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Pediatric Sickle Cell

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https://www.readbyqxmd.com/read/29785408/stroke-prevalence-in-children-with-sickle-cell-disease-in-sub-saharan-africa-a-systematic-review-and-meta-analysis
#1
Lianna J Marks, Deogratias Munube, Philip Kasirye, Ezekiel Mupere, Zhezhen Jin, Philip LaRussa, Richard Idro, Nancy S Green
Objectives . The prevalence of stroke among children with sickle cell disease (SCD) in sub-Saharan Africa was systematically reviewed. Methods . Comprehensive searches of PubMed, Embase, and Web of Science were performed for articles published between 1980 and 2016 (English or French) reporting stroke prevalence. Using preselected inclusion criteria, titles and abstracts were screened and full-text articles were reviewed. Results . Ten full-text articles met selection criteria. Cross-sectional clinic-based data reported 2...
2018: Global Pediatric Health
https://www.readbyqxmd.com/read/29784054/systematic-literature-review-and-assessment-of-patient-reported-outcome-instruments-in-sickle-cell-disease
#2
Grammati Sarri, Menaka Bhor, Seye Abogunrin, Caroline Farmer, Savita Nandal, Rashid Halloway, Dennis A Revicki
BACKGROUND: Sickle cell disease (SCD) is a chronic condition associated with high mortality and morbidity. It is characterized by acute clinical symptoms such as painful vaso-occlusive crises, which can impair health-related quality of life (HRQL). This study was conducted to identify validated patient-reported outcome (PRO) instruments for use in future trials of potential treatments for SCD. METHODS: A systematic literature review (SLR) was performed using MEDLINE and EMBASE to identify United States (US)-based studies published in English between 1997 and 2017 that reported on validated PRO instruments used in randomized controlled trials and real-world settings...
May 21, 2018: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/29773954/pain-catastrophizing-is-associated-with-poorer-health-related-quality-of-life-in-pediatric-patients-with-sickle-cell-disease
#3
Nitya Bakshi, Ines Lukombo, Inna Belfer, Lakshmanan Krishnamurti
Background: Sickle cell disease (SCD) is an inherited disorder of the red blood cells and is associated with chronic multisystem involvement. While SCD has been associated with poorer health-related quality of life (HRQoL), there is a paucity of data on the relationship of psychological covariates other than anxiety and depression and quality of life (QoL) in children with SCD. Materials and methods: We performed a cross-sectional study of psychological factors, HRQoL, and pain-related outcomes in participants with SCD and race-matched controls as part of a larger study of experimental pain phenotyping...
2018: Journal of Pain Research
https://www.readbyqxmd.com/read/29773497/indications-for-red-cell-transfusions-in-pediatric-patients
#4
REVIEW
Jill M Cholette, Suzie A Noronha, Jerard Seghatchian, Neil Blumberg
Red cell transfusions are amongst the most common therapeutic procedures in seriously ill children, particularly in the inpatient setting. This is despite the fact that there is no evidence base for most clinical settings, with the exception of patients with hemoglobinopathies, particularly thalassemia and sickle cell anemia. Obviously exsanguinating hemorrhage and life threatening anemia are urgent indications for which no other therapeutic approach is currently available. Most transfusions are, however, given prophylactically to prevent the complications of hypoxia or hemodynamic stability, based upon expert opinion and a faith in the oxygen carrying capacity and beneficial hemodynamic properties of transfused red cells...
May 10, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29749702/management-of-severe-chronic-pain-with-methadone-in-pediatric-patients-with-sickle-cell-disease
#5
Zachary LeBlanc, Chris Vance, Jason Payne, Jie Zhang, Lee Hilliard, Jeffrey D Lebensburger, Thomas H Howard
Vasocclusive pain crises are common among pediatric patients with sickle cell disease (SCD). Some patients with repeated pain crises develop chronic pain. We performed a retrospective cohort study of pediatric patients with SCD with chronic pain treated with methadone. We identified a significant reduction in pain hospitalizations following methadone treatment (0.35 ± 0.19 vs. 0.19 ± 0.17 hospitalizations/month, P = 0.016). In addition, we did not observe overt organ toxicity nor symptoms of opioid withdrawal during methadone wean...
