1,298 results match your criteria: "Nemours Alfred I. duPont Hospital for Children[Affiliation]"

Neuroanatomical alterations in young boys and adolescents with Klinefelter syndrome.

Psychiatry Res Neuroimaging

January 2025

Stanford University School of Medicine, Department of Psychiatry and Biobehavioral Sciences, 1520 Page Mill Road, Stanford, California, 94305, United States; Stanford University School of Medicine, Department of Radiology, United States; Stanford University School of Medicine, Department of Pediatrics, United States.

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Resuscitation arterial waveform quantification and outcomes in pediatric bidirectional Glenn and Fontan patients.

Pediatr Res

September 2024

Department of Anesthesiology and Critical Care Medicine, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA.

Background: Resuscitation with chest compressions and positive pressure ventilation in Bidirectional Glenn (BDG) or Fontan physiology may compromise passive venous return and accentuate neurologic injury. We hypothesized that arterial pressure and survival would be better in BDG than Fontan patients.

Methods: Secondary analyses of the Pediatric Intensive Care Quality of CPR and Improving Outcomes from Pediatric Cardiac Arrest databases.

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Background: Tracheal intubation (TI)-associated cardiac arrest (TI-CA) occurs in 1.7% of pediatric ICU TIs. Our objective was to evaluate resuscitation characteristics and outcomes between cardiac arrest patients with and without TI-CA.

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  • * A survey conducted among participants from two SCA registries received responses from 303 individuals and caregivers, covering a wide age range and various SCA types.
  • * The survey results highlighted the SCA community's main priorities for health, emphasizing the need for intervention studies and clinical treatments, indicating a strong desire for targeted research in this area.
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Background: Spinal fusion for scoliosis associated with cerebral palsy (CP) is challenging to study because specialized outcome measures are needed. Therefore, evidence in favor of the benefits of surgery has not been firmly established. This study aimed to determine if corrective spinal fusion improves health-related quality of life (HRQoL) in children with CP scoliosis at 2 years.

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Early bolus epinephrine administration during pediatric cardiopulmonary resuscitation for bradycardia with poor perfusion: an ICU-resuscitation study.

Crit Care

July 2024

Department of Anesthesiology and Critical Care Medicine, The Children's Hospital of Philadelphia, University of Pennsylvania, 3401 Civic Center Boulevard, Philadelphia, PA, 19104, USA.

Article Synopsis
  • The study focused on pediatric in-hospital CPR events, particularly those involving bradycardia with poor perfusion, to evaluate the effects of early epinephrine on survival outcomes in children.
  • Researchers analyzed data from the ICU-RESUS trial, which involved CPR events that lasted 2 minutes or longer, examining the timing of epinephrine administration and the development of pulselessness.
  • Findings indicated that early epinephrine did not significantly improve the chances of survival or favorable neurological outcomes, while a high percentage of patients experienced pulselessness within the first few minutes of CPR.
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Stroke in critically ill patients with respiratory failure due to COVID-19: Disparities between low-middle and high-income countries.

Heart Lung

October 2024

Division of Neuroscience Critical Care, Departments of Neurology, Neurosurgery, and Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Article Synopsis
  • A study was conducted to compare stroke incidences and mortality rates in critically ill COVID-19 patients from low-and middle-income countries (LMICs) and high-income countries (HICs).
  • The results showed that stroke incidence was significantly higher in LMICs (35.7 per 1000 admitted-days) compared to HICs (17.6 per 1000 admitted-days), with patients from LMICs also facing higher mortality rates (43.6% vs. 29.2%).
  • The findings highlight the need for better stroke diagnosis and healthcare resource allocation in LMICs, as both higher income status and the presence of stroke are associated with increased risk of death
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Mucopolysaccharidosis III type C (MPS IIIC) is an untreatable neuropathic lysosomal storage disease caused by a genetic deficiency of the lysosomal N-acetyltransferase, HGSNAT, catalyzing a transmembrane acetylation of heparan sulfate. HGSNAT is a transmembrane enzyme incapable of free diffusion between the cells or their cross-correction, which limits development of therapies based on enzyme replacement and gene correction. Since our previous work identified neuroinflammation as a hallmark of the CNS pathology in MPS IIIC, we tested whether it can be corrected by replacement of activated brain microglia with neuroprotective macrophages/microglia derived from a heterologous HSPC transplant.

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Therapeutic targeting in pediatric acute myeloid leukemia with aberrant HOX/MEIS1 expression.

