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Children's hospital of philadelphia

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https://www.readbyqxmd.com/read/28219061/establishing-prenatal-surgery-for-myelomeningocele-in-asia-the-singapore-consensus
#1
Tuangsit Wataganara, Suresh Seshadri, Tak Yeung Leung, Citra Matter, Monawat Ngerncham, Namtip Triyasunant, Padmakar Vidyadhar Mali, Agnhitori Biswas, Katika Nawapun, Nisarat Phithakwatchara, Alan W Flake, Mark P Johnson, Arijit Biswas, Mahesh Choolani
OBJECTIVES: The Management of Myelomeningocele Study (MOMS Trial) has inspired many fetal therapy centers (FTCs) to offer open fetal surgery for myelomeningocele (MMC). This is an initial effort to create a candidate model that can be applicable to many parts of Asia. MATERIAL AND METHODS: A limited selection of specialists from 4 established FTCs in Thailand, Hong Kong, India, and Singapore met for a round table discussion. Experts from Children's Hospital of Philadelphia (CHOP) involved in the Trial moderated the session...
February 21, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28148316/design-and-initial-results-of-a-programme-for-routine-standardised-longitudinal-follow-up-after-congenital-heart-surgery
#2
Sara K Pasquali, Chitra Ravishankar, Jennifer C Romano, Kristin Kane, Suzanne Viers, Andrea Kennedy, Nancy Burnham, Ray Lowery, Karen Uzark, Lauren Retzloff, Jonathon J Rome, Joseph W Rossano, John R Charpie, Thomas L Spray, Michael G Gaies, Richard G Ohye, J William Gaynor
BACKGROUND: With improvements in early survival following congenital heart surgery, it has become increasingly important to understand longer-term outcomes; however, routine collection of these data is challenging and remains very limited. We describe the development and initial results of a collaborative programme incorporating standardised longitudinal follow-up into usual care at the Children's Hospital of Philadelphia (CHOP) and University of Michigan (UM). METHODS: We included children undergoing benchmark operations of the Society of Thoracic Surgeons...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28140383/pavlik-harness-disease-revisited-does-prolonged-treatment-of-a-dislocated-hip-in-a-harness-adversely-affect-the-%C3%AE-angle-study-conducted-at-the-children-s-hospital-of-philadelphia-in-conjunction-with-the-international-hip-dysplasia-institute
#3
Alex L Gornitzky, Emily K Schaeffer, Charles T Price, Wudbhav N Sankar
BACKGROUND: Current dogma contends that prolonged treatment of a dislocated hip in Pavlik harness beyond 3 weeks will cause "Pavlik harness disease." To our knowledge, however, no previous studies have documented objective morphologic changes to the acetabulum from continued treatment of a persistently dislocated hip. METHODS: We retrospectively reviewed a consecutive series of infants with developmental dysplasia of the hip, below 6 months old, who failed Pavlik treatment from a single, tertiary-care pediatric hospital and a multicenter, international study group...
June 27, 2016: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28114639/incidence-of-recurrence-in-posterior-circulation-childhood-arterial-ischemic-stroke
#4
Michael Y Uohara, Lauren A Beslow, Lori Billinghurst, Brianna M Jones, Sudha K Kessler, Daniel J Licht, Rebecca N Ichord
Importance: Childhood arterial ischemic stroke (CAIS) affects approximately 1.6 per 100 000 children per year, while stroke recurs in up to 20% of patients at 5 years. Factors determining the risk of recurrence are incompletely understood. Objective: To investigate the incidence of the recurrence of CAIS in the posterior and anterior circulations to determine if the risk differs between the 2 locations. Design, Setting, and Participants: A retrospective analysis of CAIS was conducted among children enrolled in a single-center prospective consecutive cohort at The Children's Hospital of Philadelphia between January 1, 2006, and January 1, 2015...
January 23, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28112025/validation-of-the-use-of-electronic-health-records-for-classification-of-adhd-status
#5
Siobhan M Gruschow, Benjamin E Yerys, Thomas J Power, Dennis R Durbin, Allison E Curry
OBJECTIVE: To validate an electronic health record (EHR)-based algorithm to classify ADHD status of pediatric patients. METHOD: As part of an applied study, we identified all primary care patients of The Children's Hospital of Philadelphia [CHOP] health care network who were born 1987-1995 and residents of New Jersey. Patients were classified with ADHD if their EHR indicated an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) diagnosis code of "314...
