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https://www.readbyqxmd.com/read/30110527/pediatric-eosinophilic-esophagitis
#1
Stephanie Posten, Tonya Adamiak, Melissa Jensen
Eosinophilic esophagitis (EoE) is a chronic immune-mediated esophageal inflammatory disease that is becoming more widely recognized as a cause of feeding difficulties in infants and young children, as well as gastroesophageal reflux disease (GERD)-like symptoms, dysphagia, and food impaction in children and adolescents. The diagnosis of EoE is clinicopathologic, based on endoscopic, histologic, and clinical findings. Patients with suspected eosinophilic esophagitis require an endoscopy with biopsies. The diagnosis requires the presence of 15 or more intraepithelial eosinophils/HPF in at least one endoscopic esophageal mucosal biopsy...
August 2018: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/30054164/safety-of-adalimumab-in-pediatric-patients-with-polyarticular-juvenile-idiopathic-arthritis-enthesitis-related-arthritis-psoriasis-and-crohn-s-disease
#2
Gerd Horneff, Marieke M B Seyger, Dilek Arikan, Jasmina Kalabic, Jaclyn K Anderson, Andreas Lazar, David A Williams, Chen Wang, Rita Tarzynski-Potempa, Jeffrey S Hyams
OBJECTIVE: To evaluate the safety of adalimumab in pediatric patients who participated in clinical trials of juvenile idiopathic arthritis (polyarticular juvenile idiopathic arthritis and pediatric enthesitis-related arthritis), psoriasis, and Crohn's disease. STUDY DESIGN: This analysis included data from 7 global, randomized, and open-label AbbVie-sponsored clinical trials of adalimumab and their open-label extensions conducted between September 2002 and December 31, 2015 (cutoff date for ongoing studies)...
July 25, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/30016002/pediatric-cancer-families-participation-in-whole-genome-sequencing-research-in-denmark-parent-perspectives
#3
Anna Byrjalsen, Ulrik Stoltze, Karin Wadt, Lisa Lyngsie Hjalgrim, Anne-Marie Gerdes, Kjeld Schmiegelow, Ayo Wahlberg
With an impending introduction of genome sequencing into paediatric oncology to facilitate personalised medicine, this study examines parent perspectives on participating in whole genome sequencing (WGS) research in the difficult weeks following diagnosis. As an embedded part of Sequencing Tumor and Germline DNA-Implications and National Guidelines (STAGING), a project aiming to implement WGS of all newly diagnosed paediatric cancer patients in Denmark, a parent perspective study was conducted by a clinical geneticist and anthropologist to document pragmatic, social and ethical dilemmas...
July 17, 2018: European Journal of Cancer Care
https://www.readbyqxmd.com/read/29970628/oral-manifestations-of-gastroesophageal-reflux-disease-in-children-a-preliminary-observational-study
#4
Kothimbakkam Sai Sarath Kumar, Jayanthi Mungara, Nilaya Reddy Venumbaka, Poornima Vijayakumar, Deebiga Karunakaran
Aims: This study aimed to document the prevalence of oral manifestations seen among pediatric gastroesophageal reflux disease (GERD) patients in Chennai, Tamil Nadu, India. Methodology: This study was performed at various children's hospitals in Chennai. Fifty-one GERD patients who were assessed by endoscopy and 24 h pH-metry, 31 (60.78%) males and 20 (39.21%) females, aged 2-12 years (mean age, 7.43 years), comprised the study group. All patients answered a detailed frequency questionnaire related to regurgitation and acidic foods and participated in a clinical dental examination...
April 2018: Journal of the Indian Society of Pedodontics and Preventive Dentistry
https://www.readbyqxmd.com/read/29901547/a-multifaceted-intervention-to-reduce-pediatric-acid-suppressant-prescriptions-for-ger-what-have-we-learned
#5
Nina F Steutel, Miranda W Langendam, Marc A Benninga, Minke E P Jansen, Merit M Tabbers
OBJECTIVE: Acid-suppressant prescriptions for children have increased over past decades, despite guideline recommendations to prescribe prudently. Acid-suppressants are often ineffective and may lead to side-effects. We aimed to reduce inappropriate acid-suppressant prescriptions for gastro-esophageal reflux (GER) in a tertiary care setting through active implementation of national guideline recommendations and to evaluate intervention effect. METHODS: Implementation consisted of two steps...
