3,401 results match your criteria Pediatrics Sickle Cell Disease


Non-S Sickling Hemoglobin Variants: Historical, Genetic, Diagnostic, and Clinical Perspectives.

Oman Med J 2021 May 31;36(3):e261. Epub 2021 May 31.

Department of Pediatrics, Aminu Kano Teaching Hospital, Kano State, Nigeria.

Apart from hemoglobin-S (HbS), there are other Hb variants (non-S sickling Hb variants) that cause sickle cell disease. However, the profiles of these non-S sickling Hb variants have neither been collated nor harmonized. A literature search revealed 14 non-S sickling Hb variants (HbC-Harlem, HbC-Ziguinchor, HbS-Travis, HbS-Antilles, HbS-Providence, HbS-Oman, HbS-Cameroon, HbS-South End, Hb Jamaica Plain, HbC-Ndjamena, HbS-Clichy, HbS-San Martin, HbS-Wake, and HbS-São Paulo). Read More

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Prolonged QTc Interval in Nigerian Children with Sickle Cell Anemia.

Hemoglobin 2021 Jun 10:1-6. Epub 2021 Jun 10.

Department of Paediatrics, University of Calabar Teaching Hospital, Calabar, Nigeria.

Prolonged QTc interval, a risk factor for ventricular arrhythmia, occurs in sickle cell anemia. The aim of this study was to determine the risk of prolonged QTc interval and its relationship with vaso-occlusive painful crises (VOCs) and follow-up steady-state in the same children with sickle cell anemia. This prospective cohort study enrolled 38 subjects, aged 5-17 years. Read More

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SNAP: Supportive noninvasive ventilation for acute chest syndrome prevention in children with sickle cell disease.

Pediatr Blood Cancer 2021 Jun 8:e29136. Epub 2021 Jun 8.

Department of Pediatrics, Boston Medical Center, Boston, Massachusetts, USA.

Background: Acute chest syndrome (ACS) is a leading cause of morbidity and mortality among children with sickle cell disease (SCD). Preventing hypoxemia by optimizing lung aeration during sleep remains a challenge.

Objectives: To explore safety, feasibility, and tolerability of noninvasive, bi-level positive airway pressure ventilation (BiPAP) as preventative, supportive care for hospitalized, medically stable children with SCD on a general pediatric inpatient unit. Read More

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Flow-induced segregation and dynamics of red blood cells in sickle cell disease.

Phys Rev Fluids 2020 May 4;5(5). Epub 2020 May 4.

Department of Chemical and Biological Engineering, University of Wisconsin-Madison, Madison, WI 53706-1691.

Blood flow in sickle cell disease (SCD) can substantially differ from normal blood flow due to significant alterations in the physical properties of the red blood cells (RBCs). Chronic complications, such as inflammation of the endothelial cells lining blood vessel walls, are associated with SCD, for reasons that are unclear. Here, detailed boundary integral simulations are performed to investigate an idealized model flow flow in SCD, a binary suspension of flexible biconcave discoidal fluid-filled capsules and stiff curved prolate capsules that represent healthy and sickle RBCs, respectively, subjected to pressure-driven flow in a planar slit. Read More

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Adenotonsillectomy in high risk patients: Hematologic abnormalities and COVID-19 considerations.

World J Otorhinolaryngol Head Neck Surg 2021 May 29. Epub 2021 May 29.

Department of Otolaryngology-Head and Neck Surgery, University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia 19104, USA.

Adenotonsillectomy is one of the most common surgical procedures performed by otolaryngologists. It is vital that surgeons are aware of the risks in performing this surgery especially during the COVID-19 pandemic and in children with hematologic disorders. In this review we describe common hematologic disorders often noted in pediatric patients undergoing this procedure, as well as proper screening and management of these patients. Read More

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Differences between children with severe acute lower respiratory infection with or without SARS-Cov-2 infection.

J Infect 2021 Jun 3. Epub 2021 Jun 3.