October 1, 2016: Journal of Attention Disorders
https://www.readbyqxmd.com/read/28056115/a-tiered-approach-to-retinopathy-of-prematurity-screening-tarp-using-a-weight-gain-predictive-model-and-a-telemedicine-system
#6
Jaclyn Gurwin, Lauren A Tomlinson, Graham E Quinn, Gui-Shuang Ying, Agnieshka Baumritter, Gil Binenbaum
Importance: The Telemedicine Approaches to Evaluating Acute-Phase Retinopathy of Prematurity (e-ROP) Study telemedicine system of remote fundus image grading and The Children's Hospital of Philadelphia Retinopathy of Prematurity (CHOP-ROP) postnatal weight gain predictive model are 2 approaches for improving ROP screening efficiency. Current screening has low specificity for severe ROP. Objective: To describe a tiered approach to ROP screening (TARP) for identifying children who develop severe ROP using telemedicine and a predictive model synergistically...
January 5, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28045769/diverting-ileostomy-for-the-treatment-of-severe-refractory-pediatric-inflammatory-bowel-disease
#7
Elizabeth C Maxwell, Noor Dawany, Robert N Baldassano, Petar Mamula, Peter Mattei, Lindsey Albenberg, Judith R Kelsen
OBJECTIVES: Diverting ileostomy is used as a temporizing therapy in patients with perianal Crohn disease; however, little data exist regarding its use for colonic disease. The primary aim of the present study was to determine the role of diversion in severe refractory colonic inflammatory bowel disease (IBD) in a pediatric population. METHODS: Retrospective study of patients who underwent diverting ileostomy at The Children's Hospital of Philadelphia from 2000 to 2014 for the management of severe, refractory colonic IBD...
December 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28033084/effects-of-exercise-restriction-on-patients-with-anomalous-aortic-origin-of-a-coronary-artery
#8
Matthew D Elias, James Meza, Brian W McCrindle, Julie A Brothers, Stephen Paridon, Meryl S Cohen
BACKGROUND: Management of young patients with anomalous aortic origin of a coronary artery (AAOCA) may involve exercise restriction. We sought to identify the association of exercise restriction with changes over time in body mass index (BMI) and exercise capacity in this cohort. METHODS: We performed a retrospective review of patients with AAOCA seen at The Children's Hospital of Philadelphia between January 1, 1998, and August 31, 2014. Linear mixed model repeated-measures analysis assessed changes in BMI and exercise capacity...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28009100/rare-copy-number-variants-in-a-population-based-investigation-of-hypoplastic-right-heart-syndrome
#9
Aggeliki Dimopoulos, Robert J Sicko, Denise M Kay, Shannon L Rigler, Charlotte M Druschel, Michele Caggana, Marilyn L Browne, Ruzong Fan, Paul A Romitti, Lawrence C Brody, James L Mills
BACKGROUND: Hypoplastic right heart syndrome (HRHS) is a rare congenital defect characterized by underdevelopment of the right heart structures commonly accompanied by an atrial septal defect. Familial HRHS reports suggest genetic factor involvement. We examined the role of copy number variants (CNVs) in HRHS. METHODS: We genotyped 32 HRHS cases identified from all New York State live births (1998-2005) using Illumina HumanOmni2.5 microarrays. CNVs were called with PennCNV and prioritized if they were ≥20 Kb, contained ≥10 SNPs and had minimal overlap with CNVs from in-house controls, the Database of Genomic Variants, HapMap3, and Childrens Hospital of Philadelphia database...
December 23, 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
https://www.readbyqxmd.com/read/27989738/incidence-characteristics-and-survival-following-cardiopulmonary-resuscitation-in-the-quaternary-neonatal-intensive-care-unit
#10
Elizabeth E Foglia, Robert Langeveld, Lauren Heimall, Alyson Deveney, Anne Ades, Erik A Jensen, Vinay M Nadkarni
BACKGROUND: The contemporary characteristics and outcomes of cardiopulmonary resuscitation (CPR) in the neonatal intensive care unit (NICU) are poorly described. The objectives of this study were to determine the incidence, interventions, and outcomes of CPR in a quaternary referral NICU. METHODS: Retrospective observational study of infants who received chest compressions for resuscitation in the Children's Hospital of Philadelphia NICU between April 1, 2011 and June 30, 2015...
