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Pediatric Sickle Cell

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https://www.readbyqxmd.com/read/28326208/a-child-as-a-donor-for-hematopoietic-stem-cell-transplantation-bioethical-justification-a-case-study-on-sickle-cell-disease
#1
Andrea Z Pereira, Ricardo Hellman, Nelson Hamerschlak, Andrea Kondo, Polianna Mara Rodrigues de Souza, Wilson Leite Pedreira, Luiz Fernando Alves Lima Mantovani, Eduardo Juan Troster, Henrique Grunspun, Marco Aurélio Scarpinella Bueno
Hematopoietic stem cell transplantation (HSCT) is an important treatment option for children with severe and refractory sickle cell disease (SCD) with debilitating clinical complications. HSCT with cells from the bone marrow of a HLA-identical sibling used in SCD has a low mortality risk, high cure rate, and high event-free survival rate after a median follow-up of 5-6 years. However, matched donors are found in only about 20% of the patients. A boy aged 8 years with SCD had a sister, <2 years old, a fully compatible donor...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28298206/burden-and-spectrum-of-bacterial-infections-among-sickle-cell-disease-children-living-in-cameroon
#2
Anastasie Nicole Alima Yanda, Jobert Richie N Nansseu, Hubert Désiré Mbassi Awa, Sandra A Tatah, Judith Seungue, Charlotte Eposse, Paul Olivier N Koki
BACKGROUND: Although sickle cell disease (SCD) children are highly susceptible to bacterial infections (BIs), there is a dreadful lack of data related to the burden and spectrum of BIs in sub-Saharan Africa (SSA), the highest affected region with SCD. This study aimed to determine the burden and spectrum of BIs among SCD children hospitalized in a pediatric reference hospital in Cameroon, a SSA country. METHODS: We conducted a retrospective analysis of records of children hospitalized from November 2012 to August 2015 in the SCD unit of the Mother and Child Centre of the Chantal Biya Foundation, Cameroon...
March 15, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28261374/the-impact-on-quality-of-life-on-families-of-children-on-an-elimination-diet-for-non-immunoglobulin-e-mediated-gastrointestinal-food-allergies
#3
Rosan Meyer, Heather Godwin, Robert Dziubak, Julie A Panepinto, Ru-Xin M Foong, Mandy Bryon, Adriana Chebar Lozinsky, Kate Reeve, Neil Shah
BACKGROUND: The impact on health related quality of life (HRQL) has been well studied in children with Immunoglobulin E (IgE)-mediated food allergy. However limited data exists on related quality of life (QOL) of families who have a child suffering from food protein induced non-IgE mediated gastrointestinal allergies. We aimed to establish the QOL of families with children at the beginning of following an elimination diet for non-IgE mediated gastrointestinal food allergies. METHODS: A prospective, observational study was performed...
2017: World Allergy Organization Journal
https://www.readbyqxmd.com/read/28238158/acute-kidney-injury-during-a-pediatric-sickle-cell-vaso-occlusive-pain-crisis
#4
Sujatha Baddam, Inmaculada Aban, Lee Hilliard, Thomas Howard, David Askenazi, Jeffrey D Lebensburger
BACKGROUND: Patients who develop sickle cell disease (SCD) nephropathy are at a high risk for mortality. The pathophysiology of vaso-occlusive pain crisis may contribute to acute kidney injury (AKI). Non-steroidal anti-inflammatory drugs, known inducers of AKI, are used to treat pain crises. Multiple gaps exist in the knowledge about the impact of AKI in SCD. METHODS: We conducted a 2-year retrospective review of AKI events in patients admitted for vaso-occlusive crisis...
