3,800 results match your criteria Pediatrics Sickle Cell Disease


Anti-SARS-CoV-19 antibodies in children and adults with sickle cell disease: A single-site analysis in New York City.

Br J Haematol 2022 Jun 27. Epub 2022 Jun 27.

Department of Pathology and Cell Biology, Columbia University Irving Medical Center - New York Presbyterian Hospital, New York, New York, USA.

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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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Pediatric Patients With Sickle Cell Disease at a Public Hospital: Nutrition, Compliance and Early Experience With L-Glutamine Therapy.

In Vivo 2022 Jul-Aug;36(4):1761-1768

Division of Pediatric Hematology-Oncology, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, CA, U.S.A.

Background/aim: Hydration and hydroxyurea (HU) can modify sickle cell disease (SCD) severity. Optimal nutrition and L-glutamine (Gln) may provide further amelioration.

Patients And Methods: Reviews of medical records and nutrition surveys were used to investigate severity of pediatric patients with SCD in relation to nutrition, growth, hematologic parameters, and diseasemodifying agents. Read More

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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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Provider Communication and Fever Protocol for Children With Sickle Cell Disease in the Emergency Department.

Pediatr Emerg Care 2022 Jun 22. Epub 2022 Jun 22.

Division of Hematology/Oncology, Department of Pediatrics, East Carolina University, Greenville, NC.

Objective: We assessed whether prior communication between pediatric hematologists and emergency department (ED) providers reduced time to administration of parenteral antibiotics for children with sickle cell disease presenting with fever.

Methods: Patients 2 months to 21 years of age were retrospectively identified if they were followed up at our center's pediatric hematology clinic and presented to the pediatric ED with fever. Emergency department-hematology communication before patient arrival was ascertained by chart review. Read More

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Reduction in seroprevalence of viral transfusion-transmitted infections in southwest Nigeria in children with sickle cell disease using an enhanced screening strategy.

Br J Haematol 2022 Jun 19. Epub 2022 Jun 19.

Division of Hematology/Oncology, Department of Medicine, Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

Blood transfusion is an integral component in the management of children and adults with sickle cell disease (SCD). Concerns about blood safety due to the high risk of bloodborne infections in sub-Saharan Africa limits the application of this cost-effective strategy in the management of individuals with SCD. In a single-centre, retrospective, longitudinal study in southwest Nigeria, we hypothesised that the use of stringent blood donor selection, along with very sensitive enzyme-linked immunosorbent assay (ELISA) screening methods would reduce transfusion-transmitted infections (TTIs). 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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Neurocognitive screening in sickle cell disease.

Pediatr Blood Cancer 2022 Jun 18:e29861. Epub 2022 Jun 18.

Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, MD.

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Application of validated mapping algorithms between generic PedsQL scores and utility values to individuals with sickle cell disease.

Qual Life Res 2022 Jun 17. Epub 2022 Jun 17.

The Comparative Health Outcomes, Policy, and Economics (CHOICE) Institute, University of Washington, 1959 NE Pacific Street, H-375Q, Box 357630, Seattle, WA, 98195-7630, USA.

Purpose: There is a paucity of empirically estimated health state utility (HSU) values to estimate health-related quality of life among individuals with sickle cell disease (SCD). This study aims to map the Pediatric Quality of Life Inventory generic core scales (PedsQL GCS) to HSUs for children and adolescents with SCD in the United States, using published algorithms, and to assess the construct validity of these HSUs against SCD-specific PedsQL scores.

Methods: We used the published mapping algorithms identified in four published articles, in which the PedsQL GCS was mapped to either the EuroQol-5 Dimension 3-Level, Youth Version or the Child Health Utility 9-Dimension to obtain HSUs. Read More

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Role of Serum Ferritin in Predicting Outcomes of COVID-19 Infection Among Sickle Cell Disease Patients: A Systematic Review and Meta-Analysis.

Front Med (Lausanne) 2022 30;9:919159. Epub 2022 May 30.

Department of Pediatrics, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.

Patients with sickle cell disease (SCD) are at higher risk of getting severe COVID-19 infection. This systematic review and meta-analysis aimed to determine the role of serum ferritin in predicting ICU admission and mortality among patients with SCD following COVID-19 infection. A systematic search was conducted in PubMed, Scopus, Web of Science, Embase, WHO COVID-19 database, ProQuest, and Cochrane Library for articles published between 1st December 2019 to 31st November 2021. Read More

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Early Initiation of Sub-anesthetic Ketamine Infusion in Adults with Vaso-occlusive Crises is Associated with Greater Reduction in Sickle Cell Pain Intensity: A Single Center's Experience.

