Extracorporeal Membrane Oxygenation in Children with Pulmonary Atresia and Intact Ventricular Septum: Mortality and Associated Outcomes.

Data on outcomes of extracorporeal membrane oxygenation (ECMO) are limited in patients with pulmonary atresia intact ventricular septum (PAIVS). The objective of this study was to describe the use of ECMO and the associated outcomes in patients with PAIVS. We retrospectively reviewed neonates with PAIVS who received ECMO between 2009 and 2019 in 19 US hospitals affiliated with the Collaborative Research for the Pediatric Cardiac Intensive Care Society (CoRe-PCICS).

Prevention and Management of Infectious Complications in Pediatric Patients With Cancer: A Survey Assessment of Current Practices Across Children's Oncology Group Institutions.

Introduction: While clinical practice guidelines (CPGs) for pediatric oncology infection prophylaxis and management exist, few data describe actual management occurring at pediatric oncology centers.

Methods: An electronic survey querying infection management practices in nontransplant pediatric oncology patients was iteratively created by the Children's Oncology Group (COG) Cancer Control and Supportive Care Infectious Diseases Subcommittee and sent to leaders at all COG institutions, limiting each site to one response to represent their institution.

Results: The response rate was 57% (129/227 institutions).

Pharmacokinetics and Pharmacodynamics of Intravenous Magnesium Sulfate in Pediatric Acute Asthma Exacerbations.

Pediatric asthma exacerbations represent a significant cause of emergency department use and hospitalizations. Despite available treatment options, many children's exacerbations are refractory to standard therapies and require adjunct treatments. The Intravenous Magnesium: Prompt use for Asthma in Children Treated in the Emergency Department study investigated the pharmacology of intravenous magnesium sulfate (IVMg) in treating pediatric asthma exacerbations.

A 7-year-old with new-onset seizures, behavior disturbance, and encephalitis.

Refractory seizures are common in pediatric neurology; consideration of whether seizures are acutely symptomatic of an underlying disease process is critical. In this case, a previously healthy 7-year-old patient presented with intractable focal seizures in the setting of a viral prodrome and headache. This case highlights a comprehensive work up of new-onset refractory seizures in a patient with features of encephalitis and describes a diagnostic quandary that ultimately led to a rare unifying diagnosis.

Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group.

Objective: We aimed to provide an evidence-supported approach to diagnose, monitor, and treat children with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC).

Methods: The core group formulated seven PICO-structured clinical questions. A systematic literature search from inception to December 2022 was conducted by a medical librarian using MEDLINE and EMBASE.

Factors Associated with Pediatric Drowning-Associated Lung Injury.

Objective: To identify risk factors for clinically-important drowning-associated lung injury (ciDALI) in children.

Study Design: This was a cross-sectional study of children (0 through18 years) who presented to 32 pediatric emergency departments (EDs) from 2010 through 2017. We reviewed demographics, comorbidities, prehospital data, chest radiographs reports, and ED course from emergency medical services, medical, and fatality records.

Perfluorodecanoic acid (PFDA) increases oxidative stress through inhibition of mitochondrial β-oxidation.

PER: and polyfluoroalkyl substances (PFAS) are a large group of synthetic organic chemicals that are ubiquitous environmental pollutants. Among PFAS, perfluorodecanoic acid (PFDA) is one of the most toxic compounds, but the molecular basis behind its toxicity is not fully understood. In an interspecies comparison with placental cells (HTR-8/SVneo) and zebrafish embryos, we demonstrate that PFDA induces mitochondrial dysfunction and impairs fatty acid β-oxidation.

Optimizing Ewing Sarcoma and Osteosarcoma Biopsy Acquisition: A Children's Oncology Group Bone Tumor Committee Consensus Statement.

Trends in diagnostic biopsy sample collection approaches for primary bone sarcomas have shifted in the past 2 decades. Although open/incisional biopsies used to be the predominant approach to obtain diagnostic material for Ewing sarcoma and osteosarcoma, image-guided core needle biopsies have increased in frequency and are safe for patients. These procedures are less invasive and reduce recovery times but have potential limitations.

Over 100 Fontan Patients on Systemic Ventricular Assist Device Support: An ACTION Update.

As the Fontan population grows, understanding successful strategies for ventricular assist device (VAD) support of the failing Fontan circulation is needed. We performed a retrospective analysis of patients with Fontan circulation and systemic VAD support in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. Competing outcomes and Kaplan-Meier estimated survival methods were used.

A Citation Analysis of the Top 50 Most Cited Nurse Practitioner Publications.

The role of nurse practitioners (NPs) has become integral to healthcare systems worldwide. Originating in the United States over 50 years ago, it has since been adopted by countries such as Canada, the United Kingdom, and Australia. To honor the research and contributions that have shaped the NP discipline, it is valuable to review and recognize key literature that has significantly impacted its development.

Imaging of common coronary artery anomalies and imaging features important for clinical decision-making.

Anomalous coronary arteries are associated with sudden cardiac death, although only in a relatively small percentage. Although this has been a field of study for years and there are many ongoing studies of larger patient cohorts and longer term follow-up, important questions remain at present in clinical decision-making, particularly whether or not to have surgery. Advanced imaging techniques including coronary computed tomography (CT) and cardiac magnetic resonance (MR) play an important role in the workup of patients with known or suspected anomalies including delineating origin and course as well as defining presence of high-risk imaging features.

Radiographic and visual response to the type II RAF inhibitor tovorafenib in children with relapsed/refractory optic pathway glioma in the FIREFLY-1 trial.

