Accuracy of pathogen diagnostic codes for acute hematogenous musculoskeletal infections in children.

Administrative databases are powerful tools for pediatric research but lack patient-level microbiology results. This study aimed to determine the accuracy of pathogen discharge diagnosis codes for children hospitalized with acute hematogenous musculoskeletal infections (MSKIs). Medical records for 244 children hospitalized with acute hematogenous MSKIs were manually reviewed to determine which bacterial pathogen, if any, was identified for each MSKI based on microbiology results obtained during the hospitalization.

Engaging Students in a Dialysis Unit: A Pilot Study.

Background: Seeking to provide early paediatric nephrology exposure to medical students in the United States, we implemented the Kids In Dialysis, Nephrology Exposure and Education (KIDNEE) club. This club served as an educational intervention in which preclinical medical students were paired with paediatric dialysis patients, as patient buddies.

Approach: Students were recruited for involvement in the club through the medical school Paediatric Interest Group.

Six-Month Outcomes in the Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children Study.

Importance: Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited.

Objective: To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms (z score ≥2.

Effects of Food Processing on Allergenicity.

Purpose Of Review: There is an increasing awareness among clinicians that industrial and household food processing methods can increase or decrease the allergenicity of foods. Modification to allergen properties through processing can enable dietary liberations. Reduced allergenicity may also allow for lower risk immunotherapy approaches.

Treatment of focal anaplastic Wilms tumor: A report from the Children's Oncology Group AREN0321 and AREN03B2 studies.

Background: In the fifth National Wilms Tumor Study, patients received vincristine and dactinomycin (VA) without radiation for stage I focal anaplastic Wilms tumor (FAWT) and VA plus doxorubicin (DD4A) and radiation for stage II-IV FAWT. Four-year event-free survival (EFS) and overall survival (OS) for stage I FAWT were 67.5% and 88.

Structural variation in nebulin and its implications on phenotype and inheritance: establishing a dominant distal phenotype caused by large deletions.

Introduction: Structural variants (SVs) of the nebulin gene (), including intragenic duplications, deletions, and copy number variation of the triplicate region, are an established cause of recessively inherited nemaline myopathies and related neuromuscular disorders. Large deletions have been shown to cause dominantly inherited distal myopathies. Here we provide an overview of 35 families with muscle disorders caused by such SVs in .

Postprandial Hypoglycemia in a Patient With Clinical Beckwith-Wiedemann Syndrome.

A male neonate exhibited hallmark features of Beckwith-Wiedemann syndrome (BWS) including large for gestational age, macroglossia, multiple ear pits, and umbilical hernia. He had neonatal hypoglycemia, requiring a glucose infusion rate of 9.7 mg/kg/min.

Improving Access to Early Developmental Evaluation in Academic Primary Care Centers.

Introduction: Developmental disorders (DDs) affect approximately 1 in 6 children in the United States. Early identification and treatment improve developmental outcomes and child and family functioning. Disparities exist in the diagnosis of DD that leads to inequitable access to developmental services during important periods of neuroplasticity.

One is not like the other: health quality of life scores vary in neuromuscular EOS by diagnosis.

Purpose: The etiology of early-onset scoliosis (EOS) has been shown to significantly influence baseline parent-reported health-related quality of life (HrQOL). In combining these etiology groups, we obligatorily lump together many disparate diagnoses, particularly true in the neuromuscular (NM) cohort. We sought to evaluate the influence of underlying neuromuscular diagnosis on the HrQOL at 5 years following surgery for EOS.

Evolving Roles: Adolescent perspectives on shared decision making with their parents and healthcare providers.

Introduction: Shared decision making (SDM) involving adolescents presents unique challenges. To adequately support and evaluate SDM that includes adolescents, parents, and healthcare providers, it is imperative to understand adolescent perspectives. This study aims to describe the perspectives of adolescents with chronic health conditions regarding sharing medical decisions with their parents and healthcare providers.

Safety and immunogenicity of ascending doses of influenza A(H7N9) inactivated vaccine with or without MF59®.

Introduction: While it remains impossible to predict the timing of the next influenza pandemic, novel avian influenza A viruses continue to be considered a significant threat.

Methods: A Phase II study was conducted in healthy adults aged 18-64 years to assess the safety and immunogenicity of two intramuscular doses of pre-pandemic 2017 influenza A(H7N9) inactivated vaccine administered 21 days apart. Participants were randomized (n = 105 in each of Arms 1-3) to receive 3.