Pain Med 2022 Jun 16. Epub 2022 Jun 16.

Division of General Internal Medicine, Department of Internal Medicine, Virginia Commonwealth University School of Medicine, Richmond, VA, USA.

Objectives: Recurrent, severely painful episodes, known as vaso-occlusive crises (VOCs) are the hallmark of sickle cell disease (SCD) and the primary reason for hospitalization. Opioids have been the gold standard for VOC treatment without significant improvement pain outcomes. To aid analgesia and combat opioid related adverse effects (ORAEs), some SCD clinicians have trialed infusions of sub-anesthetic ketamine along with opioids to treat VOCs. 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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Sickle cell disease in children: an update of the evidence for WHO guideline development.

Authors:
Isaac Odame

Arch Dis Child 2022 Jun 15. Epub 2022 Jun 15.

Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada

Sickle cell disease (SCD), one of the most common monogenetic diseases in the world, is associated with multisystemic complications that begin in childhood. Most of the babies homozygous for the sickle haemoglobin gene are born in sub-Saharan Africa. Over the years, progress has been made with early diagnosis through newborn screening, penicillin prophylaxis, pneumococcal immunisation, transcranial Doppler (TCD) screening, hydroxyurea therapy and chronic blood transfusions with remarkably improved survival and quality of life of children with SCD. 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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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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The association between sleep disturbances and neurocognitive function in pediatric sickle cell disease.

Sleep Med 2022 May 26;97:27-35. Epub 2022 May 26.

Department of Pediatrics, Division of Hematology/Oncology, School of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA; Institute of Cancer Outcomes and Survivorship, University of Alabama at Birmingham, Birmingham, AL, USA. Electronic address:

Background And Objective: Youth with sickle cell disease (SCD) without neurological complications continue to be at increased risk of neurocognitive difficulties. Nocturnal hypoxemia is associated with neurocognitive outcomes and has been identified as a chronic complication in youth with SCD. The objective of this study was to assess the relationship between sleep disturbances and neurocognitive functioning in youth with SCD, while taking into account demographic and socioeconomic factors. Read More

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Distribution of Emergency Department Encounters and Subsequent Hospital Admissions for Children by Child Opportunity Index.

Acad Pediatr 2022 Jun 9. Epub 2022 Jun 9.

Division of Emergency Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States of America; Mary Ann & J. Milburn Smith Child Health Outcomes, Research, and Evaluation Center, Stanley Manne Children's Research Institute, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago IL, USA.

Objective: To evaluate differences in emergency utilization (ED) utilization and subsequent admission among children by Child Opportunity Index (COI).

Methods: We performed a cross-sectional study of pediatric (<18 years) encounters to 194 EDs in Illinois from 2016-2020. Each encounter was assigned the postal code-level category from the COI 2. 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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Suboptimal vancomycin levels in critically ill children with sickle cell disease and acute chest syndrome.

J Infect Chemother 2022 Jun 7. Epub 2022 Jun 7.

Department of Pharmacy Services, King Khalid University Hospital, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia. Electronic address:

Objectives: This is a pilot retrospective study to assess the effect of glomerular hyper-filtration (GHF) related to sickle cell disease (SCD) on vancomycin clearance and ultimately on therapeutic drug levels in children admitted to the pediatric intensive care unit (PICU) with acute chest syndrome (ACS).

Method: The patients' steady-state vancomycin trough levels (VTL) and the area under the curve (AUC) were compared with those of age- and gender-matched control group; matching was made at a 1:3 ratio.

Results: Twelve SCD patients with ACS and treated with vancomycin were compared with 36 non-SCD patients (control group). Read More

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New therapeutics for children with sickle cell disease: A time for celebration, caution, or both?

Pediatr Blood Cancer 2022 Aug 10;69(8):e29805. Epub 2022 Jun 10.

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.

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Clonal Hematopoiesis and the Risk of Hematologic Malignancies after Curative Therapies for Sickle Cell Disease.

J Clin Med 2022 Jun 2;11(11). Epub 2022 Jun 2.

Cellular and Molecular Therapeutics Branch, National Heart, Lung, and Blood Institute, NIH, 10 Center Drive, Bethesda, MD 20814, USA.

Sickle cell disease (SCD) is associated with severe morbidity and early mortality. Two large population studies found an increased risk for leukemia in individuals with SCD. Notably, while the relative risk of leukemia development is high, the absolute risk is low in individuals with SCD who do not receive cell-based therapies. Read More

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Long-Term Health Effects of Curative Therapies on Heart, Lungs, and Kidneys for Individuals with Sickle Cell Disease Compared to Those with Hematologic Malignancies.

J Clin Med 2022 May 31;11(11). Epub 2022 May 31.

