1,735 results match your criteria: "Primary Children's Hospital.[Affiliation]"

Papillary tumor of the pineal region: analysis of DNA methylation profiles and clinical outcomes in 76 cases.

Acta Neuropathol Commun

July 2024

Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Dr., Room 2S235, Bethesda, MD, 20892, USA.

Article Synopsis
  • Papillary tumor of the pineal region (PTPR) is a rare and unique tumor characterized by specific molecular and histopathologic features, with limited prior research on its variations and clinical presentations.
  • In a study of 76 confirmed PTPR cases, researchers identified two main methylation groups (PTPR-A and PTPR-B) and further classified PTPR-B into two subtypes (B1 and B2) based on DNA methylation profiles and genomic variations.
  • Clinical outcomes revealed that nearly half of the patients experienced tumor progression, with significant differences in outcomes among the identified subtypes, highlighting the tumor's molecular diversity and potential for recurrence.
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Objective: Treatment of neonates with anorectal malformations (ARMs) can be challenging due to variability in anatomic definitions, multiple approaches to surgical management, and heterogeneity of reported outcomes. The purpose of this systematic review is to summarize existing evidence, identify treatment controversies, and provide guidelines for perioperative care.

Methods: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee (OEBP) drafted five consensus-based questions regarding management of children with ARMs.

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Objective: Our aim was to better understand attitudes towards parental leave from the perspective of both surgeon faculty and current surgical trainees. We hypothesized that support for trainees to take parental leave would vary by year of residency graduation and by parental status.

Design: We conducted a web-based survey regarding opinions on trainee parental leave.

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Objectives: For neonates and infants with aortic valve pathology, the Ross procedure historically has been associated with high rates of morbidity and mortality. Data regarding long-term durability are lacking.

Methods: The international, multi-institutional Ross Collaborative included 6 tertiary care centers.

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Objective: The Subaxial Cervical Spine Injury Classification (SLIC) score has not been previously validated for a pediatric population. The authors compared the SLIC treatment recommendations for pediatric subaxial cervical spine trauma with real-world pediatric spine surgery practice.

Methods: A retrospective cohort study at a pediatric level 1 trauma center was conducted in patients < 18 years of age evaluated for trauma from 2012 to 2021.

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Two percent of pediatric malignancies arise primarily in the liver; roughly 60% of these cancers are hepatoblastoma (HB). Despite the rarity of these cases, international collaborative efforts have led to the consistent histological classification and staging systems, which facilitate ongoing clinical trials. Other primary liver malignancies seen in children include hepatocellular carcinoma (HCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), undifferentiated embryonal sarcoma of the liver (UESL), and hepatocellular neoplasm not otherwise specified (HCN-NOS).

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Neonatal Seizures.

Pediatr Rev

July 2024

Division of Neurology, Departments of Neurology and Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA.

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Background: Previous studies have reported racial disparities in extracorporeal membrane oxygenation (ECMO) utilization in pediatric cardiac patients.

Objectives: The objective of this study was to determine if there was racial/ethnic variation in ECMO utilization and, if so, whether mortality was mediated by differences in ECMO utilization.

Methods: This is a multicenter, retrospective cohort study of the Pediatric Cardiac Critical Care Consortium clinical registry.

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While significant progress has been made in reducing disparities within the US health care system, notable gaps remain. This article explores existing disparities within pediatric congenital heart disease care. Congenital heart disease, the most common birth defect and a leading cause of infant death, has garnered substantial attention, revealing certain disparities within the US health care system.

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Feeding Variation Among Infants in Acute Care Cardiology Units.

Pediatr Cardiol

June 2024

Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, St. Louis, MS, USA.

Infants with heart disease are at high risk of feeding difficulties and complications. Feeding practices amongst acute care cardiology units are not standardized. This study aims to describe feeding practices for infants at the time of discharge from a Pediatric Acute Care Cardiology Collaborative (PAC) center and practice variation between centers.

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Background: Oxygen saturation (Spo) screening has not led to earlier detection of critical congenital heart disease (CCHD). Adding pulse oximetry features (ie, perfusion data and radiofemoral pulse delay) may improve CCHD detection, especially coarctation of the aorta (CoA). We developed and tested a machine learning (ML) pulse oximetry algorithm to enhance CCHD detection.

