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Pediatric Sickle Cell

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https://www.readbyqxmd.com/read/28944997/desire-for-parenthood-and-reproductive-health-knowledge-in-adolescents-and-young-adults-with-sickle-cell-disease-and-their-caregivers
#1
Leena Nahata, Nicole M Caltabellotta, Kyle Ball, Sarah H O'Brien, Susan E Creary
BACKGROUND/OBJECTIVE: Sickle cell disease (SCD) and hydroxyurea have implications for fertility and reproductive health. The goal of this study was to examine desire for parenthood and reproductive health knowledge among a cohort of adolescent and young adult (AYA) with SCD receiving hydroxyurea and their caregivers at a large pediatric academic center. METHODS: Patients with SCD were approached from September 2016 to July 2017 if they were: (1) 12-20 years old, (2) prescribed hydroxyurea for at least 6 months, (3) proficient in English, and (4) accompanied by a caregiver who was proficient in English and willing to participate...
September 25, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28933357/risk-of-asymptomatic-bacteriuria-among-people-with-sickle-cell-disease-in-accra-ghana
#2
Eric S Donkor, Jonathan A Osei, Isaac Anim-Baidoo, Samuel Darkwah
Asymptomatic bacteriuria (ASB) is benign except in certain medical conditions such as pregnancy and immunosuppression. In Ghana, there are hardly any studies on urinary infections among sickle cell disease (SCD) patients, and the few studies carried out in Africa focused on pediatric SCD populations. The current study aimed to investigate the risk of ASB among SCD patients at a tertiary hospital in Ghana. This was a cross-sectional study involving 110 SCD patients and 110 age and sex matched healthy controls...
February 15, 2017: Diseases (Basel)
https://www.readbyqxmd.com/read/28911832/association-of-silent-infarcts-in-sickle-cell-anemia-with-decreased-annexin-a5-resistance
#3
Kerry A Morrone, Lydia H Pecker, Jacob Rand, Jennifer Davila, Suzette Oyeku, Jane A Little, Xue Xiaonan, Deepa Manwani
BACKGROUND: Sickle cell anemia (SCA) is characterized by abnormally shaped, adhesive RBCs that interact with white blood cells and the endothelium, leading to chronic hemolysis, vasculopathy and a prothrombotic state. About 10% of subjects with a thrombotic event in the general population will have an associated antiphospholipid (aPL) antibody. One proposed mechanism for the thrombophilic nature of aPL antibodies is the disruption of the potent anticoagulant annexin A5 or Annexin A5 resistance (A5R)...
September 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28904678/-epidemiological-profile-of-hemoglobinopathies-a-cross-sectional-and-descriptive-index-case-study
#4
Fatima Dahmani, Souad Benkirane, Jaafar Kouzih, Aziz Woumki, Hassan Mamad, Azlarab Masrar
Hemoglobinopathies are congenital disorders resultimg from hemoglobin abnormalities. Major forms are often severe, their management is difficult and associated with a great psychosocial impact on patients and their families. They are classified as rare diseases and are still insufficiently known by health professionals. This lack of knowledge is at the origin of diagnostic errors, delay in their management and therefore high morbidity and mortality rate for these patients. In 2008, the World Health Organization (WHO) has published data on hemoglobinopathies epidemiology: more than 330...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28902183/patient-characteristics-affect-the-response-to-ketamine-and-opioids-during-the-treatment-of-vaso-occlusive-episode-related-pain-in-sickle-cell-disease
#5
Raissa Nobrega, Kathy A Sheehy, Caroline Lippold, Amy L Rice, Julia C Finkel, Zenaide M N Quezado
BackgroundN-methyl-D-aspartate receptor activation has been implicated in the pathobiology of inflammatory, nociceptive and neuropathic pain, opioid tolerance, opioid-induced hyperalgesia, and central sensitization. Some of those mechanisms underlie sickle cell disease(SCD)-associated pain.MethodsWe conducted an exploratory cohort study of SCD patients who during vaso-occlusive episodes (VOEs) received subanesthetic doses of the N-methyl-D-aspartate receptor antagonist, ketamine, as an adjunct to opioids. We sought to identify predictors of changes in pain scores and of the percentage of ketamine infusions associated with meaningful changes (≥20% reduction) in pain and opioid consumption...
