Chromosome mis-segregation triggers cell cycle arrest through a mechanosensitive nuclear envelope checkpoint.

Errors during cell division lead to aneuploidy, which is associated with genomic instability and cell transformation. In response to aneuploidy, cells activate the tumour suppressor p53 to elicit a surveillance mechanism that halts proliferation and promotes senescence. The molecular sensors that trigger this checkpoint are unclear.

A lifespan-generalizable skull-stripping model for magnetic resonance images that leverages prior knowledge from brain atlases.

In magnetic resonance imaging of the brain, an imaging-preprocessing step removes the skull and other non-brain tissue from the images. But methods for such a skull-stripping process often struggle with large data heterogeneity across medical sites and with dynamic changes in tissue contrast across lifespans. Here we report a skull-stripping model for magnetic resonance images that generalizes across lifespans by leveraging personalized priors from brain atlases.

Transradial cerebral angiography in the neonate and infant: a case series.

Background: We aim to share our experience of transradial access (TRA) for cerebral angiography and intervention in five patients ranging from 6 days to 7 months of age.

Methods: In this institutional review board-approved, retrospective case series, we reviewed all patients who underwent TRA for cerebral angiography with and without intervention. We describe three techniques for radial artery cannulation, namely: (1) bareback; (2) with a micropuncture sheath; and (3) with an intravenous catheter.

Genetic Test Utilization and Cost among Families of Children Evaluated for Genetic Conditions: An Analysis of USA Commercial Claims Data.

Introduction: Healthcare payers in the USA increasingly cover genetic testing, including exome sequencing (ES), for pediatric indications. Analysis of claims data enables understanding of utilization and costs in real-world settings. The objective of this study was to describe genetic test utilization, diagnostic outcomes, and costs for children who received ES as well as for those who received less comprehensive forms of genetic testing, along with their families.

Pediatric Snowboarding-Related Concussions and Musculoskeletal Injuries Decreased From 2012 to 2022.

Purpose: To analyze mechanisms, diagnoses, and incidence of youth snowboarding-related injuries presenting to US emergency departments.

Methods: Data from the National Electronic Injury Surveillance System were analyzed for pediatric snowboarding injuries (≤18 years old) from 2012 to 2022. Data were collected for mechanism of injury, diagnosis, location of injury, and disposition.

Pediatric Bilateral Vestibular Hypofunction: A Review of 26 Cases.

Objective: Peripheral bilateral vestibular hypofunction (BVH) is a rare condition that is well-studied in the adult population, whereas characterization in children has been limited. We report a pediatric cohort of patients with BVH at a multidisciplinary, tertiary care pediatric vestibular clinic.

Methods: A record review of 832 patients with balance-related complaints in our center was conducted.

Diagnostic Accuracy of S100B in Predicting Intracranial Abnormalities on CT Imaging Following Mild Traumatic Brain Injury: A Systematic Review and Meta-analysis.

Traumatic brain injury (TBI) is a major cause of health loss and disabilities globally, burdening health care systems. Mild TBI is a common cause of emergency department visits. Computed tomography (CT) scans are the mainstay for acute TBI imaging.

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.

Intracerebroventricular B7-H3-targeting CAR T cells for diffuse intrinsic pontine glioma: a phase 1 trial.

Diffuse intrinsic pontine glioma (DIPG) is a fatal central nervous system (CNS) tumor that confers a median survival of 11 months. As B7-H3 is expressed on pediatric CNS tumors, we conducted BrainChild-03, a single-center, dose-escalation phase 1 clinical trial of repetitive intracerebroventricular (ICV) dosing of B7-H3-targeting chimeric antigen receptor T cells (B7-H3 CAR T cells) for children with recurrent or refractory CNS tumors and DIPG. Here we report results from Arm C, restricted to patients with DIPG.

Enhanced D614G and Omicron Variants Antibody Persistence in Infants at 2 Months of Age Following Maternal mRNA Booster Vaccination During Pregnancy or Postpartum.

Background: Following maternal COVID-19 vaccination, the persistence of antibodies in sera and breast milk for mothers and infants is not well characterized. We sought to describe the persistence of antibodies through 2 months after delivery in maternal and infant serum and breast milk following maternal COVID-19 mRNA vaccination and to examine differences by receipt of booster dose during pregnancy or postpartum.

Methods: This is a prospective cohort study with enrollment from July 2021 to January 2022 at 9 US academic sites.

Targeting Senescent Stemlike Subpopulations in Philadelphia Chromosome-Like Acute Lymphoblastic Leukemia.

Philadelphia chromosome-like B-cell acute lymphoblastic leukemia (Ph-like ALL) is driven by genetic alterations that induce constitutive kinase signaling and is associated with chemoresistance and high relapse risk in children and adults. Preclinical studies in the most common CRLF2-rearranged/JAK pathway-activated Ph-like ALL subtype have shown variable responses to JAK inhibitor-based therapies, suggesting incomplete oncogene addiction and highlighting a need to elucidate alternative biologic dependencies and therapeutic vulnerabilities, while the ABL-class Ph-like ALL subtype appears preferentially sensitive to SRC/ABL- or PDGFRB-targeting inhibitors. Which patients may be responsive versus resistant to tyrosine kinase inhibitor (TKI)-based precision medicine approaches remains a critical knowledge gap.

Predicting Blood Loss in Aneurysmal Bone Cyst Surgery.

Background: Preoperative estimation of intraoperative blood loss is essential for its management and literature is lacking with respect to factors influencing blood loss in aneurysmal bone cysts (ABC) surgery. The purpose of this study is to identify risk factors and predictors for blood loss in ABC surgery.

