27,186 results match your criteria Sickle Cell Anemia


Single Nucleotide Polymorphisms in , -, and and Their Relation to High Fetal Hemoglobin Levels That Alleviate Anemia.

Diagnostics (Basel) 2022 Jun 2;12(6). Epub 2022 Jun 2.

Department of Haematology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia.

Anemia is a condition in which red blood cells and/or hemoglobin (Hb) concentrations are decreased below the normal range, resulting in a lack of oxygen being transported to tissues and organs. Those afflicted with this condition may feel lethargic and weak, which reduces their quality of life. The condition may be manifested in inherited blood disorders, such as thalassemia and sickle cell disease, whereas acquired disorders include aplastic anemia, chronic disease, drug toxicity, pregnancy, and nutritional deficiency. Read More

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Predictors of Recurrent Acute Chest Syndrome in Pediatric Sickle Cell Disease: A Retrospective Case-Control Study.

Children (Basel) 2022 Jun 15;9(6). Epub 2022 Jun 15.

Department of Pediatrics, King Fahad Hospital of the University, Al-Khobar 31952, Saudi Arabia.

Acute chest syndrome (ACS) is a common cause of death in sickle cell disease (SCD) patients. Multiple studies investigated the risk factors of developing ACS; however, predictors of recurrent ACS episodes have not been thoroughly investigated. We aim to examine the clinical and laboratory predictors of recurrent ACS in pediatric patients with SCD. Read More

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Moyamoya syndrome and stroke among pediatric sickle cell disease patients in Sudan: A cross-sectional study.

Ann Med Surg (Lond) 2022 Jun 21;78:103815. Epub 2022 May 21.

Ribat Neurospine Center, Ribat University Hospital, The National Ribat University, Khartoum, Sudan.

Background: Sickle cell disease (SCD) is autosomal recessive hemolytic anemia due to hemoglobinopathy commonly in Sub-Saharan Africa, and particularly in Sudan. The disease induces a pro-inflammatory cascade in the intimal layer that leads to hyperplasia and progressive stenosis in the major vessel of the circle of Willis. This is associated with the development of Moyamoya collaterals. Read More

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Patient perception of voxelotor treatment benefit in sickle cell disease.

J Investig Med 2022 06;70(5):1316-1319

Department of Internal Medicine, The University of Texas Health Science Center at Houston, John P and Katherine G McGovern Medical School, Houston, Texas, USA.

Patients with sickle cell disease (SCD) experience a range of clinical symptoms, including acute and chronic pain, fatigue, and respiratory problems, as well as chronic organ complications that can lead to disability and accelerated mortality. Voxelotor is a first-in-class therapy that targets sickle hemoglobin polymerization, the root cause of SCD. It is approved by the US Food and Drug Administration for treatment of SCD in patients aged 4 years and older and in the European Union and United Arab Emirates for the treatment of SCD in patients aged 12 years and older. Read More

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New agents for sickle cell disease: patient perceptions of benefit in the real world.

Authors:
Robert T Means

J Investig Med 2022 06;70(5):1210-1211

Internal Medicine and Pathology, James H Quillen College of Medicine, East Tennessee State University, Johnson City, Tennessee, USA

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Transition for Adolescents and Young Adults With Sickle Cell Disease in a US Midwest Urban Center: A Multilevel Perspective on Barriers, Facilitators, and Future Directions.

J Pediatr Hematol Oncol 2022 Jul 22;44(5):e872-e880. Epub 2021 Sep 22.

Department of Pediatrics.

Background: Sickle cell disease (SCD), an inherited red blood cell disorder, primarily affects African Americans in the United States. Adolescents and young adults with SCD (AYA-SCD) are at risk of high morbidity and mortality when transitioning from pediatric to adult care. The goal of this qualitative study was to understand factors associated with optimal implementation of the AYA-SCD transition. Read More

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Clinical management of the acute complications of sickle cell anemia: 11 years of experience in a tertiary hospital.

An Pediatr (Engl Ed) 2022 Jun 18. Epub 2022 Jun 18.