June 12, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29899372/developmental-epileptic-encephalopathy-with-hypomyelination-and-brain-atrophy-associated-with-ptpn23-variants-affecting-the-assembly-of-usnrnps
#6
Robert Smigiel, Gerd Landsberg, Maximilian Schilling, Małgorzata Rydzanicz, Agnieszka Pollak, Anna Walczak, Anna Stodolak, Piotr Stawinski, Hanna Mierzewska, Maria M Sasiadek, Oliver J Gruss, Rafal Ploski
PTPN23 encodes a ubiquitously expressed non-receptor type, catalytically inactive protein-tyrosine phosphatase found in all cells including neurons. Recently, we have identified PTPN23 in a cellular screen for the systematic identification of novel regulators of survival motor neuron (SMN) function in the assembly of splicing factors (Uridine-rich small nuclear ribonucleoproteins, UsnRNPs). Based on three families, recessive PTPN23 variants have been associated with human disease tentatively, without functional studies...
June 13, 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29868522/the-role-of-proton-pump-inhibitors-in-the-management-of-pediatric-eosinophilic-esophagitis
#7
REVIEW
Carolina Gutiérrez-Junquera, Sonia Fernández-Fernández, M Luz Cilleruelo, Ana Rayo, Enriqueta Román
Eosinophilic esophagitis (EoE) is a chronic, local, immune-mediated disorder characterized by symptoms of esophageal dysfunction and the presence of a dense eosinophilic infiltrate in the esophageal mucosa. Consensus diagnostic recommendations for EoE diagnosis included absence of histological response to a proton-pump inhibitor (PPI) trial, to exclude gastro-oesophageal reflux disease (GERD)-associated esophagitis. This recommendation exposed an entity known as "proton pump inhibitor-responsive esophageal eosinophilia" (PPI-REE), which refers to patients with EoE phenotype who are PPI-responsive and do not present GERD...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29848349/the-majority-of-patients-with-newly-diagnosed-juvenile-idiopathic-arthritis-achieve-a-health-related-quality-of-life-that-is-similar-to-that-of-healthy-peers-results-of-the-german-multicenter-inception-cohort-icon
#8
Miriam Listing, Kirsten Mönkemöller, Ina Liedmann, Martina Niewerth, Claudia Sengler, Joachim Listing, Dirk Foell, Arnd Heiligenhaus, Ariane Klein, Gerd Horneff, Gerd Ganser, Johannes-Peter Haas, Jens Klotsche, Kirsten Minden
BACKGROUND: Achieving the best possible health-related quality of life (HRQoL) for a patient is an important treatment goal in juvenile idiopathic arthritis (JIA). We investigated the 36-month trajectories of HRQoL in children with JIA compared with healthy peers and identified the predictors of an unfavorable HRQoL. METHODS: Patients with a recent JIA diagnosis were enrolled in the German inception cohort study ICON. As a peer group, friends of patients of the same age and sex were asked to cooperate...
May 30, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29523922/a-novel-mechanism-for-improved-exercise-performance-in-pediatric-fontan-patients-after-cardiac-rehabilitation
#9
Samuel Wittekind, Wayne Mays, Yvette Gerdes, Sandra Knecht, John Hambrook, William Border, John Lynn Jefferies
Patients with a Fontan circulation have impaired exercise capacity. Cardiac rehabilitation (CR) has shown promise in enhancing peak exercise parameters in this population, but an improvement in submaximal exercise has not been consistently demonstrated. We assessed the hypothesis that participation in CR will be associated with more efficient oxygen extraction and ventilation during submaximal exercise. In this prospective study, pediatric Fontans completed two 60 min CR sessions per week for 12 weeks. Cardiopulmonary exercise testing and stress echocardiography were performed at baseline and last CR session, and then compared with a paired sample t test...
June 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29494321/clinical-practice-guideline-hoarseness-dysphonia-update
#10
Robert J Stachler, David O Francis, Seth R Schwartz, Cecelia C Damask, German P Digoy, Helene J Krouse, Scott J McCoy, Daniel R Ouellette, Rita R Patel, Charles Charlie W Reavis, Libby J Smith, Marshall Smith, Steven W Strode, Peak Woo, Lorraine C Nnacheta
Objective This guideline provides evidence-based recommendations on treating patients who present with dysphonia, which is characterized by altered vocal quality, pitch, loudness, or vocal effort that impairs communication and/or quality of life. Dysphonia affects nearly one-third of the population at some point in its life. This guideline applies to all age groups evaluated in a setting where dysphonia would be identified or managed. It is intended for all clinicians who are likely to diagnose and treat patients with dysphonia...
