31,554 results match your criteria sickle cell


Novel histone deacetylase inhibitor CT-101 induces γ-globin gene expression in sickle erythroid progenitors with targeted epigenetic effects.

Blood Cells Mol Dis 2021 Nov 17;93:102626. Epub 2021 Nov 17.

Department of Pediatrics, Augusta University, Augusta, GA 30912, United States of America. Electronic address:

Induction of fetal hemoglobin (HbF) expression ameliorates the clinical severity and prolong survival in persons with sickle cell disease (SCD). Hydroxyurea (HU) is the only FDA-approved HbF inducer however, additional therapeutics that produce an additive effect in SCD are needed. To this end, development of potent Class I histone deacetylase inhibitors (HDACi) for HbF induction represents a rational molecularly targeted approach. Read More

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November 2021

Predicting risk factors for thromboembolic complications in patients with sickle cell anaemia - lessons learned for prophylaxis.

J Int Med Res 2021 Dec;49(12):3000605211055385

Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman.

Objective: To assess the clinical and laboratory predictors of venous thromboembolism (VTE) in patients with sickle cell anaemia (SCA) and its relationship to morbidity and mortality.

Methods: This retrospective case-control study analysed data from patients with SCA that experienced VTE compared with matched control patients with SCA but no VTE (2:1 ratio).

Results: A total of 102 patients with SCA were enrolled (68 cases with VTE and 34 controls). Read More

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December 2021

Hepatobiliary Findings in Nigerian Children with Sickle Cell Anaemia.

West Afr J Med 2021 Oct;Vol. 38(10):917-923

Department of Medicine, College of Medicine/ Lagos University Teaching Hospital, Lagos.

Introduction: Hepatobiliary abnormalities occur commonly in sickle cell anaemia and these have been extensively reported in the adult patients. However, complications have been sparsely reported in children especially in the sub-Saharan African continent. This study aimed to highlight the hepatobiliary complications in this group of children using clinical examination, laboratory testing and abdominal ultrasonography. Read More

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October 2021

Trends in quality of care among children with sickle cell anemia.

Pediatr Blood Cancer 2021 Dec 2:e29446. Epub 2021 Dec 2.

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

Introduction: For decades, it has been recommended that children with sickle cell anemia (SCA) receive antibiotic prophylaxis to prevent serious infections and undergo transcranial Doppler (TCD) screening to identify those at highest risk of overt stroke. We assessed recent temporal trends in antibiotic prophylaxis prescription fills and TCD screening among children with SCA using validated quality measures.

Procedure: Using validated claims-based definitions, we identified children with SCA who were enrolled in Michigan or New York State (NYS) Medicaid programs (2011-2018). Read More

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December 2021

Multiplex CRISPR/Cas9 genome editing in hematopoietic stem cells for fetal hemoglobin reinduction generates chromosomal translocations.

Mol Ther Methods Clin Dev 2021 Dec 28;23:507-523. Epub 2021 Oct 28.

Stem Cell and Gene Therapy Program, Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA 98109-1024, USA.

Sickle cell disease and β-thalassemia are common monogenic disorders that cause significant morbidity and mortality globally. The only curative treatment currently is allogeneic hematopoietic stem cell transplantation, which is unavailable to many patients due to a lack of matched donors and carries risks including graft-versus-host disease. Genome editing therapies targeting either the erythroid enhancer or the promoter are already demonstrating success in reinducing fetal hemoglobin. Read More

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December 2021

INCREASED CIRCULATING COPEPTIN LEVELS ARE ASSOCIATED WITH VASO-OCCLUSIVE CRISIS AND RIGHT VENTRICULAR DYSFUNCTION IN SICKLE CELL ANEMIA.

Med Princ Pract 2021 Dec 1. Epub 2021 Dec 1.

Objective Vaso-occlusive crisis (VOC) is a common clinical manifestation of sickle cell anemia (SCA) and is associated with increased proinflammatory mediators. Copeptin is the C-terminal part of the prohormone for pro-vasopressin and seems clinically relevant in various clinical conditions. Right ventricular (RV) dysfunction significantly appears in SCA patients due to pulmonary hypertension. Read More

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December 2021

Early oral switch to combined cefixime therapy for management of osteoarticular infections in pediatric sickle cell disease patients: A descriptive analysis.