October 27, 2016: Resuscitation
https://www.readbyqxmd.com/read/27984153/incidence-characteristics-and-survival-following-cardiopulmonary-resuscitation-in-the-quaternary-neonatal-intensive-care-unit
#11
Elizabeth E Foglia, Robert Langeveld, Lauren Heimall, Alyson Deveney, Anne Ades, Erik A Jensen, Vinay M Nadkarni
BACKGROUND: The contemporary characteristics and outcomes of cardiopulmonary resuscitation (CPR) in the neonatal intensive care unit (NICU) are poorly described. The objectives of this study were to determine the incidence, interventions, and outcomes of CPR in a quaternary referral NICU. METHODS: Retrospective observational study of infants who received chest compressions for resuscitation in the Children's Hospital of Philadelphia NICU between April 1, 2011 and June 30, 2015...
January 2017: Resuscitation
https://www.readbyqxmd.com/read/27967291/novel-hypoglycemia-phenotype-in-congenital-hyperinsulinism-due-to-dominant-mutations-of-uncoupling-protein-2-ucp2
#12
Christine T Ferrara, Kara E Boodhansingh, Elenora Paradies, Fiermonte Giuseppe, Linda J Steinkrauss, Lisa Swartz Topor, Jose Bernardo Quintos, Arupa Ganguly, Diva D De Leon, Ferdinando Palmieri, Charles A Stanley
CONTEXT: The rarest genetic form of congenital hyperinsulinism (HI) has been associated with dominant inactivating mutations in uncoupling protein 2 (UCP2), a mitochondrial inner membrane carrier that modulates the oxidation of glucose vs amino acids. OBJECTIVE: To evaluate the frequency of UCP2 mutations in children with HI and phenotypic features of this form of HI. DESIGN: We examined 211 children with diazoxide-responsive HI seen at The Children's Hospital of Philadelphia (CHOP) between 1997 and October 2016...
December 14, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27939059/treatment-of-infantile-onset-spinal-muscular-atrophy-with-nusinersen-a-phase-2-open-label-dose-escalation-study
#13
Richard S Finkel, Claudia A Chiriboga, Jiri Vajsar, John W Day, Jacqueline Montes, Darryl C De Vivo, Mason Yamashita, Frank Rigo, Gene Hung, Eugene Schneider, Daniel A Norris, Shuting Xia, C Frank Bennett, Kathie M Bishop
BACKGROUND: Nusinersen is a 2'-O-methoxyethyl phosphorothioate-modified antisense drug being developed to treat spinal muscular atrophy. Nusinersen is specifically designed to alter splicing of SMN2 pre-mRNA and thus increase the amount of functional survival motor neuron (SMN) protein that is deficient in patients with spinal muscular atrophy. METHODS: This open-label, phase 2, escalating dose clinical study assessed the safety and tolerability, pharmacokinetics, and clinical efficacy of multiple intrathecal doses of nusinersen (6 mg and 12 mg dose equivalents) in patients with infantile-onset spinal muscular atrophy...
17, 2016: Lancet
https://www.readbyqxmd.com/read/27921214/external-validity-of-the-chiari-severity-index-and-outcomes-among-pediatric-chiari-i-patients-treated-with-intra-or-extra-dural-decompression
#14
Jared M Pisapia, Maxwell B Merkow, Danielle Brewington, Rosemary E Henn, Leslie N Sutton, Phillip B Storm, Gregory G Heuer
INTRODUCTION: Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI. METHODS: We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children's hospital...
December 5, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27876583/treatment-of-pediatric-forearm-midshaft-fractures-is-there-a-difference-between-types-of-orthopedic-surgeon
#15
D Lu, Z Lin, J-D Zhang, H Chen, L-J Sun
PURPOSE OF THE STUDY: The objective of this study was to compare the clinical and radiological outcomes of pediatric forearm midshaft fractures treated operatively with titanium elastic nails (TENs) by pediatric orthopedists and non-pediatric orthopedists. MATERIAL AND METHODS: We conducted a prospective cohort study of 88 children of forearm midshaft fractures, who were randomized to operative stabilization either by pediatric orthopedists (Group A, 44 cases) or by non-pediatric orthopedists (Group B, 44 cases) from April 2013 to February 2014...
