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https://www.readbyqxmd.com/read/29345595/hospital-management-of-severe-hypertriglyceridemia-in-children
#1
Ambika Ashraf, Badhma Valaiyapathi
Severe hypertriglyceridemia (HTG), i.e., triglyceride levels exceeding 1000 mg/dL, is one of the established causes of acute pancreatitis and severe abdominal pain. There are no established pediatric guidelines for treatment of children and adolescents with severe HTG. Management often includes hospital admission, fasting followed by fat free diet until the triglyceride (TG) levels are below 1000 mg/dL. Insulin infusion lowers TG levels in patients, especially in those with diabetes. Plasmapheresis is a limited option due to its restricted availability and scarcity of studies on its efficacy and safety in children...
January 16, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29344584/early-and-late-outcomes-after-cord-blood-transplantation-for-pediatric-patients-with-inherited-leukodystrophies
#2
Brigitte T A van den Broek, Kristin Page, Annalisa Paviglianiti, Janna Hol, Heather Allewelt, Fernanda Volt, Gerard Michel, Miguel Angel Diaz, Victoria Bordon, Tracey O'Brien, Peter J Shaw, Chantal Kenzey, Amal Al-Seraihy, Peter M van Hasselt, Andrew R Gennery, Eliane Gluckman, Vanderson Rocha, Annalisa Ruggeri, Joanne Kurtzberg, Jaap Jan Boelens
Leukodystrophies (LD) are devastating inherited disorders leading to rapid neurological deterioration and premature death. Hematopoietic stem cell transplantation (HSCT) can halt disease progression for selected LD. Cord blood is a common donor source for transplantation of these patients because it is rapidly available and can be used without full HLA matching. However, precise recommendations allowing care providers to identify patients who benefit from HSCT are lacking. In this study, we define risk factors and describe the early and late outcomes of 169 patients with globoid cell leukodystrophy, X-linked adrenoleukodystrophy, and metachromatic leukodystrophy undergoing cord blood transplantation (CBT) at an European Society for Blood and Marrow Transplantation center or at Duke University Medical Center from 1996 to 2013...
January 9, 2018: Blood Advances
https://www.readbyqxmd.com/read/29341904/a-rapid-and-simple-lc-ms-ms-method-for-personalized-busulfan-dosing-in-pediatric-patients-undergoing-hematological-stem-cell-transplantation-hsct
#3
Yi Xiao, Xiaohan Li, Xiaowei Fu
BACKGROUND: Busulfan is commonly used as a conditioning regimen before hematological stem cell transplantation (HSCT). There is a big inter-individual variability in busulfan exposure and the narrow therapeutic index, especially in pediatric population. Therefore, to achieve therapeutic efficacy and safety concurrently, personalized busulfan dosing, guided by pharmacokinetic study with serial plasma samples, is needed a few hours afterwards. METHODS: We used liquid-chromatography-mass spectrometry (HPLC-MS/MS) to develop, validate, and implementation of a fast, sensitive, and accurate method for busulfan measurement...
January 13, 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29333903/the-first-report-of-relative-incidence-of-inherited-white-matter-disorders-in-an-asian-country-based-on-an-iranian-bioregistry-system
#4
Mahmoud Reza Ashrafi, Zahra Rezaei, Morteza Heidari, Sedigheh Nikbakht, Reza Azizi Malamiri, Mahmoud Mohammady, Gholam Reza Zamani, Reza Shervin Badv, Parastoo Rostami, Mojtaba Movahedinia, Mostafa Qorbani, Man Amanat, Ali Reza Tavasoli
Childhood leukodystrophies are a fast-growing field of pediatric neurology practice. Epidemiologic studies on the incidence of these disorders in children show different results. This is the first report of childhood leukodystrophies incidence from Iran. The enrolled patients were recruited from the neurometabolic bioregistry system that was organized in 2010 in the Children's Medical Center, Tehran, Iran. Herein is reported the incidence rate of leukodystrophies in those patients who were residents of 2 big popular provinces near Iran's capital city Tehran, with an average child population of 2 988 800 children...
January 1, 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29333742/parvalbumin-fast-spiking-interneurons-are-selectively-altered-by-pediatric-traumatic-brain-injury
#5
Joshua Nichols, George Reed Bjorklund, Jason Newbern, Trent Anderson
Pediatric traumatic brain injury (TBI) is a leading cause of death and disability in children. Traditionally, ongoing neurodevelopment and neuroplasticity have thought to confer children with an advantage following TBI. However, recent findings indicate that the pediatric brain may be more sensitive to brain injury. Inhibitory interneurons are essential for proper cortical function and implicated in the pathophysiology of TBI, yet few studies have directly examined for TBI induced changes to interneurons themselves...