September 13, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28892533/association-of-guideline-adherent-antibiotic-treatment-with-readmission-of-children-with-sickle-cell-disease-hospitalized-with-acute-chest-syndrome
#6
David G Bundy, Troy E Richardson, Matthew Hall, Jean L Raphael, David C Brousseau, Staci D Arnold, Ram V Kalpatthi, Angela M Ellison, Suzette O Oyeku, Samir S Shah
Importance: Acute chest syndrome (ACS) is a common, serious complication of sickle cell disease (SCD) and a leading cause of hospitalization and death in both children and adults with SCD. Little is known about the effectiveness of guideline-recommended antibiotic regimens for the care of children hospitalized with ACS. Objectives: To use a large, national database to describe patterns of antibiotic use for children with SCD hospitalized for ACS and to determine whether receipt of guideline-adherent antibiotics was associated with lower readmission rates...
September 11, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28884840/hydroxycarbamide-in-children-with-sickle-cell-anemia-after-first-dose-vs-chronic-therapy-pharmacokinetics-and-predictive-models-for-drug-exposure
#7
Jeremie H Estepp, Paweł Wiczling, Joseph Moen, Guolian Kang, Joana Marie Mack, Robert Liem, Julie A Panepinto, Uttam Garg, Gregory Kearns, Kathleen A Neville
AIMS: The purpose of this work was to (1) compare pharmacokinetic (PK) parameters for hydroxycarbamide in children receiving their first dose (HCnew ) versus those receiving chronic therapy (HCchronic ), (2) assess the external validity of a published PK dosing strategy, and (3) explore the accuracy of dosing strategies based on a limited number of HC measurements. METHODS: Utilizing data from two prospective, multicenter trials of hydroxycarbamide (Pharmacokinetics of Liquid Hydroxyurea in Pediatric Patients with Sickle Cell Anemia; NCT01506544 and Single-Dose (SD) and Steady-State (SS) Pharmacokinetics of Hydroxyurea in Children and Adolescents with Sickle Cell Disease), plasma drug concentration versus time profiles were evaluated with a model independent approach in the HCnew and HCchronic groups...
September 8, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28884421/assessing-responsiveness-over-time-of-the-promis-%C3%A2-pediatric-symptom-and-function-measures-in-cancer-nephrotic-syndrome-and-sickle-cell-disease
#8
Bryce B Reeve, Lloyd J Edwards, Byron C Jaeger, Pamela S Hinds, Carlton Dampier, Debbie S Gipson, David T Selewski, Jonathan P Troost, David Thissen, Vaughn Barry, Heather E Gross, Darren A DeWalt
PURPOSE: Previous studies provided evidence for the validity of the PROMIS Pediatric measures in cross-sectional studies. This study evaluated the ability of the PROMIS Pediatric measures to detect change over time in children and adolescents with cancer, nephrotic syndrome (NS), or sickle cell disease (SCD). METHODS: Participants (8-17 years) completed measures of fatigue, pain interference, anger, anxiety, depressive symptoms, mobility, upper extremity, and peer relationships at three or four time points (T1-T4)...
September 7, 2017: Quality of Life Research
https://www.readbyqxmd.com/read/28868627/hydroxyurea-prescription-availability-and-use-for-children-with-sickle-cell-disease-in-italy-results-of-a-national-multicenter-survey
#9
Raffaella Colombatti, Giovanni Palazzi, Nicoletta Masera, Lucia Dora Notarangelo, Elisa Bonetti, Piera Samperi, Angelica Barone, Silverio Perrotta, Elena Facchini, Maurizio Miano, Giovanni Carlo Del Vecchio, Maria Elena Guerzoni, Paola Corti, Federica Menzato, Simone Cesaro, Maddalena Casale, Paolo Rigano, Gian Luca Forni, Giovanna Russo, Laura Sainati
BACKGROUND: The number of patients with sickle cell disease (SCD) has increased in Italy in the past decade due to immigration. In spite of the established efficacy of hydroxyurea (HU) in childhood, population-based data regarding its prescription and effectiveness come mainly from studies performed in adults or outside Europe. POPULATION AND METHODS: The Hydroxyurea in SCD: A Large Nation-wide Cohort Study from Italy was a retrospective cohort study of adult and pediatric patients with SCD attending 32 centers...