Eur J Med Genet

December 2023

Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands; Department of Pediatric Oncology, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands.

Article Synopsis
  • Advances in managing childhood acute myeloid leukemia (AML) have been made, but about one third of patients still face fatal outcomes due to challenges like chemoresistance and relapses.
  • Insights into the genetics of AML have revealed specific patient subsets linked to abnormalities in HOXA, MEIS1, and other key genes, suggesting avenues for targeted therapies.
  • Menin inhibitors show promise in preclinical and early-phase clinical trials for treating specific types of AML, and the PedAL/EUPAL project aims to facilitate further testing of these new agents in children.
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  • The study investigated the effectiveness of extracorporeal membrane oxygenation (ECPR) for pediatric patients who did not respond to traditional cardiopulmonary resuscitation (CPR) methods, focusing on early hemodynamics and end-tidal carbon dioxide (ET CO2) levels as potential indicators for survival and neurologic outcomes.
  • Data was collected from 97 ECPR patients across 18 ICUs from 2016-2021, revealing that most patients were under one year old and had congenital heart disease; only 41% of patients survived with favorable neurologic outcomes.
  • The study found no significant differences in blood pressure measures or chest compression rates between those who survived
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Chest compressions for pediatric organized rhythms: A hemodynamic and outcomes analysis.

Resuscitation

January 2024

Department of Anesthesiology and Critical Care Medicine, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA. Electronic address:

Aim: Pediatric cardiopulmonary resuscitation (CPR) guidelines recommend starting CPR for heart rates (HRs) less than 60 beats per minute (bpm) with poor perfusion. Objectives were to (1) compare HRs and arterial blood pressures (BPs) prior to CPR among patients with clinician-reported bradycardia with poor perfusion ("BRADY") vs. pulseless electrical activity (PEA); and (2) determine if hemodynamics prior to CPR are associated with outcomes.

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Study Design: Retrospective multicenter study.

Objective: We reviewed 15-year trends in operative factors, radiographic and quality of life outcomes, and complication rates in children with cerebral palsy (CP)-related scoliosis who underwent spinal fusion.

Summary Of Background Data: Over the past two decades, significant efforts have been made to decrease complications and improve outcomes of this population.

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Derivation of paediatric blood pressure percentiles from electronic health records.

EBioMedicine

December 2023

Children's Hospital of Philadelphia, Applied Clinical Research Center, Philadelphia, PA, USA; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania: Philadelphia, PA, USA.

Background: Identification of abnormal blood pressure (BP) in children requires normative data. We sought to examine the feasibility of using "real-world" office BP data obtained from electronic health records (EHR) to generate age-, sex- and height-specific BP percentiles for children.

Methods: Using data collected 01/01/2009-8/31/2021 from eight large children's healthcare organisations in PEDSnet, we applied a mixed-effects polynomial regression model with random slopes to generate Z-scores and BP percentiles and compared them with currently used normative BP distributions published in the 2017 American Academy of Paediatrics (AAP) Clinical Practise Guidelines (CPG).

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The Fontan Udenafil Exercise Longitudinal (FUEL) trial showed that treatment with udenafil was associated with improved exercise performance at the ventilatory anaerobic threshold in children with Fontan physiology. However, it is not known how the initiation of phosphodiesterase 5 inhibitor therapy affects heart rate and blood pressure in this population. These data may help inform patient selection and monitoring after the initiation of udenafil therapy.

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A Telemedicine Solution to Minimize Unnecessary Emergency Department Transfers for Low-acuity Pediatric Orthopaedic Patients: A Model for Cost Minimization.

J Am Acad Orthop Surg

May 2024

From the Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA (Maheu), the Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE (Shih, and Atanda), and the Hospital for Special Surgery, New York, NY (LeBrun, and Fabricant).

Introduction: Unnecessary emergency department (ED) transfers represent a notable source of excess costs and misutilization of healthcare resources, particularly with management of acute pediatric musculoskeletal injuries. This study used institutional data to create a model investigating the expected costs of a formal peer-to-peer telemedicine intervention designed to triage pediatric orthopaedic transfers, which we hypothesized would decrease healthcare costs by minimizing unwarranted ED-to-ED transfers.

Methods: In this retrospective modeling analysis, 350 pediatric orthopaedic trauma patients transferred to two in-network referral hospitals from outside facilities were identified and stratified into three groups representing how patients theoretically optimally could have been treated.