January 15, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/29313283/edmonton-obesity-staging-system-among-pediatric-patients-a-validation-and-obesogenic-risk-factor-analysis
#6
M G Grammatikopoulou, M Chourdakis, K Gkiouras, P Roumeli, D Poulimeneas, E Apostolidou, I Chountalas, I Tirodimos, O Filippou, S Papadakou-Lagogianni, T Dardavessis
PURPOSE: The Edmonton Obesity Staging System for Pediatrics (EOSS-P) is a useful tool, delineating different obesity severity tiers associated with distinct treatment barriers. The aim of the study was to apply the EOSS-P on a Greek pediatric cohort and assess risk factors associated with each stage, compared to normal weight controls. METHODS: A total of 361 children (2-14 years old), outpatients of an Athenian hospital, participated in this case-control study by forming two groups: the obese (n = 203) and the normoweight controls (n = 158)...
January 8, 2018: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/29306262/considerations-for-physicians-using-ketamine-for-sedation-of-children-in-emergency-departments
#7
Woo Sung Kim, Ji Yeon Ku, Hanbyul Choi, Hyo Jeong Choi, Ho Jung Kim, Bora Lee
OBJECTIVE: Ketamine use in emergency departments (EDs) for procedural sedation and analgesia is becoming increasingly common. However, few studies have examined patient factors related to adverse events associated with ketamine. This study investigated factors for consideration when using ketamine to sedate pediatric ED patients. METHODS: The study included pediatric patients receiving ketamine for laceration repair in the ED. Before sedation, information was collected about upper respiratory tract infection symptoms, allergy history, and fasting time...
December 2017: Clinical and Experimental Emergency Medicine
https://www.readbyqxmd.com/read/29303370/the-effects-of-subcutaneous-insulin-infusion-versus-multiple-insulin-injections-on-glucose-variability-in-young-adults-with-type-1-diabetes-the-2-year-follow-up-of-the-observational-metro-study
#8
Maria Ida Maiorino, Giuseppe Bellastella, Ofelia Casciano, Paolo Cirillo, Vittorio Simeon, Paolo Chiodini, Michela Petrizzo, Maurizio Gicchino, Ornella Romano, Paola Caruso, Dario Giugliano, Katherine Esposito
BACKGROUND: Type 1 diabetic patients have high instability of daily glucose levels. The aim of this study was to evaluate the long-term effects of continuous subcutaneous insulin infusion (CSII) therapy, compared with multiple daily injections of insulin (MDI), on glucose variability, in young type 1 diabetic patients transitioned to the adult diabetes care. METHODS: Patients aged 18-30 years and considered eligible for insulin pump therapy were included in the study...
January 5, 2018: Diabetes Technology & Therapeutics
https://www.readbyqxmd.com/read/29298340/the-association-between-a-body-shape-index-and-cardiovascular-risk-in-overweight-and-obese-children-and-adolescents
#9
Chiara Mameli, Nir Y Krakauer, Jesse C Krakauer, Alessandra Bosetti, Chiara Matilde Ferrari, Norma Moiana, Laura Schneider, Barbara Borsani, Teresa Genoni, Gianvincenzo Zuccotti
A Body Shape Index (ABSI) and normalized hip circumference (Hip Index, HI) have been recently shown to be strong risk factors for mortality and for cardiovascular disease in adults. We conducted an observational cross-sectional study to evaluate the relationship between ABSI, HI and cardiometabolic risk factors and obesity-related comorbidities in overweight and obese children and adolescents aged 2-18 years. We performed multivariate linear and logistic regression analyses with BMI, ABSI, and HI age and sex normalized z scores as predictors to examine the association with cardiometabolic risk markers (systolic and diastolic blood pressure, fasting glucose and insulin, total cholesterol and its components, transaminases, fat mass % detected by bioelectrical impedance analysis) and obesity-related conditions (including hepatic steatosis and metabolic syndrome)...
2018: PloS One
https://www.readbyqxmd.com/read/29290033/effects-of-obesity-and-metabolic-syndrome-on-cardiovascular-outcomes-in-pediatric-kidney-transplant-recipients-a-longitudinal-study
#10
Kristen Sgambat, Sarah Clauss, K Y Lei, Juizhou Song, Shaik O Rahaman, Margaret Lasota, Asha Moudgil
BACKGROUND: Obesity and metabolic syndrome (MS) are common after kidney transplantation, but their contribution to adverse cardiovascular (CV) outcomes in children are not well known. A prospective, controlled, longitudinal cohort study was conducted to investigate the effects of obesity and MS on left ventricular hypertrophy (LVH) and myocardial strain in pediatric kidney transplant recipients. METHODS: Transplant recipients (n = 42) had anthropometrics [body mass index (BMI), waist circumference, waist-to-height ratio], biochemical parameters (fasting glucose, lipid panel, HbA1c%), and echocardiogram with speckle tracking analysis for strain measured at 1, 18, and 30 months post-transplant...
