Characterization of Factors Associated With Death in Deceased Patients With Mitochondrial Disorders: A Multicenter Cross-Sectional Survey.

Background And Objectives: Mitochondrial disorders are multiorgan disorders resulting in significant morbidity and mortality. We aimed to characterize death-associated factors in an international cohort of deceased individuals with mitochondrial disorders.

Methods: This cross-sectional multicenter observational study used data provided by 26 mitochondrial disease centers from 8 countries from January 2022 to March 2023.

Revised Process for ACNS Guidelines Development.

The development of clinical practice guidelines is an evolving field. In response to the need for consistent, evidence-based medical practice, the American Clinical Neurophysiology Society identified the need to update the Society's guideline development process. The American Clinical Neurophysiology Society Guidelines Committee created an action plan with the goal of improving transparency and rigor for future guidelines and bringing existing guidelines to current standards.

High immunoproteasome and low constitutive proteasome subunit levels correlate with sensitivity to bortezomib-containing chemotherapy: update from the Children's Oncology Group AALL1231 trial.

Not available.

Mental Health and Wellness Among Plastic Surgeons During Oral Board Certification Examination Preparation.

Background: The field of plastic and reconstructive surgery has recently made a dramatic shift toward attention to mental health, with residency curricula expanding to include training on burnout prevention and the promotion of well-being. After graduation, however, new surgeons face a difficult year of preparation for the oral board certification examination. This group of young surgeons remains largely unstudied and undersupported throughout what may be the most challenging period of their educational journey.

Introduction to the Wellness Mini-series. Burnout, Disengagement, and Other Psychological Quagmires: It's Not All in Your Head.

Surgery is a state of mind. The brain controls the hands. As a result, "technical skills" are more mental than technical.

Prevalence of Asymptomatic Acetabular Labrum Abnormalities in the Active Pediatric Population.

Background: The prevalence of labral tears in asymptomatic active adults has been reported, but the prevalence of labral tears and other incidental hip lesions in the asymptomatic active pediatric population remains unclear. The purpose of this study was to determine the prevalence of hip abnormalities detected on 3T MRI in an active pediatric population with no hip symptoms and to compare with hip abnormalities found in children and adolescents who underwent an MRI for a hip-related condition.

Methods: After IRB approval, pediatric patients self-reporting as athletes and presenting with isolated, acute-onset knee pain requiring knee MRI were prospectively recruited to undergo 3T MRI of their asymptomatic contralateral hip (ASx).

Increased serum GM-CSF at diagnosis of biliary atresia is associated with improved biliary drainage.

Background: The immune heterogeneity of biliary atresia (BA) presents a challenge for development of prognostic biomarkers. This study aimed to identify early immune signatures associated with biliary drainage after Kasai Portoenterostomy (KPE).

Methods: Serum samples, liver slides, and clinical data were obtained from patients enrolled in the NIDDK-supported Childhood Liver Disease Research Network.

Anaphylaxis definition, overview, and clinical support tool: 2024 consensus report.

Background: The 2006 National Institute of Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network (NIAID/FAAN) anaphylaxis criteria are widely used in clinical care and research. In 2020, the World Allergy Organization (WAO) published modified criteria that have not been uniformly adopted. Different criteria contribute to inconsistent care and research outcomes.

Transforming Transplantation Access: A Federal Directive for Comprehensive Pre-Waitlisting Data Collection.

There is substantial variation in access to transplantation across the United States that is not entirely explained by the availability of donor organs. Barriers to transplantation and variation in care among patients with end-stage organ disease exist prior to patients' placement on a transplant waiting list as well as following waitlist placement. However, there are currently no national data available to examine rates and variations in key care processes related to pre-listing, including transplant referral, evaluation, or candidate selection.

Regional Anesthesia and Pain Outcomes After Anterior Cruciate Ligament Reconstruction Surgery in Pediatric Patients: Society of Pediatric Anesthesia Improvement Network.

Background: Optimal perioperative pain management is unknown for adolescent patients undergoing anterior cruciate ligament reconstruction (ACLR). The study aimed to determine the association of nerve blocks with short- and long-term pain outcomes and factors influencing self-reported neurological symptoms.

Methods: We performed a multisite, prospective observational study of adolescent patients undergoing ACLR.

Anisotropic hydrogel microelectrodes for intraspinal neural recordings in vivo.

Creating durable, motion-compliant neural interfaces is crucial for accessing dynamic tissues under in vivo conditions and linking neural activity with behaviors. Utilizing the self-alignment of nano-fillers in a polymeric matrix under repetitive tension, here, we introduce conductive carbon nanotubes with high aspect ratios into semi-crystalline polyvinyl alcohol hydrogels, and create electrically anisotropic percolation pathways through cyclic stretching. The resulting anisotropic hydrogel fibers (diameter of 187 ± 13 µm) exhibit fatigue resistance (up to 20,000 cycles at 20% strain) with a stretchability of 64.