May 11, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29730055/funduscopic-examination-and-sd-oct-in-detecting-sickle-cell-retinopathy-among-pediatric-patients
#6
Jing Jin, Robin Miller, Jonathan Salvin, Sharon Lehman, Dorothy Hendricks, Amanda Friess, Lauren Libfraind
PURPOSE: To compare the results of fundus examination and spectral domain optic coherence tomography (SD-OCT) in detecting retinal changes in pediatric patients with sickle cell disease at a single center. METHODS: In this prospective study, conducted over a period of 19 months, consecutive African American patients with sickle cell disease underwent complete ophthalmologic examination, and SD-OCT images of the maculas of both eyes were obtained. RESULTS: A total of 69 (37 males) patients aged 5-20 years (mean 12...
May 3, 2018: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/29724144/arrested-pneumatization-of-the-sphenoid-sinus-mimicking-skull-base-tumors-mri-prevalence-in-children-with-haematologic-diseases
#7
Taner Arpaci
OBJECTIVE: Arrested pneumatization of the sphenoid sinus (APSS) is a developmental anatomic variation but may be confused with serious diseases of the skull base. The purpose of this study was to investigate the prevalence of APSS in pediatric patients with haemotologic diseases like sickle cell anemia (SCA), thalassemia and leukemia. MATERIALS AND METHODS: One hundred and eight pediatric patients (43 girl, 65 boy; age range, 4-18 years; median age, 13 years) with haemotologic diseases who underwent at least one magnetic resonance imaging (MRI) of the head and neck between 2010-2017 in a single instution were included...
May 3, 2018: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29712666/randomized-phase-2-trial-of-monthly-vitamin-d-to-prevent-respiratory-complications-in-children-with-sickle-cell-disease
#8
Margaret T Lee, Meyer Kattan, Ilene Fennoy, Stephen M Arpadi, Rachel L Miller, Serge Cremers, Donald J McMahon, Jeri W Nieves, Gary M Brittenham
In sickle cell disease, respiratory infection and asthma may lead to respiratory complications that are a leading cause of morbidity and mortality. Vitamin D has anti-infective and immunomodulatory effects that may decrease the risk for respiratory infections, asthma, and acute chest syndrome. We conducted a randomized double-blind active-controlled clinical trial to determine whether monthly oral vitamin D3 can reduce the rate of respiratory events in children with sickle cell disease. Seventy sickle cell subjects, ages 3-20 years, with baseline records of respiratory events over 1 year before randomization, underwent screening...
May 8, 2018: Blood Advances
https://www.readbyqxmd.com/read/29687892/metabolomics-evaluation-of-early-storage-red-blood-cell-rejuvenation-at-4%C3%A2-c-and-37%C3%A2-c
#9
Sarah Gehrke, Amudan J Srinivasan, Rachel Culp-Hill, Julie A Reisz, Andrea Ansari, Alan Gray, Matthew Landrigan, Ian Welsby, Angelo D'Alessandro
BACKGROUND: Refrigerated red blood cell (RBC) storage results in the progressive accumulation of biochemical and morphological alterations collectively referred to as the storage lesion. Storage-induced metabolic alterations can be in part reversed by rejuvenation practices. However, rejuvenation requires an incubation step of RBCs for 1 hour at 37°C, limiting the practicality of providing "on-demand," rejuvenated RBCs. We tested the hypothesis that the addition of rejuvenation solution early in storage as an adjunct additive solution would prevent-in a time window consistent with the average age of units transfused to sickle cell recipients at Duke (15 days)-many of the adverse biochemical changes that can be reversed via standard rejuvenation, while obviating the incubation step...
April 24, 2018: Transfusion
https://www.readbyqxmd.com/read/29667721/diverse-manifestations-of-acute-sickle-cell-hepatopathy-in-pediatric-patients-with-sickle-cell-disease-a-case-series
#10
Lydia H Pecker, Nidhi Patel, Susan Creary, Anil Darbari, Emily Riehm Meier, Deepika S Darbari, Ross M Fasano
The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis...