J Infect Public Health 2021 Nov 17;15(1):1-6. Epub 2021 Nov 17.

Center for Health Outcomes & PharmacoEconomic Research, University of Arizona, Tucson, AZ, USA; Department of Clinical Pharmacy, Prince Sattam Bin Abdulaziz University, Alkharj, Saudi Arabia.

Background: The treatment of osteoarticular infections in pediatric patients with sickle cell disease (SCD) is a challenging task for the practitioner. The aim of this study is to evaluate cefixime for the treatment of osteoarticular infections in pediatric SCD patients by retrospective design.

Methods: This study was done in the pediatric hospital of King Saud Medical City, Riyadh, Saudi Arabia. Read More

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November 2021

First report of successful treatment for hemoglobin Bristol-Alesha by hemopoietic stem cell transplantation.

Ann Hematol 2021 Dec 1. Epub 2021 Dec 1.

Department of Hematology of Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, People's Republic of China.

HBB gene mutations lead to many kinds of diseases, of which, except for the two most common diseases of thalassemia and sickle cell anemia, rare kinds of hemolytic anemia, such as hemoglobin Bristol-Alesha, are rarely reported, no ideal treatment in clinic. A child suffered from chronic recurrent hemolytic attacks and the related genes of hereditary hemolytic anemia were detected on her. Hematopoietic stem cell transplantation was conducted in the treatment of the patient. Read More

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December 2021

Preferences for Using a Mobile App in Sickle Cell Disease Self-management: Descriptive Qualitative Study.

JMIR Form Res 2021 Nov 30;5(11):e28678. Epub 2021 Nov 30.

Department of Health Policy, Vanderbilt University School of Medicine, Nashville, TN, United States.

Background: Individuals with sickle cell disease (SCD) and their caregivers may benefit from technology-based resources to improve disease self-management.

Objective: This study explores the preferences regarding a mobile health (mHealth) app to facilitate self-management in adults with SCD and their caregivers living in urban and rural communities.

Methods: Five community listening sessions were conducted in 2 urban and rural communities among adults with SCD and their caregivers (N=43). Read More

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November 2021

Sickle cell disease patients in two London trusts: Genotyping including RH variants.

Transfus Med 2021 Dec 1. Epub 2021 Dec 1.

Haematology Department, Imperial College Healthcare NHS Trust, London, UK.

Background: All SCD patients need extended RBC antigen typing (by serology or genotyping) for provision of extended RH, K matched blood and to guide RBC selection in those with complex transfusion requirements. Genotyping can also identify RH variants which can cause sensitisation even when extended RH phenotypically matched blood is provided and alloantibodies associated with RH variants can cause HTRs.

Objectives: To review the use of RBC genotyping in SCD patients at two London trusts (ICHNT, LNWH) with a focus on RH variants. Read More

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December 2021

Longitudinal assessment of adhesion to vascular cell adhesion molecule-1 at steady state and during vaso-occlusive crises in sickle cell disease.

Br J Haematol 2021 Dec 1. Epub 2021 Dec 1.

Functional Fluidics, Children's Hospital of Michigan, Detroit, MI, USA.

Sickle cell disease (SCD) is characterized by frequent and unpredictable vaso-occlusive crises (VOCs). Sickle erythrocytes (SSRBCs) contribute to VOCs by participating in a series of adhesive events with blood cells and the vascular endothelium. Adhesion assays have been used to evaluate the relationship between SSRBC adhesion and SCD severity. Read More

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December 2021

Non-Invasive Prenatal Test for β-Thalassemia and Sickle Cell Disease Using Probe Capture Enrichment and Next-Generation Sequencing of DNA in Maternal Plasma.

J Appl Lab Med 2021 Nov 26. Epub 2021 Nov 26.

Department of Genetics and Genomics, Children's Hospital Oakland Research Institute, Oakland, CA, USA.

