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https://www.readbyqxmd.com/read/29141082/diagnostic-accuracy-of-pediatric-teledermatology-using-parent-submitted-photographs-a-randomized-clinical-trial
#1
Daniel M O'Connor, Olivia S Jew, Marissa J Perman, Leslie A Castelo-Soccio, Flaura K Winston, Patrick J McMahon
Importance: Advances in smartphone photography (both quality and image transmission) may improve access to care via direct parent-to-clinician telemedicine. However, the accuracy of diagnoses that are reliant on parent-provided photographs has not been formally compared with diagnoses made in person. Objective: To assess whether smartphone photographs of pediatric skin conditions taken by parents are of sufficient quality to permit accurate diagnosis. Design, Setting, and Participants: A prospective study was conducted among 40 patient-parent dyads at a pediatric dermatology clinic at the Children's Hospital of Philadelphia from March 1 to September 30, 2016, to assess concordance between diagnoses made by an independent pediatric dermatologist based on in-person examination and those based on parental photographs...
November 15, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/29112080/%C3%AE-lactam-therapeutic-drug-management-in-the-picu
#2
Jeffrey J Cies, Wayne S Moore, Adela Enache, Arun Chopra
OBJECTIVES: To determine whether contemporary β-lactam anti-infective dosing recommendations in critically ill children achieve concentrations associated with maximal anti-infective activity. The secondary objective was to describe the microbiological and clinical outcomes associated with β-lactam therapeutic drug management. DESIGN: Electronic Medical Record Review. SETTING: A 189-bed, freestanding children's tertiary care teaching hospital in Philadelphia, PA...
November 3, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/29091557/single-dose-gene-replacement-therapy-for-spinal-muscular-atrophy
#3
Jerry R Mendell, Samiah Al-Zaidy, Richard Shell, W Dave Arnold, Louise R Rodino-Klapac, Thomas W Prior, Linda Lowes, Lindsay Alfano, Katherine Berry, Kathleen Church, John T Kissel, Sukumar Nagendran, James L'Italien, Douglas M Sproule, Courtney Wells, Jessica A Cardenas, Marjet D Heitzer, Allan Kaspar, Sarah Corcoran, Lyndsey Braun, Shibi Likhite, Carlos Miranda, Kathrin Meyer, K D Foust, Arthur H M Burghes, Brian K Kaspar
BACKGROUND: Spinal muscular atrophy type 1 (SMA1) is a progressive, monogenic motor neuron disease with an onset during infancy that results in failure to achieve motor milestones and in death or the need for mechanical ventilation by 2 years of age. We studied functional replacement of the mutated gene encoding survival motor neuron 1 (SMN1) in this disease. METHODS: Fifteen patients with SMA1 received a single dose of intravenous adeno-associated virus serotype 9 carrying SMN complementary DNA encoding the missing SMN protein...
November 2, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29080380/nutritional-risk-factors-predict-severe-acute-graft-versus-host-disease-and-early-mortality-in-pediatric-allogeneic-hematopoietic-stem-cell-transplantation
#4
Eva H Kerby, Yimei Li, Kelly D Getz, Elizabeth C Smith, Laura T Smith, Nancy J Bunin, Alix E Seif
BACKGROUND: Malnutrition is a pro-inflammatory state, yet data on nutritional risk factors and development of acute graft-versus-host disease (aGVHD) are extremely limited. PROCEDURE: We conducted a retrospective cohort analysis of pediatric patients up to age 21 years who underwent allogeneic hematopoietic stem cell transplantation (HSCT) at the Children's Hospital of Philadelphia from January 2011 to September 2014 to determine whether malnutrition was associated with development of aGVHD and early mortality...
October 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29059263/effect-of-financial-incentives-on-glucose-monitoring-adherence-and-glycemic-control-among-adolescents-and-young-adults-with-type-1-diabetes-a-randomized-clinical-trial
#5
Charlene A Wong, Victoria A Miller, Kathryn Murphy, Dylan Small, Carol A Ford, Steven M Willi, Jordyn Feingold, Alexander Morris, Yoonhee P Ha, Jingsan Zhu, Wenli Wang, Mitesh S Patel
Importance: Glycemic control often deteriorates during adolescence and the transition to young adulthood for patients with type 1 diabetes. The inability to manage type 1 diabetes effectively during these years is associated with poor glycemic control and complications from diabetes in adult life. Objective: To determine the effect of daily financial incentives on glucose monitoring adherence and glycemic control in adolescents and young adults with type 1 diabetes...