Department of Pediatrics, Vanderbilt-Ingram Cancer Center, Vanderbilt University Medical Center, 395 Preston Research Building, 2220 Pierce Ave., Nashville, TN 37232, USA.

The goal of curing children and adults with sickle cell disease (SCD) is to maximize benefits and minimize intermediate and long-term adverse outcomes so that individuals can live an average life span with a high quality of life. While greater than 2000 individuals with SCD have been treated with curative therapy, systematic studies have not been performed to evaluate the long-term health effects of hematopoietic stem cell transplant (HSCT) in this population. Individuals with SCD suffer progressive heart, lung, and kidney disease prior to curative therapy. Read More

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In-Depth Immunological Typization of Children with Sickle Cell Disease: A Preliminary Insight into Its Plausible Correlation with Clinical Course and Hydroxyurea Therapy.

J Clin Med 2022 May 27;11(11). Epub 2022 May 27.

Pediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy.

Sickle cell disease (SCD) is a condition of functional hypo-/a-splenism in which predisposition to bacterial infections is only a facet of a wide spectrum of immune-dysregulation disorders forming the clinical expression of a peculiar immunophenotype. The objective of this study was to perform an in-depth immunophenotypical characterization of SCD pediatric patients, looking for plausible correlations between immunological biomarkers, the impact of hydroxyurea (HU) treatment and clinical course. This was an observational case-control study including 43 patients. 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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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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Health related quality of life in children with sickle cell disease: A systematic review and meta-analysis.

Blood Rev 2022 May 25:100982. Epub 2022 May 25.

Hematology, Oncology, Transplant Program, Alberta Children's Hospital, 28 Oki Dr NW, Calgary, AB T3B 6A8, Canada; Department of Oncology, Division of Psychosocial Oncology, Cumming School of Medicine, University of Calgary, 3330 Hospital Dr NW, Calgary, AB T2N 4N1, Canada. Electronic address:

This review had three aims: 1) describe the measures used to assess health-related quality of life (HRQL) in pediatric patients diagnosed with sickle cell disease (SCD); 2) document the biopsychosocial factors related to HRQL in pediatric patients diagnosed with SCD; and 3) complete a meta-analysis comparing HRQL in pediatric patients diagnosed with SCD to healthy controls. Included studies were published in English, quantitatively assessed HRQL as a primary aim, in both SCD and controls, and included participants between 0 and 21 years of age. The final review included 66 articles, with a total of 8642 participants with SCD, 4 months-21 years of age, and 62,458 controls, 5-27 years of age. Read More

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Salubrinal induces fetal hemoglobin expression via the stress-signaling pathway in human sickle erythroid progenitors and sickle cell disease mice.

PLoS One 2022 31;17(5):e0261799. Epub 2022 May 31.

Department of Biochemistry and Cancer Biology, Augusta University, Augusta, GA, United States of America.

Sickle cell disease (SCD) is an inherited blood disorder caused by a mutation in the HBB gene leading to hemoglobin S production and polymerization under hypoxia conditions leading to vaso-occlusion, chronic hemolysis, and progressive organ damage. This disease affects ~100,000 people in the United States and millions worldwide. An effective therapy for SCD is fetal hemoglobin (HbF) induction by pharmacologic agents such as hydroxyurea, the only Food and Drug Administration-approved drug for this purpose. Read More

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Mental health impacts of the COVID-19 pandemic on children with underlying health and disability issues, and their families and health care providers.

Paediatr Child Health 2022 Jun 9;27(Suppl 1):S33-S39. Epub 2022 May 9.

Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.

Objectives: The COVID-19 pandemic has impacted mental health at a population level. Families of children with health vulnerabilities have been disproportionately affected by pandemic-related policies and service disruptions as they substantially rely on the health and social care system. We elicited the impact of the COVID-19 pandemic on children with health and disability-related vulnerabilities, their families, and their health care providers (HCPs). Read More

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Does glucose-6-phosphate dehydrogenase deficiency worsen the clinical features of sickle cell disease? A multi-hospital-based cross-sectional study.

Hematology 2022 Dec;27(1):590-595

Department of Internal Medicine, Faculty of Medicine and Pharmacy, University of Kisangani, Kisangani, Democratic Republic of the Congo.

Background: The impact of glucose-6-phosphate dehydrogenase deficiency(G-6-PD) on the clinical course of sickle cell disease(SCD) is still controversial. The objectives of this study were to determine the prevalence of G-6-PD deficiency in patients with SCD and its effect on their clinical course.

Methods: A cross-sectional study of 122 SCD patients and 211 healthy blood donors was conducted in Kisangani city. Read More

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December 2022