DNAJB1-PKAc Kinase Is Expressed in Young Patients with Pediatric Liver Cancers and Enhances Carcinogenic Pathways.

Hepatoblastoma (HBL) and fibrolamellar hepatocellular carcinoma (FLC) are the most common liver malignancies in children and young adults. FLC oncogenesis is associated with the generation of the fusion kinase, DNAJB1-PKAc (J-PKAc). J-PKAc has been found in 90% of FLC patients' tumors but not in other liver cancers.

Cpeb1 remodels cell type-specific translational program to promote fear extinction.

Protein translation is crucial for fear extinction, a process vital for adaptive behavior and mental health, yet the underlying cell-specific mechanisms remain elusive. Using a Tet-On 3G genetic approach, we achieved precise temporal control over protein translation in the infralimbic medial prefrontal cortex () during fear extinction. In addition, our results reveal that the disruption of cytoplasmic polyadenylation element binding protein 1 (Cpeb1) leads to notable alterations in cell type-specific translational programs, thereby affecting fear extinction.

Probing the Neurodynamic Mechanisms of Cognitive Flexibility in Depressed Individuals with Autism Spectrum Disorder.

Autism spectrum disorder (ASD) is characterized by deficits in social behavior and executive function (EF), particularly in cognitive flexibility. Whether transcranial magnetic stimulation (TMS) can improve cognitive outcomes in patients with ASD remains an open question. We examined the acute effects of prefrontal TMS on cortical excitability and fluid cognition in individuals with ASD who underwent TMS for refractory major depression.

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).

Sox10 is required for systemic initiation of bone mineralization.

Heterozygous variants in SOX10 cause congenital syndromes affecting pigmentation, digestion, hearing, and neural development, primarily attributable to failed differentiation or loss of non-skeletal neural crest derivatives. We report here an additional, previously undescribed requirement for Sox10 in bone mineralization. Neither crest- nor mesoderm-derived bones initiate mineralization on time in zebrafish sox10 mutants, despite normal osteoblast differentiation and matrix production.

The Role of Environmental Exposures on Survival After Non-Small Cell Lung Cancer Resection.

Background: Socioeconomic status and pollution exposure have been described as risk factors for poor survival in patients with non-small cell lung cancer (NSCLC). However, the relationship between these factors is complex and inadequately studied. This study aimed to evaluate the relationship between environmental and social factors and their impact on survival after NSCLC resection.

Functional genotype classification groups distinguish disease severity in recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic disorder due to pathogenic variants in the COL7A1 gene. In this study we determined the association between different categories of COL7A1 variants and clinical disease severity in 236 RDEB patients in North America. Published reports or in-silico predictions were used to assess the impact of pathogenic variants in COL7A1 on type VII collagen (C7) protein function.

Utility of Monitoring Fasting Phase During Pediatric Colonic Manometry Studies.

Introduction: Colonic manometry (CM) is a diagnostic procedure used to evaluate pediatric patients with refractory constipation, fecal incontinence, Hirschsprung disease, and pediatric intestinal pseudo-obstruction. Pan-colonic high-amplitude propagated contractions (HAPCs), measured by CM, reflect an intact neuromuscular function of the colon. Current guidelines recommend starting CM with fasting recording for 1-2 h, but no prior evaluation has determined the diagnostic yield of the fasting phase.

The Influence of Project ECHO and Integrated Behavioral Health in Primary Care on Emergency Department Visits Among Youth Diagnosed with Depression.

Rates of depression among youth and emergency department (ED) visits for un- or under-treated symptoms are on the rise. Early identification and treatment of depression is imperative at the patient, program, system, and population levels. This paper examines the individual and cumulative impact of Project ECHO and the inclusion of IBH services in pediatric primary care practices on mental health-related ED rates among youth diagnosed with depression for those practices.

Circle of Willis Variants and Their Association with Outcome after Successful Revascularization of Anterior Large Vessel Occlusion.

Background And Purpose: Leptomeningeal collaterals have been associated with better outcomes in large-vessel stroke, but little is known about how the Circle of Willis (CoW) collaterals affect stroke outcomes. We aimed to determine the relationship between three anatomically distinct CoW subtypes and 90-day outcomes in acute ischemic stroke patients after successful revascularization via endovascular thrombectomy (EVT).

Materials And Methods: We performed a retrospective analysis of patients treated with successful EVT for large-vessel occlusion at a comprehensive stroke center between May 2016 and November 2023.