March 2018: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29494316/clinical-practice-guideline-hoarseness-dysphonia-update-executive-summary
#11
Robert J Stachler, David O Francis, Seth R Schwartz, Cecelia C Damask, German P Digoy, Helene J Krouse, Scott J McCoy, Daniel R Ouellette, Rita R Patel, Charles Charlie W Reavis, Libby J Smith, Marshall Smith, Steven W Strode, Peak Woo, Lorraine C Nnacheta
Objective This guideline provides evidence-based recommendations on treating patients presenting with dysphonia, which is characterized by altered vocal quality, pitch, loudness, or vocal effort that impairs communication and/or quality of life. Dysphonia affects nearly one-third of the population at some point in its life. This guideline applies to all age groups evaluated in a setting where dysphonia would be identified or managed. It is intended for all clinicians who are likely to diagnose and treat patients with dysphonia...
March 2018: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29491751/infantile-gastroesophageal-reflux-adherence-to-treatment-guidelines-in-the-hospital-setting
#12
Heather A Long, Lauren Solski, Jill A Rebuck, Colin Bridgeman
OBJECTIVES: Recent guidelines defined and differentiated the management of gastroesophageal reflux (GER) and gastroesophageal reflux disease (GERD). The guidelines recommend against using empiric acid suppression therapy for infantile GER. The primary objective of this study was to assess inpatient guideline adherence regarding management of infantile GER through the perspective of pharmacists. Secondary objectives included assessing pharmacist comfort level with differentiation between GER and GERD, observing current trends in practice relating to the primary objective, and determining the availability of institution-specific guidelines that address the management of infantile GER...
January 2018: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29481737/subcutaneous-abatacept-in-patients-with-polyarticular-course-juvenile-idiopathic-arthritis-results-from-a-phase-iii-open-label-study
#13
Hermine I Brunner, Nikolay Tzaribachev, Gabriel Vega-Cornejo, Ingrid Louw, Alberto Berman, Inmaculada Calvo Penadés, Jordi Antón, Francisco Ávila-Zapata, Rubén Cuttica, Gerd Horneff, Ivan Foeldvari, Vladimir Keltsev, Daniel J Kingsbury, Diego Oscar Viola, Rik Joos, Bernard Lauwerys, Maria Eliana Paz Gastañaga, Maria Elena Rama, Carine Wouters, John Bohnsack, Johannes Breedt, Michel Fischbach, Thomas Lutz, Kirsten Minden, Tatiana Miraval, Mahmood M T M Ally, Nadina Rubio-Pérez, Elisabeth Solau Gervais, Riana van Zyl, Xiaohui Li, Marleen Nys, Robert Wong, Subhashis Banerjee, Daniel J Lovell, Alberto Martini, Nicolino Ruperto
OBJECTIVE: To investigate the pharmacokinetics, effectiveness, and safety of subcutaneous (SC) abatacept treatment over 24 months in patients with polyarticular-course juvenile idiopathic arthritis (JIA). METHODS: In this phase III, open-label, international, multicenter, single-arm study, patients with polyarticular JIA (cohort 1, ages 6-17 years and cohort 2, ages 2-5 years) in whom treatment with ≥1 disease-modifying antirheumatic drug was unsuccessful received weight-tiered SC abatacept weekly: 10 to <25 kg (50 mg), 25 to <50 kg (87...
July 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29470322/pediatric-gastroesophageal-reflux-clinical-practice-guidelines-joint-recommendations-of-the-north-american-society-for-pediatric-gastroenterology-hepatology-and-nutrition-and-the-european-society-for-pediatric-gastroenterology-hepatology-and-nutrition
#14
Rachel Rosen, Yvan Vandenplas, Maartje Singendonk, Michael Cabana, Carlo DiLorenzo, Frederic Gottrand, Sandeep Gupta, Miranda Langendam, Annamaria Staiano, Nikhil Thapar, Neelesh Tipnis, Merit Tabbers
This document serves as an update of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) 2009 clinical guidelines for the diagnosis and management of gastroesophageal reflux disease (GERD) in infants and children and is intended to be applied in daily practice and as a basis for clinical trials. Eight clinical questions addressing diagnostic, therapeutic and prognostic topics were formulated...
