3,056 results match your criteria Pediatrics Sickle Cell Disease


Transient Hypoxia Model Revealed Cerebrovascular Impairment in Anemia Using BOLD MRI and Near-Infrared Spectroscopy.

J Magn Reson Imaging 2020 Jul 9. Epub 2020 Jul 9.

Department of Biomedical Engineering, University of Southern California, Los Angeles, California, USA.

Background: Obstructive sleep apnea and nocturnal oxygen desaturations, which are prevalent in sickle cell disease (SCD) and chronic anemia disorders, have been linked to risks of stroke and silent cerebral infarcts (SCI). Cerebrovascular response to intermittent desaturations has not been well studied and may identify patients at greatest risk.

Purpose: To investigate the cerebral dynamic response to induced desaturation in SCD patients with and without SCI, chronic anemia, and healthy subjects. Read More

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http://dx.doi.org/10.1002/jmri.27210DOI Listing

A National Measurement Framework to Assess and Improve Sickle Cell Care in 4 US Regions.

Public Health Rep 2020 Jul 8:33354920935068. Epub 2020 Jul 8.

50980 National Institute for Children's Health Quality, Boston, MA, USA.

Objectives: Coordinated measurement strategies are needed to inform collaborative approaches to improve access to and quality of care for persons with sickle cell disease (SCD). The objective of our study was to develop a multilevel measurement strategy to assess improvements in access to and quality of care for persons with SCD in 4 US regions.

Methods: From 2014 through 2017, regional grantees in the Sickle Cell Disease Treatment Demonstration Program collected administrative and patient-level electronic health record (EHR) data to assess quality improvement initiatives. Read More

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http://dx.doi.org/10.1177/0033354920935068DOI Listing

The impact of pre-existing HLA and red blood cell antibodies on transfusion support and engraftment in sickle cell disease after nonmyeloablative hematopoietic stem cell transplantation from HLA-matched sibling donors: A prospective, single-center, observational study.

EClinicalMedicine 2020 Jul 28;24:100432. Epub 2020 Jun 28.

Cellular and Molecular Therapeutics Branch, National Institutes of Health, Bethesda, MD, United States.

Background: Hematopoietic stem cell transplantation (HSCT) is curative for patients with sickle cell disease (SCD). Prior to HSCT, patients with SCD commonly receive RBC transfusions with some becoming RBC or HLA alloimmunized. This alloimmunization may impact post-HSCT transfusion requirements and donor engraftment. Read More

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http://dx.doi.org/10.1016/j.eclinm.2020.100432DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7327930PMC

Sickle cell disease in Sri Lanka: clinical and molecular basis and the unanswered questions about disease severity.

Orphanet J Rare Dis 2020 Jul 6;15(1):177. Epub 2020 Jul 6.

Department of Medicine, University of Kelaniya, Ragama, Sri Lanka.

Background: Though case reports and limited case series of Sickle cell disease in Sri Lanka have been reported previously, no attempt has been made hitherto to undertake a comprehensive genotypic-phenotypic analysis of this "rare" group of patients.

Results: All accessible Sickle cell disease patients, totaling 60, including, 51 Sickle β-thalassaemia and 9 homozygous sickle patients were enrolled from seven thalassaemia treatment centres between December 2016-March 2019. The majority of patients were of Sinhalese ethnicity (n = 52, 86. Read More

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http://dx.doi.org/10.1186/s13023-020-01458-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7339547PMC

Use of dual lumen ports for red blood cell exchange: A comparison of adults and children with sickle cell disease.

J Clin Apher 2020 Jul 6. Epub 2020 Jul 6.

Center for Cancer and Blood Disorders, Phoenix Children's Hospital, Phoenix, Arizona, USA.

Purpose: Sickle cell patients receiving chronic RBC exchange require a form of long-term central venous access if peripheral access is inadequate. In adults, dual lumen (DL) ports have been utilized but associated with greater procedure complications and duration when compared to other forms of access. In the pediatric sickle cell population, the use of DL ports for RBC exchange has not been well described. Read More

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http://dx.doi.org/10.1002/jca.21807DOI Listing

Rapid progression to end-stage renal disease in a child with IgA-dominant infection-related glomerulonephritis associated with parvovirus B19.

CEN Case Rep 2020 Jul 3. Epub 2020 Jul 3.

