Whole-Body MRI in Children: Concepts and Controversies- Expert Panel Narrative Review.

The use of whole-body MRI (WBMRI) in children, from infancy to adolescence, has expanded rapidly over the past decade, with increasing uptake and a broadening range of clinical indications. Current indications include screening for presymptomatic lesions in cancer predisposition syndromes; tumor staging in known malignancies; investigating fevers of unknown origin; as well as diagnosing and monitoring rheumatologic diseases, vascular anomalies and neuromuscular disorders. This AJR Expert Panel Narrative Review aims to offer a comprehensive discussion of WBMRI in pediatric patients, exploring protocols and other technical considerations, clinical indications, implementation challenges and troubleshooting, as well as controversies in widespread adoption, while considering emerging trends and directions.

Outcomes for Children With Congenital Heart Disease Undergoing Ventricular Assist Device Implantation: An ACTION Registry Analysis.

Background: There are no contemporary reports that highlight the national outcomes for children with congenital heart disease (CHD) undergoing ventricular assist device (VAD) implantation.

Objectives: This study sought to evaluate differences in VAD outcomes for children with CHD to those with non-CHD as well as those with univentricular CHD to those with biventricular CHD.

Methods: Data for CHD and non-CHD patients from the multicenter ACTION (Advanced Cardiac Therapies Improving Outcomes Network) undergoing VAD implantation from April 2018 to February 2023 were included.

Studying Rare Movement Disorders: From Whole-Exome Sequencing to New Diagnostic and Therapeutic Approaches in a Modern Genetic Clinic.

Rare movement disorders often have a genetic etiology. New technological advances have increased the odds of achieving genetic diagnoses: next-generation sequencing (NGS) (whole-exome sequencing-WES; whole-genome sequencing-WGS) and long-read sequencing (LRS). In 2017, we launched a WES program for patients with rare movement disorders of suspected genetic etiology.

In toto biological framework: Modeling interconnectedness during human development.

Recent advancements in pluripotent stem cell and synthetic tissue technology have brought significant breakthroughs in studying early embryonic development, particularly within the first trimester of development in humans. However, during fetal stage development, investigating further biological events represents a major challenge, partly due to the evolving complexity and continued interaction across multiple organ systems. To bridge this gap, we propose an "in toto" biological framework that leverages a triad of technologies: synthetic tissues, intravital microscopy, and computer vision to capture in vivo cellular morphodynamics, conceptualized as single-cell choreography.

Practical approach to quantitative liver and pancreas MRI in children.

Quantitative abdominal magnetic resonance imaging (MRI) offers non-invasive, objective assessment of diseases in the liver, pancreas, and other organs and is increasingly being used in the pediatric population. Certain quantitative MRI techniques, such as liver proton density fat fraction (PDFF), R2* mapping, and MR elastography, are already in wide clinical use. Other techniques, such as liver T1 mapping and pancreas quantitative imaging methods, are emerging and show promise for enhancing diagnostic sensitivity and treatment monitoring.

Growth, Body Composition, and Strength of Children With Cystic Fibrosis Treated With Elexacaftor/Tezacaftor/Ivacaftor (ETI).

Objective: We prospectively monitored rates of change for growth, body mass and composition, muscle strength, and FEV1 in 6-11-year-olds initiating ETI therapy, comparing them to those of US reference children. We assessed factors potentially contributing to rate of change and report ranges of individual variation.

Methods: Body composition was assessed using bioelectrical impedance analysis (BIA), and rates of change were analyzed using linear mixed effects regression models.

Klotho mutation does not accelerate intervertebral disc aging in mice.

Aging is a risk factor for several chronic conditions, including intervertebral disc degeneration and associated back pain. Disc pathologies include loss of reticular-shaped nucleus pulposus cells, disorganization of annulus fibrosus lamellae, reduced disc height, and increased disc bulging. Sonic hedgehog, cytokeratin 19, and extracellular matrix proteins are markers of healthy disc.

TClC effectively suppresses the growth and metastasis of NSCLC via polypharmacology.

Despite significant advances in targeted therapies and immunotherapies, non-small cell lung cancer (NSCLC) continues to present a global health challenge, with a modest five-year survival rate of 28 %, largely due to the emergence of treatment-resistant and metastatic tumors. In response, we synthesized a novel bioactive compound, ethyl 6-chlorocoumarin-3-carboxylyl L-theanine (TClC), which significantly inhibited NSCLC growth, epithelial mesenchymal transition (EMT), migration, and invasion and tumor growth and metastasis without inducing toxicity. TClC disrupts autocrine loops that promote tumor progression, particularly in stem-like CD133-positive NSCLC (CD133+ LC) cells, which are pivotal in tumor metastasis.

Propensity Score Analysis of Possible Medication Effects on Outcomes in Patients With Systemic Right Ventricles.

Background: Patients with systemic right ventricle (SRV), either d-transposition of the great arteries following an atrial switch procedure or congenitally corrected transposition of the great arteries, develop severe right ventricular dysfunction, prompting appropriate medical therapy. However, the efficacy of beta-blockers and angiotensin receptor blockers or angiotensin-converting enzyme inhibitors (ACEI) in SRV patients is unproven.

Objectives: The objective of this study was to determine the effects of ACEI/ARB and beta-blockers on outcomes in SRV patients after accounting for likely cofounders affecting their use.

Virtual and In-Person Multiple Mini-interviews: A Comparison of Two Modalities in Regard to Bias.

Purpose: To examine the characteristics between virtual multiple mini-interview (vMMI) and in-person interviews (ipMMI) in regard to difference in performance between applicant-reported gender identity and racial groups.