Methods: An IRB-approved retrospective review was performed from 2011 to 2021 at a pediatric tertiary care center.

Epithelial outgrowth through mesenchymal rings drives alveologenesis.

Determining how alveoli are formed and maintained is critical to understanding lung organogenesis and regeneration after injury. To study the cellular dynamics of this critical stage of lung development, we have used scanned oblique-plane illumination microscopy of living lung slices to observe alveologenesis in real time at high resolution over several days. Contrary to the prevailing notion that alveologenesis occurs by airspace subdivision via ingrowing septa, we find that alveoli form by ballooning epithelial outgrowth supported by contracting mesenchymal ring structures.

Tgfβ signaling stimulates glycolysis to promote the genesis of synovial joint interzone in developing mouse embryonic limbs.

The initial interzone cells for synovial joints originate from chondrocytes, but such critical transition is minimally understood. With single-cell RNA sequencing (scRNA-seq) of murine embryonic knee joint primordia, we discovered that heightened expression of glycolysis genes characterized developing interzone cells when compared to flanking chondrocytes. Conditional deletion of the glucose transporters and/or , in either the incipient pre-skeletal mesenchyme with or in chondrocytes with , disrupted interzone formation dose-dependently.

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.

Using Heart Rate and Behaviors to Predict Effective Intervention Strategies for Children on the Autism Spectrum: Validation of a Technology-Based Intervention.

Many children on the autism spectrum engage in challenging behaviors, like aggression, due to difficulties communicating and regulating their stress. Identifying effective intervention strategies is often subjective and time-consuming. Utilizing unobservable internal physiological data to predict strategy effectiveness may help simplify this process for teachers and parents.

Understanding the Patient Experience with Twice-Nightly Sodium Oxybate Therapy for Narcolepsy: A Social Listening Experiment.

Background/objectives: Narcolepsy is a chronic neurologic disorder associated with substantial challenges that affect the social, emotional, and financial quality-of-life domains. A social listening analysis and structured survey were conducted to better understand the candid perspective of people with narcolepsy (PWN) and their experience with twice-nightly sodium oxybate (SXB).

Methods: To characterize conversations and experiences in narcolepsy communities where SXB was mentioned, a social media analysis was conducted from August 2011 to October 2022.

Lysyl Oxidase Mediates Proliferation and Differentiation in the Esophageal Epithelium.

In homeostatic conditions, the basal progenitor cells of the esophagus differentiate into a stratified squamous epithelium. However, in the setting of acid exposure or inflammation, there is a marked failure of basal cell differentiation, leading to basal cell hyperplasia. We have previously shown that lysyl oxidase (LOX), a collagen crosslinking enzyme, is upregulated in the setting of allergic inflammation of the esophagus; however, its role beyond collagen crosslinking is unknown.

The Proteomics of T-Cell and Early T-Cell Precursor (ETP) Acute Lymphocytic Leukemia: Prognostic Patterns in Adult and Pediatric-ETP ALL.

Background: The 5-year overall survival (OS) rates of T-cell lymphocytic leukemia (T-ALL) are better for children (>90%) compared to adults (~57%). The early T-cell precursor (ETP) T-ALL subtype is prognostically unfavorable in adults, but less significant in pediatric T-ALL, and the diagnosis and prognosis of "near"-ETP is controversial. We compared protein and RNA expression patterns in pediatric and adult T-ALL to identify prognostic subgroups, and to further characterize ETP and near-ETP T-ALL in both age groups.

Cannabis use before and after metabolic and bariatric surgery: literature review.

As accessibility and legalization of cannabis rise throughout the United States (US), programs have sought guidance about whether its use should be considered a contraindication or, if not a contraindication, what recommendations patients should receive regarding appropriate use before and after metabolic and bariatric surgery (MBS). In this review, medical, nutritional, pharmacological, and psychological considerations are presented by a multidisciplinary group of members of the American Society for Metabolic and Bariatric Surgery (ASMBS). Research suggests several risks associated with long-term cannabis use in the general population, but research in the MBS population, specifically, is limited.

Comparison of Ultrasound Characteristics of Peripapillary Hyperreflective Ovoid Mass-Like Structures (PHOMS) and Optic Disc Drusen in Children.

Background: In children, pseudopapilledema is frequently caused by peripapillary hyperreflective ovoid mass-like structures (PHOMS) or optic disc drusen (ODD). While enhanced depth imaging (EDI) OCT can identify both, lack of cooperation, especially from younger children due to the duration of testing, often necessitates the use of B-scan ultrasound (BSUS). This study investigated whether PHOMS are hyperreflective on BSUS and if BSUS can differentiate PHOMS from ODD.

Disparities in Surgical Intervention in Pediatric Patients With Submucous Cleft Palate and Velopharyngeal Dysfunction.

Objective: To evaluate factors impacting access to and timing of surgery in patients with submucous cleft palate (SMCP) and velopharyngeal dysfunction (VPD).

Study Design: Retrospective cohort study.

Setting: Single academic medical center.

A Longitudinal Exploration of -Related Hemiplegic Migraine in Children Using Electronic Medical Records.

Background And Objectives: Since the initial description of related hemiplegic migraine (HM), the phenotypic spectrum has expanded from mild episodes in neurotypical individuals to potentially life-threatening events frequently seen in individuals with developmental and epileptic encephalopathies. However, the overall longitudinal course throughout childhood remains unknown.

Methods: We analyzed HM and seizure history from electronic medical records in individuals with -related HM, delineating frequency and severity of events in monthly increments through a standardized approach.