47 results match your criteria: "Children's Hospital of Philadelphia Philadelphia Pennsylvania USA.[Affiliation]"

Importance: Objective measures of lung function are critical for assessing respiratory outcomes of prematurity. Among extremely low gestational age neonates (ELGANs) (< 29 weeks gestational age), high rates of neurodevelopmental impairment may interfere with lung function testing. Impulse oscillometry (IOS) is a noninvasive test of respiratory system mechanics not requiring forced expiration.

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Vascular malformations of the aortic arch and its main branches such as cervical aortic arch and high-riding brachiocephalic artery can present as pulsatile neck swelling. Although this entity is rare, an initial Doppler ultrasound is suggested before intervention to avoid complications from unidentified aberrant vessels.

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Diagnosis of craniofacial morphea (CM) relies upon clinical examination of progressive craniofacial changes. We assess the utility of 3D stereophotogrammetry in documenting asymmetry of the face compared to clinical notetaking. This retrospective study of 3D images and clinical documentation included 32 patients (mean age 15.

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Intestinal webs are either congenital or acquired. There are few reported cases of either chemotherapy or nonsteroidal anti-inflammatory medications leading to acquired intestinal webs in adults. There are limited descriptions of endoscopic interventions used for therapy of numerous duodenal webs in pediatrics.

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Objectives: While guidelines exist for the diagnosis and management of pediatric celiac disease (CeD), current practices in North America are not well-described. This study aimed to explore current practice patterns to identify gaps and direct future clinical, training and research initiatives.

Methods: A 23-item survey designed by the Celiac Disease Special Interest Group was distributed electronically to its members.

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Background/aims: Nonalcoholic fatty liver disease (NAFLD) is prevalent among children, and lifestyle modification is the primary treatment approach. However, the optimal exercise duration, frequency, and intensity for managing NAFLD remain undefined. This study aimed to gain insights from the patient perspective by examining exercise behaviors, preferences, and barriers in children with NAFLD.

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Objective: Pediatric tracheostomy is associated with high morbidity and mortality, yet clinician knowledge and quality of tracheostomy care may vary widely. In situ simulation is effective at detecting and mitigating related latent safety threats, but evaluation via retrospective video review has disadvantages (eg, delayed analysis, and potential data loss). We evaluated whether a novel mobile application is accurate and reliable for assessment of in situ tracheostomy emergency simulations.

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Children with pulmonary hypertension (PH) often demonstrate limited exercise capacity. Data support exercise as an effective nonpharmacologic intervention among adults with PH. However, data on exercise training in children and adolescents are limited, and characteristics of the optimal exercise program in pediatric PH have not been identified.

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Introduction: Generation Z learners are entering emergency medicine (EM) residency training, bringing unique learning preferences that influence their engagement with residency education. To optimally teach and motivate this incoming generation of learners, EM educators must understand and adapt to the changing instructional landscape.

Methodology: The Simulation Leaders Advancing the Next Generation in Emergency Medicine (SLANG-EM) Workgroup was created to identify effective educational strategies for Generation Z learners entering EM.

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Type II D-2-Hydroxyglutaric aciduria (T2D2HGA) is caused by a gain-of-function pathogenic variant in Isocitrate Dehydrogenase 2 (IDH2). Patients with T2D2HGA commonly present with developmental delay, seizures, cardiomyopathy, and arrhythmias. The recently approved IDH2-inhibitor Enasidenib targets the p.

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Simulation training plays a vital role in modern medical education, fostering safe skill development. Task-trainer manikin and cadaveric airway management training (CAMT) offer realistic airway management practice. Simulation allows learners the opportunity to manage high-risk, low-frequency scenarios, including difficult airways and massive airway contamination, common in emergent airway management.

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Pulmonary vasodilator treatment can improve hemodynamics, right ventricular function, symptoms, and survival in pediatric pulmonary hypertension (PH). However, clinical trial data are lacking due to many constraints. One major limitation is the lack of relevant trial endpoints reflective of hemodynamics or functional status in patients in whom standard exercise testing is impractical, unreliable, or not reproducible.

