13,495 results match your criteria: "the Children's Hospital of Philadelphia.[Affiliation]"

Case: We discuss a 16-year-old adolescent boy presenting with a minimally displaced greenstick fracture of the distal third ulnar diaphysis sustained during a fall playing football. Initial treatment consisted of in situ casting followed by removable forearm splinting. The patient returned 3 months postinjury with complete forearm motion loss.

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Background: The published reports examining socioeconomic factors and their relationship to osteosarcoma presentation and treatment suggest an association between lower socioeconomic status and a worse response to chemotherapy and lower survivorship. However, the driving factors behind these disparities are unclear. The Child Opportunity Index was developed by diversitydatakids.

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Brain connectivity arises from interactions across biophysical scales, ranging from molecular to cellular to anatomical to network level. To date, there has been little progress toward integrated analysis across these scales. To bridge this gap, from a unique cohort of 98 individuals, we collected antemortem neuroimaging and genetic data, as well as postmortem dendritic spine morphometric, proteomic and gene expression data from the superior frontal and inferior temporal gyri.

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Ivabradine use in children.

Heart Rhythm

November 2024

Division of Cardiology, The Children's Hospital of Philadelphia and Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

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The rapid growth in consumer-facing mobile and sensor technologies has created tremendous opportunities for patient-driven personalized health management. The diagnosis and management of cardiac arrhythmias are particularly well suited to benefit from these easily accessible consumer health technologies. In particular, smartphone-based and wrist-worn wearable electrocardiogram (ECG) and photoplethysmography (PPG) technology can facilitate relatively inexpensive, long-term rhythm monitoring.

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Dynamic sampling of a surveillance state enables DNA proofreading by Cas9.

Cell Chem Biol

October 2024

Center for Computational and Genomic Medicine, The Children's Hospital of Philadelphia, 3501 Civic Center Boulevard, Philadelphia, PA 19104, USA; Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, 3620 Hamilton Walk, Philadelphia, PA 19104-6059, USA. Electronic address:

Article Synopsis
  • CRISPR-Cas9 enhances genome editing by using programmable RNA for precise targeting, but understanding how it distinguishes between on-target and off-target DNA is still unclear.* -
  • The study utilizes methyl-based NMR to reveal a specific protein conformation that helps Cas9 recognize DNA mismatches, particularly at the PAM-distal end.* -
  • Findings indicate that a modified version of Cas9 (HiFi Cas9) improves the accuracy of DNA recognition by stabilizing a "surveillance state," which shifts the protein's dynamics away from its active state, enhancing our understanding of Cas9's selective functions.*
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The case for microbial intervention at weaning.

Gut Microbes

October 2024

Division of Infectious Disease, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Weaning, the transition from a milk-based diet to solid food, coincides with the most significant shift in gut microbiome composition in the lifetime of most mammals. Notably, this period also marks a "window of opportunity" where key components of the immune system develop, and host-microbe interactions shape long-term immune homeostasis thereby influencing the risk of autoimmune and inflammatory diseases. This review provides a comprehensive analysis of the changes in nutrition, microbiota, and host physiology that occur during weaning.

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Background: Friedreich ataxia (FRDA) results in progressive impairment in gait, upper extremity coordination, and speech. Currently, these symptoms are assessed through expert examination at clinical visits. Such in-clinic assessments are time-consuming, subjective, of limited sensitivity, and provide only a limited perspective of the daily disability of patients.

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PTB airway compression complicated by vascular abnormalities and cardiac involvement: A case series demonstrating diagnosis and management.

Pediatr Pulmonol

January 2025

Department of Forensic Medicine and Imaging, Institute of Forensic Medicine, University of Zurich, Zurich, Switzerland.

Introduction: Lymphobronchial tuberculosis (LBTB) is a tuberculous lymphadenopathy causing airway compression in young children. While it can occur in older children due to factors such as airway size, wall weakness, and immune reconstitutions, severe airway obstruction is more common in younger children.

Methods: Chest X-rays show airway compression, while bronchoscopy is the gold standard for confirming TB-induced airway compression.

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Article Synopsis
  • * Conducted across 26 children's hospitals in the US from March 2020 to May 2023, the research involved analyzing data from over 172,000 eligible children and young adults aged 5 to 20 with confirmed COVID-19.
  • * The findings aim to establish a clear association between pre-infection BMI categories—ranging from healthy weight to severe obesity—and the likelihood of experiencing PASC, with statistical analyses adjusting for various demographic and clinical
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Recently, the U.S. Surgeon General issued an advisory highlighting the lack of knowledge about the safety of ubiquitous social media use on adolescent mental health.

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Background And Aims: Bowel smooth muscle experiences mechanical stress constantly during normal function, and pathologic mechanical stressors in disease states. We tested the hypothesis that pathologic mechanical stress could alter transcription to induce smooth muscle phenotypic class switching.

Methods: Primary human intestinal smooth muscle cells (HISMCs), seeded on electrospun aligned poly-ε-caprolactone nano-fibrous scaffolds, were subjected to pathologic, high frequency (1 Hz) uniaxial 3% cyclic stretch (loaded) or kept unloaded in culture for 6 hours.

