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Children's hospital of philadelphia

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March 2018: World Journal for Pediatric & Congenital Heart Surgery
Lisa M Fahey, Prasanna M Chandramouleeswaran, Shaobo Guan, Alain J Benitez, Glenn T Furuta, Seema S Aceves, Mei-Lun Wang, Chris A Liacouras, Amanda B Muir, Patrick Ma Sleiman, Hakon Hakonarson, Jonathan M Spergel, Antonella Cianferoni
OBJECTIVES: TSLP has been shown to be associated with eosinophilic esophagitis (EoE). Specifically, children with EoE often have the nucleotides AA or AG instead of GG at the single nucleotide polymorphism position RS3806932. Presently, the phenotypic characteristics in EoE children with the TSLP EoE risk allele are unknown. METHODS: A retrospective analysis was performed of all children with EoE who had TSLP genotyping at The Children's Hospital of Philadelphia from 2008-2014...
March 6, 2018: Clinical and Translational Gastroenterology
Rebecca D Ganetzky, Marni J Falk
BACKGROUND: Intravenous (IV) arginine has been reported to ameliorate acute metabolic stroke symptoms in adult patients with Mitochondrial Encephalopathy with Lactic Acidosis and Stroke-like Episodes (MELAS) syndrome, where its therapeutic benefit is postulated to result from arginine acting as a nitric oxide donor to reverse vasospasm. Further, reduced plasma arginine may occur in mitochondrial disease since the biosynthesis of arginine's precursor, citrulline, requires ATP. Metabolic strokes occur across a wide array of primary mitochondrial diseases having diverse molecular etiologies that are likely to share similar pathophysiologic mechanisms...
February 2, 2018: Molecular Genetics and Metabolism
Christopher E Gaw, Keith W Hamilton, Jeffrey S Gerber, Julia E Szymczak
BACKGROUND The decision to utilize antimicrobials in end-of-life situations is complex. Understanding the reasons why physicians prescribe antimicrobials in this patient population is important for informing the design of antimicrobial stewardship interventions. METHODS A 51-item survey containing both closed and open-ended questions on end-of-life antimicrobial use was administered to physicians affiliated with the University of Pennsylvania and Children's Hospital of Philadelphia from January through April 2017...
February 12, 2018: Infection Control and Hospital Epidemiology
Zakia Hammal, Jeffrey F Cohn, Erin R Wallace, Carrie L Heike, Craig B Birgfeld, Harriet Oster, Matthew L Speltz
OBJECTIVE: To compare facial expressiveness (FE) of infants with and without craniofacial macrosomia (cases and controls, respectively) and to compare phenotypic variation among cases in relation to FE. DESIGN: Positive and negative affect was elicited in response to standardized emotion inductions, video recorded, and manually coded from video using the Facial Action Coding System for Infants and Young Children. SETTING: Five craniofacial centers: Children's Hospital of Los Angeles, Children's Hospital of Philadelphia, Seattle Children's Hospital, University of Illinois-Chicago, and University of North Carolina-Chapel Hill...
January 1, 2018: Cleft Palate-craniofacial Journal
Andrew Joseph McCarty, Jeffrey M Miller, Heather Fecteau, Jeffrey W Pennington, Lisa Kessler
Many medical institutions have converted to a digital model for record keeping due to the Health Information Technology for Economic and Clinical Health Act. This Act provides incentives to health care systems to accelerate and encourage the adoption of electronic health record (EHR) systems. The pedigree as a tool in medicine provides an efficient method to assess and represent an individual's health and family health risks that may otherwise not be apparent in the medical record in a clearly identifiable way (Schuette, J...
January 25, 2018: Journal of Genetic Counseling
Allan F Simpao, Luis M Ahumada, Beatriz Larru Martinez, Ana M Cardenas, Talene A Metjian, Kaede V Sullivan, Jorge A Gálvez, Bimal R Desai, Mohamed A Rehman, Jeffrey S Gerber
BACKGROUND:  Hospitals use antibiograms to guide optimal empiric antibiotic therapy, reduce inappropriate antibiotic usage, and identify areas requiring intervention by antimicrobial stewardship programs. Creating a hospital antibiogram is a time-consuming manual process that is typically performed annually. OBJECTIVE:  We aimed to apply visual analytics software to electronic health record (EHR) data to build an automated, electronic antibiogram ("e-antibiogram") that adheres to national guidelines and contains filters for patient characteristics, thereby providing access to detailed, clinically relevant, and up-to-date antibiotic susceptibility data...