Department of Pediatric Nephrology, School of Medicine, Tokyo Women's Medical University, 8-1, Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Parvovirus B19 (PVB19) has been known to cause acute glomerulonephritis and nephrotic syndrome with various renal histologic patterns, such as endocapillary glomerulonephritis and collapsing glomerulopathy. Remission is achieved spontaneously or by treatment with steroid and/or immunosuppressants in most patients, except those with sickle cell anemia or two APOL1 risk alleles. In this study, we report the case of a previously healthy 5-year-old boy with infection-related glomerulonephritis (IRGN) associated with PVB19 that progressed to end-stage renal disease (ESRD). Read More

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http://dx.doi.org/10.1007/s13730-020-00501-wDOI Listing

Hemoglobin A and fructosamine correlate in a patient with sickle cell disease and diabetes on chronic transfusion therapy.

Pediatr Blood Cancer 2020 Jul 3:e28499. Epub 2020 Jul 3.

University of North Carolina School of Medicine, Chapel Hill, North Carolina.

In patients with sickle cell disease (SCD) and diabetes mellitus (DM), hemoglobin A (HbA ) is unreliable and the American Diabetes Association recommends monitoring long-term glycemia by measuring serum glucose, but use of serum fructosamine (SF), a measurement independent of red cell lifespan, has been reported. SF as a screen for DM in SCD, however, is not standardized and its relationship to serum glucose has not been validated. Further, screening for DM was not adequately addressed in the 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines for SCD management. Read More

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http://dx.doi.org/10.1002/pbc.28499DOI Listing

Hematopoietic stem cell transplantation for people with sickle cell disease.

Cochrane Database Syst Rev 2020 Jul 3;7:CD007001. Epub 2020 Jul 3.

Paediatrics Department, National Hospital, Abuja, Nigeria.

Background: Sickle cell disease is a genetic disorder involving a defect in the red blood cells due to its sickled hemoglobin. The main therapeutic interventions include preventive and supportive measures. Hematopoietic stem cell transplantations are carried out with the aim of replacing the defective cells and their progenitors (hematopoietic (i. Read More

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http://dx.doi.org/10.1002/14651858.CD007001.pub5DOI Listing

The association between timely opioid administration and hospitalization in children with sickle cell disease presenting to the emergency department in acute pain.

Pediatr Blood Cancer 2020 Jul 2:e28268. Epub 2020 Jul 2.

Medical College of Wisconsin, Milwaukee, Wisconsin.

Introduction: The National Heart, Lung, and Blood Institute guidelines for sickle cell disease (SCD) pain crisis management recommend opioids within 60 minutes of emergency department (ED) registration and every 30 minutes thereafter until acute pain is managed. These guidelines are based on expert opinion without published, supporting data.

Objective: To evaluate the association between timely ED opioid administration and hospitalization rates in children with SCD. Read More

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http://dx.doi.org/10.1002/pbc.28268DOI Listing

In Vitro Lymphocyte Functions in Undernourished Children With Sickle Cell Anemia.

Ochsner J 2020 ;20(2):151-160

Department of Pediatrics, Division of Hematology/Oncology, Louisiana State University Health Sciences Center, New Orleans, LA.

Children with sickle cell disease (SCD) often suffer from growth deficits and impaired immunity. However, the association between mild to moderate malnutrition and in vitro lymphocyte function has not been well studied. The goal of this study was to investigate the effects of undernutrition on lymphocyte functions in children with SCD. Read More

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http://dx.doi.org/10.31486/toj.19.0024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7310185PMC
January 2020

Potential Risk Factors Associated With Graft Failure of Haploidentical Hematopoietic Stem Cell Transplantation (HSCT) in Children With Sickle Cell Disease (SCD).

J Pediatr Hematol Oncol 2020 Jun 26. Epub 2020 Jun 26.

Department of Pediatrics, Division of Hematology/Oncology, University of Florida, UF Health Shands Children's Hospital.

Nonmyeloablative (NMA) haploidentical hematopoietic stem cell transplantation for sickle cell disease has significantly increased donor availability for transplant and is increasingly used as curative treatment. The authors describe 3 pediatric patients who rejected grafts after an NMA regimen, previously reported to result in good engraftment rates in the mainly adult population. In this manuscript, potential factors contributing to rejection are described and discussed. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001873DOI Listing

Folic acid supplementation in children with sickle cell disease: study protocol for a double-blind randomized cross-over trial.

Trials 2020 Jun 29;21(1):593. Epub 2020 Jun 29.