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29661609/assessing-the-effect-of-an-educational-intervention-on-nurses-and-patient-care-assistants-comprehension-and-documentation-of-functional-ability-in-pediatric-patients-with-sickle-cell-disease
#11
Katherine M Bernier, Megan Strobel, Ruth Lucas
PURPOSE: In 2014, the Youth Acute Pain Functional Ability Questionnaire (YAPFAQ) was developed to investigate patient's self-rated functional ability during times of acute pain in the inpatient clinical setting. Although it has great potential, the application of this tool has not been made a standard of care. The purpose of this multiple methods study was to determine if, through an educational intervention, hospital staff could consistently document the YAPFAQ in children with sickle cell disease (SCD) during a vaso-occlusive episode...
April 13, 2018: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/29659914/cognitive-abilities-moderate-the-effect-of-disease-severity-on-health-related-quality-of-life-in-pediatric-sickle-cell-disease
#12
Steven J Hardy, Sarah E Bills, Shane M Wise, Kristina K Hardy
Objective: Complications that can arise from sickle cell disease (SCD) have the potential to negatively affect health-related quality of life (HRQL). SCD manifests in varying degrees of severity, but effects on HRQL are not uniform. Cognitive abilities influence HRQL in other pediatric groups, potentially through variability in treatment adherence and psychological coping. This study examined the effect of SCD severity on HRQL and explored cognitive abilities as a moderator of this relationship...
April 6, 2018: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/29653206/late-effects-screening-guidelines-after-hematopoietic-cell-transplantation-hct-for-hemoglobinopathy-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international-conference-on-late-effects-after-pediatric-hct
#13
Shalini Shenoy, Javid Gaziev, Emanuele Angelucci, Allison King, Monica Bhatia, Angela Smith, Dorine Bresters, Anne E Haight, Christine N Duncan, Josu de la Fuente, Andrew C Dietz, K Scott Baker, Michael A Pulsipher, Mark C Walters
Allogeneic hematopoietic cell transplantation (HCT) can halt organ damage and eliminate symptoms in hemoglobin disorders, including sickle cell disease (SCD) and thalassemia major (TM). Managing the residual manifestations of pre-HCT disease complications and the long-term effects of HCT requires systematic monitoring, follow-up, and intervention when indicated. Late complications vary with age and disease status at HCT, and with transplant variables such as preparative regimen, donor source and compatibility, and immune reconstitution...
April 10, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29577801/early-identification-of-barriers-and-facilitators-to-self-management-behaviors-in-pediatric-patients-with-sickle-cell-disease-to-minimize-hematopoietic-cell-transplantation-complications
#14
Ginny L Schulz, Rebecca H Foster, Valerie Kennedy Lang, Alison Towerman, Shalini Shenoy, Brea-Anne Lauer, Elizabeth Molzon, Megan Holtmann
Hematopoietic cell transplantation (HCT) is an elective, curative treatment option for patients with sickle cell disease (SCD). Transplant requires extensive self-management behaviors to be successful. The purpose of this study was to describe potential barriers and facilitators to self-management in a group of pediatric patients with SCD prior to HCT and their medical outcomes post-HCT. A multiple case study approach was used to describe 4 pairs of transplant recipients grouped by age, donor type, and donor source...
March 1, 2018: Journal of Pediatric Oncology Nursing: Official Journal of the Association of Pediatric Oncology Nurses
https://www.readbyqxmd.com/read/29555644/biological-impact-of-%C3%AE-genes-%C3%AE-haplotypes-and-g6pd-activity-in-sickle-cell-anemia-at-baseline-and-with-hydroxyurea
#15
Françoise Bernaudin, Cécile Arnaud, Annie Kamdem, Isabelle Hau, Françoise Lelong, Ralph Epaud, Corinne Pondarré, Serge Pissard
Sickle cell anemia (SCA), albeit monogenic, has heterogeneous phenotypic expression, mainly related to the level of hemoglobin F (HbF). No large cohort studies have ever compared biological parameters in patients with major β-globin haplotypes; ie, Senegal (SEN), Benin (BEN), and Bantu/Central African Republic (CAR). The aim of this study was to evaluate the biological impact of α genes, β haplotypes, and glucose-6-phosphate dehydrogenase (G6PD) activity at baseline and with hydroxyurea (HU). Homozygous HbS patients from the Créteil pediatric cohort with available α-gene and β-haplotype data were included (n = 580; 301 females and 279 males) in this retrospective study...