Post-graduate Program in Health Sciences, Federal University of Bahia School of Medicine, Salvador, Brazil; Department of Paediatrics, Federal University of Bahia School of Medicine, Salvador, Brazil.

Aim: To compare clinical features and outcome of children with severe acute lower respiratory infection (ALRI) with or without SARS-CoV-2 infection admitted to Paediatric Intensive Care Unit (PICU).

Methods: For this retrospective cohort study, all children aged<17 years admitted with severe ALRI at a PICU, in Salvador, Brazil were evaluated. Investigation of SARS-CoV-2 infection was performed by real-time reverse-transcription PCR. Read More

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Topical Vapocoolant-Associated Vaso-occlusive Event in a 10-year-old with Sickle Cell Disease.

Pain Manag Nurs 2021 Jun 2. Epub 2021 Jun 2.

Department of Pediatrics, University of Illinois at Chicago, Chicago, Illinois. Electronic address:

Vapocoolant sprays are convenient forms of cold temperature analgesia. These sprays may not be suitable for all patients with particular concern for patients with sickle cell disease. To prevent any further cases from occurring, we propose adding a more specific cautionary statement to the manufacturer guidelines. Read More

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Base editing of haematopoietic stem cells rescues sickle cell disease in mice.

Nature 2021 Jun 2. Epub 2021 Jun 2.

Merkin Institute of Transformative Technologies in Healthcare, Broad Institute of Harvard and MIT, Cambridge, MA, USA.

Sickle cell disease (SCD) is caused by a mutation in the β-globin gene HBB. We used a custom adenine base editor (ABE8e-NRCH) to convert the SCD allele (HBB) into Makassar β-globin (HBB), a non-pathogenic variant. Ex vivo delivery of mRNA encoding the base editor with a targeting guide RNA into haematopoietic stem and progenitor cells (HSPCs) from patients with SCD resulted in 80% conversion of HBB to HBB. Read More

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Trends in Technology Assistance Among Patients With Childhood Onset Chronic Conditions.

Hosp Pediatr 2021 Jun 2. Epub 2021 Jun 2.

Divisions of Hospital Based Medicine and.

Objective: To describe the prevalence, types, and trends over time of medical technology assistance (TA) in patients at the age of transition to adult care with childhood onset chronic conditions (COCCs) cared for at children's hospitals.

Patients And Methods: In this retrospective repeated annual cross-sectional cohort study of the Pediatric Health Information Systems inpatient data, patients with at least 1 hospitalization from January 1, 2008, to December 31, 2018 with a selected COCC were included. The COCCs investigated were brain and spinal cord malformation, cerebral palsy, heart and great vessel malformation, cystic fibrosis, sickle cell anemia, and chronic renal failure. Read More

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Diagnosis of Sickle Cell Disease and HBB Haplotyping in the Era of Personalized Medicine: Role of Next Generation Sequencing.

J Pers Med 2021 May 23;11(6). Epub 2021 May 23.

Department of Pediatrics, Faculty of Medicine, Kuwait University, P.O. Box 24923, Safat 13110, Kuwait.

Hemoglobin genotype and HBB haplotype are established genetic factors that modify the clinical phenotype in sickle cell disease (SCD). Current methods of establishing these two factors are cumbersome and/or prone to errors. The throughput capability of next generation sequencing (NGS) makes it ideal for simultaneous interrogation of the many genes of interest in SCD. Read More

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Safety of 3 Tesla Magnetic Resonance Imaging in Patients with Sickle Cell Disease.

Radiol Res Pract 2021 11;2021:5531775. Epub 2021 May 11.

Department of Radiology, Vanderbilt University Medical Center, Nashville, TN, USA.