Food, Nutrition, and Health, Faculty of Land and Food Systems, The University of British Columbia, 2205 East Mall, Vancouver, British Columbia, V6T 1Z4, Canada.

Background: Sickle cell disease (SCD) is a genetic disorder which causes dysfunctional red blood cells (RBC) and is thought to increase requirements for folate, an essential B vitamin, due to increased RBC production and turnover in the disease. High-dose supplementation with 1-5 mg/d folic acid, synthetic folate, has been the standard recommendation for children with SCD. There is concern about whether children with SCD need such high doses of folic acid, following mandatory folic acid fortification of enriched grains in Canada, and advancements in medical therapies which extend the average lifespan of RBCs. Read More

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http://dx.doi.org/10.1186/s13063-020-04540-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7325072PMC

Epigenetic Reexpression of Hemoglobin F Using Reversible LSD1 Inhibitors: Potential Therapies for Sickle Cell Disease.

ACS Omega 2020 Jun 9;5(24):14750-14758. Epub 2020 Jun 9.

Department of Drug Discovery and Biomedical Sciences, Medical University of South Carolina, 70 President St., Charleston, South Carolina 29414, United States.

Sickle cell disease (SCD) is caused by a single nucleotide polymorphism on chromosome 11 in the β-globin gene. The resulting mutant hemoglobin S (HbS) is a poor oxygen transporter and causes a variety of vascular symptoms and organ failures. At birth, the DRED epigenetic complex forms and silences the γ-globin gene, and fetal hemoglobin (HbF, 2 α-, and 2 γ-subunits) is replaced by adult HbA (αβ) or HbS (αβ) in SCD patients. Read More

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http://dx.doi.org/10.1021/acsomega.0c01585DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7315572PMC

Advances in Sickle Cell Disease Management.

Adv Pediatr 2020 Aug 14;67:57-71. Epub 2020 May 14.

Division of Hematology/Oncology, Department of Pediatrics, University of South Florida Morsani College of Medicine, Tampa, FL, USA. Electronic address:

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http://dx.doi.org/10.1016/j.yapd.2020.03.001DOI Listing

Implementing newborn screening for sickle cell disease as part of immunisation programmes in Nigeria: a feasibility study.

Lancet Haematol 2020 Jul;7(7):e534-e540

Department of Epidemiology and Biostatistics, School of Public Health, Imperial College London, London, UK.

Background: Sickle cell disease is highly prevalent in sub-Saharan Africa, where it accounts for substantial morbidity and mortality. Newborn screening is paramount for early diagnosis and enrolment of affected children into a comprehensive care programme. Up to now, this strategy has been greatly impaired in resource-poor countries, because screening methods are technologically and financially intensive; affordable, reliable, and accurate methods are needed. Read More

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http://dx.doi.org/10.1016/S2352-3026(20)30143-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322555PMC

Haptoglobin Therapeutics and Compartmentalization of Cell-Free Hemoglobin Toxicity.

Trends Mol Med 2020 Jul 21;26(7):683-697. Epub 2020 Mar 21.

Division of Internal Medicine, University Hospital, Zurich, Switzerland. Electronic address:

Hemolysis and accumulation of cell-free hemoglobin (Hb) in the circulation or in confined tissue compartments such as the subarachnoid space is an important driver of disease. Haptoglobin is the Hb binding and clearance protein in human plasma and an efficient antagonist of Hb toxicity resulting from physiological red blood cell turnover. However, endogenous concentrations of haptoglobin are insufficient to provide protection against Hb-driven disease processes in conditions such as sickle cell anemia, sepsis, transfusion reactions, medical-device associated hemolysis, or after a subarachnoid hemorrhage. Read More

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http://dx.doi.org/10.1016/j.molmed.2020.02.004DOI Listing

Cardiovascular Risk and Cardiovascular Health Behaviours in the Transition from Childhood to Adulthood.

Can J Cardiol 2020 Jun 22. Epub 2020 Jun 22.

Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.

The prevention and management of cardiovascular risk factors during the transition from childhood to adulthood is critically important in defining cardiovascular health trajectories. Unfortunately, many young people fall out of clinical care during this important time, leading to worsening cardiovascular risk and missed opportunities to modify future outcomes. The field of healthcare transition has evolved to support young people with complex health needs in developing self-management and self-advocacy skills in order to promote positive health outcomes despite changes in healthcare providers and resources. Read More

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http://dx.doi.org/10.1016/j.cjca.2020.05.041DOI Listing

Hydroxyurea Dose Escalation for Sickle Cell Anemia in Sub-Saharan Africa.

