol 2019 Apr;41(3):e141-e145

Reference Center for Nutrition of Persons with Sickle Cell Disease (NUTRIFAL), Rio de Janeiro, Brazil.

Children with sickle cell anemia (SCA) often exhibit nutritional deficiencies and are at high risk of dying before the age of 5 years. Ensuring adequate nutrition is a critical part of health care for such children. This study aimed to investigate the association between nutritional status, nutrient intake, and food diversity in children with SCA. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001377DOI Listing
April 2019
1 Read

Psoriasis treatment in patients with sickle cell disease.

Cutis 2018 Feb;102(2):93-94

Department of Dermatology, The University of Tennessee Health Science Center, Memphis, USA.

Plaque psoriasis is a chronic inflammatory disease driven by the proliferation of T cells and the production of several immunomodulators such as tumor necrosis factor (TNF) α. Tumor necrosis factor α plays a key role in multiple inflammatory conditions, including psoriatic arthritis, rheumatoid arthritis, and hidradenitis suppurativa. We present a patient with plaque psoriasis and sickle cell disease who began treatment with the TNF-α inhibitor adalimumab. Read More

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February 2018
8 Reads

Impact of Sickle Cell Anemia on Inpatient Morbidity After Spinal Fusion.

Clin Spine Surg 2019 Mar 19. Epub 2019 Mar 19.

Department of Orthopaedics, University of California, Irvine, Orange, CA.

Study Design: A retrospective study.

Objective: To determine the impact of sickle cell anemia on perioperative outcomes and resource utilization in elective spinal fusion surgery.

Summary Of Background Data: Sickle cell anemia has been identified as an important surgical risk factor in otolaryngology, cardiothoracic surgery, general surgery, and total joint arthroplasty. Read More

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http://dx.doi.org/10.1097/BSD.0000000000000819DOI Listing
March 2019
2 Reads

Autosplenectomy in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH).

Case Rep Hematol 2019 12;2019:3146965. Epub 2019 Feb 12.

Houston Methodist Hospital, Department of Medicine, 6550 Fannin St, Houston, TX 77030, USA.

Autosplenectomy (AS) is a known complication of diseases such as sickle cell anemia, celiac disease, and inflammatory bowel disease. We report the first known case of AS due to paroxysmal nocturnal hemoglobinuria (PNH). A 24-year-old Caucasian male had evidence of hemolytic anemia at the age of 14 and was diagnosed with PNH at the age of 16. Read More

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http://dx.doi.org/10.1155/2019/3146965DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390241PMC
February 2019
5 Reads

Children with sickle cell anemia and APOL1 genetic variants develop albuminuria early in life.

Haematologica 2019 Mar 19. Epub 2019 Mar 19.

Division Pediatric Hematology and Oncology, University of Alabama at Birmingham, USA

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http://dx.doi.org/10.3324/haematol.2018.212779DOI Listing
March 2019
3 Reads

Hydroxyurea (hydroxycarbamide) for transfusion-dependent β-thalassaemia.

Cochrane Database Syst Rev 2019 03 16;3:CD012064. Epub 2019 Mar 16.

Department of Pediatric Hematology and Oncology, National Institute of Blood Disease & Bone Marrow Transplant, ST 2/A Block 17 Gulshan-e-Iqbal, KDA Scheme 24, Karachi, Sindh, Pakistan, 75300.

Background: Hydroxyurea (hydroxycarbamide) promotes the production of foetal haemoglobin (HbF) by reactivating gamma-genes. Evidence has shown clinical benefits of hydroxyurea in people with sickle cell anemia; however, only a few studies have assessed this treatment in people with beta (β)-thalassaemia.

Objectives: The primary objective is to review the efficacy of hydroxyurea in reducing or ameliorating the requirement of blood transfusions in people with transfusion-dependent β-thalassaemia. Read More

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http://dx.doi.org/10.1002/14651858.CD012064.pub2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421980PMC
March 2019
8 Reads

Effect of heterozygous beta thalassemia on HbA1c levels in individuals without diabetes mellitus: A cross sectional study.