Sickle cell disease (SCD) is a well-characterized hemoglobinopathy affecting more than 20 million individuals worldwide and carries an increased risk of cerebral vasculopathy, cerebral infarct, and stroke. As mechanisms of cerebral infarction in SCD are partly attributable to microvascular vaso-occlusive crises, manifesting as altered cerebral blood flow and associated impaired oxygen delivery, magnetic resonance imaging (MRI) methods that can quickly provide a comprehensive perspective on structural and functional disease status, without exogenous contrast administration or ionizing radiation, have emerged as crucial clinical tools for surveillance. However, early MRI work in suspended erythrocytes containing hemoglobin S at 0. Read More

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Clinical Utilities of Electrocardiography in the Diagnosis of Myocardial Ischemia in Children With Sickle Cell Anemia: Correlation With Serum Cardiac Troponin I.

J Pediatr Hematol Oncol 2021 May 31. Epub 2021 May 31.

Department of Pediatrics, Ekiti State University Department of Pediatrics, Ekiti State University Teaching Hospital, Ado-Ekiti Department of Pediatrics and Child Health, Obafemi Awolowo University Departments of Pediatrics Chemical Pathology, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria.

Background: Sickle cell anemia (SCA) is associated with recurrent vaso-occlusive crisis (VOC) and the risk of myocardial ischemia (MI). This study investigated the utility of electrocardiography (ECG) and cardiac troponin I (cTnI) in diagnosing MI during VOC.

Materials And Methods: Children with SCA 5 to 15 years of age in VOC (patients) and age-matched and sex-matched steady-state controls were studied. Read More

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Klotho Level as a Marker of Low Bone Mineral Density in Egyptian Sickle Cell Disease Patients.

J Pediatr Hematol Oncol 2021 May 31. Epub 2021 May 31.

Departments of Pediatrics Clinical Pathology Radiology, Cairo University Ministry of Health, Cairo, Egypt.

Bone involvement of sickle cell disease (SCD) patients varies from acute clinical manifestations of painful vaso-occlusive crises or osteomyelitis to more chronic affection of bone mineral density (BMD) and debilitating osteonecrosis and osteoporosis. Secreted klotho protein is involved in calcium (Ca) reabsorption in the kidney. This study aimed to measure serum klotho levels in children with SCD to determine the possibility of using it as a marker of low BMD in children with SCD in correlation with a dual-energy x-ray absorptiometry scan. Read More

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Transcranial Doppler Ultrasonography Imaging Studies in Children with Sickle Cell Anaemia in a Tertiary Hospital, Abuja, Nigeria.

West Afr J Med 2021 May;38(5):460-464

Department of Paediatrics, National Hospital, Abuja, Nigeria.

Background: Stroke risk in sickle cell anaemia (SCA) can be detected by abnormalities of cerebral blood flow velocities (CBFV), measurable by Transcranial Doppler Imaging (TCDI) ultrasonography. This has become an important strategy in the routine care of children with SCA globally.

Objectives: To determine the pattern of CBFV and document the proportion of children with SCA at risk of stroke at National Hospital Abuja (NHA), Nigeria using TCDI. Read More

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Chlorine inhalation induces acute chest syndrome in humanized sickle cell mouse model and ameliorated by postexposure hemopexin.

Redox Biol 2021 May 17;44:102009. Epub 2021 May 17.

Department of Anesthesiology and Perioperative Medicine, School of Medicine, University of Alabama at Birmingham, AL, USA.

Triggering factors of Acute Chest Syndrome (ACS) is a leading cause of death in patients with Sickle Cell Disease (SCD) and targeted therapies are limited. Chlorine (Cl) inhalation happens frequently, but its role as a potential trigger of ACS has not been determined. In this study, we hypothesized that Cl exposure resembling that in the vicinity of industrial accidents induces acute hemolysis with acute lung injury, reminiscent of ACS in humanized SCD mice. Read More

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Seizures risk factors in sickle cell disease. The cooperative study of sickle cell disease.

Seizure 2021 May 15;89:107-113. Epub 2021 May 15.

Department of Pediatrics, King Hussein Cancer Center (KHCC), Amman, Jordan.