Background: Noninvasive prenatal testing (NIPT) of chromosomal aneuploidies based on next-generation sequencing (NGS) analysis of fetal DNA in maternal plasma is well established, but testing for autosomal recessive disorders remains challenging. NGS libraries prepared by probe capture facilitate the analysis of the short DNA fragments plasma. This system has been applied to the β-hemoglobinopathies to reduce the risk to the fetus. Read More

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November 2021

Performance of an HRP-2 based (First Response®) and p-LDH-based (Optimal®) rapid diagnostic tests for diagnosis of malaria in paediatric sickle cell disease patients.

Clin Infect Dis 2021 Nov 26. Epub 2021 Nov 26.

Centre for Medical Parasitology at Department of Immunology and Microbiology, University of Copenhagen and Department of Clinical Microbiology and Department of Infectious Diseases, Copenhagen University Hospital (Rigshospitalet), Copenhagen, Denmark.

Background: Rapid diagnostic tests (RDTs) have been extensively evaluated and play an important role in malaria diagnosis. However, the accuracy of RDT for malaria diagnosis in patients with sickle cell disease (SCD) is unknown.

Methods: We compared the performance of a histidine rich protein 2 (HRP-2) - based RDT (First Response®), and a lactate dehydrogenase (LDH) - based RDT (Optimal®) with routine microscopy as reference standard in 445 SCD children with an acute febrile illness in Accra, Ghana. Read More

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November 2021

Nationwide retrospective study of critically ill adults with sickle cell disease in France.

Sci Rep 2021 Nov 30;11(1):23132. Epub 2021 Nov 30.

Service de Médecine Intensive Réanimation, Centre Hospitalier Universitaire Hôtel-Dieu, 30 Bd. Jean Monnet, 44093, Nantes Cedex 1, France.

Little is known about patients with sickle cell disease (SCD) who require intensive care unit (ICU) admission. The goals of this study were to assess outcomes in patients admitted to the ICU for acute complications of SCD and to identify factors associated with adverse outcomes. This multicenter retrospective study included consecutive adults with SCD admitted to one of 17 participating ICUs. Read More

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November 2021

Clinical depression in children and adolescents with sickle cell anaemia: influencing factors in a resource-limited setting.

BMC Pediatr 2021 12 1;21(1):533. Epub 2021 Dec 1.

Department of Paediatrics, Faculty of Medical Sciences, College of Medicine, University of Nigeria, Ituku-Ozalla Campus, Enugu, Nigeria.

Background: Sickle cell anaemia (SCA) is the commonest monogenic haematologic disorder resulting from the inheritance of homozygous mutant haemoglobin genes from both parents. Some factors have been identified as important in explaining the variability in depression in sickle cell anaemia (SCA). Information on this is limited in a resource-limited setting like Nigeria. Read More

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December 2021

Deferiprone vs deferoxamine for transfusional iron overload in SCD and other anemias: a randomized, open-label, noninferiority study.

Blood Adv 2021 Nov 30. Epub 2021 Nov 30.

Ain Shams University, Cairo, Egypt.

Many people with sickle cell disease (SCD) or other anemias require chronic blood transfusions, which often causes iron overload and requires chelation therapy. The iron chelator deferiprone is often used in individuals with thalassemia syndromes, but data in patients with SCD are limited. This open-label study (NCT02041299) assessed the efficacy and safety of deferiprone in patients with SCD or other anemias receiving chronic transfusion therapy. Read More

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November 2021

Breaking the Cycle: Care Coordination Interventions and Sickle Cell Readmissions.

Authors:
Naph'tali B Edge

Prof Case Manag 2022 Jan-Feb 01;27(1):12-18

Naph'tali B. Edge, DNP, MBA, MSN, BSN, is a vice president of care coordination & operations at a large academic, safety net, Level 1 trauma center in Atlanta, GA. His work includes case management and social work operations, transfer center, bed management, patient flow, and other aspects of hospital operations throughout the continuum of care. He is now on the Board and Board President for the Sickle Cell Association of Houston.

Purpose/objectives: Approximately 100,000 people in the United States are affected by sickle cell disease (SCD). Sickle cell disease represents one of the highest readmitting diagnoses at this organization. The purpose of this study is to determine the impact of implementing care coordination interventions to reduce hospital readmissions of patients with SCD. Read More

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November 2021

Integrating deep learning with microfluidics for biophysical classification of sickle red blood cells adhered to laminin.

PLoS Comput Biol 2021 Nov 29;17(11):e1008946. Epub 2021 Nov 29.