October 23, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/29021134/nih-funds-pediatric-data-resource-center
#6
(no author information available yet)
Children's Hospital of Philadelphia will lead a collaborative effort-funded with $14.8 million from the NIH-to pool genomic and phenotypic data from tens of thousands of patients to study the causes of pediatric cancer and structural birth defects.
October 11, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28922265/-stuck-in-the-icu-caring-for-children-with-chronic-critical-illness
#7
Carrie M Henderson, Erin P Williams, Miriam C Shapiro, Emily Hahn, Laura Wright-Sexton, Nancy Hutton, Renee D Boss
OBJECTIVE: Neonatal ICUs and PICUs increasingly admit patients with chronic critical illness: children whose medical complexity leads to recurrent and prolonged ICU hospitalizations. We interviewed participants who routinely care for children with chronic critical illness to describe their experiences with ICU care for pediatric chronic critical illness. DESIGN: Semi-structured interviews. Interviews were transcribed and analyzed for themes. SETTING: Stakeholders came from five regions (Seattle, WA; Houston, TX; Jackson, MS; Baltimore, MD; and Philadelphia, PA)...
September 15, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28917444/developing-a-hospital-wide-fertility-preservation-service-for-pediatric-and-young-adult-patients
#8
Claire A Carlson, Thomas F Kolon, Peter Mattei, Wendy Hobbie, Clarisa R Gracia, Sue Ogle, Jill P Ginsberg
PURPOSE: Gonadal damage is a common consequence of treatment for pediatric malignancies. Nononcologic conditions may also utilize treatments with potential impact on fertility. Models for oncology fertility preservation programs have emerged and demonstrate that a multidisciplinary team approach can have a positive impact on referral patterns, appropriate risk counseling, and access to fertility preservation options. Expansion of programmatic breadth is needed, providing improved care to nonmalignant conditions where the disease itself may impact reproductive health or treatment modalities...
September 13, 2017: Journal of Adolescent Health: Official Publication of the Society for Adolescent Medicine
https://www.readbyqxmd.com/read/28916010/competencies-and-training-guidelines-for-behavioral-health-providers-in-pediatric-primary-care
#9
REVIEW
Wanjiku F M Njoroge, Ariel A Williamson, Jennifer A Mautone, Paul M Robins, Tami D Benton
This article focuses on the cross-discipline training competencies needed for preparing behavioral health providers to implement integrated primary care services. After a review of current competencies in the disciplines of child and adolescent psychiatry, psychology, and social work, cross-cutting competencies for integrated training purposes are identified. These competencies are comprehensive and broad and can be modified for use in varied settings and training programs. An existing and successful integrated care training model, currently implemented at Children's Hospital of Philadelphia, is described...
October 2017: Child and Adolescent Psychiatric Clinics of North America
https://www.readbyqxmd.com/read/28886309/the-correlation-between-pax5-deletion-and-patients-survival-in-iranian-children-with-precursor-b-cell-acute-lymphocytic-leukemia
#10
A Moafi, A Zojaji, R Salehi, S Najafi Dorcheh, S Rahgozar
Despite advances in treatment, children with acute lymphoblastic leukemia (ALL) still experience drug resistance and relapse. Several gene mutations are involved in the onset of this disease and resistance to therapy. The present study examines the incidence of IKZF1, CDKN2A/B, PAX5, EBF1, ETV6, BTG1, RB1, JAK2, and Xp22.33 gene deletions/duplications associated with pediatric ALL in Iran and investigates the possible effect of these mutations on drug resistance. Three-year disease-free survival (3DFS) was evaluated for children diagnosed with Philadelphia negative precursor-B-cell ALL hospitalized at Sayed-al-Shohada Hospital, Isfahan-Iran, from January 2009 until December 2012...
