400 results match your criteria: "the Children's Hospital of Philadelphia and the University of Pennsylvania[Affiliation]"

Schizophrenia (SZ) is a highly heterogeneous disorder in both its symptoms and risk factors. One of the most prevalent genetic risk factors for SZ is the hemizygous microdeletion at chromosome 22q11.2 (22q11DS) that confers a 25-fold increased risk.

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Objective: Compare rates of hypoxaemia during transpyloric and gastric feedings in very preterm infants with severe bronchopulmonary dysplasia.

Design: N-of-1 multiple crossover trials with individual patient and pooled data analyses.

Setting: Level IV intensive care nursery.

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Background: Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations.

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Background: Lengthening temporalis myoplasty (LTM) and cross-face nerve graft with free gracilis muscle transfer (CFNG-FGMT) are the 2 most common procedures used to restore dynamic facial animation and improve facial symmetry. There has not been direct comparison or consensus. Here, the authors compare our experience with respect to muscle activity, symmetry, and excursion.

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Background: Characteristics of neonatal tracheal intubations (TI) may vary between the neonatal intensive care unit (NICU) and delivery room (DR). The impact of the setting on TI outcomes is not well characterized.

Objective: The aim of this study was to define variation in neonatal TI practice between settings, and identify the association between setting and TI success and safety outcomes.

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Objective: To assess the frequency of gastrostomy tube (GT) placement in extremely low birth weight (ELBW) infants, associated comorbidities, and long-term outcomes.

Study Design: Analysis of ELBW infants from 25 centers enrolled in the National Institute of Child Health and Human Development Neonatal Research Network's Generic Database and Follow-up Registry from 2006 to 2012. Frequency of GT placement before 18-22 months, demographic and medical factors associated with GT placement, and associated long-term outcomes at 18-22 months of corrected age were described.

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Background Whether racial and neighborhood characteristics are associated with bystander cardiopulmonary resuscitation ( BCPR ) in pediatric out-of-hospital cardiac arrest ( OHCA ) is unknown. Methods and Results An analysis was conducted of CARES (Cardiac Arrest Registry to Enhance Survival) for pediatric nontraumatic OHCA s from 2013 to 2017. An index (range, 0-4) was created for each arrest based on neighborhood characteristics associated with low BCPR (>80% black; >10% unemployment; <80% high school; median income, <$50 000).

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Most preterm infants breathe at birth, but need additional respiratory support due to immaturity of the lung and respiratory control mechanisms. To avoid lung injury, the focus of respiratory support has shifted from invasive towards non-invasive ventilation. However, applying effective non-invasive ventilation is difficult due to mask leak and airway obstruction.

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Dosage Counts: Correcting Trisomy-21-Related Phenotypes in Human Organoids and Xenografts.

Cell Stem Cell

June 2019

Department of Psychiatry, The Children's Hospital of Philadelphia and The University of Pennsylvania Perelman School of Medicine, ARC 517, 3615 Civic Center Blvd., Philadelphia, PA 19104, USA. Electronic address:

Studies in mice suggest that Olig2 gene dosage alters cerebral cortical interneuron development and contributes to trisomy-21/Down-syndrome-related intellectual disability. Xu et al. (2019) extend these studies through the remarkable use of cerebral organoid and human iPSC/mouse brain chimera experimental systems that provide an opportunity for the development of novel therapeutics.

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Objective: To evaluate the effect of blood sampling from the placental end of the umbilical cord compared with initial blood sampling from neonates, on the need for first packed red blood cell transfusion in extremely preterm infants. We hypothesized that cord blood sampling could delay the time to first blood transfusion.

Study Design: In this single-center, assessor blind, randomized controlled trial, we included extremely low birth weight neonates <28 weeks of gestational age at birth.

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The Pediatric Cell Atlas: Defining the Growth Phase of Human Development at Single-Cell Resolution.

Dev Cell

April 2019

Department of Biomedical Informatics, University of Cincinnati College of Medicine, and Cincinnati Children's Hospital Medical Center, Division of Biomedical Informatics, Cincinnati, OH 45229, USA.