February 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28229097/pediatric-hypovitaminosis-d-molecular-perspectives-and-clinical-implications
#5
Rafiu Ariganjoye
Vitamin D, a secosteroid, is essential for the development and maintenance of healthy bone in both the adult and pediatric populations. Low level of 25-hydroxy vitamin D (25-(OH)-D) is highly prevalent in children worldwide and has been linked to various adverse health outcomes including rickets, osteomalacia, osteomalacic myopathy, sarcopenia, and weakness, growth retardation, hypocalcemia, seizure and tetany, autism, cardiovascular diseases, diabetes mellitus, cancers (prostate, colon, breast), infectious diseases (viral, tuberculosis), and autoimmune diseases, such as multiple sclerosis and Hashimoto's thyroiditis...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28221266/body-mass-index-and-the-association-with-vaso-occlusive-crises-in-pediatric-sickle-cell-disease
#6
Andrea Zivot, Nataly Apollonsky, Edward Gracely, Deepti Raybagkar
BACKGROUND: Children with sickle cell disease (SCD) historically have been underweight and have poor overall growth. Recent studies have demonstrated a trend toward obesity in pediatric SCD populations. MATERIALS AND METHODS: Through retrospective chart review of patients with SCD followed at our center, we collected patient's data, including body mass index (BMI), weight percentiles, sickle cell genotype, baseline hemoglobin, medical and psychiatric comorbidities, 25-hydroxy vitamin D level, treatment with hydroxyurea, and chronic transfusions...
February 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28192225/a-randomized-trial-of-yoga-for-children-hospitalized-with-sickle-cell-vaso-occlusive-crisis
#7
Karen Moody, Bess Abrahams, Rebecca Baker, Ruth Santizo, Deepa Manwani, Veronica Carullo, Doris Eugenio, Aaron Carroll
CONTEXT: Sickle cell disease (SCD) vaso-occlusive crisis (VOC) remains an important cause of acute pain in pediatrics and the most common SCD complication. Pain management recommendations in SCD include nonpharmacological interventions. Yoga is one nonpharmacological intervention that has been shown to reduce pain in some populations, however, evidence is lacking in children with VOC. OBJECTIVES: The primary objective of this study was to compare the effect of yoga versus an attention control on pain in children with VOC...
February 9, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28185829/a-systematic-review-of-the-literature-for-severity-predictors-in-children-with-sickle-cell-anemia
#8
Emily Riehm Meier, Ross M Fasano, Paul R Levett
All patients with HbSS (SCA) share the same genetic mutation but the clinical phenotype is variable and difficult to predict early in life. A reliable severity predictor would be invaluable toward directing therapeutic decisions in those patients at highest risk of SCA complications. A search of PubMed, Cochrane Clinical Trials Register, and Scopus was performed to determine which SCA severity predictors have been validated in pediatric patients. The full text of 94 of the 590 references identified was reviewed based on the title/abstract...
February 2, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28181950/clinicopathologic-and-molecular-pathology-of-collecting-duct-carcinoma-and-related-renal-cell-carcinomas
#9
REVIEW
An Na Seo, Ghilsuk Yoon, Jae Y Ro
Collecting duct carcinoma (CDC) and related tumors [ie, renal medullary carcinoma (RMC)] are rare types of highly aggressive renal cell carcinomas (RCC) with poor prognosis. Because of the rarity and diagnostic uncertainty of them, their molecular pathology and significance have not yet been fully elucidated. CDC, RMC, fumarate hydratase-deficient RCC (including hereditary leiomyomatosis and RCC-associated RCC HLRCC-RCC), and recently reported anaplastic lymphoma kinase (ALK)-rearrangement RCC have significant morphologic overlaps, but they are separately distinct entities having different molecular pathway and clinical settings...