March 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29466083/laparoscopic-nissen-fundoplication-an-excellent-treatment-of-gerd-related-respiratory-symptoms-in-children-results-of-a-multicentric-study
#15
Ciro Esposito, Amulya Saxena, Sabine Irtan, Holger Till, Maria Escolino
INTRODUCTION: Respiratory manifestations of gastroesophageal reflux disease (GERD), particularly chronic cough, are being recognized with increased frequency in children. This survey aimed to investigate the efficacy of laparoscopic Nissen fundoplication for treatment of GERD-related respiratory symptoms not responsive to medical therapy in neurological normal children. MATERIALS AND METHODS: We collected data of children with GERD-related respiratory complaints not responsive to medical therapy who underwent laparoscopic Nissen fundoplication in four European centers of Pediatric Surgery over a 10-year period...
February 21, 2018: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/29442203/cyclic-vomiting-syndrome-epidemiology-diagnosis-and-treatment
#16
REVIEW
Sanjay Bhandari, Pinky Jha, Abhishek Thakur, Abhipsa Kar, Harrison Gerdes, Thangam Venkatesan
Cyclic-vomiting syndrome (CVS) is a chronic functional gastrointestinal disorder characterized by recurrent episodes of nausea and vomiting. Although once thought to be a pediatric disorder, there has been a considerable increase in recognition of CVS in adults. The exact pathogenesis is unknown and several theories have been proposed. Migraine and CVS share a similar pathophysiology as suggested by several studies. Since there are no specific biomarkers available for this disorder, physicians should rely on Rome criteria for the diagnosis...
April 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/29439587/clinical-management-of-pediatric-achalasia
#17
Marinde van Lennep, Michiel P van Wijk, Taher I M Omari, Marc A Benninga, Maartje M J Singendonk
Achalasia is a rare esophageal motility disorder. Much of the literature is based on the adult population. In adults, guidance of therapeutic approach by manometric findings has led to improvement in patient outcome. Promising results have been achieved with novel therapies such as PerOral Endoscopic Myotomy (POEM). Areas covered: In this review, we provide an overview of the novel diagnostic and therapeutic tools for achalasia management and in what way they will relate to the future management of pediatric achalasia...
April 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29413212/gastroesophageal-reflux-regurgitation-in-the-infant-population
#18
REVIEW
Teresa D Ferguson
Gastroesophageal reflux (GER) is common in infancy and mainly treated through nonpharmacological interventions. Knowing the early warning signs of GER is important for nursing assessment. Untreated GER can become acute when an infant fails to gain weight and has recurrent, forceful vomiting. Further investigation of gastroesophageal reflux disease (GERD) is indicated when failure to gain weight, irritability, swallowing difficulties, regurgitation, and respiratory complications occur and should trigger referral to pediatric specialists...
March 2018: Critical Care Nursing Clinics of North America
https://www.readbyqxmd.com/read/29394212/survey-on-the-adherence-to-the-2009-naspghan-espghan-gastroesophageal-reflux-guidelines-by-brazilian-paediatricians
#19
Sarah Cristina Fontes Vieira, Flavia Matos Gurgel, Maria Zuleide Leão, Tiê Emídio Costa-Silva, Ikaro Daniel de Carvalho Barreto, Paulo Ricardo Saquete Martins-Filho, Mário César Vieira, Ricardo Queiroz Gurgel
OBJECTIVE: The aim of the study was to evaluate the management of gastroesophageal reflux in children among Brazilian pediatricians and adherence to the 2009 North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN), and European Society of Gastroenterology, Hepatology, and Nutrition (ESPGHAN) Guideline in Brazil. METHODS: An observational cross-sectional study was conducted, applying a standard questionnaire with 12 questions about gastroesophageal reflux (GER) and gastroesophageal reflux disease (GERD) management in infants, children, and adolescents to the pediatricians during the 37th Brazilian Pediatrics Congress in October, 2015...
July 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29391259/comparative-genomics-analysis-of-clostridium-difficile-epidemic-strain-dh-nap11-106
#20
Larry K Kociolek, Dale N Gerding, David W Hecht, Egon A Ozer
Clostridium difficile PCR ribotype 106 (also identified as restriction endonuclease analysis [REA] group DH) recently emerged as the most common strain causing C. difficile infection (CDI) among US adults. We previously identified this strain predominating our pediatric cohort. Pediatric clinical CDI isolates previously characterized by REA underwent antibiotic resistance testing and whole genome sequencing. Of 134 isolates collected from children, 31 (23%) were REA group DH. We performed a comparative genomics analysis to identify DH-associated accessory genes...
April 2018: Microbes and Infection
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