Servicio de Pediatría, Hospital Universitario Cruces, Osakidetza, Barakaldo, Bizkaia, Spain; Biocruces Bizkaia Health Research Institute, Barakaldo, Bizkaia, Spain. Electronic address:

Introduction: Sickle cell disease is an emerging anemia in Europe leading to high morbidity with severe acute complications requiring hospital admission and chronic consequences. The management of these patients is complex and needs interdisciplinary care. The objective is to analyze clinical characteristics and management of patients with sickle cell disease admitted for acute complications. Read More

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Examining resilience of individuals living with sickle cell disease in the COVID-19 pandemic.

BMC Psychol 2022 Jun 20;10(1):156. Epub 2022 Jun 20.

Social and Behavioral Research Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Background: The COVID-19 pandemic has impacted the physical and mental health of people worldwide including those living with genetic conditions. Sickle cell disease (SCD) is a hematologic chronic disease that causes multisystem damage and morbidity. Individuals living with SCD have had to continue managing their care for their chronic disease while following public health measures to protect against infection with COVID-19. Read More

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COVID19 vaccination in adults with sickle cell disease is not associated with increases in rates of pain crisis.

Hematology 2022 Dec;27(1):742-744

Montefiore Medical Center, Bronx, NY, USA.

People with sickle cell disease (SCD) are more vulnerable to hospitalization, pneumonia, and pain following COVID-19 infection. However, given the association between the inflammatory response and vaso-occlusive crises in SCD and a case report of vaso-occlusive crises following administration of the ChAdOx1 nCov-195-7/AstraZeneca vaccine, there is concern that the administration of COVID-19 vaccines in people with SCD might provoke a vaso-occlusive crisis. To address this critical gap in knowledge, we sought to examine acute care usage for vaso-occlusive crisis and frequency and severity of side effects following COVID-19 vaccination among patients at the Montefiore Sickle Cell Center for Adults. Read More

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December 2022

Gestion des crises vaso-occlusives par les patients atteints de drépanocytose.

Rech Soins Infirm 2022 ;147(4):82-91

Introduction : Sickle cell disease, while it is the most common genetic disease in France, is little known to caregivers and the general public.Context : Only a few studies have examined the daily lives of people suffering from this disease.Objectives : The purpose of this project was to describe the specific strategies helping adult patients to manage the symptoms of their disease. Read More

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Effects of pregnancy on cardiac structure and function in women with sickle cell anemia: a longitudinal comparative study.

J Matern Fetal Neonatal Med 2022 Jun 20:1-6. Epub 2022 Jun 20.

Department of Obstetrics and Gynaecology, Lagos University Teaching Hospital, Lagos, Nigeria.

Background: Sickle cell anemia is the commonest hemoglobinopathy in pregnant Nigerian women, and cardiac manifestations are a significant feature of the disease especially in pregnancy. Pregnant women with sickle cell anemia are at high risk of morbidity and mortality and cardiac dysfunction in them increases this risk and may compromise their post-partum health.

Objective: To evaluate the cardiac size and function in women with sickle cell anemia (HbSS) during late pregnancy and postpartum. Read More

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Hyperkalemia and Metabolic Acidosis Occur at a Higher eGFR in Sickle Cell Disease.

Kidney360 2022 Apr 3;3(4):608-614. Epub 2022 Feb 3.

Department of Medicine, Division of Hematology & Oncology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

Background: People with sickle cell disease (SCD) have an elevated estimated glomerular filtration rate (eGFR) compared with the general population, and this may alter the usual creatinine-based eGFR cutoffs for which physiologic evidence of kidney dysfunction is apparent. This study aimed to identify eGFR thresholds for hyperkalemia and metabolic acidosis in patients with SCD.

Methods: This was a cross-sectional analysis of 733 patients with severe (hemoglobin SS or S -thalassemia) SCD genotype, 238 patients with moderate (hemoglobin SC or S -thalassemia) SCD genotype, and 1333 age- and sex-matched African Americans from the National Health and Nutrition Examination Survey (NHANES). Read More

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Neutrophil gelatinase-associated lipocalin is elevated in children with acute kidney injury and sickle cell anemia, and predicts mortality.

Kidney Int 2022 Jun 16. Epub 2022 Jun 16.