Clin Chim Acta 2019 Mar 13;494:132-137. Epub 2019 Mar 13.

First Department of Propaedeutic Medicine, Medical School, National and Kapodistrian University of Athens, Athens, Greece.

Aims: To investigate the effect of heterozygous β-thalassemia on HbA1c levels in a population without diabetes mellitus (DM).

Methods: Using a cross-sectional design, HbA1c levels were compared between two groups of 100 consecutive carriers of β-thalassemia and 100 healthy controls matched for age, gender and BMI, taking into account fasting serum glucose and fructosamine levels. The effect of hemoglobin concentration on HbA1c was also examined. Read More

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http://dx.doi.org/10.1016/j.cca.2019.03.1611DOI Listing
March 2019
2 Reads

Incidence of Pulmonary Embolism in Sickle Cell Anemia Patients Undergoing Computed Tomography Pulmonary Angiography in the Emergency Department.

J Thorac Imaging 2019 Mar 11. Epub 2019 Mar 11.

Department of Radiology and Radiologic Sciences, Medical University of South Carolina, Charleston, SC.

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http://dx.doi.org/10.1097/RTI.0000000000000409DOI Listing
March 2019
5 Reads

Unprepared and Misinformed Parents of Children with Sickle Cell Disease: Time to Rethink Awareness Campaigns.

Cureus 2018 Dec 31;10(12):e3806. Epub 2018 Dec 31.

Cardiothoracic Surgery, Lagos State University Teaching Hospital, Lagos, NGA.

Worldwide, sickle cell disease (SCD) poses a significant public health concern. It causes recurrent morbidity, and premature death is a distinct possibility, especially in Nigeria, which bears half the world's burden of SCD patients. Nigeria has yet to establish a newborn screening program; consequently, most affected children are diagnosed between one and three years of age when a health problem arises. Read More

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http://dx.doi.org/10.7759/cureus.3806DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402737PMC
December 2018
3 Reads

Prevalence and Genetic Analysis of - and -Thalassemia and Sickle Cell Anemia in Southwest Iran.

J Epidemiol Glob Health 2018 Dec;8(3-4):189-195

Research Center of Thalassemia and Hemoglobinopathies, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.

This prospective study assessed the prevalence and genetic analysis of - and -thalassemia and sickle cell anemia (SCA) in Southwest Iran. Hematological indices were measured in 17,581 couples living in Khuzestan Province, Southwest Iran. Individuals with mean corpuscular volume <80, mean corpuscular hemoglobin <27, hemoglobin A2 ≥3/5 were considered as -thalassemia traits. Read More

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http://dx.doi.org/10.2991/j.jegh.2018.04.103DOI Listing
December 2018
4 Reads

Etiology and Outcomes of Thrombotic Microangiopathies.

Clin J Am Soc Nephrol 2019 Apr 12;14(4):557-566. Epub 2019 Mar 12.

Service de Néphrologie-hypertension, Dialyses, Transplantation Rénale, Hôpital Bretonneau et hôpital Clocheville,

Background And Objectives: Thrombotic microangiopathies constitute a diagnostic and therapeutic challenge. Secondary thrombotic microangiopathies are less characterized than primary thrombotic microangiopathies (thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome). The relative frequencies and outcomes of secondary and primary thrombotic microangiopathies are unknown. Read More

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http://dx.doi.org/10.2215/CJN.11470918DOI Listing
April 2019
3 Reads

Haematological values in steady-state sickle cell anaemia patients and matched heamoglobin AA Controls in a Rural Area of Eastern Gabon.

Niger Postgrad Med J 2019 Jan-Mar;26(1):13-17

Paul Moukambi Regional Hospital Centre of Koula-Moutou (CHRPM), Koula-Moutou, Gabon.

