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Pediatric Sickle Cell

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https://www.readbyqxmd.com/read/28078488/hydroxyurea-treatment-effect-on-children-with-sickle-cell-disease-and-obstructive-sleep-apnea
#1
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-hct-for-children-with-sickle-cell-disease-and-thalassemia-a-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international
#2
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 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 (SCD) 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 due to 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
#3
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
#4
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
https://www.readbyqxmd.com/read/27957790/transcranial-doppler-in-hemoglobin-sc-disease
#5
Camilo Vieira, Carolina Nogueira Costa de Oliveira, Ludmila Araújo Borges de Figueiredo, Rayra Pereira Santiago, Corynne Stephanie Ahouefa Adanho, Sanzio Silva Santana, Caroline Lang Burak, Isa Menezes Lyra, Marilda Souza Goncalves
BACKGROUND: Stroke is a severe clinical disorder in sickle cell disease (SCD), and few studies have evaluated transcranial Doppler (TCD) flow velocities in hemoglobin SC disease (HbSC). The guidelines for stroke risk are based on evaluations in sickle cell anemia (SCA) or HbS/β thalassemia. PROCEDURE: In this study, we compare cerebral blood flow in patients with SCD stratified by genotypes. A total of 1,664 pediatric patients with SCD underwent TCD velocity screening, and the time-averaged maximum mean velocity (TAMM) was determined in the middle cerebral artery (MCA), anterior cerebral artery (ACA), and distal intracranial internal carotid artery (ICA) ...
December 13, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27932225/hospitalizations-of-children-with-sickle-cell-disease-in-the-brazilian-unified-health-system-in-the-state-of-minas-gerais
#6
Ana Paula P C Fernandes, Fernanda A Avendanha, Marcos B Viana
OBJECTIVE: To identify and characterize hospital admissions and readmissions in the Brazilian Unified Public Health System (Sistema Único de Saúde [SUS]) in children with sickle cell disease diagnosed by the Minas Gerais Newborn Screening Program between 1999 and 2012. METHODS: Hospital Admission Authorizations with the D57 (International Classification of Diseases-10) code in the fields of primary or secondary diagnosis were retrieved from the SUS Databank (1999-2012)...
December 5, 2016: Jornal de Pediatria
https://www.readbyqxmd.com/read/27905689/development-of-quality-indicators-for-transition-from-pediatric-to-adult-care-in-sickle-cell-disease-a-modified-delphi-survey-of-adult-providers
#7
Amy E Sobota, Nishita Shah, Jennifer W Mack
BACKGROUND: Transition from pediatric to adult care is a vulnerable time for young adults with sickle cell disease (SCD); however, improvements in transition are limited by a lack of quality indicators. The purpose of this study was to establish quality indicators for transition in SCD and to determine the optimal timing between the final pediatric visit and the first adult provider visit. PROCEDURE: We conducted a modified Delphi survey to reach a consensus on which quality indicators are most important for a successful transition...
December 1, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27902670/ketamine-infusion-for-pain-control-in-acute-pediatric-sickle-cell-painful-crises
#8
Jonathan M Hagedorn, Evelyn C Monico
OBJECTIVES: Sickle cell disease is the most common hemoglobin variant in the world and can present with recurrent vaso-occlusive painful crises. Pain control in these patients is often difficult and requires a multimodal approach. In patients with uncontrolled pain after traditional pain control methods, ketamine infusions have proven beneficial. Unfortunately, no source alone had described ketamine infusions for pediatric sickle cell patients. Our objective was to provide a thorough definitive reference for health care providers regarding the use of ketamine infusion for pain control in pediatric sickle cell painful crises...
November 29, 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27887664/translating-sickle-cell-guidelines-into-practice-for-primary-care-providers-with-project-echo
#9
Lisa M Shook, Christina B Farrell, Karen A Kalinyak, Stephen C Nelson, Brandon M Hardesty, Angeli G Rampersad, Kay L Saving, Wanda J Whitten-Shurney, Julie A Panepinto, Russell E Ware, Lori E Crosby
BACKGROUND: Approximately 100,000 persons with sickle cell disease (SCD) live in the United States, including 15,000 in the Midwest. Unfortunately, many patients experience poor health outcomes due to limited access to primary care providers (PCPs) who are prepared to deliver evidence-based SCD care. Sickle Treatment and Outcomes Research in the Midwest (STORM) is a regional network established to improve care and outcomes for individuals with SCD living in Indiana, Illinois, Michigan, Minnesota, Ohio, and Wisconsin...