N Engl J Med 2020 06;382(26):2524-2533

From the Ryan White Center for Pediatric Infectious Diseases and Global Health, Department of Pediatrics, Indiana University, Indianapolis (C.C.J.); the Department of Pediatrics and Child Health, Makerere University (R.O.O., H.A.H., C.N., P.K., C.M.N.), Global Health Uganda (R.O.O., C.N.), and Mulago Hospital (P.K.) - all in Kampala, Uganda; the Division of Hematology, Department of Pediatrics (T.S.L., A.L., R.E.W.), and the Global Health Center (R.E.W.), Cincinnati Children's Hospital Medical Center, and the University of Cincinnati College of Medicine (A.L., R.E.W.) - all in Cincinnati; and the Department of Pediatrics, Centre Hospitalier Universitaire Sainte-Justine, University of Montreal, Montreal (H.A.H.).

Background: Hydroxyurea has proven safety, feasibility, and efficacy in children with sickle cell anemia in sub-Saharan Africa, with studies showing a reduced incidence of vaso-occlusive events and reduced mortality. Dosing standards remain undetermined, however, and whether escalation to the maximum tolerated dose confers clinical benefits that outweigh treatment-related toxic effects is unknown.

Methods: In a randomized, double-blind trial, we compared hydroxyurea at a fixed dose (approximately 20 mg per kilogram of body weight per day) with dose escalation (approximately 30 mg per kilogram per day). Read More

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http://dx.doi.org/10.1056/NEJMoa2000146DOI Listing

Thrombin generation in children with sickle cell Anemia is Higher in the presence of platelets.

Transfus Apher Sci 2020 Jun 16:102852. Epub 2020 Jun 16.

University of Texas Southwestern, 5323 Harry Hines Boulevard, Dallas, TX, 75390, USA.

Cellular and plasma interactions underlie hypercoagulability in sickle cell anemia (SCA). In healthy adults, thrombin generation (TG), a biomarker of hypercoagulability, is similar in plasma with and without platelets. Studies investigating TG in SCA using platelet-poor plasma (PPP) show conflicting results. Read More

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http://dx.doi.org/10.1016/j.transci.2020.102852DOI Listing

Emergency Department Provider Survey Regarding Acute Sickle Cell Pain Management.

J Pediatr Hematol Oncol 2020 Jun 19. Epub 2020 Jun 19.

Indiana University School of Medicine.

Sickle cell disease (SCD) can lead to potentially devastating complications that occur secondary to vaso-occlusion. Current national clinical guidelines are largely based on expert opinion, resulting in significant variation of management. Provider awareness regarding emergency department (ED) management of vaso-occlusive crises (VOC) remains unknown. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001843DOI Listing

Socioeconomic status dependent medical complexities in children with sickle cell disease in Saudi Arabia.

Saudi J Biol Sci 2020 Jul 12;27(7):1781-1787. Epub 2020 Mar 12.

Applied Nutrition Group, King Fahd Medical Research Center, King Abdulaziz University, Jeddah, Saudi Arabia.

A look into the associations of socioeconomic status (SES) with prevalence of various complications in sickle cell disease (SCD) is necessary, for an improvement of societal norms, governmental health policies and strategies. We therefore studied the influence of SES indices on certain hematological and clinical parameters in children with SCD in Saudi Arabia. We included 32 female and 33 male patients aged 5-16 years, who were classified based upon their family income. Read More

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http://dx.doi.org/10.1016/j.sjbs.2020.03.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7296505PMC

Label-free hematology analysis using deep-ultraviolet microscopy.

Proc Natl Acad Sci U S A 2020 Jun 19;117(26):14779-14789. Epub 2020 Jun 19.

Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, GA 30332;

Hematological analysis, via a complete blood count (CBC) and microscopy, is critical for screening, diagnosing, and monitoring blood conditions and diseases but requires complex equipment, multiple chemical reagents, laborious system calibration and procedures, and highly trained personnel for operation. Here we introduce a hematological assay based on label-free molecular imaging with deep-ultraviolet microscopy that can provide fast quantitative information of key hematological parameters to facilitate and improve hematological analysis. We demonstrate that this label-free approach yields 1) a quantitative five-part white blood cell differential, 2) quantitative red blood cell and hemoglobin characterization, 3) clear identification of platelets, and 4) detailed subcellular morphology. Read More

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http://dx.doi.org/10.1073/pnas.2001404117DOI Listing

American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain.