August 30, 2017: Cellular and Molecular Biology
https://www.readbyqxmd.com/read/28873224/disparities-in-prognosis-communication-among-parents-of-children-with-cancer-the-impact-of-race-and-ethnicity
#11
Maya F Ilowite, Angel M Cronin, Tammy I Kang, Jennifer W Mack
BACKGROUND: Most parents of children with cancer say they want detailed information about their child's prognosis. However, prior work has been conducted in populations of limited diversity. The authors sought to evaluate the impact of parental race/ethnicity on prognosis communication experiences among parents of children with cancer. METHODS: In total, 357 parents of children with cancer and the children's physicians were surveyed at Dana-Farber Cancer Institute/Boston Children's Hospital and Children's Hospital of Philadelphia...
October 15, 2017: Cancer
https://www.readbyqxmd.com/read/28832883/association-between-mitochondrial-dna-haplogroup-variation-and-autism-spectrum-disorders
#12
Dimitra Chalkia, Larry N Singh, Jeremy Leipzig, Maria Lvova, Olga Derbeneva, Anita Lakatos, Dexter Hadley, Hakon Hakonarson, Douglas C Wallace
Importance: Autism spectrum disorders (ASD) are characterized by impairments in social interaction, communication, and repetitive or restrictive behavior. Although multiple physiologic and biochemical studies have reported defects in mitochondrial oxidative phosphorylation in patients with ASD, the role of mitochondrial DNA (mtDNA) variation has remained relatively unexplored. Objective: To assess what impact mitochondrial lineages encompassing ancient mtDNA functional polymorphisms, termed haplogroups, have on ASD risk...
November 1, 2017: JAMA Psychiatry
https://www.readbyqxmd.com/read/28826991/parents-preferences-regarding-public-reporting-of-outcomes-in-congenital-heart-surgery
#13
Mallory L Irons, J William Gaynor, Thomas L Spray, Chris Feudtner
BACKGROUND: Calls for public reporting of outcomes in congenital heart surgery have led to several different reporting schemes, including a star rating system and benchmark procedure-specific mortality data tables. Important unanswered questions remain about the optimal format and content of public reporting of congenital heart surgery outcomes. METHODS: In conjunction with three parent advocacy groups, we developed a questionnaire to gauge parents' attitudes regarding the format and content of an "optimal" public reporting scheme...
August 19, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28768252/amniotic-fluid-concentrations-of-glial-fibrillary-acidic-protein-do-not-correlate-with-prenatal-metrics-in-fetuses-with-myelomeningocele
#14
Aimee G Kim, Enrico Danzer, Julie S Moldenhauer, Nahla Khalek, Lauren E McClain, Lindsay N Waqar, Holly L Hedrick, Mark P Johnson, N Scott Adzick, William H Peranteau, Alan W Flake
INTRODUCTION: We investigated the correlation of amniotic fluid (AF) concentrations of glial fibrillary acidic protein (GFAP) with prenatal features of myelomeningocele (MMC) and neurodevelopmental outcome after fetal MMC (fMMC) surgery. MATERIALS AND METHODS: AF was collected during fMMC surgery between December 2012 and November 2015. AF-GFAP concentration was determined by ELISA. Retrospective chart review identified the characteristics of the defect. Data regarding delivery and 1-year neurodevelopmental outcome was collected from The Children's Hospital of Philadelphia fMMC Registry...
July 29, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28758365/evaluation-of-the-safety-of-iron-dextran-with-parenteral-nutrition-in-the-paediatric-inpatient-setting
#15
Dale Lee, Diane Barsky, Rachel Hughes, Julia Driggers, Nicole Poupard, Maria Mascarenhas
AIM: Practitioners often avoid administering iron dextran in parenteral nutrition (PN) for hospitalised children because of the concern for anaphylaxis. The primary aim of the present study was to determine the risk of anaphylaxis associated with exposure to PN containing iron dextran in the inpatient setting. METHODS: Charts were reviewed for all children admitted to The Children's Hospital of Philadelphia from January 1, 2011 to December 30, 2013 who received PN containing low molecular weight (LMW) iron dextran...
July 7, 2017: Nutrition & Dietetics: the Journal of the Dietitians Association of Australia
https://www.readbyqxmd.com/read/28748621/outcomes-of-matched-sibling-donor-bone-marrow-transplantation-in-children-using-single-agent-calcineurin-inhibitors-as-prophylaxis-for-graft-versus-host-disease
#16
Caitlin W Elgarten, Danielle E Arnold, Nancy J Bunin, Alix E Seif
BACKGROUND: Optimal graft versus host disease (GVHD) prophylaxis prevents severe manifestations without excess immunosuppression. Standard prophylaxis includes a calcineurin inhibitor (CNI) with low-dose methotrexate. However, single-agent CNI may be sufficient prophylaxis for a defined group of patients. Single-agent CNI has been used for GVHD prophylaxis for human leukocyte antigen (HLA)-matched sibling donor (MSD) bone marrow transplants (BMTs) in young patients at the Children's Hospital of Philadelphia for over 20 years...