2016: Medical Education Online
https://www.readbyqxmd.com/read/27814292/hemorheological-alterations-in-sickle-cell-anemia-and-their-clinical-consequences-the-role-of-genetic-modulators
#10
Marisa Silva, Sofia Vargas, Andreia Coelho, Alexandra Dias, Teresa Ferreira, Anabela Morais, Raquel Maia, Paula Kjöllerström, João Lavinha, Paula Faustino
Sickle cell anemia (SCA) is an autosomal recessive disease caused by the HBB:c.20A>T mutation that leads to hemoglobin S synthesis. The disease presents with high clinical heterogeneity characterized by chronic hemolysis, recurrent episodes of vaso-oclusion and infection. This work aimed to characterize by in silico studies some genetic modulators of severe hemolysis and stroke risk in children with SCA, and understand their consequences at the hemorheological level.Association studies were performed between hemolysis biomarkers as well as the degree of cerebral vasculopathy and the inheritance of several polymorphic regions in genes related with vascular cell adhesion and vascular tonus in pediatric SCA patients...
November 4, 2016: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/27803072/factors-associated-with-the-prophylactic-prescription-of-a-bowel-regimen-to-prevent-opioid-induced-constipation
#11
Nancy Y Chen, Eugene Nguyen, Sheree M Schrager, Christopher J Russell
OBJECTIVE: Identify factors associated with the prophylactic prescription of a bowel regimen with an inpatient opioid prescription. METHODS: This was a retrospective cohort study from June 1, 2013, to October 31, 2014 of pediatric inpatients prescribed an oral or intravenous opioid on the general medical/surgical floors. We identified patients with or without a prophylactic prescription of a bowel regimen. We obtained patient demographics, prescriber training level and service and used multivariate logistic regression to analyze the factors associated with prophylactic bowel regimen and opioid prescription...
November 2016: Hospital Pediatrics
https://www.readbyqxmd.com/read/27802215/sickle-cell-anemia-nitric-oxide-related-genetic-modifiers-of-hematological-and-biochemical-parameters
#12
Laura Aguiar, Andreia Matos, Ângela Gil, Conceiçã Afonso, Lígia Braga, Lavinha João, Paula Kjollerstrom, Paula Faustino, Manuel Bicho, Ângela Inácio
BACKGROUND: Sickle cell anemia (SCA) is an inherited blood disorder. SCA patients present clinical and hematologic variability that cannot be only explained by the single mutation in the beta-globin gene. Others genetic modifiers and environmental effects are important for the clinical phenotype. SCA patients present arginine deficiency that contributes to a lower nitric oxide (NO) bioactivity. OBJECTIVE: The aim of this work is to determine the association between hematological and biochemical parameters and genetic variants from eNOS gene, in pediatric SCA patients...
October 14, 2016: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/27798391/pain-management-for-sickle-cell-disease-in-the-pediatric-emergency-department-medications-and-hospitalization-trends
#13
Chantel Cacciotti, Sarah Vaiselbuh, Eleny Romanos-Sirakis
The majority of emergency department (ED) visits and hospitalizations for patients with sickle cell disease (SCD) are pain related. Adequate and timely pain management may improve quality of life and prevent worsening morbidities. We conducted a retrospective chart review of pediatric patients with SCD seen in the ED, selected by sickle cell-related ICD-9 codes. A total of 176 encounters were reviewed from 47 patients to record ED pain management and hospitalization trends. Mean time to pain medication administration was 63 minutes...
October 23, 2016: Clinical Pediatrics
https://www.readbyqxmd.com/read/27796476/cognitive-and-fine-motor-deficits-in-a-pediatric-sickle-cell-disease-cohort-of-mixed-ethnic-origin
#14
Luise Burkhardt, Stephan Lobitz, Elisabeth Koustenis, Stefan Mark Rueckriegel, Pablo Hernáiz Driever
Cerebrovascular disease is an important feature of pediatric sickle cell disease (SCD) and may lead to cognitive and motor impairment. Our cross-sectional study examined the incidence and severity of these impairments in a pediatric cohort without clinical cerebrovascular events from Berlin of mixed ethnic origin. Thirty-two SCD patients (mean age 11.14 years, range 7.0-17.25 years; males 14) were evaluated for full-scale intelligence (IQ) (German version WISC-III), fine motor function (digital writing tablet), and executive function (planning, attention, working memory, and visual-spatial abilities) with the Amsterdam Neuropsychological Tasks (ANT) program and the Tower of London (ToL)...