March 2017: Advances in Anatomic Pathology
https://www.readbyqxmd.com/read/28175446/198%C3%A2-effectiveness-of-surgical-revascularization-for-stroke-prevention-in-pediatric-patients-with-sickle-cell-disease-and-moyamoya-syndrome
#10
Wuyang Yang, Jose Luis Porras, Risheng Xu, Tomas Garzon-Muvdi, Justin M Caplan, Geoffrey P Colby, Alexander Lewis Coon, Rafael J Tamargo, Judy Huang, Edward Sanghoon Ahn
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
https://www.readbyqxmd.com/read/28159432/large-vessel-vasculopathy-in-children-with-sickle-cell-disease-a-magnetic-resonance-imaging-study-of-infarct-topography-and-focal-atrophy
#11
Kristin P Guilliams, Melanie E Fields, Dustin K Ragan, Yasheng Chen, Cihat Eldeniz, Monica L Hulbert, Michael M Binkley, James N Rhodes, Joshua S Shimony, Robert C McKinstry, Katie D Vo, Hongyu An, Jin-Moo Lee, Andria L Ford
BACKGROUND: Large-vessel vasculopathy (LVV) increases stroke risk in pediatric sickle cell disease beyond the baseline elevated stroke risk in this vulnerable population. The mechanisms underlying this added risk and its unique impact on the developing brain are not established. METHODS: We analyzed magnetic resonance imaging and angiography scans of 66 children with sickle cell disease and infarcts by infarct density heatmaps and Jacobian determinants, a metric utilized to delineate focal volume change, to investigate if infarct location, volume, frequency, and cerebral atrophy differed among hemispheres with and without LVV...
December 7, 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/28141731/growth-and-documentation-deficits-where-to-start-in-helping-families
#12
Wilhelmina Hernandez, Deborah A Frank, Samantha Morton, Carmen Palacios, Marilyn Augustyn
Pedro is a 2-year 7-month-old boy who was presented for the first time after a visit to a local emergency room (ER) for diarrhea. At the time of his birth, his undocumented mother, Clara, was 20 years old, uninsured, and with limited English proficiency living approximately 400 miles south of our practice in a different state. Although she had been brought to the United States as a child, she had never finished high school. Pedro was born what mother thought was full-term at his parent's home, where a lay midwife assisted during the delivery...
February 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28125022/pediatric-exercise-testing-value-and-implications-of-peak-oxygen-uptake
#13
REVIEW
Paolo T Pianosi, Robert I Liem, Robert G McMurray, Frank J Cerny, Bareket Falk, Han C G Kemper
Peak oxygen uptake (peak V ˙ O 2 ) measured by clinical exercise testing is the benchmark for aerobic fitness. Aerobic fitness, estimated from maximal treadmill exercise, is a predictor of mortality in adults. Peak V ˙ O 2 was shown to predict longevity in patients aged 7-35 years with cystic fibrosis over 25 years ago. A surge of exercise studies in young adults with congenital heart disease over the past decade has revealed significant prognostic information. Three years ago, the first clinical trial in children with pulmonary arterial hypertension used peak V ˙ O 2 as an endpoint that likewise delivered clinically relevant data...
January 24, 2017: Children
https://www.readbyqxmd.com/read/28100970/sickle-cell-disease-with-cyanotic-congenital-heart-disease-long-term-outcomes-in-5-children
#14
Glen J Iannucci, Olufolake A Adisa, Matthew E Oster, Michael McConnell, William T Mahle
Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction...
December 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28099399/the-association-of-serum-25-hydroxyvitamin-d-with-biomarkers-of-hemolysis-in-pediatric-patients-with-sickle-cell-disease
#15
Samuel A Adegoke, Josefina A P Braga, Adekunle D Adekile, Maria S Figueiredo
Although vitamin D deficiency (VDD) has been linked to anemia among sickle cell disease (SCD), its relationship with hemolysis is unclear. Serum 25-hydroxyvitamin D and biomarkers of hemolysis (hemoglobin [Hb]/hematocrit, reticulocyte percentage, absolute reticulocyte, and lactate dehydrogenase [LDH] levels) in 36 hydroxyurea-naive SCD children were quantified. Correlations were significantly positive with Hb/hematocrit (r=0.40, P=0.017; r=0.45, P=0.006, respectively); inverse with reticulocyte percentage, absolute reticulocyte, and LDH (r=-0...
