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Pediatric Sickle Cell

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https://www.readbyqxmd.com/read/28538501/invasive-pneumococcal-disease-in-patients-with-sickle-cell-disease
#1
Pournima Navalkele, Bülent Özgönenel, Eric McGrath, Paul Lephart, Sharada Sarnaik
BACKGROUND: Patients with sickle cell disease (SCD) are at risk of fatal sepsis with encapsulated bacteria, such as Streptococcus pneumoniae, because of the inherent autosplenectomy that occurs in SCD. This risk is thwarted with oral penicillin prophylaxis during the first 5 years of life, and with stringent vaccination against S. pneumoniae alongside routine childhood immunization. But compared with the general African American pediatric population, the rate of invasive pneumococcal disease (IPD) in patients with SCD still remains high, resulting in hospitalization and fatality...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28500682/red-blood-cell-transfusions-during-sickle-cell-anemia-vaso-occlusive-crises-a-report-from-the-magnesium-in-crisis-magic-study
#2
Monica L Hulbert, Julie A Panepinto, J Paul Scott, Robert I Liem, Lawrence J Cook, Timothy Simmons, David C Brousseau
BACKGROUND: Little is known about red blood cell (RBC) transfusion practices for children hospitalized for a sickle cell vaso-occlusive pain crisis (VOC). We hypothesized that transfusion would be associated with the development of acute chest syndrome (ACS), lower hemoglobin (Hb) concentration, and lack of hydroxyurea therapy. STUDY DESIGN AND METHODS: This is a secondary analysis of all children admitted for a sickle cell pain crisis enrolled in the Magnesium in Crisis (MAGiC) randomized trial; all had HbSS or S-β(0) thalassemia...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28490230/acceptability-of-in-utero-hematopoietic-cell-transplantation-for-sickle-cell-disease
#3
Ryan M Antiel, Scott D Halpern, Evelyn M Stevens, Jesse D Vrecenak, Chavis A Patterson, Trudy Tchume-Johnson, Kim Smith-Whitley, William H Peranteau, Alan W Flake, Lamia P Barakat
BACKGROUND: In utero hematopoietic cell transplantation (IUHCT) has curative potential for sickle cell disease (SCD) but carries a risk of fetal demise. METHODS: We assessed the conditions under which parents of children with SCD and young adults with SCD would consider IUHCT in a future pregnancy, given a 5% fixed risk of fetal demise. Participants were randomized to consider a hypothetical cure rate (20%, 40%, or 70%). Subsequently, cure rate was either increased or decreased depending on the previous answer to reveal the lowest acceptable rate...
May 1, 2017: Medical Decision Making: An International Journal of the Society for Medical Decision Making
https://www.readbyqxmd.com/read/28488469/extramedullary-hematopoiesis-presenting-as-bilateral-glomus-tympanicum-first-case-report-in-a-pediatric-patient-and-with-bilateral-presentation
#4
Leonard N Chen, Alexandra Espinel, Brian Reilly, Dragos C Luca
To the best of our knowledge, this is the first case report of middle ear extramedullary hematopoiesis (EMH) in a pediatric patient as well as the first bilateral presentation reported in both children and adults. We report a 13-year-old African-American female with sickle cell disease who developed bilateral hearing loss, with magnetic resonance imaging findings consistent with bilateral glomus tympanicum (GT). Upon excisional biopsy, however, EMH was diagnosed histologically. Besides its novelty, this case highlights the importance of considering EMH in the differential diagnosis of GT including cases with bilateral presentation that may be otherwise highly suggestive of the familial form of GT...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28481580/neurocognitive-deficits-in-children-with-chronic-health-conditions
#5
Bruce E Compas, Sarah S Jaser, Kristen Reeslund, Niral Patel, Janet Yarboi
Over 4 million children in the United States suffer from chronic health conditions, including cancer, sickle cell disease, and diabetes. Because of major advances in the early identification and treatment of these conditions, survival rates for these children continue to rise, and the majority now lives into adulthood. However, increases in survival have come with costs related to long-term effects of disease processes and treatments. Foremost among these consequences is impairment in brain development and neurocognitive function that may affect a substantial portion of children with chronic health conditions and follow many into adulthood...