Blood Adv 2020 Jun;4(12):2656-2701

Department of Emergency Medicine, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.

Background: The management of acute and chronic pain for individuals living with sickle cell disease (SCD) is a clinical challenge. This reflects the paucity of clinical SCD pain research and limited understanding of the complex biological differences between acute and chronic pain. These issues collectively create barriers to effective, targeted interventions. Read More

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http://dx.doi.org/10.1182/bloodadvances.2020001851DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322963PMC

Association between hospital admissions and healthcare provider communication for individuals with sickle cell disease.

Hematology 2020 Dec;25(1):229-240

Department of Hematology/Oncology, UCSF Benioff Children's Hospital Oakland, Oakland, CA, USA.

To test the hypothesis that caregivers' or adult participants' low ratings of provider communication are associated with more hospital admissions among adults and children with sickle cell disease (SCD), respectively. Secondarily, we determined whether there was an association between the caregivers' or participants' health literacy and rating of providers' communication. Primary data were collected from participants through surveys between 2014 and 2016, across six sickle cell centers throughout the U. Read More

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http://dx.doi.org/10.1080/16078454.2020.1780737DOI Listing
December 2020

Perceptions and Practice of Early Diagnosis of Sickle Cell Disease by Parents and Physicians in a Southwestern State of Nigeria.

ScientificWorldJournal 2020 31;2020:4801087. Epub 2020 May 31.

Department of Community Medicine, Obafemi Awolowo University, Ile-Ife, Osun State, Nigeria.

Background: Early sickle cell disease (SCD) diagnosis has shown promise in combating SCD in many countries. The aim of this study was to assess the practice and perception of early SCD diagnosis among a group of parents and physicians in Nigeria. . Read More

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http://dx.doi.org/10.1155/2020/4801087DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7281802PMC

Knowledge and awareness of individual sickle cell genotype among adolescents in a unity school in Southeast, Nigeria: a cross-sectional study.

Int J Adolesc Med Health 2020 Jun 9. Epub 2020 Jun 9.

Healthy Sunrise Foundation, Las Vegas, Nevada, USA.

Objective Our study sought to assesses the knowledge and awareness of individual sickle cell genotype among adolescents. Methods Participants were recruited from a large school in Southeast Nigeria where adult prevalence of sickle cell trait is 25%. Data was collected through a 50-item survey with previously pretested questions that assessed awareness of individual genotype, general knowledge of sickle cell disease, perception of sickle cell trait and sickle cell anaemia (SCA). Read More

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http://dx.doi.org/10.1515/ijamh-2019-0149DOI Listing

Polymorphisms of the receptor for advanced glycation end products as vasculopathy predictor in sickle cell disease.

Pediatr Res 2020 Jun 16. Epub 2020 Jun 16.

Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Background: The genetic variants of the receptor for advanced glycation end products (RAGE) gene have been associated with vascular disease risk. The objective of this work was to explore the association of three single-nucleotide polymorphisms (SNPs) of RAGE gene (374T/A, 429T/C, and G82S) with vascular complications in SCD.

Methods: The study was conducted on 40 children with SCD and 40 healthy children served as controls. Read More

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http://dx.doi.org/10.1038/s41390-020-1014-3DOI Listing

Paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 mimicking Kawasaki disease (Kawa-COVID-19): a multicentre cohort.

Ann Rheum Dis 2020 Jun 11. Epub 2020 Jun 11.

General Paediatrics, Department of Infectious Disease and Internal Medicine, Reference centre for Rheumatic, AutoImmune and Systemic diseases in children (RAISE), Paris, France, Robert Debré University Hospital, AP-HP, Paris, France

Background: Current data suggest that COVID-19 is less frequent in children, with a milder course. However, over the past weeks, an increase in the number of children presenting to hospitals in the greater Paris region with a phenotype resembling Kawasaki disease (KD) has led to an alert by the French national health authorities.

Methods: Multicentre compilation of patients with KD in Paris region since April 2020, associated with the detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ('Kawa-COVID-19'). Read More

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http://dx.doi.org/10.1136/annrheumdis-2020-217960DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7299653PMC

Comment on "Low educational status of head of household, as a proxy for poverty, is associated with severe anaemia amongst children with sickle cell disease living in a low-resource setting: evidence from the SPRING trial".

Br J Haematol 2020 Jun 11. Epub 2020 Jun 11.