Background: Due to their anatomical locations, optic pathway gliomas (OPGs) can rarely be cured by resection. Given the importance of preserving visual function, we analyzed radiological and visual acuity (VA) outcomes for the type II RAF inhibitor tovorafenib in the OPG subgroup of the phase 2 FIREFLY-1 trial.

Methods: FIREFLY-1 investigated the efficacy (arm 1, n=77), safety, and tolerability (arms 1/2) of tovorafenib (420 mg/m2 once weekly; 600 mg maximum) in patients with BRAF-altered relapsed/refractory pediatric low-grade glioma (pLGG).

Skin Graftless Dorsal Metacarpal Island VY Advancement Flap vs. Dorsal Rectangular Flap with Skin Grafting in 213 Simple Syndactyly Releases.

Background: Simple syndactyly is a common congenital upper extremity difference. Traditional reconstructive approaches utilize skin grafts; however, recent studies suggest skin graftless techniques may improve outcomes. We hypothesized that patients who underwent reconstruction with a skin graftless dorsal metacarpal island VY advancement flap would have fewer postoperative complications compared to those released with a dorsal rectangular flap with skin grafts.

Bladder exstrophy-epispadias-cloacal exstrophy complex: characteristics, aetiologies, and epidemiologic findings.

Bladder exstrophy-epispadias-cloacal exstrophy complex (BEEC) is a spectrum of congenital urologic anomalies that involve the bladder, urethra, genitalia, and pelvic musculoskeletal system, and can affect urinary continence, sexual health, and fertility. BEEC includes a wide spectrum of anatomical abnormalities with different levels of severity: epispadias represents the mildest phenotype, classic bladder exstrophy (CBE) is the most common defect, and cloacal exstrophy (CE) - often referred to as omphalocele, exstrophy, imperforate anus, and spinal defects (OEIS) complex - is the most severe form. BEEC disorders cause significant health problems and affect the health-related quality of life (QoL) of affected individuals.

Development of a model to predict electroencephalographic seizures in neonates with hypoxic ischemic encephalopathy treated with therapeutic hypothermia.

Objective: Electroencephalographic seizures (ES) are common in neonates with hypoxic-ischemic encephalopathy (HIE), but identification with continuous electroencephalographic (EEG) monitoring (CEEG) is resource-intensive. We aimed to develop an ES prediction model.

Methods: Using a prospective observational study of 260 neonates with HIE undergoing CEEG, we identified clinical and EEG risk factors for ES, evaluated model performance with area under the receiver operating characteristic curve (AUROC), and calculated test characteristics emphasizing high sensitivity.

Newborn Screening for Critical Congenital Heart Disease: A New Algorithm and Other Updated Recommendations: Clinical Report.

Critical congenital heart disease (CCHD) screening was added to the US Recommended Uniform Screening Panel in 2011 and adopted by all US states and territories by 2018. In addition to reviewing key developments in CCHD screening since the initial American Academy of Pediatrics (AAP) endorsement in 2011, this clinical report provides 3 updated recommendations. First, a new AAP algorithm has been endorsed for use in CCHD screening.

Outcomes Following Fecal Diversion for Intractable Hirschsprung Associated Enterocolitis: A Study From the Pediatric Colorectal and Pelvic Learning Consortium.

Background: Hirschsprung associated enterocolitis (HAEC) is a challenging problem in a subset of children with Hirschsprung disease (HD). In refractory cases, fecal diversion may be required. The aim of this study was to characterize patients who require fecal diversion for HAEC management and examine their long-term outcomes.

Impact of COVID-19 on newborn hearing screening (NBHS) and early hearing detection and intervention (EHDI) services: A statewide analysis of differences and policy implications.

Introduction: This study investigates the pandemic's impact on newborn hearing screening (NBHS) and access to hearing services for children in Utah. Specifically, it explores the differences in NBHS rates, diagnostic hearing testing, early intervention enrollment, and congenital cytomegalovirus (cCMV) screening before and during the pandemic.

Methods: Utilizing a comprehensive statewide Early Hearing Detection and Intervention (EHDI) database, we analyzed data from January 2017 to December 2021, excluding a 6-month period preceding March 16, 2020, to eliminate potential confounders related to pandemic onset.

Blinatumomab in Standard-Risk B-Cell Acute Lymphoblastic Leukemia in Children.

Background: B-cell acute lymphoblastic leukemia (B-cell ALL) is the most common childhood cancer. Despite a high overall cure rate, relapsed B-cell ALL remains a leading cause of cancer-related death among children. The addition of the bispecific T-cell engager molecule blinatumomab (an anti-CD19 and anti-CD3 single-chain molecule) to therapy for newly diagnosed standard-risk (as defined by the National Cancer Institute) B-cell ALL in children may improve outcomes.

Bilateral rib-to-pelvis growth-friendly spinal surgery for congenital kyphosis in the child with myelomeningocele delays but does not prevent a definitive procedure.

Purpose: To determine the efficacy of bilateral rib-to-pelvis distraction instrumentation for the growth-friendly surgical treatment of congenital kyphosis in children with myelomeningocele.

Methods: The clinical courses of nine children (five males, four females), mean age 4.3 years (range 1.

CD33-CD123 IF-THEN Gating Reduces Toxicity while Enhancing the Specificity and Memory Phenotype of AML-Targeting CAR-T Cells.

Our study demonstrates the use of "IF-THEN" SynNotch-gated CAR-T cells targeting CD33 and CD123 in AML reduces off-tumor toxicity. This strategy enhances T-cell phenotype, improves expansion, preserves HSPCs, and mitigates cytokine release syndrome-addressing critical limitations of existing AML CAR-T therapies.