May 2017: American Psychologist
https://www.readbyqxmd.com/read/28475298/a-modified-noninvasive-screening-protocol-for-pulmonary-hypertension-in-children-with-sickle-cell-disease-who-should-be-sent-for-invasive-evaluation
#6
Christian Lilje, Jordan Harry, Kelly K Gajewski, Renee V Gardner
BACKGROUND: Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) tend to be overestimated if based exclusively on Doppler-derived tricuspid regurgitant velocity (TRV) as surrogate noninvasive marker with a cutoff ≥2.5 m/s. OBJECTIVES: We aimed to better define a subgroup of pediatric SCD patients who should be sent for invasive evaluation of pulmonary artery pressure (PAP) based on a modified echocardiographic PH screening protocol that implements evidence from Doppler-catheter comparative studies...
May 5, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28450986/-major-sickle-cell-syndromes-and-infections-associated-with-this-condition-in-children-in-burkina-faso
#7
Sonia Douamba, Kisito Nagalo, Laure Tamini, Ismaël Traoré, Madibèlè Kam, Fla Kouéta, Diarra Yé
INTRODUCTION: This study aims to investigate infections in children with major sickle cell syndrome. METHODS: We conducted a monocentric descriptive retrospective hospital study in Ouagadougou, Burkina Faso, over a ten-year period. All children with major sickle cell syndrome (homozygous SS and double heterozygous SC, SDPunjab, Sβ thalassemic, SOArab and SE) hospitalized for microbiologically confirmed infections were enrolled in the study. RESULTS: One hundred and thirty-three patients met our inclusion criteria...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28444215/behavioral-and-pharmacological-adherence-in-pediatric-sickle-cell-disease-parent-child-agreement-and-family-factors-associated-with-adherence
#8
Page H Klitzman, Julia K Carmody, Mary H Belkin, David M Janicke
This study aimed to evaluate agreement between children and parents on a measure of behavioral and pharmacological adherence in children with sickle cell disease (SCD), and the associations among family factors (i.e., problem-solving skills, routines, communication) and adherence behaviors. In all, 85 children (aged 8-18 years) with SCD and their parents completed questionnaires assessing individual and family factors. Overall parent-child agreement on an adherence measure was poor, particularly for boys and older children...
April 21, 2017: Journal of Pediatric Psychology
https://www.readbyqxmd.com/read/28424766/the-need-for-hematology-nurse-education-in-low-and-middle-income-countries-a-community-case-study-in-tanzania
#9
Julie M Buser
Hematology-related diseases, such as anemia, malaria, sickle cell disease (SCD), and blood cancers, have differing rates of survival between high-income and low- and middle-income countries (LMICs). Nurses in LMICs have an unmet need for specialty training and education to address hematology and hemato-oncology disorders. A gap in the literature exists about hematology nurse education and clinical service demands in LMICs. This community case study documents a collaborative hematology and basic hemato-oncology education program to sustainably strengthen nurse capacity at a national referral hospital and university in Tanzania...
2017: Frontiers in Public Health
https://www.readbyqxmd.com/read/28417347/-serological-markers-of-viral-hepatitis-b-and-c-in-children-with-sickle-cell-disease-monitored-in-the-pediatrics-department-at-the-university-hospital-of-bobo-dioulasso-burkina-faso
#10
S A Kissou, M Koura, A Sawadogo, A-S Ouédraogo, H Traoré, E Kamboulé, W W F Zogona, B Nacro
Viral hepatitis B and C are universal public health problems. Burkina Faso is a high endemic area for hepatitis B. Patients with sickle cell disease are at risk. The aim of this study was to investigate and quantify the serological markers of viral hepatitis B and C among pediatric patients with sickle cell disease. This was a descriptive cross-sectional study, which took place from July to November 2014 at the Department of Pediatrics, University Hospital of Bobo- Dioulasso. The study included 44 SS and 26 SC sickle cell patients...