Epidemiologic Investigation of Protozoa and Soil-Transmitted Helminths in Starr County, Texas.

The burden of pathogenic enteric protozoa and soil-transmitted helminths among impoverished populations living on the Texas-Mexico border is unclear. We conducted a cross-sectional study on an ongoing longitudinal cohort of 616 adults residing in Starr County, Texas. A total of 359 adults were screened for four protozoa and five soil-transmitted helminths by using real-time polymerase chain reaction.

Cardiopulmonary Exercise Testing.

AbstractBecause symptoms of cardiopulmonary disease often occur with exertion, cardiopulmonary exercise testing (CPET) has a unique role in the assessment of patient symptoms, disease severity, prognosis, and response to therapy. In addition to the evaluation of cardiovascular and pulmonary physiology, CPET provides an assessment of the interaction of the cardiovascular and pulmonary systems with the musculoskeletal, nervous, and hematological systems. In this article, we review key CPET variables, protocols, and clinical indications.

An Exploratory Analysis of Early Care Differences and Risk of Post-Maxillary Advancement VPI in Individuals With Cleft Palate.

Objective: To investigate whether differences in early cleft care increase risk of velopharyngeal insufficiency (VPI) after maxillary advancement.

Design: Retrospective cohort study.

Setting: Large pediatric tertiary care hospital.

MetaboLINK is a novel algorithm for unveiling cell-specific metabolic pathways in longitudinal datasets.

Introduction: In the rapidly advancing field of 'omics research, there is an increasing demand for sophisticated bioinformatic tools to enable efficient and consistent data analysis. As biological datasets, particularly metabolomics, become larger and more complex, innovative strategies are essential for deciphering the intricate molecular and cellular networks.

Methods: We introduce a pioneering analytical approach that combines Principal Component Analysis (PCA) with Graphical Lasso (GLASSO).

Cell surface protein-protein interaction profiling for biological network analysis and novel target discovery.

The secretome is composed of cell surface membrane proteins and extracellular secreted proteins that are synthesized via secretory machinery, accounting for approximately one-third of human protein-encoding genes and playing central roles in cellular communication with the external environment. Secretome protein-protein interactions (SPPIs) mediate cell proliferation, apoptosis, and differentiation, as well as stimulus- or cell-specific responses that regulate a diverse range of biological processes. Aberrant SPPIs are associated with diseases including cancer, immune disorders, and illness caused by infectious pathogens.

BCG-Induced DNA Methylation Changes Improve Coronavirus Disease 2019 Vaccine Immunity Without Decreasing the Risk for Severe Acute Respiratory Syndrome Coronavirus 2 Infection.

Background: The BCG vaccine induces trained immunity, an epigenetic-mediated increase in innate immune responsiveness. Therefore, this clinical trial evaluated if BCG-induced trained immunity could decrease coronavirus disease 2019 (COVID-19)-related frequency or severity.

Methods: A double-blind, placebo-controlled clinical trial of healthcare workers randomized participants to vaccination with BCG TICE or placebo (saline).

"It's Like You're Feeding Your Child Twice": Barriers and Facilitators to Human Milk Feeding Children With Cystic Fibrosis.

Background: Cystic Fibrosis Foundation guidelines recommend human milk (HM) as the ideal source of nutrition for children with CF (cwCF). Despite known pulmonary and nutritional benefits, fewer cwCF ever receive HM compared to the general population. Early nutrition choices are preference-sensitive, yet little is known about the factors that impede or sustain HM feeding among parents of cwCF.

Caught in the Crossfire: A Single Institution's 10-Year Experience with Pediatric Firearm Injury.

Introduction: Pediatric firearm-related injuries are now the leading cause of death among children in the United States. We sought to characterize the experience of a large free-standing children's hospital treating children with firearm injuries.

Methods: We reviewed all 2012-2022 gunshot wound encounters using the institutional trauma database of an urban Level 1 pediatric trauma center in Texas.

New approaches in childhood IgE-mediated food allergy treatment.

Purpose Of Review: This review aims to provide an overview of the current and future treatment options for children with food allergies (FAs), highlighting the latest research findings and the potential impact of these new approaches on improving patients' and caregivers' quality of life.

Recent Findings: In the last decade, many promising approaches have emerged as an alternative to the standard avoidance of the culprit food with the risk of severe accidental reactions. Desensitization through oral immunotherapy has been introduced in clinical settings as a therapeutic approach, and more recently also omalizumab.

Sudden cardiac death associated with fatty liver disease.

Background: Fatty liver disease or steatotic liver disease (SLD) affects 25% of the global population and has been associated with heart disease. However, there is a lack of postmortem studies in the context of sudden cardiac death (SCD).

Objectives: To investigate the relationship between SLD and SCD.