Department of Pediatrics, Ryan White Center for Pediatric Infectious Disease and Global Health, Indiana University School of Medicine, Indianapolis, Indiana. Electronic address:

Urine neutrophil gelatinase-associated lipocalin (NGAL) is a biomarker of acute kidney injury that has been adapted to a urine dipstick test. However, there is limited data on its use in low-and-middle-income countries where diagnosis of acute kidney injury remains a challenge. To study this, we prospectively enrolled 250 children with sickle cell anemia aged two to 18 years encompassing 185 children hospitalized with a vaso-occlusive pain crisis and a reference group of 65 children attending the sickle cell clinic for routine care follow up. Read More

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The oral ferroportin inhibitor vamifeport improved hemodynamics in a mouse model of sickle cell disease.

Blood 2022 Jun 17. Epub 2022 Jun 17.

Vifor (International) Ltd., St Gallen, Switzerland.

Sickle cell disease (SCD) is an inherited hemolytic anemia caused by a single point mutation in the beta‑globin gene of hemoglobin that leads to synthesis of sickle hemoglobin (HbS) in red blood cells (RBCs). HbS polymerizes in hypoxic conditions, leading to intravascular hemolysis, release of free hemoglobin and heme, and increased adhesion of blood cells to endothelial vasculature, which causes painful vaso-occlusion and organ damage. HbS polymerization kinetics are strongly dependent on the intracellular HbS concentration; a relatively small reduction in cellular HbS concentration may prevent HbS polymerization and its sequelae. Read More

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Economic burden of sickle cell disease in Brazil.

PLoS One 2022 16;17(6):e0269703. Epub 2022 Jun 16.

Department of Hematology/Oncology, Santa Casa Medical School of Sao Paulo, Sao Paulo, Brazil.

Background: Sickle cell disease (SCD) may cause several impacts to patients and the whole society. About 4% of the population has the sickle cell trait in Brazil, and 60,000 to 100,000 have SCD. However, despite recognizing the significant burden of disease, little is known about SCD costs. Read More

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Uptake and usage of proguanil as malaria chemoprophylaxis and the socio-economic determinants of proguanil usage in children with sickle cell anemia in Benin City.

Niger J Clin Pract 2022 Jun;25(6):903-908

Department of Child Health, University of Benin Teaching Hospital; Department of Child Health, University of Benin, Edo State, Nigeria.

Background: Proguanil is currently the recommended drug used for malaria chemoprophylaxis in children with Sickle cell anaemia (SCA).

Aims: This study aims to determine the uptake and usage of proguanil as malaria chemoprophylaxis and the socioeconomic determinants of its usage in children aged 6-59 months. This was a descriptive cross-sectional study carried out in two major sickle cell clinics in Benin City, Edo state, Nigeria. Read More

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SARS-CoV-2 omicron variant may present with severe sickle cell painful crisis: A report of two cases.

Clin Case Rep 2022 Jun 9;10(6):e05934. Epub 2022 Jun 9.

Department of Hematology and Medical Oncology Hamad Medical Corporation Doha Qatar.

Coronavirus disease 2019 (COVID-19) is a respiratory viral illness that is caused by coronavirus 2 (SARS-CoV-2). The disease often presents with non-specific symptoms such as fever, headache, and fatigue, accompanied by respiratory symptoms (e.g. Read More

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Primary Prevention of Stroke in Children With Sickle Cell Anemia in Nigeria: Protocol for a Mixed Methods Implementation Study in a Community Hospital.

JMIR Res Protoc 2022 Jun 13;11(6):e37927. Epub 2022 Jun 13.

Program in Occupational Therapy, Department of Medicine, Pediatrics, Surgery, and Education, Washington University School of Medicine, St. Louis, MO, United States.

Background: In Nigeria, approximately 150,000 children with sickle cell anemia (SCA) are born annually, accounting for more than half of all SCA births worldwide. Without intervention, about 11% of children with SCA will develop a stroke before their 20th birthday. Evidence-based practices for primary stroke prevention include screening for abnormal transcranial Doppler (TCD) measurements coupled with regular blood transfusion therapy for at least one year, followed by hydroxyurea (HU) therapy indefinitely. Read More

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Hemophagocytic lymphohistiocytosis diagnosed by bone marrow trephine biopsy in living post-COVID-19 patients: case report and mini-review.