April 17, 2017: Bulletin de la Société de Pathologie Exotique
https://www.readbyqxmd.com/read/28409222/incidental-splenic-nodules-found-on-mr-imaging-done-for-assessment-of-iron-overload-in-children
#11
Rayan A Ahyad, Christopher Z Lam, Omid Shearkhani, Oscar M Navarro
BACKGROUND: MR imaging is used to assess iron overload in patients with hemoglobinopathies and in those who have undergone multiple blood transfusions. Sometimes splenic nodules are found incidentally on these examinations and this may cause diagnostic uncertainty. OBJECTIVE: To determine the prevalence, imaging characteristics and evolution of splenic nodules found on MR imaging for iron overload evaluation. MATERIALS AND METHODS: Retrospective review of all MR imaging examinations performed for iron overload assessment from 2005 to 2015 in a tertiary pediatric hospital...
April 13, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28394851/development-and-validation-of-the-self-reported-promis-pediatric-pain-behavior-item-bank-and-short-form-scale
#12
Natoshia R Cunningham, Susmita Kashikar-Zuck, Constance Mara, Kenneth R Goldschneider, Dennis A Revicki, Carlton Dampier, David D Sherry, Lori Crosby, Adam Carle, Karon F Cook, Esi M Morgan
Pain behaviors are important indicators of functioning in chronic pain; however, no self-reported pain behavior instrument has been developed for pediatric populations. The purpose of this study was to create a brief pediatric measure of patient-reported pain behaviors as part of the Patient-Reported Outcome Measurement Information System (PROMIS). A pool of 47 candidate items for this measure had been previously developed through qualitative research. In this study, youth with chronic pain associated with juvenile fibromyalgia, juvenile idiopathic arthritis, or sickle cell disease (ages 8-18 years) from 3 pediatric centers completed all 47 candidate items for development of the pain behavior item bank along with established measures of pain interference, depressive symptoms, fatigue, average pain intensity, and pain catastrophizing...
April 5, 2017: Pain
https://www.readbyqxmd.com/read/28382567/evaluating-risk-factors-for-chronic-kidney-disease-in-pediatric-patients-with-sickle-cell-anemia
#13
Jeffrey D Lebensburger, Gary R Cutter, Thomas H Howard, Paul Muntner, Daniel I Feig
BACKGROUND: Patients with sickle cell anemia (SCA) have an increased prevalence of nephropathy and mortality from chronic kidney disease (CKD). METHODS: We evaluated the association of hyperuricemia and nocturnal hypertension with lower estimated glomerular filtration rate (eGFR) using cystatin-C in patients aged 10-21 years with the HbSS or HbSB0 form of the disease during a non-acute clinic visit. eGFR and uric acid measurements were obtained in 83 and 81 participants, respectively, and 24-h ambulatory blood pressure monitoring (ABPM) was performed in 44 participants...
April 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28353009/acute-kidney-injury-in-children-with-sickle-cell-disease-compounding-a-chronic-problem
#14
EDITORIAL
Cherry Mammen, Mei Lin Bissonnette, Douglas G Matsell
In an article recently published in Pediatric Nephrology, Baddam and colleagues discuss the relatively underreported clinical problem of repeated episodes of acute kidney injury (AKI) in children with sickle cell disease (SCD). Their report is a cautionary note about the importance of repeated kidney injury on the background of underlying chronic kidney injury and its potential implications on long-term kidney outcome. In children and adults with SCD, this includes the effects of repeated vaso-occlusive crises and the management of these painful episodes with non-steroidal anti-inflammatory drugs...
March 28, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28341428/renal-papillary-necrosis-in-patients-with-sickle-cell-disease-how-to-recognize-this-forgotten-diagnosis
#15
REVIEW
Michaël M E L Henderickx, Tim Brits, Karen De Baets, Mattias Seghers, Philip Maes, Dominique Trouet, Stefan De Wachter, Gunter De Win
INTRODUCTION: Renal papillary necrosis is not commonly seen in daily practice, but can have severe consequences when it is not diagnosed in time. It is known to be associated with sickle cell hemoglobinopathies; however a wide range of etiologies are possible, and it is therefore not the first diagnosis clinicians consider in patients with sickle cell disease who present with hematuria. METHODS: A literature search was performed to summarize the current knowledge about renal papillary necrosis associated with sickle cell disease...
March 7, 2017: Journal of Pediatric Urology
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
#16
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
#17
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
#18
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
#19
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
#20
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
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