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Article Synopsis
  • Hepatic artery complications (HACs) after pediatric liver transplantation can lead to significant health issues, and this study aims to understand how common they are, how they are managed, and what outcomes arise from these complications.
  • The research utilizes the international HEPATIC Registry, collecting data on pediatric patients under 18 who experienced HAC within the last 20 years, focusing on survival rates and treatment success.
  • Ethical approval will be obtained from all participating sites, and findings will be shared at conferences and in academic journals, with the study registered on ClinicalTrials.gov (NCT05818644).
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Hodgkin lymphoma (HL) occurs throughout the lifespan but is one of the most common cancers in adolescents and young adults (AYA; 15-39 years). HL has become a highly curable disease with survival rates surpassing 90%, including patients with high-risk and advanced stage disease. Unfortunately, intensive treatment carries a risk of short- and long-term toxicity.

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Article Synopsis
  • The study evaluates the safety, tolerability, and effectiveness of tofacitinib in children and adolescents with juvenile idiopathic arthritis (JIA) in a long-term extension (LTE) study.
  • Out of 225 patients treated with tofacitinib, a high percentage experienced adverse events (AEs), although serious AEs were relatively low, with a focus on infection rates and disease activity.
  • Results showed significant improvement in disease activity scores over time, with a notable increase in patients achieving inactive disease status after 48 months of treatment.
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Background: Prediction of outcomes in perinatal arterial ischemic stroke (PAIS) is challenging. We performed a systematic review and meta-analysis to determine whether infarct characteristics can predict outcomes in PAIS.

Methods: A systematic search was conducted using five databases in January 2023.

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Background: Cervical spine injuries in children are uncommon but potentially devastating; however, indiscriminate neck imaging after trauma unnecessarily exposes children to ionising radiation. The aim of this study was to derive and validate a paediatric clinical prediction rule that can be incorporated into an algorithm to guide radiographic screening for cervical spine injury among children in the emergency department.

Methods: In this prospective observational cohort study, we screened children aged 0-17 years presenting with known or suspected blunt trauma at 18 specialised children's emergency departments in hospitals in the USA affiliated with the Pediatric Emergency Care Applied Research Network (PECARN).

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Importance: Pediatric consensus guidelines recommend antibiotic administration within 1 hour for septic shock and within 3 hours for sepsis without shock. Limited studies exist identifying a specific time past which delays in antibiotic administration are associated with worse outcomes.

Objective: To determine a time point for antibiotic administration that is associated with increased risk of mortality among pediatric patients with sepsis.

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Playing Telephone: Characterizing the Interfacility Referral Process to the PICU.

Pediatr Crit Care Med

June 2024

Both authors: Department of Pediatrics and Intermountain Primary Children's Hospital, Utah School of Medicine, Salt Lake City, UT.

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Method of home tube feeding and 2-3-year neurodevelopmental outcome.

J Perinatol

November 2024

Department of Pediatrics, Division of Neonatology, University of Utah, Salt Lake City, UT, USA.

Objective: To describe the Bayley Scales of Infant Development 3rd Edition (Bayley-III) of infants discharged home receiving tube feeds.

Study Design: Retrospective review of infants discharged with nasogastric or gastrostomy tube feeds and completed a Bayley-III assessment at 2-3-years of age through a neonatal follow-up program. Results were reported using descriptive statistics.

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Anaplastic sarcoma of the kidney (DICER1-sarcoma of the kidney): A report from the International Pleuropulmonary Blastoma/DICER1 Registry.

Pediatr Blood Cancer

August 2024

International Pleuropulmonary Blastoma/DICER1 Registry, Children's Minnesota, Minneapolis, Minnesota, USA.

Background: Anaplastic sarcoma of the kidney (ASK) is a DICER1-related neoplasm first identified as a distinctive tumor type through the evaluation of unusual cases of putative anaplastic Wilms tumors. Subsequent case reports identified the presence of biallelic DICER1 variants as well as progression from cystic nephroma, a benign DICER1-related neoplasm. Despite increasing recognition of ASK as a distinct entity, the optimal treatment remains unclear.

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Background: Li-Fraumeni syndrome (LFS) is an inherited cancer predisposition syndrome with an estimated prevalence of 1 in 3,000-5,000 individuals. LFS poses a significant cancer risk throughout the lifespan, with notable cancer susceptibility in childhood. Despite being predominantly inherited, up to 20% of cases arise .

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Article Synopsis
  • Fetal imaging studies are used to assess the prognosis of congenital diaphragmatic hernia (CDH) and the need for therapy, but recent findings suggest these predictions may not be accurate anymore due to improved survival rates.
  • A study on 58 left-sided CDH cases revealed that contemporary survival rates were significantly higher across all severity classifications compared to past studies, indicating that current prognostic criteria are outdated.
  • The authors argue that these overly pessimistic imaging criteria could result in unnecessary fetal interventions, stressing the need for new prognostic guidelines in future research.
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