Department of Physics, Case Western Reserve University, Cleveland, Ohio, United States of America.

Sickle cell disease, a genetic disorder affecting a sizeable global demographic, manifests in sickle red blood cells (sRBCs) with altered shape and biomechanics. sRBCs show heightened adhesive interactions with inflamed endothelium, triggering painful vascular occlusion events. Numerous studies employ microfluidic-assay-based monitoring tools to quantify characteristics of adhered sRBCs from high resolution channel images. Read More

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November 2021

The Alberta Newborn Screening Approach for Sickle Cell Disease: The Advantages of Molecular Testing.

Int J Neonatal Screen 2021 Nov 16;7(4). Epub 2021 Nov 16.

Newborn Metabolic Screening and Biochemical Genetics Laboratory, University of Alberta Hospital, Alberta Precision Laboratories, Edmonton, AB T6G 2B7, Canada.

Sickle cell disease (SCD), a group of inherited red blood cell (RBC) disorders caused by pathogenic variants in the beta-globin gene (), can cause lifelong disabilities and/or early mortality. If diagnosed early, preventative measures significantly reduce adverse outcomes related to SCD. In Alberta, Canada, SCD was added to the newborn screening (NBS) panel in April 2019. Read More

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November 2021

Case report of active pulmonary TB in a sickle cell disease patient.

Ann Med Surg (Lond) 2021 Dec 16;72:103073. Epub 2021 Nov 16.

Department of Medical Oncology, Hematology Section, National Center for Cancer Care & Research, Hamad Medical Corporation, Doha, Qatar.

Both Sickle cell disease (SCD) and Tuberculosis (TB) are prevalent, and mostly concentrated in similar geographic areas. Since there is an overlap of the possible presenting symptoms of SCD complications and TB, a low threshold for suspicion of TB in these patients is recommended. We present a case of an active TB infection in a SCD patient that presented mainly with chronic cough. Read More

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December 2021

Neurodevelopmental Outcomes in Preterm Children with Sickle Cell Disease.

J Int Neuropsychol Soc 2021 Nov 29:1-11. Epub 2021 Nov 29.

Department of Psychology, University of South Carolina, Columbia, SC, USA.

Objectives: To explore the combined effect of pediatric sickle cell disease (SCD) and preterm birth on cognitive functioning.

Methods: Cognitive functioning was examined in children ages 6-8 with high risk SCD genotypes born preterm (n = 20) and full-term (n = 59) and lower risk SCD genotypes/no SCD born preterm (n = 11) and full-term (n = 99) using tests previously shown to be sensitive to SCD-related neurocognitive deficits. Factorial ANOVAs and log linear analyses were conducted to examine the relationship between SCD risk, preterm birth status, and cognitive outcomes. Read More

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November 2021

Clinical outcomes of COVID-19 in patients with sickle cell disease and sickle cell trait: A critical appraisal of the literature.

Blood Rev 2021 Nov 20:100911. Epub 2021 Nov 20.

Department of Radiology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, NY 10461, USA. Electronic address:

Individuals with sickle cell disease (SCD) and sickle cell trait (SCT) have many risk factors that could make them more susceptible to COVID-19 critical illness and death compared to the general population. With a growing body of literature in this field, a comprehensive review is needed. We reviewed 71 COVID-19-related studies conducted in 15 countries and published between January 1, 2020, and October 15, 2021, including a combined total of over 2000 patients with SCD and nearly 2000 patients with SCT. Read More

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November 2021

Sickle Cell Anemia and Infection.

Pathogens 2021 Nov 4;10(11). Epub 2021 Nov 4.

Department of Blood Borne Parasites, Lindsley F. Kimball Research Institute, New York Blood Center, New York, NY 100065, USA.

is an intraerythrocytic, obligate Apicomplexan parasite that has, in the last century, been implicated in human infections via zoonosis and is now widespread, especially in parts of the USA and Europe. It is naturally transmitted by the bite of a tick, but transfused blood from infected donors has also proven to be a major source of transmission. When infected, most humans are clinically asymptomatic, but the parasite can prove to be lethal when it infects immunocompromised individuals. Read More

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November 2021

Adipose-Derived Stem Cell: "Treat or Trick".