J Mol Histol 2022 Jun 14. Epub 2022 Jun 14.

Department of Pathology, Faculty of Medicine, Medical School, School of Health Sciences, University of Thessaly, Biopolis, 41500, Larissa, Greece.

Hemophagocytic lymphohistiocytosis (HLH) constitutes a life-threatening inflammatory syndrome. Postmortem histological findings of bone marrow (BM) from COVID-19 patients showed histiocytosis and hemophagocytosis and supported the hypothesis that secondary HLH (sHLH) may be triggered by SARS-CoV-2 infection. However, there are a limited number of sHLH cases in which trephine has been performed in living post-COVID-19 patients. Read More

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Screening and diagnosis of hemoglobinopathies in Germany: Current state and future perspectives.

Sci Rep 2022 Jun 13;12(1):9762. Epub 2022 Jun 13.

Department of Hematology and Stem Cell Transplantation, West German Cancer Center, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.

This monocentric study conducted at the Pediatric and Adult Hemoglobinopathy Outpatient Units of the University Hospital of Essen summarizes the results of hemoglobinopathies diagnosed between August 2018 and September 2021, prior to the introduction of a general newborn screening (NBS) for SCD in Germany (October 2021). In total, 339 patients (pts.), 182 pediatric [50. Read More

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Longitudinal Study of Glomerular Hyperfiltration in Adults with Sickle Cell Anemia: A Multicenter Pooled Analysis.

Blood Adv 2022 Jun 13. Epub 2022 Jun 13.

University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States.

Glomerular hyperfiltration is common in young sickle cell anemia (SCA) patients and precedes development of overt kidney disease. In this multicenter pooled cohort, we characterized hyperfiltration and its decline to normal range in adult patients. Glomerular filtration rate (GFR) was estimated using the creatinine-based 2009 CKD-EPI equation omitting race adjustment and the 2021 CKD-EPI equation. Read More

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Epicatechin exerts dual action to shield sickling and hydroxyurea-induced myelosuppression: Implication in sickle cell anemia management.

Toxicol Appl Pharmacol 2022 Jun 9;449:116113. Epub 2022 Jun 9.

PK-PD Toxicology Division, CSIR-Indian Institute of Integrative Medicine, Jammu 180001, India; Academy of Scientific and Innovative Research (AcSIR), Ghaziabad 201002, India. Electronic address:

Hydroxyurea (HU) is the key drug to treat Sickle cell anemia (SCA). However, its treatment is associated with the liability of myelosuppression. The present study aimed to investigate the potential of epicatechin as a supplementation therapy for the symptomatic management of SCA under HU therapy. Read More

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Knowledge, attitude and premarital screening practices for sickle cell disease among young unmarried adults in an urban community in Lagos, Nigeria.

Pan Afr Med J 2022 6;42. Epub 2022 May 6.

Department of Haematology and Blood Transfusion, College of Medicine, University of Lagos, Lagos, Nigeria.

Introduction: sickle cell disease (SCD) refers to a group of inherited blood disorders that are life-long and affect many people globally. An estimate of 2.3% of the Nigerian population suffer from SCD and about 25% of adults have the sickle cell gene. Read More

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The Effects of Sickle Cell Disease on the Quality of Life: A Focus on the Untold Experiences of Parents in Tanzania.

Int J Environ Res Public Health 2022 06 4;19(11). Epub 2022 Jun 4.

Department of Development Studies, School of Public Health and Social Sciences, Muhimbili University of Health and Allied Sciences, Dar es Salaam P.O. Box 65013, Tanzania.

Tanzania is among the top five countries with a high burden of sickle cell disease (SCD) in the world. Even though the effects of SCD on quality of life have been documented in other countries including Nigeria and the United States of America, few are known from Tanzania. Therefore, this study focused on evaluating the effects of SCD on the quality of life among children living with SCD and their parents. Read More

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Hematopoietic Stem Cell Gene-Addition/Editing Therapy in Sickle Cell Disease.