September 4, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28860042/oct-angiography-and-ultra-widefield-fluorescein-angiography-for-early-detection-of-adolescent-sickle-retinopathy
#10
Daniel A Pahl, Nancy S Green, Monica Bhatia, Margaret T Lee, Jonathan S Chang, Maureen Licursi, Courtney Briamonte, Elana Smilow, Royce W S Chen
PURPOSE: Using standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescents with sickle cell disease (SCD). We performed a prospective, observational clinical study to determine if ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCT-A) detect more frequent retinopathy in adolescents with SCD. DESIGN: Cross-sectional study. METHODS: Setting: Institutional...
August 28, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28859033/longitudinal-analysis-of-echocardiographic-abnormalities-in-children-with-sickle-cell-disease
#11
Jamie K Harrington, Usha Krishnan, Zhezhen Jin, Christopher Mardy, Serge Kobsa, Margaret T Lee
BACKGROUND: Cardiac abnormalities have been described in echocardiograms of children with sickle cell disease (SCD). However, longitudinal studies investigating progression of echocardiographic abnormalities across the pediatric age spectrum in SCD are lacking. METHODS: A retrospective longitudinal analysis of 829 echocardiograms from pediatric patients with SCD at steady-state was performed. Left heart parameters included left ventricular end-systolic, end-diastolic diameters, fractional shortening, and mass...
August 30, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28852282/the-bone-pain-crisis-of-sickle-cell-disease-and-malaria-observations-from-gujarat-india
#12
Jyotish Patel, Bharati Patel, Graham R Serjeant
BACKGROUND: Sickle cell disease is a common problem across central India, but its clinical features may differ from that in African populations. There is a need to define the features of sickle cell disease in India, and the current study addresses some features of the bone pain crisis. OBJECTIVES: The objective of the study was to describe the epidemiology of the bone pain crisis of sickle cell disease in Gujarat and explore the relationship with infection by Plasmodium vivax...
July 2017: Indian Journal of Community Medicine
https://www.readbyqxmd.com/read/28840600/red-blood-cell-minor-antigen-mismatches-during-chronic-transfusion-therapy-for-sickle-cell-anemia
#13
Marianne E M Yee, Cassandra D Josephson, Anne M Winkler, Jennifer Webb, Naomi L C Luban, Traci Leong, Sean R Stowell, Ross M Fasano
BACKGROUND: Red blood cell (RBC) alloimmunization occurs at a high frequency in sickle cell anemia (SCA) despite serologic matching for Rh (C/c, E/e) and K antigens. RBC minor antigen genotyping allows for prediction of antigens and RH variants that may lead to alloimmunization. STUDY DESIGN AND METHODS: RBC antigen genotyping was performed on chronically transfused pediatric SCA patients, using PreciseType human erythrocyte antigen (HEA), RHCE, and RHD BeadChip arrays...
August 24, 2017: Transfusion
https://www.readbyqxmd.com/read/28816916/developmental-screening-in-pediatric-sickle-cell-disease-disease-related-risk-and-screening-outcomes-in-4-year-olds
#14
Jeffrey Schatz, Alyssa Schlenz, Laura Reinman, Kelsey Smith, Carla W Roberts
OBJECTIVE: Studies of early child development in sickle cell disease (SCD) have found modest associations between disease-related risks and developmental status in infants and toddlers, but such associations are evident by early elementary school. We screened 4-year-old children with SCD using 2 screening strategies to assess if biomedical risk factors for neurologic disease are related to developmental screening outcomes at this intermediate age. METHODS: Seventy-seven 4-year-old children with SCD (M = 4...