Department of Paediatrics, College of Medicine, Ekiti State University, Ado Ekiti, Ekiti State, Nigeria.

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http://dx.doi.org/10.1111/bjh.16816DOI Listing

Optical coherence tomography (OCT) and OCT angiography allow early identification of sickle cell maculopathy in children and correlate it with systemic risk factors.

Graefes Arch Clin Exp Ophthalmol 2020 Jun 9. Epub 2020 Jun 9.

Department of Medicine- Ophthalmology, University of Udine, Udine, Italy.

Purpose: To determine the presence of sickle cell retinopathy and maculopathy and to identify associations between markers of hemolysis and systemic and ocular manifestations in children affected by sickle cell disease.

Methods: Eighteen children with sickle cell disease, aged 5-16 years, underwent complete eye examination including best-corrected visual acuity, slit-lamp biomicroscopy, ophthalmoscopy after pharmacological mydriasis, spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCTA). Blood test results and clinical history information were collected for each child, including fetal hemoglobin (HbF), hemoglobin (Hb), hematocrit (Htc), mean corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), reticulocytes percentage (%ret), lactic dehydrogenase (LDH), total and direct bilirubin, glomerular filtration rate, number of painful crises, acute chest syndromes, and splenic sequestration. Read More

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http://dx.doi.org/10.1007/s00417-020-04764-yDOI Listing

Severe and fatal forms of COVID-19 in children.

Arch Pediatr 2020 Jul 4;27(5):235-238. Epub 2020 Jun 4.

Pediatric Intensive Care Unit, Necker-Enfants-Malades University Hospital, APHP, Centre-Université de Paris, Paris, France.

Objectives: The aim of this study was to describe severe forms of novel coronavirus disease 2019 in children, including patient characteristics, clinical, laboratory, and imaging findings, as well as the disease management and outcomes.

Methods: This was a retrospective, single-center, observational study conducted in a pediatric intensive and high-dependency care unit (PICU, HDU) in an urban hospital in Paris. All patients, aged from 1 month to 18 years, admitted for confirmed or highly suspected SARS-CoV-2 were included. Read More

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http://dx.doi.org/10.1016/j.arcped.2020.05.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7269941PMC

Automated screening of sickle cells using a smartphone-based microscope and deep learning.

NPJ Digit Med 2020 22;3:76. Epub 2020 May 22.

Electrical and Computer Engineering Department, University of California, Los Angeles, CA 90095 USA.

Sickle cell disease (SCD) is a major public health priority throughout much of the world, affecting millions of people. In many regions, particularly those in resource-limited settings, SCD is not consistently diagnosed. In Africa, where the majority of SCD patients reside, more than 50% of the 0. Read More

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http://dx.doi.org/10.1038/s41746-020-0282-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7244537PMC

Sickle cell disease clinical phenotypes in Nigeria: A preliminary analysis of the Sickle Pan Africa Research Consortium Nigeria database.

Blood Cells Mol Dis 2020 Apr 23;84:102438. Epub 2020 Apr 23.

Centre of Excellence for Sickle Cell Disease Research & Training, University of Abuja (CESRTA), Abuja, Federal Capital Territory, Nigeria. Electronic address:

Background/objective: Sickle cell disease (SCD) is a monogenic disease with multiple phenotypic expressions. Previous studies describing SCD clinical phenotypes in Nigeria were localized, with limited data, hence the need to understand how SCD varies across Nigeria.

Method: The Sickle Pan African Research Consortium (SPARCO) with a hub in Tanzania and collaborative sites in Tanzania, Ghana and Nigeria, is establishing a single patient-consented electronic database with a target of 13,000 SCD patients. Read More

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http://dx.doi.org/10.1016/j.bcmd.2020.102438DOI Listing
April 2020
2.646 Impact Factor

The Heterogeneity of Pediatric Knee Infections: A Retrospective Analysis.

J Pediatr Orthop 2020 Jul;40(6):314-321

Departments of Orthopaedics and Rehabilitation.

Background: Musculoskeletal infection is a major cause of morbidity in the pediatric population. Despite the canonical teaching that an irritable joint and signs of infection likely represent an infected joint space, recent evidence in the pediatric hip has demonstrated that alternative diagnoses are equally or more likely and that combinations of pathologies are common. The knee is the second most commonly infected joint in children, yet there remains a paucity of available data regarding the epidemiology and workup of the infected pediatric knee. Read More

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http://dx.doi.org/10.1097/BPO.0000000000001425DOI Listing

Sexual health of French adolescents with sickle cell disease.