Cells 2022 Jun 4;11(11). Epub 2022 Jun 4.

Cellular and Molecular Therapeutics Branch, National Heart Lung and Blood Institutes (NHLBI)/National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health (NIH), Bethesda, MD 20892, USA.

Autologous hematopoietic stem cell (HSC)-targeted gene therapy provides a one-time cure for various genetic diseases including sickle cell disease (SCD) and β-thalassemia. SCD is caused by a point mutation (20A > T) in the β-globin gene. Since SCD is the most common single-gene disorder, curing SCD is a primary goal in HSC gene therapy. Read More

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Safety and Acceptance of Acupuncture and Acupressure in Children, Adolescents, and Young Adults Undergoing Hematopoietic Stem Cell Transplant.

Cancer Nurs 2022 Jun 9. Epub 2022 Jun 9.

Author Affiliations: Palliative Care, Children's National Hospital, Washington, DC (Drs Bettini, Idiokitas, Mahmood, and Jacobs); and Capital Caring Hospice, Falls Church, Virginia (Dr Steinhorn).

Background: Hematopoietic stem cell transplant (HSCT) is associated with significant morbidity and high symptom burden including mucositis pain, nausea, and vomiting. There is little documentation in the literature regarding acupuncture or acupressure for children undergoing HSCT.

Objective: The purpose of this study was to determine the safety and acceptance of acupuncture and acupressure in children undergoing HSCT in a large tertiary care children's hospital. Read More

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Functional foods: promising therapeutics for Nigerian Children with sickle cell diseases.

Heliyon 2022 Jun 2;8(6):e09630. Epub 2022 Jun 2.

Department of Nursing, Achievers University, Owo, Nigeria.

Sickle cell disease (SCD), also known as sickle cell anemia (SCA) is one of the structural hemoglobinopathies that occurs due to a single nucleotide mutation from GAG to GTG, which changes the amino acid of a β-globin chain of hemoglobin (Hb) from glutamate to valine. This singular mutation results to disorderliness in red blood cells (RBCs) with advent of changes in RBC morphology and other pathological conditions. In the 1980s, intermittent red blood cell transfusions, opioids, and penicillin prophylaxis were the only available therapy for SCA and were commonly reserved for acute, life threatening complications. Read More

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Sex as an Independent Risk Factor for Venous Thromboembolism in Sickle Cell Disease: A Cross-Sectional Study.

J Womens Health (Larchmt) 2022 Jun 8. Epub 2022 Jun 8.

Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA.

Venous thromboembolism (VTE) affects up to 25% of individuals with sickle cell disease (SCD), but risk factors are not well characterized. We sought to measure the prevalence of VTE among SCD patients in our health system and to describe the relationship between medical history, biological sex, and VTE. We performed a retrospective chart review of SCD patients who visited an outpatient hematology clinic within Penn Medicine between June 2014 and June 2019. Read More

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Oxidative stress and associated clinical manifestations in malaria and sickle cell (HbSS) comorbidity.

PLoS One 2022 8;17(6):e0269720. Epub 2022 Jun 8.

Department of Biomedical Sciences, School of Allied Health Sciences, University of Cape Coast, Cape Coast, Ghana.

In Ghana, uncomplicated malaria and sickle cell disease (SCD) is common, hence comorbidity is not farfetched. However, the extent of oxidative stress and the array of clinical manifestations in this comorbidity (presence of both malaria and SCD) has not been fully explored. This study highlights the impact of uncomplicated malaria on SCD. Read More

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Voxelotor for the treatment of sickle cell disease in pediatric patients.

Expert Rev Hematol 2022 Jun 7;15(6):485-492. Epub 2022 Jun 7.

Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon.

Introduction: Sickle cell disease (SCD) describes a group of heritable blood disorders caused by the polymerization of sickle hemoglobin (HbS). HbS polymerization leads to anemia and vaso-occlusion, a process that impedes delivery of oxygen to tissues throughout the body, resulting in end-organ damage (EOD). Given the lifelong complications associated with SCD, identification and treatment of early symptoms in childhood is increasingly important. Read More

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