August 4, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28815799/characterization-of-opioid-use-in-sickle-cell-disease
#15
Jin Han, Jifang Zhou, Santosh L Saraf, Victor R Gordeuk, Gregory S Calip
PURPOSE: Opioid analgesics are commonly used to treat vaso-occlusive pain episodes in sickle cell disease (SCD), but comprehensive evidence characterizing opioid use in this patient population is limited. Our objective was to characterize opioid use patterns among SCD patients using a large nationwide database. METHODS: A large, US medical claims database was utilized to identify a cohort of 3882 SCD patients, and characteristics of opioid use were analyzed. Clinical variables including age, gender, medication use, health care utilization, and medical history were evaluated for correlations with opioid use...
August 16, 2017: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/28801954/an-analysis-of-inpatient-pediatric-sickle-cell-disease-incidence-costs-and-outcomes
#16
Laura M Bou-Maroun, Fabien Meta, Curtis J Hanba, Andrew D Campbell, Gregory A Yanik
OBJECTIVE: To identify characteristics of pediatric sickle cell disease (SCD) hospitalizations and to examine admission demographics and medical expenditures. METHODS: Admissions with SCD were identified from the 2009 and 2012 releases of the Healthcare and Cost Utilization Project's Kids Inpatient Database. Disease-specific secondary diagnoses including acute chest syndrome (ACS), vaso-occlusive pain crisis (VOC), splenic sequestration, and stroke/transient ischemic attack were analyzed for patient and hospital demographics...
August 12, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28783617/an-age-dependent-response-to-hydroxyurea-in-pediatric-sickle-cell-anemia-patients-with-alpha-thalassemia-trait
#17
Lisa Figueiredo, Kerry Morrone, Catherine Wei, Karen Ireland, Hillel W Cohen, Catherine Driscoll, Deepa Manwani
Hydroxyurea (HU) is a key drug therapy for individuals with sickle cell anemia (SCA), yet its clinical and hematologic responses can be variable. Various studies have reported the role of α-thalassemia as one of the most prevalent heritable traits that may modify HU response. We provide data from 62 pediatric and adolescent patients with SCA, 26 with co-inherited α-thalassemia trait. Our data suggest that altered hematologic and clinical responses to HU therapy are noted in adolescent SCA individuals with co-inherited α-thalassemia trait...
July 31, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28745855/managing-acute-complications-of-sickle-cell-disease-in-pediatric-patients-digest
#18
Sathyaseelan Subramaniam, Jennifer H Chao, Pradip Chaudhari
Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management...
November 22, 2016: Pediatric Emergency Medicine Practice
https://www.readbyqxmd.com/read/28743191/successful-utilization-of-an-electronic-pain-diary-in-a-multinational-phase-3-interventional-study-of-pediatric-sickle-cell-anemia
#19
Lori E Heath, Matthew M Heeney, Carolyn C Hoppe, Samuel Adjei, Tsiri Agbenyega, Mohamed Badr, Nicoletta Masera, Chunmei Zhou, Patricia B Brown, Joseph A Jakubowski, Carlton Dampier
BACKGROUND/AIMS: Patients with sickle cell anemia can experience recurrent pain episodes, which affect quality of life. The reported prevalence of pain is higher in studies using patient diaries than in healthcare facility utilization data. Determining Effects of Platelet Inhibition on Vaso-Occlusive Events was a multinational study that assessed the efficacy and safety of prasugrel in reducing the rate of vaso-occlusive events in children with sickle cell anemia (NCT01794000) and included an electronic patient-reported outcome diary to record pain occurrence...
July 1, 2017: Clinical Trials: Journal of the Society for Clinical Trials
https://www.readbyqxmd.com/read/28737601/new-ways-to-detect-pediatric-sickle-cell-retinopathy-a-comprehensive-review
#20
Daniel A Pahl, Nancy S Green, Monica Bhatia, Royce W S Chen
Sickle retinopathy reflects disease-related vascular injury of the eye, which can potentially result in visual loss from vitreous hemorrhage or retinal detachment. Here we review sickle retinopathy among children with sickle cell disease, describe the epidemiology, pediatric risk factors, pathophysiology, ocular findings, and treatment. Newer, more sensitive ophthalmological imaging modalities are available for retinal imaging, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography...
July 21, 2017: Journal of Pediatric Hematology/oncology
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