Eur J Contracept Reprod Health Care 2020 Jun 4:1-6. Epub 2020 Jun 4.

Pediatrics Department, Centre Hospitalier Intercommunal de Créteil (CHIC), Créteil, France.

Focussing on sickle cell disease (SCD), the objective of this study was to assess adolescents' sexual heath experience in the context of their chronic illness. We included teenagers from 14 to 19 years old followed for SCD in a hospital located in Créteil (France) from March 2017 to February 2018. Their sexual health experience was assessed by a self-questionnaire with three key themes: contraceptive experience, awareness of sexuality with chronic disease and level of information on the genetic transmission of the disease. Read More

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http://dx.doi.org/10.1080/13625187.2020.1774868DOI Listing

Fusobacterium nucleatum Multiple Liver Abscesses in an Adolescent With Hemoglobin SC Disease.

J Pediatr Hematol Oncol 2020 Jun 2. Epub 2020 Jun 2.

Department of Pediatrics, Division of Pediatric Hematology-Oncology, Charles-Bruneau Cancer Center, CHU Sainte-Justine, Montreal University.

Liver abscesses are poorly known in sickle cell disease. We report here multiple liver abscesses occurring in a 17-year-old patient with hemoglobin SC disease. A Fusobacterium nucleatum was identified on cyst puncture. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001852DOI Listing

Pulmonary disease burden in Hispanic and non-Hispanic children with sickle cell disease.

Pediatr Pulmonol 2020 Jun 2. Epub 2020 Jun 2.

Division of Pediatric Pulmonology, Columbia University Medical Center, Vagelos College of Physicians and Surgeons, New York, New York.

Rationale: Pulmonary complications are the leading cause of morbidity and mortality in sickle cell disease (SCD) patients. Research in SCD has predominantly been conducted on African-Americans, and the disease burden of SCD in other races and ethnicities, including Hispanic patients, is not well characterized.

Objective: To compare pulmonary disease burden between Hispanic and non-Hispanic ethnic groups among children with SCD. Read More

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http://dx.doi.org/10.1002/ppul.24883DOI Listing

Literacy assessment of preimplantation genetic patient education materials exceed national reading levels.

J Assist Reprod Genet 2020 May 29. Epub 2020 May 29.

Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Purpose: In vitro fertilization with preimplantation genetic testing (IVF+PGT-M) reduces the risk of having a child affected by a heritable condition, yet only one-third of eligible patients are aware of this reproductive option. Access to education materials written at appropriate literacy levels could raise patients' awareness, but there is a mismatch between patient reading ability and the literacy demand of most materials. This study aimed to systematically identify written education materials on IVF+PGT-M and evaluate their literacy levels. Read More

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http://dx.doi.org/10.1007/s10815-020-01837-zDOI Listing

Racism and Health-Related Quality of Life in Pediatric Sickle Cell Disease: Roles of Depression and Support.

J Pediatr Psychol 2020 May 29. Epub 2020 May 29.

Department of Psychology, Georgia State University.

Introductions: Sickle cell disease (SCD) is the most commonly inherited blood disorder in the United States, and it predominately affects the Black community. SCD is characterized by a number of symptoms, including unpredictable pain, which can lead to hospitalizations. Data indicate that people with SCD experience racism and ample data indicate that racism predicts depressive symptoms and impaired health-related quality of life (HRQOL). Read More

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http://dx.doi.org/10.1093/jpepsy/jsaa035DOI Listing

Evaluation of the efficacy and safety of deferiprone compared with deferasirox in paediatric patients with transfusion-dependent haemoglobinopathies (DEEP-2): a multicentre, randomised, open-label, non-inferiority, phase 3 trial.

Lancet Haematol 2020 Jun;7(6):e469-e478

Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus, Valenzano, Italy.

Background: Transfusion-dependent haemoglobinopathies require lifelong iron chelation therapy with one of the three iron chelators (deferiprone, deferasirox, or deferoxamine). Deferasirox and deferiprone are the only two oral chelators used in adult patients with transfusion-dependent haemoglobinopathies. To our knowledge, there are no randomised clinical trials comparing deferiprone, a less expensive iron chelator, with deferasirox in paediatric patients. Read More

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http://dx.doi.org/10.1016/S2352-3026(20)30100-9DOI Listing

Assessment of Patient and Caregiver Attitudes and Approaches to Decision-Making Regarding Bone Marrow Transplant for Sickle Cell Disease: A Qualitative Study.