Background: In Gabon, universal neonatal screening of sickle cell disease is not carried out in rural areas, often leading to late detection of the disease. However, complete blood counts are available in rural areas.

Materials And Methods: We evaluated the haematological parameters of 45 homozygous steady-state sickle cell anaemia (SCA) patients and compared them with 45 sex- and age-matched Haemoglobin AA controls in Koula-Moutou, a rural area in Eastern Gabon. Read More

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http://dx.doi.org/10.4103/npmj.npmj_182_18DOI Listing
March 2019
2 Reads

Survival Probability in Patients with Sickle Cell Anemia Using the Competitive Risk Statistical Model.

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019022. Epub 2019 Mar 1.

Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti. HEMORIO, Rio de Janeiro, RJ, Brazil.

The clinical picture of patients with sickle cell anemia (SCA) is associated with several complications some of which could be fatal. The objective of this study is to analyze the causes of death and the effect of sex and age on survival of Brazilian patients with SCA. Data of patients with SCA who were seen and followed at HEMORIO for 15 years were retrospectively collected and analyzed. Read More

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http://dx.doi.org/10.4084/MJHID.2019.022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402554PMC
March 2019
3 Reads

Hydroxyurea reduces cerebral metabolic stress in patients with sickle cell anemia.

Blood 2019 Mar 11. Epub 2019 Mar 11.

Department of Neurology, Washington University School of Medicine, St. Louis, MO, United States;

Chronic transfusion therapy (CTT) prevents stroke in selected patients with sickle cell anemia (SCA). We have shown that CTT mitigates signatures of cerebral metabolic stress, reflected by elevated oxygen extraction fraction (OEF), which likely drives stroke risk reduction. The region of highest OEF falls within the border zone, where cerebral blood flow (CBF) nadirs, and OEF in this region was reduced after CTT. Read More

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http://dx.doi.org/10.1182/blood-2018-09-876318DOI Listing
March 2019
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New insights into the links between hypoxia and iron homeostasis.

Curr Opin Hematol 2019 May;26(3):125-130

Department Endocrinology Metabolism and Diabetes, INSERM U1016, Institut Cochin.

Purpose Of Review: This review outlines recent discoveries on the crosstalk between oxygen metabolism and iron homeostasis, focusing on the role of HIF-2 (hypoxia inducible factor-2) in the regulation of iron metabolism under physiopathological conditions.

Recent Findings: The importance of the hepcidin/ferroportin axis in the modulation of intestinal HIF-2 to regulate iron absorption has been recently highlighted. Latest advances also reveal a direct titration of the bone morphogenetic proteins by the erythroferrone contributing to liver hepcidin suppression to increase iron availability. Read More

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http://dx.doi.org/10.1097/MOH.0000000000000494DOI Listing
May 2019
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Haemolysis in sickle cell anaemia: effects of polymorphisms in α-globin gene regulatory elements.

Br J Haematol 2019 Mar 10. Epub 2019 Mar 10.

Department of Medicine, Boston University School of Medicine, Boston, MA, USA.

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http://dx.doi.org/10.1111/bjh.15852DOI Listing
March 2019
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Double-Blind Clinical Trial of Arginine Supplementation in the Treatment of Adult Patients with Sickle Cell Anaemia.

Adv Hematol 2019 3;2019:4397150. Epub 2019 Feb 3.

Graduate Programme in Development and Technological Innovation of Medicines, Federal University of Ceará, Fortaleza, Brazil.

Background: Sickle cell anaemia (SCA) is the most prevalent monogenic disease in Brazil. In SCA, haemoglobin S (HbS) is formed, which modifies red blood cell morphology. Intravascular haemolysis occurs, in which free Hb and free radicals degrade nitric oxide (NO) and release arginase, which reduces arginine levels. Read More

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http://dx.doi.org/10.1155/2019/4397150DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378076PMC
February 2019
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Sickle cell retinopathy: What we now understand using optical coherence tomography angiography. A systematic review.