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Animal models of epilepsy are critical in drug development and therapeutic testing, but dominant methods for pharmaceutical evaluation face a tradeoff between higher throughput and etiological relevance. For example, in temporal lobe epilepsy, a type of epilepsy where seizures originate from limbic structures like the hippocampus, the main screening models are either based on acutely induced seizures in wild type, naïve animals or spontaneous seizures in chronically epileptic animals. Both types have their disadvantages - the acute convulsant or kindling induced seizures do not account for the myriad neuropathological changes in the diseased, epileptic brains, and spontaneous behavioral seizures are sparse in the chronically epileptic models, making it time-intensive to sufficiently power experiments.

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Chloride ions play a critical role in neuronal inhibition through the activity of chloride-permeable GABA receptor channels. Ion transporters, chloride channels, and immobile ion species tightly regulate intracellular chloride concentrations. Several studies related to epilepsy suggest that chloride extrusion function may decrease in an activity-dependent manner.

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Ten challenges and opportunities in computational immuno-oncology.

J Immunother Cancer

October 2024

Center for Computational and Genomic Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

Article Synopsis
  • Immuno-oncology is revolutionizing cancer treatment, but most patients do not see long-lasting benefits, indicating a need for further advancements in the field.
  • Computational immuno-oncology combines biomedical data science with oncology and immunology to enhance the development of effective immunotherapy treatments from research to clinical application.
  • The review highlights 10 key challenges and opportunities in computational immuno-oncology, stressing the need for strong computational methods and teamwork to adapt to rapid changes in clinical demands and technology.
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Response to the Letter to the Editor.

J Pediatr Urol

October 2024

Division of Pediatric Urology, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, USA.

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Article Synopsis
  • * In this study, preterm fetal lambs were subjected to reduced oxygen levels using the EXTEND model, resulting in noticeable growth restrictions and changes in lung structure compared to those with normal oxygen levels.
  • * Key findings included fewer blood vessels, increased muscularization in peripheral vessels, enlarged airspaces, and a drop in specific lung cell types and proteins crucial for lung function, indicating that fetal hypoxia mainly affects airway and surfactant production development.
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Cardiac MRI Predictors of Arrhythmic Sudden Cardiac Events in Patients With Fontan Circulation.

J Am Coll Cardiol

December 2024

Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. Electronic address: https://twitter.com/nkwcardiomd.

Article Synopsis
  • Patients with congenital heart disease, particularly those who have undergone the Fontan procedure for single ventricle issues, face a high risk of sudden cardiac events (SCE) and early mortality, highlighting the importance of identifying risk factors.
  • In a study involving 3,132 Fontan patients, 3.5% experienced SCE over an average follow-up of 4 years, with 36% of those cases resulting in death.
  • Key risk factors for SCE included higher NYHA functional class, history of protein-losing enteropathy or plastic bronchitis, large end-diastolic volume index, and reduced ejection fraction, all of which can help in predicting and preventing such events.
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Temporal trends demonstrate improved survival for many types of common pediatric cancer. Studies have not examined improvement in very rare pediatric cancers or compared these improvements to more common cancers. In this cohort study of the Surveillance, Epidemiology, and End Results (SEER) registry, we examined patients from 1975 to 2016 who were 0-19 years of age at the time of diagnosis.

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Objective: To assess the screening ability of a high body mass index (BMI) for high adiposity among 8- to 19-year-olds.

Study Design: This cross-sectional study included 6454 National Health and Nutrition Survey participants from 2011 through 2018. Fat and lean mass were measured with dual-energy X-ray absorptiometry.

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Article Synopsis
  • - The study utilized data from the FORCE registry to analyze cardiac magnetic resonance (CMR) metrics in healthier Fontan patients, aiming to create Fontan-specific z-scores that consider different ventricular morphologies.
  • - The "healthier" cohort consisted of 885 patients, primarily children, who were assessed for ventricular size and function against those with adverse outcomes, revealing that those with left ventricle morphology had better metrics.
  • - Comparisons showed adults had lower blood flow in the ascending aorta than children, and patients with adverse outcomes tended to have higher heart volumes and mass, which suggests a link between ventricular morphology and patient prognosis post-Fontan surgery.
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Ventricular Dysfunction in Patients With Congenital Diaphragmatic Hernia Who Die After Repair.

J Pediatr Surg

January 2025

Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA; Department of Pediatric General Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

Background: Quantitative echocardiographic (echo) measures of ventricular function predict mortality in pediatric pulmonary hypertension (PH), but studies in congenital diaphragmatic hernia (CDH)-related PH are limited. Few studies report quantitative echo data beyond the first week of life in CDH non-survivors.

Methods: A single-center retrospective, cross-sectional, cohort study included CDH patients born between January 2013 and April 2022 who survived to surgical repair but died during the neonatal hospitalization.

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The gut microbiota plays a critical role in human health and disease. Microbial community assembly and succession early in life are influenced by numerous factors. In turn, assembly of this microbial community is known to influence the host, including immune system development, and has been linked to outcomes later in life.

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SCN8A developmental and epileptic encephalopathy (DEE) is a severe epilepsy syndrome resulting from mutations in the voltage-gated sodium channel Nav1.6, encoded by the gene SCN8A. Nav1.

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