November 19, 2016: Orthopaedics & Traumatology, Surgery & Research: OTSR
https://www.readbyqxmd.com/read/27763634/phenotypic-predictors-and-final-diagnoses-in-patients-referred-for-rasopathy-testing-by-targeted-next-generation-sequencing
#16
Elizabeth J Bhoj, Zhenming Yu, Qiaoning Guan, Rebecca Ahrens-Nicklas, Kajia Cao, Minjie Luo, Tanya Tischler, Matthew A Deardorff, Elaine Zackai, Avni B Santani
INTRODUCTION: RASopathies include disorders generally characterized by developmental delay, specific heart defects, short stature, cardiac hypertrophy, and facial dysmorphisms. Next-generation sequencing (NGS)-based panels have widespread acceptance as a diagnostic tool for RASopathies. MATERIALS AND METHODS: The first 126 patients evaluated by clinical examination and the NGS RASopathy panel at the Children's Hospital of Philadelphia were enrolled. We calculated diagnosis rate, correlated reported clinical findings with positive or negative test results, and identified final molecular diagnoses in 28/96 patients who tested negative for RASopathies...
October 20, 2016: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/27681852/emergency-preparedness-for-a-mass-gathering-the-2015-papal-visit-to-philadelphia
#17
Emma Paras, Matthew Butler, Brian F Maguire, Rich Scarfone
In September 2015, Pope Francis visited Philadelphia, Pennsylvania, and led outdoor events over the course of 2 days. As a result, The Children's Hospital of Philadelphia (CHOP) was faced with the challenge of planning for and responding to a mass gathering that was anticipated to be one of the largest in US history. As a result of unprecedented traffic restrictions, it was anticipated that patients, staff, and vendors would have severe challenges accessing the hospital, along with impaired communications from widespread cell phone service disruption...
September 29, 2016: Disaster Medicine and Public Health Preparedness
https://www.readbyqxmd.com/read/27670804/medical-and-psychological-factors-related-to-pain-in-adults-with-pediatric-onset-spinal-cord-injury-a-biopsychosocial-model
#18
C B Murray, K Zebracki, K M Chlan, A C Moss, L C Vogel
STUDY DESIGN: A cross-sectional study. OBJECTIVES: The aim of this study is to determine medical and psychological correlates of pain in individuals with pediatric-onset spinal cord injury (SCI). SETTING: Shriners Hospitals for Children-Chicago, Philadelphia and Northern California (USA). METHODS: A total of 187 adults who had sustained an injury before 19 years of age completed interviews that included medical information, standardized measures of psychological functioning (Beck Anxiety Inventory and Patient Health Questionnaire) and a comprehensive pain questionnaire to assess the location, frequency, intensity and duration of pain and distress and disability related to pain...
September 27, 2016: Spinal Cord
https://www.readbyqxmd.com/read/27647117/improving-access-to-vision-screening-in-urban-philadelphia-elementary-schools
#19
Lisa A Hark, Eileen L Mayro, Judie Tran, Michael Pond, Rachel Schneider, Justin Torosian, Melanie Snitzer, Nooreen Dabbish, Alex V Levin
BACKGROUND: The Wills Eye Vision Screening Program for Children is a community-based vision screening program for children in urban Philadelphia elementary schools that aims to provide vision screening, remedy refractive error by providing glasses, and refer children with suspected nonrefractive eye disease for eye care. METHODS: Children in grades K-5 from 45 Philadelphia elementary schools were screened for distance and near visual acuity, stereopsis, and color vision from January 2014 to June 2015...
September 16, 2016: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/27631740/pediatric-asthma-care-coordination-in-underserved-communities-a-quasiexperimental-study
#20
Mary R Janevic, Shelley Stoll, Margaret Wilkin, Peter X K Song, Alan Baptist, Marielena Lara, Gilberto Ramos-Valencia, Tyra Bryant-Stephens, Victoria Persky, Kimberly Uyeda, Julie Kennedy Lesch, Wen Wang, Floyd J Malveaux
OBJECTIVES: To assess the effect of care coordination on asthma outcomes among children in underserved urban communities. METHODS: We enrolled children, most of whom had very poorly or not well-controlled asthma, in medical-social care coordination programs in Los Angeles, California; Chicago, Illinois; Philadelphia, Pennsylvania; and San Juan, Puerto Rico in 2011 to 2014. Participants (n = 805; mean age = 7 years) were 60% male, 50% African American, and 42% Latino...
November 2016: American Journal of Public Health
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