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Unlabelled: Ferredoxin-2 (FDX2) is an electron transport protein required for iron-sulfur clusters biosynthesis. Pathogenic variants in have been associated with autosomal recessive -related disorder characterized by mitochondrial myopathy with or without optic atrophy and leukoencephalopathy. We described a new case harboring compound heterozygous variants in who presented with recurrent rhabdomyolysis with severe episodes affecting respiratory muscle.

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The coronavirus disease 2019 (COVID-19) pandemic posed new challenges in health care delivery for patients of all ages. These included inadequate personal protective equipment, workforce shortages, and unknowns related to a novel virus. Children have been uniquely impacted by COVID-19, both from the system of care and socially.

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Dihydrolipoamide dehydrogenase (DLD; E3) oxidizes lipoic acid. Restoring the oxidized state allows lipoic acid to act as a necessary electron sink for the four mitochondrial keto-acid dehydrogenases: pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, branched-chain α-keto-acid dehydrogenase, and 2-oxoadipate dehydrogenase. DLD deficiency (DLDD) is caused by biallelic pathogenic variants in .

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Hematologic malignancy is a risk factor for severe coronavirus disease 2019 (COVID-19) in adults; however, data specific to children with leukemia are limited. High-quality infectious adverse event data from the ongoing Children's Oncology Group (COG) standard-risk B acute lymphoblastic leukemia/lymphoma (ALL/LLy) trial, AALL1731, were analyzed to provide a disease-specific estimate of SARS-CoV-2 infection outcomes in pediatric ALL. Of 253 patients with reported infections, the majority (77.

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Objective: Cervical spine imaging decision-making for pediatric traumas is complex and multidisciplinary. Implementing a risk assessment tool has the potential to reduce variation in these decisions and unnecessary radiation exposure for pediatric patients. We sought to determine how emergency department-trauma team dynamics may affect implementation of such a tool.

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Key Clinical Message: This case report aims to increase awareness that pulmonary artery aneurysms may occur as a complication of neglected patent ductus arteriosus and should be sought in children with ill-treated congenital heart diseases.

Abstract: Pulmonary artery aneurysm is a rare anomaly with an autopsy prevalence of 1:14,000. These aneurysms can arise secondary to various etiologies, with congenital causes identified in 25% of cases and congenital heart diseases (CHD) responsible for more than half of these cases.

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Background: The last few years have seen an increased focus on diversity, equity, and inclusion (DEI) initiatives across organizations. Simulation has been used in varying degrees for teaching about DEI topics with emergency medicine; however, there are no established best practices or guidelines on this subject. To further examine the use of simulation for DEI teachings, the DEISIM work group was created as a collaboration between the Society of Academic Emergency Medicine (SAEM) Simulation Academy and the Academy for Diversity and Inclusion in Emergency Medicine (ADIEM).

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Background: Simulation provides consistent opportunities for residents to practice high-stakes, low-frequency events such as pediatric resuscitations. To increase standardization across North American residency programs, the Emergency Medicine Resident Simulation Curriculum for Pediatrics (EM ReSCu Peds) was developed. However, access to high-quality simulation/pediatric expertise is not uniform.

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Continuous subcutaneous (SubQ) treprostinil is an effective therapy for pediatric patients diagnosed with pulmonary hypertension (PH). To date, the clinical characteristics and factors associated with failure to tolerate this therapy have not been described. The purpose was to describe patient-reported factors contributing to SubQ treprostinil intolerance in pediatric patients with PH.

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Objective: To assess the efficacy of liraglutide 3.0 mg, a glucagon-like peptide-1 (GLP-1) receptor agonist, for binge eating disorder (BED).

Methods: Adults with a body mass index (BMI) ≥ 27 kg/m enrolled in a pilot, 17-week double-blind, randomized controlled trial of liraglutide 3.

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Asparagine synthetase (ASNS) catalyzes the synthesis of asparagine (Asn) from aspartate and glutamine. Biallelic mutations in the gene result in ASNS Deficiency (ASNSD). Children with ASNSD exhibit congenital microcephaly, epileptic-like seizures, and continued brain atrophy, often leading to premature mortality.

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