Methods: Retrospective multiple mini-interview (MMI) data from two vMMI interview cycles (2021 and 2022) consisting of 627 applicants and four ipMMI cycles (2017-2020) consisting of 2248 applicants. Comparisons were made between applicant subgroups including reported gender (male and female) and minority status (URiM and non-URiM).

International consensus guidelines on the implementation and monitoring of vosoritide therapy in individuals with achondroplasia.

Achondroplasia is the most common genetic form of short-limbed skeletal dysplasia (dwarfism). Clinical manifestations and complications can affect individuals across the lifespan, including the need for adaptations for activities of daily living, which can affect quality of life. Current international guidelines focus on symptomatic management, with little discussion regarding potential medication, as therapeutic options were limited at the time of their publication.

Phasic dopamine release in two different rat models of attention-deficit/hyperactivity disorder: Spontaneously hypertensive rats (SHR) versus Lphn3 knockout rats.

We examined DA activity in the medial prefrontal cortex (mPFC) and nucleus accumbens core (NAcc) in two Different Rat Models of Attention-Deficit/Hyperactivity Disorder: Spontaneously Hypertensive Rats (SHR) Versus Lphn3 Knockout Rats. We examined baseline stimulation-evoked phasic DA release, half-life, and DA autoreceptor (DAR) functioning in the mPFC and NAcc, as well as the response to nomifensine (10 mg/kg, IP), a DA transporter (DAT) blocker, on these measures in the NAcc. Both rat models were hypodopaminergic, with notable regional and mechanistic differences.

Rhinoconjunctivitis symptoms in children and adolescents with asthma: Longitudinal clustering analysis.

Background: Rhinoconjunctivitis phenotypes are conventionally described on the basis of symptom severity, duration and seasonality, and aeroallergen sensitization. It is not known whether these phenotypes fully reflect the patterns of symptoms seen at a population level.

Objective: We sought to identify phenotypes of rhinoconjunctivitis on the basis of symptom intensity and seasonality using an unbiased approach and to compare their characteristics.

Transplacental signals involved in the programming effects of prenatal psychosocial stress on neurodevelopment.

Exposure to psychosocial stress during pregnancy has been associated with the emergence of neurodevelopmental and neuropsychiatric disorders in offspring. The placenta is known to orchestrate various functions that are essential for normal fetal development, including the brain. It has therefore been postulated that alterations in such functions, and downstream signaling, have the potential to dramatically affect brain developmental trajectories and contribute to adverse neurodevelopmental outcomes.

System vaccinology analysis of predictors and mechanisms of antibody response durability to multiple vaccines in humans.

We performed a systems vaccinology analysis to investigate immune responses in humans to an H5N1 influenza vaccine, with and without the AS03 adjuvant, to identify factors influencing antibody response magnitude and durability. Our findings revealed a platelet and adhesion-related blood transcriptional signature on day 7 that predicted the longevity of the antibody response, suggesting a potential role for platelets in modulating antibody response durability. As platelets originate from megakaryocytes, we explored the effect of thrombopoietin (TPO)-mediated megakaryocyte activation on antibody response longevity.

Systematic Review of Pain Assessment Measures Used in Pediatric Acute Lymphoblastic Leukemia.

Background: Pain is a prevalent, frequent, and often persistent symptom among children with acute lymphoblastic leukemia (ALL). Despite its high prevalence, pain has remained understudied, and no evidence-based recommendations exist for how best to assess and treat pain in this population. Without proper assessment, clinical efforts to improve pain management in pediatric ALL will be ineffective.

Engraft: A Collaborative Learning Health Network for Enhanced Transplant and Cellular Therapy Outcomes.

The Engraft Learning Health Network (LHN) aims to improve outcomes for patients undergoing transplant and cellular therapy (TCT) through a collaborative, data-driven approach. Engraft brings together diverse stakeholders, including clinicians, patients, caregivers, and institutions, to standardize best practices and accelerate the dissemination of innovations in TCT care. By establishing a multicenter, real-world clinical registry focused on rapid-cycle quality improvement (QI) and implementation research, Engraft seeks to reduce variability in clinical practice to improve TCT outcomes across centers.

Are enteral devices risk factors for central line-associated bloodstream infections in children with intestinal failure?

Background & Aims: Central line-associated bloodstream infections (CLABSI) represent one of the most common and serious complications in children with intestinal failure (IF). This study aimed to assess if there is an association between the use of enteral devices (feeding tubes and stomas) with rate of CLABSI after adjusting for clinically relevant factors. Second, association between enteral devices with time to first CLABSI event was evaluated.

A Needs Assessment of Labor and Delivery Nurses Performing NRP in the Delivery Room.

Introduction: Labor and delivery (L&D) nurses are often responsible for initiating the critical first steps of the Neonatal Resuscitation Program (NRP). Identification of knowledge gaps and underlying reasons for NRP non-adherence is crucial for designing educational interventions.

Methods: A convenience sample of 37 L&D nurses from two delivery hospitals were enrolled.

User-defined virtual sensors: A new solution to the problem of temporal plus epilepsy sources.

Objective: The most common medically resistant epilepsy (MRE) involves the temporal lobe (TLE), and children designated as temporal plus epilepsy (TLE+) have a five-times increased risk of postoperative surgical failure. This retrospective, blinded, cross-sectional study aimed to correlate visual and computational analyses of magnetoencephalography (MEG) virtual sensor waveforms with surgical outcome and epilepsy classification (TLE and TLE+).

Methods: Patients with MRE who underwent MEG and iEEG monitoring and had at least 1 year of postsurgical follow-up were included in this retrospective analysis.