Biomedicines 2021 Nov 5;9(11). Epub 2021 Nov 5.

Reconstructive Sciences Unit, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia.

Stem cells have been widely used for treating disease due to the various benefits they offer in the curing process. Several treatments using stem cells have undergone clinical trials, such as cell-based therapies for heart disease, sickle cell disease, thalassemia, etc. Adipose-derived stem cells are some of the many mesenchymal stem cells that exist in our body that can be harvested from the abdomen, thighs, etc. Read More

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November 2021

Characteristics associated with the perceived likelihood to become parents among young adults with sickle cell disease or sickle cell trait in the USA.

J Genet Couns 2021 Nov 26. Epub 2021 Nov 26.

Department of Biobehavioral Nursing Science, College of Nursing, University of Florida, Gainesville, Florida, USA.

Individuals with sickle cell disease (SCD) and individuals with sickle cell trait (SCT) have different health trajectories, but it is unknown whether sociodemographic and clinical characteristics are associated with their likelihood to be a parent. The purpose of this study was to examine the sociodemographic and clinical characteristics associated with perceived likelihood-to-parent among a cohort of young adults with SCD or SCT in the USA. The participants were 234 young adults (82 males, 152 females) who had either SCD (n = 138) or SCT (n = 96). Read More

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November 2021

Prognostic factors associated with COVID-19 related severity in sickle cell disease.

Blood Cells Mol Dis 2021 Nov 17;92:102627. Epub 2021 Nov 17.

Lindsley F. Kimball Research Institute, New York Blood Center, New York, NY, United States of America; Department of Medicine, Division of Hematology-Oncology, Zucker School of Medicine, Northwell Health, NY, United States of America. Electronic address:

Background: Equipoise exists regarding sickle cell disease (SCD) as a risk factor for COVID-19 disease severity and variables that increase risk of COVID-19 severity in SCD. Given our health system's large SCD patient catchment, we analyzed our own experience in this regard.

Study Methods: Retrospective analysis of the clinical course and factors associated with need for hospitalization and ICU admission of SCD patients diagnosed with COVID-19 through the Northwell Health system from March 1 to Dec 31, 2020. Read More

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November 2021

Deep Conservation of -Like RHG Gene Family Homologs in Winged Insects Revealed by "Taxon Hopping" BLAST.

Authors:
Markus Friedrich

Insects 2021 Oct 21;12(11). Epub 2021 Oct 21.

Department of Biological Sciences, Wayne State University, 5047 Gullen Mall, Detroit, MI 48202, USA.

Together with (), the paralogs (), (), and (RHG) control a critical switch in the induction of programmed cell death. RHG homologs have been identified in other dipteran and lepidopteran species but not beyond. Revisiting this issue with a "taxon hopping" BLAST search strategy in current genome and transcriptome resources, I detected high confidence RHG homologs in Coleoptera, Hymenoptera, Hemiptera, and Dictyoptera. Read More

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October 2021

Total Hip Replacement in Sickle Cell Disease Patients with Avascular Necrosis of Head of Femur: A Retrospective Observational Study.

Indian J Orthop 2021 Oct 28;55(5):1225-1231. Epub 2021 Mar 28.

Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India.

Background: Femoral head avascular necrosis leads to osteoarthritis of the hip joint and affects its functional capacity in sickle cell disease patients. The functional outcomes of total hip replacement (THR) on patients with congruous joints who underwent hip replacement after having a failed joint preservation surgery are unknown. This study aimed to compare the functional outcomes of THR in patients with sickle cell disease having avascular necrosis with and without loss of hip joint congruency. Read More

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October 2021

Clinical and physiological characteristics of, medically treated, chronic thromboembolic pulmonary hypertension patients in Saudi Arabia: A single center experience.

Ann Thorac Med 2021 Oct-Dec;16(4):347-353. Epub 2021 Oct 26.

Department of Medicine, Division of Pulmonary Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is uncommon but well-known sequel of venous thromboembolism (VTE). At present, it is the only potential curable subtype of pulmonary hypertension. The aim of this study is to describe the medically treated-CTEPH patients' characteristics in a single specialized PH center in Saudi Arabia. Read More

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October 2021