January 2018: Applied Clinical Informatics
Adam C Adler, Stephanie Helman, Cynthia Field, Judith J Stellar, Lauren A Brown, Nicole Omensetter, Andrew T Costarino, Mahsun Yuerek, Karen S Bender
OBJECTIVES: To reduce the number of ischemic arterial catheter injuries in children with congenital or acquired heart disease. DESIGN: This is a quality improvement study with pre- and postintervention groups. SETTING: University-affiliated pediatric cardiac center in a quaternary care freestanding children's hospital. PATIENTS: All patients with an indwelling peripheral arterial catheter placed in the Children's Hospital of Philadelphia Cardiac Center associated with an admission to the Cardiac Intensive Cardiac Unit from January 2015 to July 2017 are included...
March 2018: Pediatric Critical Care Medicine
Elizabeth B Froh, Jill Vanderpool, Diane L Spatz
Human milk donated to a milk bank can become contaminated in a number of ways, but processes exist to eradicate pathogenic bacterial growth. Donor human milk may be cultured before or after pasteurization or both. The purpose of this article is to describe standard operations of the Mothers' Milk Bank of the Children's Hospital of Philadelphia, best practices to limit the bacterial contamination of donor human milk, and implications for future research.
January 2, 2018: Journal of Obstetric, Gynecologic, and Neonatal Nursing: JOGNN
Kathryn L Kreicher, Michael J Bauschard, Clarice S Clemmens, Concetta Maria Riva, Ted A Meyer
BACKGROUND: The purpose of this study was to evaluate hearing impairment in pediatric patients with cystic fibrosis (CF). METHODS: This is a retrospective analysis of the AudGen database generated by Children's Hospital of Philadelphia. Audiograms were analyzed for type of hearing loss (HL), pure-tone-average (PTA), laterality, and change in hearing over time. Medical charts were reviewed to identify factors that influence development and progression of hearing loss...
December 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Madison A Grasty, Richard F Ittenbach, Carol Knightly, Cynthia B Solot, Marsha Gerdes, Judy C Bernbaum, Gil Wernovsky, Thomas L Spray, Susan C Nicolson, Robert R Clancy, Daniel J Licht, Elaine Zackai, J William Gaynor, Nancy B Burnham
OBJECTIVES: To investigate the prevalence of hearing loss after cardiac surgery in infancy, patient and operative factors associated with hearing loss, and the relationship of hearing loss to neurodevelopmental outcomes. STUDY DESIGN: Audiologic and neurodevelopmental evaluations were conducted on 348 children who underwent repair of congenital heart disease at the Children's Hospital of Philadelphia as part of a prospective study evaluating neurodevelopmental outcomes at 4 years of age...
January 2018: Journal of Pediatrics
Robert J Smith, Sarah B Klieger, Salwa E Sulieman, Emily Berger, James R Treat, Brian T Fisher
OBJECTIVES: Cutaneous lesions are often the first marker of invasive mold infection, which can cause substantial morbidity in immunocompromised children. The purpose of this study was to describe the evaluation and outcomes of immunocompromised children who presented with findings requiring skin biopsy because of concern about invasive infection. In children who were biopsied, we sought to determine the factors predictive of invasive mold infection. METHODS: A retrospective review was conducted at the Children's Hospital of Philadelphia...
January 2018: Pediatric Dermatology
Sarah E Hopkins
PURPOSE OF REVIEW: Purpose of review Acute flaccid myelitis is a polio-like illness defined by the acute onset of flaccid paralysis in the setting spinal MRI demonstrating a longitudinal lesion in the gray matter of the cord. This paper aims to review the current state of knowledge and key clinical points for the diagnosis and management of acute flaccid myelitis. RECENT FINDINGS: Recent findings There were clusters of AFM noted in California and Colorado in 2014, with additional cases across the USA that year, and another spike in cases in 2016...
November 28, 2017: Current Treatment Options in Neurology
Roberto De Sanctis, Marika Pane, Giorgia Coratti, Concetta Palermo, Daniela Leone, Maria Carmela Pera, Emanuela Abiusi, Stefania Fiori, Nicola Forcina, Lavinia Fanelli, Simona Lucibello, Elena S Mazzone, Francesco Danilo Tiziano, Eugenio Mercuri
The advent of clinical trials has highlighted the need for natural history studies reporting disease progression in type 1 spinal muscular atrophy. The aim of this study was to assess functional changes using the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) scale in a cohort of type 1 infants. Nutritional and respiratory longitudinal data were also recorded. Patients were classified according to the severity of the phenotype and age of onset. SMN2 copies were also assessed...
January 2018: Neuromuscular Disorders: NMD
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...
December 1, 2017: JAMA Dermatology
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...
February 2018: Critical Care Medicine
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
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
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...
December 1, 2017: JAMA Pediatrics
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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.
December 2017: Cancer Discovery
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