October 29, 2016: Annals of Hematology
https://www.readbyqxmd.com/read/27778040/modern-american-scurvy-experience-with-vitamin-c-deficiency-at-a-large-children-s-hospital
#15
Farahnaz Golriz, Lane F Donnelly, Sridevi Devaraj, Raj Krishnamurthy
BACKGROUND: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism. OBJECTIVE: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations...
October 24, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27775898/managing-acute-complications-of-sickle-cell-disease-in-pediatric-patients
#16
REVIEW
Sathyaseelan Subramaniam, Jennifer H Chao
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 2016: Pediatric Emergency Medicine Practice
https://www.readbyqxmd.com/read/27771375/spleen-histology-in-children-with-sickle-cell-disease-and-hereditary-spherocytosis-hints-on-the-disease-pathophysiology
#17
Marco Pizzi, Fabio Fuligni, Luisa Santoro, Elena Sabattini, Martina Ichino, Rita De Vito, Pietro Zucchetta, Raffaella Colombatti, Laura Sainati, Piergiorgio Gamba, Rita Alaggio
Hereditary spherocytosis (HS) and sickle cell disease (SCD) are associated with splenomegaly and spleen dysfunction in pediatric patients. Scant data exist on possible correlations between spleen morphology and function in HS and SCD. This study aimed to assess the histological and morphometric features of HS and SCD spleens, in order to get possible correlations with disease pathophysiology. In a large series of spleens from SCD, HS and control patients the following parameters were considered: (i) macroscopic features; (ii) lymphoid follicle (LF) density; (iii) presence of peri-follicular marginal zones (MZs); (iv) presence of Gamna-Gandy bodies; (v) density of CD8-positive sinusoids; (vi) density of CD34-positive microvessels; (vii) presence/distribution of fibrosis and SMA-positive myoid cells; (viii) density of CD68-positive macrophages...
October 19, 2016: Human Pathology
https://www.readbyqxmd.com/read/27761932/hydroxyurea-effectiveness-in-children-and-adolescents-with-sickle-cell-anemia-a-large-retrospective-population-based-cohort
#18
Maa-Ohui Quarmyne, Wei Dong, Rodney Theodore, Sonia Anand, Vaughn Barry, Olufolake Adisa, Iris D Buchanan, James Bost, Robert C Brown, Clinton H Joiner, Peter A Lane
The clinical efficacy of hydroxyurea in patients with sickle cell anemia (SCA) has been well established. However, data about its clinical effectiveness in practice is limited. We evaluated the clinical effectiveness of hydroxyurea in a large pediatric population using a retrospective cohort, pre-post treatment study design to control for disease severity selection bias. The cohort included children with SCA (SS, Sβ(0) thalassemia) who received care at Children's Healthcare of Atlanta (CHOA) and who initiated hydroxyurea in 2009-2011...
January 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27722086/mri-based-cerebrovascular-reactivity-using-transfer-function-analysis-reveals-temporal-group-differences-between-patients-with-sickle-cell-disease-and-healthy-controls
#19
Jackie Leung, James Duffin, Joseph A Fisher, Andrea Kassner
OBJECTIVES: Cerebrovascular reactivity (CVR) measures the ability of cerebral blood vessels to change their diameter and, hence, their capacity to regulate regional blood flow in the brain. High resolution quantitative maps of CVR can be produced using blood-oxygen level-dependent (BOLD) magnetic resonance imaging (MRI) in combination with a carbon dioxide stimulus, and these maps have become a useful tool in the clinical evaluation of cerebrovascular disorders. However, conventional CVR analysis does not fully characterize the BOLD response to a stimulus as certain regions of the brain are slower to react to the stimulus than others, especially in disease...
2016: NeuroImage: Clinical
https://www.readbyqxmd.com/read/27721706/hemoglobin-threshold-for-blood-transfusion-in-a-pediatric-intensive-care-unit
#20
Madhuradhar Chegondi, Jun Sasaki, André Raszynski, Balagangadhar R Totapally
OBJECTIVE: To evaluate the hemoglobin threshold for red cell transfusion in children admitted to a pediatric intensive care unit (PICU). METHODS: Retrospective chart review study. Tertiary care PICU. Critically ill pediatric patients requiring blood transfusion. No intervention. RESULTS: We analyzed the charts of all children between 1 month and 21 years of age who received packed red blood cell (PRBC) transfusions during a 2-year period. The target patients were identified from our blood bank database...
July 2016: Transfusion Medicine and Hemotherapy
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