JAMA Netw Open 2020 May 1;3(5):e206742. Epub 2020 May 1.

Department of Pediatrics, Division of Hematology-Oncology-BMT, Emory University School of Medicine, Atlanta, Georgia.

Importance: Bone marrow transplant (BMT) is a potentially curative treatment for sickle cell disease (SCD). Patient and caregiver attitudes toward BMT for SCD and the willingness to accept risks of BMT vary, but these attitudes are not well understood.

Objective: To understand patient and caregiver perceptions of and attitudes toward BMT for SCD and decision-making about BMT. Read More

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http://dx.doi.org/10.1001/jamanetworkopen.2020.6742DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7260617PMC

Perceptions of US Adolescents and Adults With Sickle Cell Disease on Their Quality of Care.

JAMA Netw Open 2020 May 1;3(5):e206016. Epub 2020 May 1.

University of California, San Francisco, Benioff Children's Hospital Oakland, Oakland.

Importance: Sickle cell disease (SCD) is the most common inherited red blood cell disorder in the United States, and previous studies have shown that individuals with SCD are affected by multiple health disparities, including stigmatization, inequities in funding, and worse health outcomes, which may preclude their ability to access quality health care. This needs assessment was performed as part of the Sickle Cell Disease Implementation Consortium (SCDIC) to assess barriers to care that may be faced by individuals with SCD.

Objective: To assess the SCD-related medical care experience of adolescents and adults with SCD. Read More

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http://dx.doi.org/10.1001/jamanetworkopen.2020.6016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7260622PMC

Improving the Quality of Care for Adolescents and Adults With Sickle Cell Disease-It's a Long Road.

JAMA Netw Open 2020 May 1;3(5):e206377. Epub 2020 May 1.

Division of Hematology, Department of Medicine, Duke University School of Medicine, Durham, North Carolina.

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http://dx.doi.org/10.1001/jamanetworkopen.2020.6377DOI Listing

Influenza vaccine effectiveness and disease burden in children and adolescents with sickle cell disease: 2012-2017.

Pediatr Blood Cancer 2020 Aug 29;67(8):e28358. Epub 2020 May 29.

Emory University School of Medicine, Department of Pediatrics, Division of Infectious Diseases, Atlanta, Georgia.

Background: Data are limited on the burden of influenza and seasonal influenza vaccine effectiveness (VE) in children with sickle cell disease (SCD).

Methods: We used a prospectively collected clinical registry of SCD patients 6 months to 21 years of age to determine the influenza cases per 100 patient-years, vaccination rates, and a test-negative case-control study design to estimate influenza VE against medically attended laboratory-confirmed influenza infection. Influenza-positive cases were randomly matched to test-negative controls on age and influenza season in 1:1 ratio. Read More

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http://dx.doi.org/10.1002/pbc.28358DOI Listing

Homozygous sickle cell disease in Central India & Jamaica: A comparison of newborn cohorts.

Indian J Med Res 2020 04;151(4):326-332

Sickle Cell Trust (Jamaica), Kingston, Jamaica.

Background & Objectives: Homozygous sickle cell (SS) disease in Central India runs a more severe clinical course than reports from other areas of India. The current study was undertaken to compare the disease in Central India (Nagpur) with that in Jamaica, both populations defined by newborn screening.

Methods: The Nagpur cohort included infants born to sickling-positive mothers from May 2008 to 2012, examined by high-pressure liquid chromatography and DNA analysis. Read More

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http://dx.doi.org/10.4103/ijmr.IJMR_1946_18DOI Listing

Outcomes of a preoperative risk-based transfusion assignment protocol in sickle cell disease patients: a single-center retrospective study from Saudi Arabia.

Pediatr Hematol Oncol 2020 May 27:1-11. Epub 2020 May 27.

Princess Noorah Oncology Center, King Abdul-Aziz Medical City, Ministry of National Guard - Health Affairs, Jeddah, Saudi Arabia.

Many patients with sickle cell disease (SCD) need surgical management during their lifetime. The best approach for preoperative transfusion in SCD is still to be determined. In this single-center retrospective study, we included HBSS/HBS-Beta-thalassemia patients younger than 16 years of age who underwent surgery between January 2008 and July 2019. Read More

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http://dx.doi.org/10.1080/08880018.2020.1767738DOI Listing