December 30, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29285534/comparison-of-quality-and-output-of-different-optimal-perimetric-testing-approaches-in-children-with-glaucoma
#11
Dipesh E Patel, Phillippa M Cumberland, Bronwen C Walters, Isabelle Russell-Eggitt, John Brookes, Maria Papadopoulos, Peng Tee Khaw, Ananth C Viswanathan, David Garway-Heath, Mario Cortina-Borja, Jugnoo S Rahi
Importance: There is limited evidence to support the development of guidance for visual field testing in children with glaucoma. Objective: To compare different static and combined static/kinetic perimetry approaches in children with glaucoma. Design, Setting, and Participants: Cross-sectional, observational study recruiting children prospectively between May 2013 and June 2015 at 2 tertiary specialist pediatric ophthalmology centers in London, England (Moorfields Eye Hospital and Great Ormond Street Hospital)...
December 28, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/29276865/high-frequency-oscillatory-ventilation-versus-conventional-mechanical-ventilation-in-pediatric-acute-respiratory-distress-syndrome-a-randomized-controlled-study
#12
Ahmed El-Nawawy, Azza Moustafa, Hassan Heshmat, Ahmed Abouahmed
El-Nawawy A, Moustafa A, Heshmat H, Abouahmed A. High frequency oscillatory ventilation versus conventional mechanical ventilation in pediatric acute respiratory distress syndrome: A randomized controlled study. Turk J Pediatr 2017; 59: 130-143. The aim of this prospective randomized study is to compare the outcomes of the early use of either high frequency oscillation (HFO) or conventional mechanical ventilation (CMV) in patients with pediatric acute respiratory distress syndrome (PARDS). We allocated two hundred PARDS patients over 5 years in 1:1 ratio to either mode...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29248949/robot-assisted-extravesical-ureteral-reimplantation-revur-for-unilateral-vesico-ureteral-reflux-in-children-results-of-a-multicentric-international-survey
#13
Ciro Esposito, Lorenzo Masieri, Henri Steyaert, Maria Escolino, Raffaele Cerchione, Angela La Manna, Chiara Cini, Thomas S Lendvay
PURPOSE: This multicentric international retrospective study aimed to report the outcome of robot-assisted extravesical ureteral reimplantation (REVUR) in patients with unilateral vesico-ureteral reflux (VUR). METHODS: The medical records of 55 patients (35 girls, 20 boys) underwent REVUR in four international centers of pediatric robotic surgery for primary unilateral VUR were retrospectively reviewed. Patients' average age was 4.9 years. The preoperative grade of reflux was III in 12...
December 16, 2017: World Journal of Urology
https://www.readbyqxmd.com/read/29242622/assessment-of-disturbed-glucose-metabolism-and-surrogate-measures-of-insulin-sensitivity-in-obese-children-and-adolescents
#14
Christian L Roth, Clinton Elfers, Christiane S Hampe
BACKGROUND: With the rising prevalence of obesity and type 2 diabetes (T2D) in obese children, it is becoming imperative to detect disturbed glucose metabolism as early as possible in order to prevent T2D development. SUBJECTS/METHODS: Cross-sectional study of 92 obese children (median age 11.7 years, 51% female) and 7 lean children (median age 11.4 years, 57% female) who underwent an oral glucose tolerance test (OGTT) in a tertiary pediatric care center. Glucose tolerance was assessed and different indices for β-cell function, insulin sensitivity and insulin secretion were calculated...
December 14, 2017: Nutrition & Diabetes
https://www.readbyqxmd.com/read/29239916/early-experience-with-clinimacs-prodigy-ccs-ifn-gamma-system-in-selection-of-virus-specific-t-cells-from-third-party-donors-for-pediatric-patients-with-severe-viral-infections-after-hematopoietic-stem-cell-transplantation
#15
Krisztián Kállay, Csaba Kassa, Marienn Réti, Éva Karászi, János Sinkó, Vera Goda, Anita Stréhn, Katalin Csordás, Orsolya Horváth, Attila Szederjesi, Szabolcs Tasnády, Apor Hardi, Gergely Kriván
Viral reactivation is a frequent complication of allogeneic hematopoietic stem cell transplantation especially in children. For refractory cases, rapid virus-specific T-cell therapy would be ideally implemented within a few days. Over the course of a year in our pediatric cohort of 43 allogeneic transplantation, 9 patients fulfilled criteria for virus-specific T-cell therapy. Viral infections were due to cytomegalovirus (CMV) in 3, Epstein-Barr virus (EBV) in 2, and adenovirus (AdV) in 1 case, whereas >1 virus was detected in 3 cases...
