Dysregulation of Airway and Systemic Interferon Responses Promote Asthma Exacerbations in Urban Children.

Background: Determining why some upper respiratory illnesses provoke asthma exacerbations remains an unmet need.

Objective: To identify transcriptome-wide gene expression changes associated with colds that progress to exacerbation.

Methods: 208 urban children (6-17 years) with exacerbation-prone asthma were prospectively monitored for up to two cold illnesses.

Validation and performance of MicroVue sC5b-9 Plus ELISA on the Dynex DS2 platform.

Background: The complement membrane attack complex involves C5b-mediated assembly of C6-C9 polymers to form pores in cell membranes during complement activation. Inactive complexes can become soluble C5b-9 (sC5b-9) when they bind to Protein S. Elevated sC5b-9 levels are associated with increased risk of hematopoietic stem cell transplant-associated thrombotic microangiopathy (TA-TMA), a serious condition which can be improved with eculizumab therapy.

What dimensions of school climate promote adaptive functioning in adolescents with ADHD? A prospective longitudinal study.

Background: The study of ADHD has predominantly focused on individual-level risk-factors, and less is known about contextual factors that promote adaptive functioning.

Aims: The present study is the first to evaluate the longitudinal association between five dimensions of school climate (academic expectations, student engagement, disciplinary structure, respect for students, willingness to seek help) and student outcomes, and whether ADHD symptom severity moderates those associations.

Methods And Procedures: Participants included 274 adolescents (45 % female) who completed assessments in 8th (T1) and 10th (T2) grades.

Successful Long-Term Management of NF1 Congenital Tibial Dysplasia with Prophylactic Bypass Graft and Bracing: A Case Report.

Case: An 8-year-old girl with NF1 and congenital tibial dysplasia with significant bilateral tibial bowing underwent McFarland procedures. Now age 22 with 14-year follow-up indicating sustained correction and healing of her bilateral limb deformities.

Conclusion: This case illustrates the McFarland procedure provided adequate corrective treatment of this deformity and remains a success at long-term follow-up.

Loss of Cpt1a results in elevated glucose-fueled mitochondrial oxidative phosphorylation and defective hematopoietic stem cells.

Hematopoietic stem cells (HSCs) rely on self-renewal to sustain stem cell potential and undergo differentiation to generate mature blood cells. Mitochondrial fatty acid β-oxidation (FAO) is essential for HSC maintenance. However, the role of Carnitine palmitoyl transferase 1a (CPT1A), a key enzyme in FAO, remains unclear in HSCs.

Cognitive Disengagement Syndrome and Autism Traits are Empirically Distinct from each Other and from Other Psychopathology Dimensions.

Recently, an association between cognitive disengagement syndrome (CDS), formerly sluggish cognitive tempo, and autism has been documented, but it is not known if the association is due to overlapping autism and CDS traits or if CDS is empirically distinct from autism. Mothers rated 2,209 children 4-17 years (1,177 with autism, 725 with ADHD-Combined type, and 307 with ADHD-Inattentive type) on the Pediatric Behavior Scale. Factor analysis of the Pediatric Behavior Scale items indicated that CDS and autism traits are empirically distinct from each other without cross-loading and are distinct from eight other factors (attention deficit, impulsivity, hyperactivity, oppositional behavior, irritability/anger, conduct problems, depression, and anxiety).

Utilization of Palliative Care in Cardiogenic Shock Patients: A Retrospective Analysis of the National Inpatient Sample Database, 2020.

Cardiogenic shock (CS) is one of the leading causes of death in patients with myocardial infarction, myocarditis, and congestive heart failure. The utilization patterns of specialist palliative care (PC) consultation in these patients are currently unknown. To determine the utilization of PC in patients with CS and the overall comorbidities of that population.

The modern use of hydroxyurea for children with sickle cell anemia.

Over the past 40 years, the introduction and refinement of hydroxyurea therapy has led to remarkable progress for the care of individuals with sickle cell anemia (SCA). From initial small proof-of-principle studies to multi-center Phase 3 controlled clinical trials and then numerous open-label studies, the consistent benefits of once-daily oral hydroxyurea have been demonstrated across the lifespan. Elevated fetal hemoglobin (HbF) serves as the most important treatment response, as HbF delays sickle hemoglobin polymerization and reduces erythrocyte sickling.

Mechanisms of exercise-related neurocardiogenic syncope and the relationship between resting and dynamic cardiac testing.

Objective: Syncope is common among pediatric patients and is rarely pathologic. The mechanisms for symptoms during exercise are less well understood than the resting mechanisms. Additionally, inert gas rebreathing analysis, a non-invasive examination of haemodynamics including cardiac output, has not previously been studied in youth with neurocardiogenic syncope.

Anomalous Left Coronary Artery from the Pulmonary Artery in Three Patients with MYRF-Associated Cardiac-Urogenital Syndrome.

Cardiac-Urogenital Syndrome (CUGS) is a recently identified genetic disease characterized by urogenital, diaphragmatic, ophthalmic, and cardiac abnormalities caused by heterozygous pathogenic variants in the Myelin Regulatory Factor (MYRF) gene. The complete spectrum of disease characteristics and prevalence is not yet defined. This report documents the first known cases of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in MYRF-associated Cardiac-Urogenital Syndrome (MYRF-CUGS).