Article Synopsis
  • Single-cell gene expression studies in mammalian tissues reveal important stage-specific molecular processes that are crucial for understanding different cell types and their developmental pathways.
  • The authors propose the creation of a Pediatric Cell Atlas to be integrated into the Human Cell Atlas consortium, which will create detailed single-cell profiles of gene expression in human tissues and organs.
  • This Pediatric Cell Atlas will enhance existing research on adults and development, offering valuable insights into pediatric health issues and the genetic and environmental factors affecting health throughout one's lifetime.
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Vancomycin Prescribing and Therapeutic Drug Monitoring in Children With and Without Acute Kidney Injury After Cardiac Arrest.

Paediatr Drugs

April 2019

Department of Anesthesiology and Critical Care Medicine, Children's Hospital of Philadelphia and The University of Pennsylvania Perelman School of Medicine, 3401 Civic Center Blvd, 6th Floor Wood Building, Room 6117, Philadelphia, PA, 19104, USA.

Background: Acute kidney injury (AKI) commonly occurs after cardiac arrest. Those subsequently treated with vancomycin are at additional risk for drug-induced kidney injury.

Objective: We aimed to determine whether opportunities exist for improved drug monitoring after cardiac arrest.

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Spinocerebellar Ataxia Type 2 (SCA2) is an autosomal dominant disease characterized by progressive degeneration of the cerebellum, brain stem, and spinal cord. SCA2 is caused by spontaneous misfolding and aggregate formation from abnormal CAG trinucleotide repeat expansion in the coding region of the ATXN2 gene. Here we describe the generation of two distinct iPSC lines from patients with SCA2.

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Hurry up and wait, then activate and translate!

Sci Immunol

January 2019

Department of Pediatrics, Allergy Immunology Division, the Children's Hospital of Philadelphia and the University of Pennsylvania, Philadelphia, PA 19104, USA. Institute for Immunology, Perelman School of Medicine, Philadelphia, PA 19104, USA. Email:

The immunometabolic pivot from reliance on fatty acid oxidation to glycolysis during T cell activation is, in part, reliant on the regulation of translation of key metabolic enzymes.

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Purpose: To summarize the use of continuous electroencephalographic monitoring (cEEG) in the diagnosis and management of pediatric convulsive status epilepticus (CSE) and subsequent non-convulsive seizures (NCS) with a focus on available guidelines and infrastructure. In addition, we provide an overview of quantitative EEG (QEEG) for the identification of NCS in critically ill children.

Methods: We performed a review of the medical literature on the use of cEEG and QEEG in pediatric CSE.

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Objective: To develop a postoperative mortality case-mix adjustment model to facilitate assessment of cardiac ICU quality of care, and to describe variation in adjusted cardiac ICU mortality across hospitals within the Pediatric Cardiac Critical Care Consortium.

Design: Observational analysis.

Setting: Multicenter Pediatric Cardiac Critical Care Consortium clinical registry.

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Objective: To assess whether length of hospital stay is decreased among moderately preterm infants weaned from incubator to crib at a lower vs higher weight.

Study Design: This trial was conducted in the Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network. Infants with gestational ages 29-33 weeks, birthweight <1600 g, and in an incubator were randomly assigned to a weaning weight of 1600 or 1800 g.

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One, two, three-how many cells for me?

Sci Immunol

September 2018

Department of Pediatrics, Allergy Immunology Division, the Children's Hospital of Philadelphia and the University of Pennsylvania, Philadelphia, PA 19104, USA. Email:

An alternate strategy is described for enumerating peripheral immune cells in health and disease by epigenetic quantitative PCR.

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The effect of video laryngoscopy on adverse events during neonatal tracheal intubation is unknown. In this single site retrospective cohort study, video laryngoscopy was independently associated with decreased risk for adverse events during neonatal intubation.

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Objectives: To examine growth, sedation needs, and participation in developmental activities before and after tracheostomy among infants with severe bronchopulmonary dysplasia.

Methods: Retrospective analysis of infants born at <32 weeks' gestation or birth weights <1500 g with severe BPD who underwent tracheostomy placement between January 1, 2010 and December 31, 2016 in a quaternary referral newborn and infant intensive care unit. Changes in growth parameters and frequency/type of participation in physical therapy sessions performed during the 4-weeks before tracheostomy and 4-weeks after the first tracheostomy tube change were compared.

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The use of prophylactic indomethacin in very preterm infants is controversial. The last randomized controlled trial (RCT) to study this therapy enrolled infants over 20 years ago. More recently, observational studies have investigated the association between exposure to prophylactic indomethacin and neonatal morbidities and mortality.

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