Introduction: Although evidence suggests that neurological complications, including seizures and epilepsy, are more common among sickle cell disease (SCD) patients, few studies have assessed the risk factors of developing seizures among SCD patients METHODS: We used a nested case-control study design to compare pediatric and adult SCD patients who experienced seizures with patients who did not experience any seizure regarding clinical and laboratory parameters. We conducted a secondary analysis using the data from the Cooperative Study of Sickle Cell Disease in this study RESULTS: There were 153 out of 2804 (5.5%) pediatric patients who had seizures with a median age of 8. Read More

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COVID-19 outcomes in a large pediatric hematology-oncology center in Houston, Texas.

Pediatr Hematol Oncol 2021 May 25:1-14. Epub 2021 May 25.

Texas Children's Cancer and Hematology Center and Baylor College of Medicine, Department of Pediatrics, Section of Hematology-Oncology, Houston, Texas, USA.

An understanding of the behavior of SARS-CoV-2 in pediatric hematology-oncology patients is essential to the optimal management of these patients during the COVID-19 pandemic. This study describes the characteristics and outcomes of COVID-19 disease in children with cancer or hematologic disorders treated at a large children's hospital. A retrospective cohort study was conducted at Texas Children's Cancer and Hematology Center from January 1, 2020 to September 30, 2020. Read More

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Psychosocial risk and health care utilization in pediatric sickle cell disease.

Pediatr Blood Cancer 2021 May 24:e29139. Epub 2021 May 24.

Aflac Cancer and Blood Disorders Center, Children's Health care of Atlanta, Atlanta, Georgia, USA.

Introduction: Pain and complications related to pediatric sickle cell disease (SCD) are associated with higher health care utilization. In other pediatric chronic conditions, psychosocial screening can help identify children and families at risk of increased health care utilization to guide resource allocation, address treatment needs, and improve care. This study aimed to investigate the utility of psychosocial screening in predicting increased health care utilization among youth with SCD. Read More

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Rapid and automated quantitation of dense red blood cells: A robust biomarker of hydroxyurea treatment response.

Blood Cells Mol Dis 2021 May 11;90:102576. Epub 2021 May 11.

Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States of America; Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States of America; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States of America. Electronic address:

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Engaging Caregivers and Providers of Children With Sickle Cell Anemia in Shared Decision Making for Hydroxyurea: Protocol for a Multicenter Randomized Controlled Trial.

JMIR Res Protoc 2021 May 21;10(5):e27650. Epub 2021 May 21.

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States.

Background: Sickle cell anemia (SCA) is a genetic blood disorder that puts children at a risk of serious medical complications, early morbidity and mortality, and high health care utilization. Until recently, hydroxyurea was the only disease-modifying treatment for this life-threatening disease and has remained the only option for children younger than 5 years. Evidence-based guidelines recommend using a shared decision-making (SDM) approach for offering hydroxyurea to children with SCA (HbSS or HbS/β0 thalassemia) aged as early as 9 months. Read More

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Decision making for hematopoietic stem cell transplantation in pediatric, adolescent, and young adult patients with a hemoglobinopathy-Shared or not?

Pediatr Blood Cancer 2021 May 18:e29099. Epub 2021 May 18.

Willem-Alexander Children's Hospital, Department of Pediatrics, Leiden University Medical Centre, Leiden, The Netherlands.

Background: Hematopoietic stem cell transplantation (HSCT) offers an established curative option for sickle cell disease (SCD) and thalassemia patients but is associated with significant risks. Decision making is a complex process and shared decision making (SDM) could be a fitting approach in case of such preference-sensitive decisions. This study investigated what level of SDM is used in conversations with hemoglobinopathy patients and/or their caregivers considering HSCT as a curative treatment option. Read More

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Mortality and Causes of Death From Sickle Cell Disease in The Netherlands, 1985-2017.

J Pediatr Hematol Oncol 2021 May 18. Epub 2021 May 18.