July 27, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28730089/an-international-survey-of-classification-and-treatment-choices-for-group-d-retinoblastoma
#17
Christina Scelfo, Jasmine H Francis, Vikas Khetan, Thomas Jenkins, Brian Marr, David H Abramson, Carol L Shields, Jacob Pe'er, Francis Munier, Jesse Berry, J William Harbour, Andrey Yarovoy, Evandro Lucena, Timothy G Murray, Pooja Bhagia, Evelyn Paysse, Samuray Tuncer, Guillermo L Chantada, Annette C Moll, Tatiana Ushakova, David A Plager, Islamov Ziyovuddin, Carlos A Leal, Miguel A Materin, Xun-Da Ji, Jose W Cursino, Rodrigo Polania, Hayyam Kiratli, Charlotta All-Ericsson, Rejin Kebudi, Santosh G Honavar, Vicktoria Vishnevskia-Dai, Sidnel Epelman, Anthony B Daniels, Jeanie D Ling, Fousseyni Traore, Marco A Ramirez-Ortiz
AIM: To determine which IIRC scheme was used by retinoblastoma centers worldwide and the percentage of D eyes treated primarily with enucleation versus globe salvaging therapies as well as to correlate trends in treatment choice to IIRC version used and geographic region. METHODS: An anonymized electronic survey was offered to 115 physicians at 39 retinoblastoma centers worldwide asking about IIRC classification schemes and treatment patterns used between 2008 and 2012...
2017: International Journal of Ophthalmology
https://www.readbyqxmd.com/read/28715553/validation-of-the-children-s-hospital-of-philadelphia-retinopathy-of-prematurity-chop-rop-model
#18
MULTICENTER STUDY
Gil Binenbaum, Gui-Shuang Ying, Lauren A Tomlinson
Importance: The Children's Hospital of Philadelphia Retinopathy of Prematurity (CHOP ROP) model uses birth weight (BW), gestational age at birth (GA), and weight gain rate to predict the risk of severe retinopathy of prematurity (ROP). In a model development study, it predicted all infants requiring treatment, while greatly reducing the number of examinations compared with current screening guidelines. Objective: To validate the CHOP ROP model in a multicenter cohort that is large enough to obtain a precise estimate of the model's sensitivity for treatment-requiring ROP...
August 1, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28604931/motor-vehicle-crash-risk-among-adolescents-and-young-adults-with-attention-deficit-hyperactivity-disorder
#19
Allison E Curry, Kristina B Metzger, Melissa R Pfeiffer, Michael R Elliott, Flaura K Winston, Thomas J Power
Importance: Attention-deficit/hyperactivity disorder (ADHD) often persists into adolescence, when motor vehicle crash risk peaks. We know little about when adolescents with ADHD get licensed and, once they do, the extent to which they have increased crash risk compared with adolescents without ADHD. Objectives: To examine the association between ADHD and both driver licensing and crash involvement and whether it varies by sex, licensing age, and/or being prescribed ADHD medication at licensure...
August 1, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28598945/long-term-outcomes-of-pediatric-cardiac-patients-supported-by-extracorporeal-membrane-oxygenation
#20
Matthew D Elias, Barbara-Jo Achuff, Richard F Ittenbach, Chitra Ravishankar, Thomas L Spray, Stephanie Fuller, Lisa M Montenegro, J William Gaynor, Matthew J O'Connor
OBJECTIVE: Extracorporeal membrane oxygenation is an important form of short-term mechanical support in children with cardiac disease, but information on long-term outcomes and quality of life is limited. The primary objective of this study was to determine the long-term outcomes of children previously supported by extracorporeal membrane oxygenation for cardiac etiologies. DESIGN: A retrospective analysis was performed on patients with cardiac disease managed with extracorporeal membrane oxygenation between January 1, 1995, and December 31, 2012, at the Children's Hospital of Philadelphia...
August 2017: Pediatric Critical Care Medicine
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