January 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28099398/utilizing-a-novel-mobile-health-selfie-application-to-improve-compliance-to-iron-chelation-in-pediatric-patients-receiving-chronic-transfusions
#16
Sarah Leonard, Lindsay M Anderson, Jude Jonassaint, Charles Jonassaint, Nirmish Shah
Iron chelation therapy can prevent iron overload for pediatric patients with sickle cell disease and β-thalassemia major; however, adherence is suboptimal. Therefore, we developed an intensive training program (ITP), to improve medication management and disease knowledge. The objectives were to determine feasibility of the ITP and its preliminary impact on adherence, disease knowledge, and health outcomes. Pediatric patients were recruited to participate in the ITP over a 90-day period and were followed for 6 months...
April 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28078488/hydroxyurea-treatment-effect-on-children-with-sickle-cell-disease-and-obstructive-sleep-apnea
#17
Anthony J Grady, Jane S Hankins, Brent Haberman, Robert Schoumacher, Rose Mary Stocks
BACKGROUND: While hydroxyurea is the mainstay of treatment for many of the comorbidities associated with sickle cell disease, its effect on obstructive sleep apnea has not been fully investigated. The purpose of this project is to help characterize the effects of hydroxyurea on obstructive sleep apnea in children with sickle cell disease and determine its therapeutic role in the condition. METHODS: Chart review was conducted on two pediatric patients with sickle cell disease who experienced resolution of obstructive sleep apnea following hydroxyurea administration...
January 11, 2017: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/28065838/current-results-and-future-research-priorities-in-late-effects-after-hematopoietic-stem-cell-transplantation-for-children-with-sickle-cell-disease-and-thalassemia-a-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international
#18
Shalini Shenoy, Emanuele Angelucci, Staci D Arnold, K Scott Baker, Monica Bhatia, Dorine Bresters, Andrew C Dietz, Josu De La Fuente, Christine Duncan, Javid Gaziev, Allison A King, Michael A Pulsipher, Angela R Smith, Mark C Walters
Sustained donor engraftment after allogeneic hematopoietic cell transplantation (HCT) converts to healthy donor hemoglobin synthesis and halts disease symptoms in patients with sickle cell disease and thalassemia major. A disease-free survival probability that exceeds 90% has been reported when HCT using an HLA-matched sibling donor is performed in young patients with low-risk disease or treatment-related risk factors. Alternate donor HCT and HCT in adults is performed infrequently because of a higher risk profile...
January 5, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28008687/hot-off-the-press-which-febrile-children-with-sickle-cell-disease-need-a-chest-x-ray
#19
Justin Morgenstern, Corey Heitz, William K Milne
This retrospective chart review examined the rate of acute chest syndrome (ACS) in febrile children (aged 3 months to 21 years) with sickle cell disease and used recursive partitioning to determine which clinical factors were predictive of a diagnosis of ACS. Over the course of 2 years, 697 children made 1837 visits to one of two pediatric emergency departments. ACS was diagnosed in 185 (10%) of the visits. This article is protected by copyright. All rights reserved.
December 23, 2016: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
https://www.readbyqxmd.com/read/28000344/emergency-department-utilization-by-californians-with-sickle-cell-disease-2005-2014
#20
Susan T Paulukonis, Lisa B Feuchtbaum, Thomas D Coates, Lynne D Neumayr, Marsha J Treadwell, Elliott P Vichinsky, Mary M Hulihan
BACKGROUND: Clinical care for children and adults living with sickle cell disease (SCD) is often provided in the emergency department (ED). Population-based surveillance data can be used to describe the ED utilization patterns of this patient population. PROCEDURE: A cohort of pediatric and adult California patients with SCD was identified from multiple data sources, and 10 years (2005-2014) of their treat-and-release ED utilization data were analyzed. RESULTS: Among a cohort of 4,636 patients with SCD, 4,100 (88%) had one or more treat-and-release ED visits...
December 21, 2016: Pediatric Blood & Cancer
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