March 27, 2018: Blood Advances
https://www.readbyqxmd.com/read/29550053/early-initiation-of-inhaled-corticosteroids-does-not-decrease-acute-chest-syndrome-morbidity-in-pediatric-patients-with-sickle-cell-disease
#16
Alexis Leonard, Nihal Godiwala, Nicole Herrera, Robert McCarter, Matthew Sharron, Emily Riehm Meier
Acute chest syndrome (ACS) is a leading cause of mortality in patients with sickle cell disease (SCD). Systemic corticosteroids decrease ACS severity, but the risk of readmission for vaso-occlusive crises (VOC) has limited their use. The efficacy of inhaled corticosteroids (ICS) as a safer alternative is currently unknown. An observational, historic cohort study compared patients with SCD with ACS who received ICS at admission (ICS) to those who did not (non-ICS). Outcome measures included rates of transfusion, oxygen requirement, BiPAP initiation, PICU transfer, intubation, readmission, hospital cost, and length of stay...
July 2018: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29532199/temporal-relationship-between-daily-pain-and-actigraphy-sleep-patterns-in-pediatric-sickle-cell-disease
#17
Karin Fisher, Andrea M Laikin, Katianne M Howard Sharp, Catherine A Criddle, Tonya M Palermo, Cynthia W Karlson
Limited research is available on the relationship between objective sleep patterns and pain in children with SCD. Research in other chronic pain populations suggests that the effect of sleep disruption on pain may be stronger than the effect of pain on sleep that night. To examine the bi-directional relationship between objective sleep patterns and daily pain in a pediatric SCD sample. Participants were 30 African American children with SCD 8-18 years (13 ± 2.8 years; 66.7% female) with frequent pain...
June 2018: Journal of Behavioral Medicine
https://www.readbyqxmd.com/read/29530458/-from-pediatric-care-to-adult-medicine-transition-of-sickle-cell-patients-a-french-monocentric-study
#18
M Colinart-Thomas, V Noël, G Roques, S Gordes-Grosjean, M Abely, C Pluchart
Sickle cell disease, a hemoglobin disorder with autosomal recessive transmission, is one of the most common genetic diseases screened in France. Thanks to early management, 95% of sickle cell patients reach adulthood and require transition from pediatric care to adult care. Through a retrospective study of records from serious sickle cell patients over 17 years old, followed in the hematology-oncology pediatric unit of Reims University Hospital Center in France, we analyzed transition conditions, compared pediatric and adult management, and proposed a plan for transition care...
April 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29520844/children-with-sickle-cell-anemia-with-normal-transcranial-doppler-ultrasounds-and-without-silent-infarcts-have-a-low-incidence-of-new-strokes
#19
Lori C Jordan, Dionna O Roberts Williams, Mark J Rodeghier, Brittany V Covert Greene, Maria R Ponisio, James F Casella, Robert C McKinstry, Michael J Noetzel, Fenella J Kirkham, Emily R Meier, Beng Fuh, Melissa McNaull, Sharada Sarnaik, Suvankar Majumdar, Timothy L McCavit, Michael R DeBaun
In a prospective cohort study, we tested the hypothesis that children with sickle cell anemia (SCA) with normal transcranial Doppler ultrasound (TCD) velocities and without silent cerebral infarcts (SCIs) would have a lower incidence rate of new neurological events (strokes, seizures or transient ischemic attacks) compared to children with normal TCD measurements and SCIs, not receiving regular blood transfusions. Nonrandomized participants from the silent cerebral infarct transfusion (SIT) Trial who had screening magnetic resonance imaging (MRI) of the brain and normal TCD measurements were included...
March 9, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29512881/parent-pain-catastrophizing-predicts-child-depressive-symptoms-in-youth-with-sickle-cell-disease
#20
Alana Goldstein-Leever, Lindsey L Cohen, Carlton Dampier, Soumitri Sil
BACKGROUND: Youth with sickle cell disease (SCD) are at risk for recurrent pain and depressive symptoms, both of which contribute to poorer health outcomes. Furthermore, youth and family coping with child pain, including pain catastrophizing, is known to be associated with poorer psychosocial adjustment and greater functional disability among youth with SCD. In particular, child catastrophizing about pain and parent catastrophizing about their child's pain have been linked to increased pain and depressive symptoms in youth with chronic pain conditions...
March 7, 2018: Pediatric Blood & Cancer
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