Department of Pediatric Hematology, Amalia Children's Hospital, Radboudumc, University Medical Center, Nijmegen Departments of Pediatric Hematology General Pediatrics, Emma Children's Hospital Departments of Clinical Epidemiology, Biostatistics and Bioinformatics Hematology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

In the Netherlands, between 1985 and 2007 secular changes in the health care of patients with sickle cell disease (SCD) have taken place, such as penicillin prophylaxis, vaccination programs and stroke prevention. We investigated the number and causes of death in a cohort of 298 SCD patients, established in 2007, before introduction of neonatal screening, to determine preventable deaths. All patients were diagnosed with SCD before the age of 18 (median age at diagnosis 5. Read More

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Establishing Sickle Cell Disease Stroke Prevention Teams in Africa is Feasible: Program Evaluation Using the RE-AIM Framework.

J Pediatr Hematol Oncol 2021 May 18. Epub 2021 May 18.

Department of Pediatrics, Vanderbilt University Medical Center, Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease Department of Pediatrics, Division of Pediatric Neurology Health Policy, Vanderbilt Institute for Global Health, Vanderbilt University Medical Center Department of Neurology, Vanderbilt University Medical Center, Vanderbilt University, Nashville, TN Brown School of Social Work, Washington University of St. Louis, St. Louis, MO Departments of Pediatrics Radiology Histopathology, Bayero University/Aminu Kano Teaching Hospital Department of Computer Science, Bayero University Departments of Radiology Pediatrics, Murtala Mohammed Specialist Hospital Department of Administration, Aminu Kano Teaching Hospital, Kano Departments of Radiology Hematology and Blood Transfusion Department of Pediatrics, Barau Dikko Teaching Hospital/Kaduna State University, Kaduna, Nigeria.

We used the Reach, Effectiveness, Adoption, Implementation, and Maintenance (RE-AIM) framework to evaluate a Stroke Prevention Team's readiness to prevent strokes in children with sickle cell anemia living in northern Nigeria. The NIH sponsored Stroke Prevention Trial in Nigeria included a goal of a sustainable stroke prevention program. The program's 1-year reach for transcranial Doppler screening was 14. Read More

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Concurrent Assessment of Deformability and Adhesiveness of Sickle Red Blood Cells by Measuring Perfusion of an Adhesive Artificial Microvascular Network.

Front Physiol 2021 28;12:633080. Epub 2021 Apr 28.

Department of Biomedical Engineering, University of Houston, Houston, TX, United States.

Biomarker development is a key clinical research need in sickle cell disease (SCD). Hemorheological parameters are excellent candidates as abnormal red blood cell (RBC) rheology plays a critical role in SCD pathophysiology. Here we describe a microfluidic device capable of evaluating RBC deformability and adhesiveness concurrently, by measuring their effect on perfusion of an artificial microvascular network (AMVN) that combines microchannels small enough to require RBC deformation, and laminin (LN) coating on channel walls to model intravascular adhesion. Read More

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Comparing the Effectiveness of Education Versus Digital Cognitive Behavioral Therapy for Adults With Sickle Cell Disease: Protocol for the Cognitive Behavioral Therapy and Real-time Pain Management Intervention for Sickle Cell via Mobile Applications (CaRISMA) Study.

JMIR Res Protoc 2021 May 14;10(5):e29014. Epub 2021 May 14.

Department of Medicine, University of Pittsburgh, Pittsburgh, PA, United States.

Background: Patients with sickle cell disease (SCD) experience significant medical and psychological stressors that affect their mental health, well-being, and disease outcomes. Digital cognitive behavioral therapy (CBT) has been used in other patient populations and has demonstrated clinical benefits. Although evidence-based, nonpharmacological interventions for pain management are widely used in other populations, these treatments have not been well studied in SCD. Read More

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Clinical Features and Outcome of Sickle Cell Disease in a Tertiary Center in Northern Lebanon: A Retrospective Cohort Study in a Local, Hospital-Associated Registry.

Hemoglobin 2021 May 13:1-7. Epub 2021 May 13.

Novartis Pharma Services Inc., Beirut, Lebanon.

Sickle cell disease affects more than 30 million people worldwide, including 0.1% of the population in Lebanon. It is characterized by unpredictable and painful vaso-occlusive crises (VOCs) that may lead to serious complications. Read More

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