December 12, 2017: Journal of Immunotherapy
https://www.readbyqxmd.com/read/29222858/obesity-lipid-profiles-and-oxidative-stress-in-children-after-liver-transplantation
#16
Piotr Czubkowski, Aldona Wierzbicka, Joanna Pawłowska, Irena Jankowska, Piotr Socha
PURPOSE: In adult liver transplant recipients, coronary artery disease and congestive heart failure are significant cause of morbidity and mortality. This may be attributed to the long-term immunosuppressive treatment, mostly with calcineurin inhibitors and steroids, which in long-term may be associated with hyperlipidemia, oxidative stress and cardiovascular complications. Since such data for children is sparse, the aim of this study was to assess the lipid and oxidative stress markers after pediatric liver transplantation (LTx)...
December 10, 2017: Acta Biochimica Polonica
https://www.readbyqxmd.com/read/29211219/incidence-and-treatment-of-chylothorax-in-children-undergoing-corrective-surgery-for-congenital-heart-diseases
#17
Nicolle Martin Christofe, Cristiane Felix Ximenes Pessotti, Laércio Paiva, Ieda Biscegli Jatene
INTRODUCTION: Chylothorax is a lymphatic extravasation into pleural cavity and its incidence is 0.25%-5.3% in children undergoing cardiac surgery. OBJECTIVE: To evaluate the incidence of chylothorax in pediatrics patients operated, linking it in each surgical intervention. Evaluate treatment types and efficiency. METHODS: Retrospective study using medical records of children undergoing cardiac surgery in the Hospital do Coração between 2004 and 2014...
September 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29207608/gsta1-diplotypes-affect-busulfan-clearance-and-toxicity-in-children-undergoing-allogeneic-hematopoietic-stem-cell-transplantation-a-multicenter-study
#18
Marc Ansari, Patricia Huezo-Diaz Curtis, Chakradhara Rao S Uppugunduri, Mohammed Aziz Rezgui, Tiago Nava, Vid Mlakar, Laurence Lesne, Yves Théoret, Yves Chalandon, Lee L Dupuis, Tao Schechter, Imke H Bartelink, Jaap J Boelens, Robbert Bredius, Jean-Hugues Dalle, Saba Azarnoush, Petr Sedlacek, Victor Lewis, Martin Champagne, Christina Peters, Henrique Bittencourt, Maja Krajinovic
Busulfan (BU) dose adjustment following therapeutic drug monitoring contributes to better outcome of hematopoietic stem cell transplantation (HSCT). Further improvement could be achieved through genotype-guided BU dose adjustments. To investigate this aspect, polymorphism within glutathione S transferase genes were assessed. Particularly, promoter haplotypes of the glutathione S transferase A1 (GSTA1) were evaluated in vitro, with reporter gene assays and clinically, in a pediatric multi-center study (N =138) through association with BU pharmacokinetics (PK) and clinical outcomes...
October 31, 2017: Oncotarget
https://www.readbyqxmd.com/read/29200143/the-predictors-for-positive-yield-abdominal-computed-tomography-in-pediatric-abdominal-trauma
#19
Abdulrahman M Alzahem, Soundappan S V Soundappan, Daniel T Cass
OBJECTIVES: Abdominal computed tomography (ACT) use in the initial evaluation of pediatric abdominal trauma is liberal in most instances. The aim of this study was to identify the predictors for a positive yield ACT scan in this population. METHODS: A prospective, cohort, single-center observational study was conducted at Children's Hospital at Westmead, New South Wales, from January 2008 to June 2015 on 240 pediatric abdominal trauma patients who had abdominal computed tomography...
December 1, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29199155/determination-of-procalcitonin-pct-in-micro-method-on-aqt90-flex%C3%A2-analyzer-and-in-vitro-stability-study
#20
Louis Mine, Morgane Petit, Valérie Nivet-Antoine, Jean-Louis Beaudeux, Carole Hennequin
Radiometer® has developed a point-of-care test for fast PCT measurement on whole blood in micromethod on a AQT90 FLEX® instrument. We have verified the analytical performances of the AQT90 FLEX® PCT assay in heparinized macrotube and EDTA microtube for pediatric use, according to modified French Society of Clinical Biology (SFBC) protocol to the requirements of the standard NF EN ISO 15189: 2012. The samples (n=61, 30 macrotubes, 31 microtubes) were analyzed by the Brahms Kryptor Compact Plus® reference method vs the AQT90 FLEX®...
December 4, 2017: Annales de Biologie Clinique
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