Multi-site, multi-vendor development and validation of a deep learning model for liver stiffness prediction using abdominal biparametric MRI.

Background: Chronic liver disease (CLD) is a substantial cause of morbidity and mortality worldwide. Liver stiffness, as measured by MR elastography (MRE), is well-accepted as a surrogate marker of liver fibrosis.

Purpose: To develop and validate deep learning (DL) models for predicting MRE-derived liver stiffness using routine clinical non-contrast abdominal T1-weighted (T1w) and T2-weighted (T2w) data from multiple institutions/system manufacturers in pediatric and adult patients.

Long-term kidney outcomes in survivors of Wilms tumor: a single-center retrospective cohort study.

Background: Several studies have investigated long-term kidney outcomes in survivors of Wilms tumor (WT). However, many have small sample sizes, and there is a wide variation in reported outcomes. The aim of this study is to investigate the long-term kidney outcomes in survivors of WT (S-WT), including those patients considered to be at high risk for poor kidney outcomes, and using updated estimated glomerular filtration rate (eGFR) equations.

A National Assessment of Pediatric Surgical Critical Care Fellowship Structure: Defining the Training Experience.

Background: Pediatric Surgical Critical Care (PSCC) is a unique specialty incorporating fundamental principles of surgical, neonatal, and pediatric critical care. This study aims to characterize the current landscape of PSCC training to identify opportunities for educational standardization and improvement.

Methods: An anonymous electronic survey-based assessment was distributed to the program directors (PDs) of all current ACGME-accredited PSCC fellowships (n = 14).

Neuromodulation Strategies in Lennox-Gastaut Syndrome: Practical Clinical Guidance from the Pediatric Epilepsy Research Consortium.

Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy characterized by multiple drug-resistant seizure types, cognitive impairment, and distinctive electroencephalographic patterns. Neuromodulation techniques, including vagus nerve stimulation (VNS), deep brain stimulation (DBS), and responsive neurostimulation (RNS), have emerged as important treatment options for patients with LGS who do not respond adequately to antiseizure medications. This review, developed with input from the Pediatric Epilepsy Research Consortium (PERC) LGS Special Interest Group, provides practical guidance for clinicians on the use of these neuromodulation approaches in patients with LGS.

Fate of Semilunar Valves From Discarded Hearts at the Time of Pediatric Heart Retransplantation.

Background: Partial heart transplantation (PHT) is a novel procedure for children in need of a growing valve replacement option. One challenge is identifying suitable donor valves. Semilunar heart valves from patients receiving a retransplant may be a source, however their functionality and growth potential especially at the time of retransplant are unknown.

Association Between Early Immunosuppression Center Variability and One-Year Outcomes After Pediatric Liver Transplant.

Background: Despite the existence of institutional protocols, liver transplant centers often have variability in early immunosuppression practices. We aimed to measure within-center variability in early immunosuppression after pediatric liver transplant (LT) and examine its association with one-year outcomes.

Methods: We analyzed pediatric LTs from 2013 to 2018 in the United Network for Organ Sharing registry, with data aggregated by center.

Prevention of Infections Among Pediatric Solid Organ Transplant Recipients With Asplenia or Hyposplenism.

Pediatric solid organ transplant (SOT) recipients with splenic dysfunction are at increased risk for infections, and tailored guidance on the management of asplenia/hyposplenism among SOT recipients is often lacking. The purpose of this article is to provide practice recommendations via a frequently asked questions (FAQs) format that focuses on three main domains: the identification of asplenia/hyposplenism among SOT recipients/candidates, prophylactic strategies for mitigating the risk of invasive disease associated with splenic dysfunction in the context of transplantation, and the provision of appropriate patient counseling on the risks associated with asplenia/hyposplenism. Answers to the FAQs are based on international expert opinion informed by practices for managing splenic dysfunction and associated data in other populations with asplenia.

Patient experience with eosinophilic esophagitis symptoms and impacts on daily life based on in-trial qualitative interviews.

Purpose: Eosinophilic esophagitis (EoE), a chronic immune-mediated progressive disease, causes dysphagia, food impaction, abdominal pain, vomiting, and heartburn. EoE requires long-term monitoring and can affect quality of life owing to its symptoms and associated emotional and social burden. This study aimed to understand patients' experiences with EoE.

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.

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.

Longitudinal response to standard of care in pediatric metabolic dysfunction-associated steatotic liver disease: Rates of improvement and worsening, and factors associated with outcomes.

Background Aims: Longitudinal outcomes in children with metabolic dysfunction-associated steatotic liver disease (MASLD) remain unclear due to the absence of a standardized monitoring approach. This study aimed to 1) define improvement and worsening in children with MASLD, 2) estimate rates of improvement or deterioration with standard of care (SOC) over one and two years, and 3) identify baseline and longitudinal factors associated with improvement or worsening.

Approach And Results: Using data from two large randomized controlled trials, we derived definitions for composite improvement and worsening of MASLD based on associations between changes in ALT, GGT, and liver histology after one and two years.

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.

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.