Blood Rev 2019 05 4;35:32-42. Epub 2019 Mar 4.

Postgraduate course, Bahiana School of Medicine and Public Health, Av. Dom João VI, 275, Brotas, 40290-000 Salvador, BA, Brazil.

For over four decades, efforts have been underway for the evaluation of sickle cell retinopathy (SCR) in an attempt to identify peripheral high-risk vascular abnormalities based on Goldberg's classification (gold-standard) (1971). The macula is an area in the center of the retina that is responsible for high-resolution central vision and is also affected in SCR. With the development of new technologies for retinal imaging, the macula became a main focus of interest in the study of sickle cell disease (SCD). Read More

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http://dx.doi.org/10.1016/j.blre.2019.03.001DOI Listing
May 2019
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5.565 Impact Factor

Abnormal submaximal cardiopulmonary exercise parameters predict impaired peak exercise performance in sickle cell anemia patients.

Pediatr Blood Cancer 2019 Jun 7;66(6):e27703. Epub 2019 Mar 7.

Division of Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Purpose: Sickle cell anemia (SCA) patients frequently have many comorbidities, including diastolic dysfunction (DD) and exercise intolerance. SCA patients often cannot reach maximal effort on exercise testing; little is known regarding whether submaximal exercise parameters can predict abnormal maximal exercise results in SCA patients and if there are any possible associations with DD.

Methods: A prospective longitudinal study was performed in SCA patients. Read More

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http://dx.doi.org/10.1002/pbc.27703DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6472963PMC
June 2019
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2.562 Impact Factor

Respiratory resistance and reactance in adults with sickle cell anemia: Part 2-Fractional-order modeling and a clinical decision support system for the diagnosis of respiratory disorders.

PLoS One 2019 7;14(3):e0213257. Epub 2019 Mar 7.

Biomedical Instrumentation Laboratory-Institute of Biology and Faculty of Engineering, and BioVasc Research Laboratory-Institute of Biology, State University of Rio de Janeiro, Rio de Janeiro, Brazil.

Background: A better understanding of sickle cell anemia (SCA) and improvements in drug therapy and health policy have contributed to the emergence of a large population of adults living with this disease. The mechanisms by which SCA produces adverse effects on the respiratory system of these patients are largely unknown. Fractional-order (FrOr) models have a high potential to improve pulmonary clinical science and could be useful for diagnostic purposes, offering accurate models with an improved ability to mimic nature. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0213257PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6405112PMC
March 2019
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Sickle cell diseases: What can nuclear medicine offer?

Hell J Nucl Med 2019 Jan-Apr;22(1):2-3. Epub 2019 Mar 7.

Nuclear Medicine Unit, Interdisciplinary Department of Medicine, University of Bari "Aldo Moro", Bari, Italy.

Sickle cell disease (SCD) is the best known haemoglobinopathy, caused by a mutation substituting valina for glutamic acid at position 6 of the beta-globin chain of adult hemoglobin A, resulting in hemoglobin S (HbS). The homozygous HbS disease (HbSS), an autosomal recessive disorder, is the most common form and the Mediterranean area, along with sub-Saharian African and India, have the highest prevalence (1%-15%). In particular, Sicily with a prevalence of 2%-5%, is among the most interested regions. Read More

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http://dx.doi.org/10.1967/s002449910950DOI Listing
March 2019
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The 60th American Society of Hematology Annual Meeting & Exposition.

Authors:
Walter Alexander

P T 2019 Mar;44(3):138-142

We report on two presentations on β-thalassemia and myelodysplastic syndromes, three presentations on chronic lymphocytic leukemia, and others on perioperative oral anticoagulation, sickle-cell anemia, diffuse large B-cell lymphoma, and multiple myeloma. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6385735PMC
March 2019
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