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Cognition, Academic Achievement, Adaptive Behavior, and Quality of Life in Child and Adolescent Boys with Klinefelter Syndrome.

J Dev Behav Pediatr

September 2023

Center of Interdisciplinary Brain Sciences Research, Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Stanford, CA.

Objective: Klinefelter syndrome (KS; 47, XXY), the most common sex chromosome aneuploidy in males, is characterized by testicular failure and testosterone deficiency as well as a variety of cognitive, social, and emotional challenges. In the current study, we aimed to clarify the cognitive-behavioral profile of peripubertal boys with KS using measures of cognition, academic achievement, adaptive behavior, and quality of life.

Method: We compared 47 boys with KS (7-16 years of age) with 55 performance IQ-matched boys without KS on measures of cognition (WISC-V), executive function (BRIEF-2), academic achievement (KTEA-3), adaptive behavior (Vineland-3), and quality of life (PROMIS).

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Association of CPR simulation program characteristics with simulated and actual performance during paediatric in-hospital cardiac arrest.

Resuscitation

October 2023

Department of Pediatrics, Children's Hospital of Michigan, Central Michigan University, 3901 Beaubien Blvd, Detroit, MI 48201, USA. Electronic address:

Aim: To evaluate associations between characteristics of simulated point-of-care cardiopulmonary resuscitation (CPR) training with simulated and actual intensive care unit (ICU) CPR performance, and with outcomes of children after in-hospital cardiac arrest.

Methods: This is a pre-specified secondary analysis of the ICU-RESUScitation Project; a prospective, multicentre cluster randomized interventional trial conducted in 18 ICUs from October 2016-March 2021. Point-of-care bedside simulations with real-time feedback to allow multidisciplinary ICU staff to practice CPR on a portable manikin were performed and quality metrics (rate, depth, release velocity, chest compression fraction) were recorded.

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Many factors affect patient outcome after congenital heart surgery, including the complexity of the heart disease, pre-operative status, patient specific factors (prematurity, nutritional status and/or presence of comorbid conditions or genetic syndromes), and post-operative residual lesions. The esidual esion core is a novel tool for assessing whether specific residual cardiac lesions after surgery have a measurable impact on outcome. The goal is to understand which residual lesions can be tolerated and which should be addressed prior to leaving the operating room.

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Molecular Trojan Horses for treating lysosomal storage diseases.

Mol Genet Metab

November 2023

Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA; Department of Molecular Biology, Faculty of Biology, University of Gdansk, Gdansk, Poland; Faculty of Arts and Sciences, University of Delaware, Newark, DE, USA; Department of Pediatrics, Graduate School of Medicine, Gifu University, Gifu, Japan; Department of Pediatrics, Thomas Jefferson University, Philadelphia, PA, USA. Electronic address:

Lysosomal storage diseases (LSDs) are caused by monogenic mutations in genes encoding for proteins related to the lysosomal function. Lysosome plays critical roles in molecule degradation and cell signaling through interplay with many other cell organelles, such as mitochondria, endoplasmic reticulum, and peroxisomes. Even though several strategies (i.

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Natural History of Pediatric Hand and Wrist Ganglion Cysts: Longitudinal Follow-Up of a Prospective, Dual-Center Cohort.

J Hand Surg Am

October 2023

The Children's Hospital of Philadelphia, Philadelphia, PA; Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Electronic address:

Purpose: This investigation describes the outcomes of pediatric ganglion cysts in a prospective cohort that elected not to undergo cyst aspiration or surgical treatment. Our primary aim was to investigate the rate of spontaneous resolution over time among the subset of patients who did not undergo specific treatments.

Methods: Children (aged ≤18 years) who presented to the clinic with ganglion cysts of the hand or wrist were enrolled in a prospective two-center registry between 2017 and 2021.

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Purpose: To evaluate intraoperative monitoring (IOM) alerts and neurologic deficits during severe pediatric spinal deformity surgery.

Methods: Patients with a minimum Cobb angle of 100° in any plane or a scheduled vertebral column resection (VCR) with minimum 2-year follow-up were prospectively evaluated (n = 243). Preoperative, immediate postoperative, and 2-year postoperative neurologic status were reported.

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The Fontan Udenafil Exercise Longitudinal Trial: Subgroup Analysis.

Pediatr Cardiol

December 2023

Division of Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine, 34th Street and Civic Center Blvd, Philadelphia, PA, 19104, USA.

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.

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