Outcomes following resective and disconnective strategies in the treatment of epileptic spasms: a systematic review of the literature and individual patient data meta-analysis.

Epileptic spasms (ES) are a unique seizure type typically presenting in the form of infantile epileptic spasms syndrome (IESS) with characteristic hypsarrhythmia on scalp EEG and a preponderance with developmental delay or regression. While pharmacotherapy is the mainstay of treatment, surgical options, including disconnective or resective procedures, are increasingly recognized as viable therapeutic options for recurrent or persistent ES. However, limited data on safety, effectiveness, and prognostic factors hinder informed decision-making regarding surgery indications, timing, and intervention type.

Capnocytophaga canimorsus in Iliac Artery Mycotic Aneurysm: The Role of Molecular Diagnostics.

BACKGROUND The bacterial organism Capnocytophaga canimorsus is an oral commensal of cats and dogs and can cause life-threatening infections like mycotic aneurysm, meningitis, and sepsis. Mycotic aneurysms occur when microbial infections cause arterial wall degeneration. Difficulty in diagnosing Capnocytophaga canimorsus infection can occur due to the bacteria's fastidious nature and laboratory testing limitations, contributing to the infection's high morbidity and mortality.

Multicentre study conducted across centres in the USA, Europe and Australia to assess the safety and effectiveness of a bilateral hypoglossal nerve stimulation system for the treatment of obstructive sleep apnoea in adults: a protocol for a pivotal, multicentre, open-label, single-arm study.

Introduction: Obstructive sleep apnoea (OSA) is characterised by blood oxygen desaturations and sleep disruptions manifesting undesirable consequences. Existing treatments including oral appliances, positive airway pressure (PAP) therapy and surgically altering the anatomy of the pharynx have drawbacks including poor long-term adherence or often involving irreversible, invasive procedures. Bilateral hypoglossal nerve stimulation (HNS) is a new treatment for managing OSA, and this study is intended to determine whether an HNS system is a safe and effective treatment option for adults with OSA.

Society of American Gastrointestinal and Endoscopic Surgeons guidelines development: Clinical Practice Guideline Update Standard Operating Procedure.

Introduction: The SAGES Guidelines Committee creates evidence-based clinical practice guidelines (CPGs). Updates which incorporate new evidence into the guidelines are necessary to maintain relevance for clinical use. A description of our standard operating procedure for this process is described here, which contributes to SAGES' commitment to producing high-quality clinical recommendations.

A discrete choice experiment with health professions trainees to improve the urban-rural health care access disparity in Appalachia: Study protocol.

Globally, those who live in rural areas experience significant barriers to accessing health care due to a maldistribution of health care providers. Those who live in rural areas in the Appalachian region of the United States face one of the worst shortages of health care providers despite experiencing more complex health needs compared to Americans in more affluent, urban areas. Prior research has failed to identify effective solutions to narrow the provider maldistribution, despite it being a policy focus for decades.

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.

Age-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based NORSE/FIRES Family Registry.

Febrile infection-related epilepsy syndrome (FIRES) is a rare clinical presentation of refractory status epilepticus following a febrile infection. This study analyzes data from the NORSE/FIRES Family Registry, an international web-based registry available in six languages with data entered by patients, families, and clinicians to explore clinical presentations, survivorship, and long-term outcomes in adult and pediatric FIRES patients. We characterize and examine differences in demographics, prodromal symptoms, seizure frequency, anti-seizure medications (ASMs), quality of life, cognition, mood, and anxiety in adults vs pediatric populations with FIRES.

Radial to Median Sensory Nerve Transfers to Restore Sensate Key Pinch: A Cadaveric Assessment of Size Match and Implications for Transfer.

Background: Loss of key-pinch sensation after median nerve injury poses significant functional detriment. Nerve transfers are utilized to improve function after nerve injury and size matching of donor and recipient nerves is important to optimize success. This anthropometric study investigates the anatomy of the superficial branch of the radial nerve (SBRN) to the thumb and index finger and explores radial to median sensory nerve transfers, a necessary but not heavily discussed facet of nerve transfers for the hand.

Protein tyrosine phosphatase PTPH1 potentiates receptor tyrosine kinase HER2 oncogenesis via a PDZ-coupled and phosphorylation-driven scaffold.

Cancer cell overexpresses numerus proteins, however, how these up-regulated proteins, especially those enzymatically opposite kinases and phosphatases, act together to promote oncogenesis is unknown. Here, we reported that protein tyrosine phosphatase H1 (PTPH1) is a scaffold protein for receptor tyrosine kinase (HER2) to potentiate breast tumorigenesis. PTPH1 utilizes its PDZ domain to bind HER2, p38γ, PBK, and YAP1 and to increase HER2 nuclear translocation, stemness, and oncogenesis.

Introducing the Next Era in Assessment.

In this introduction, the guest editors of the "Next Era in Assessment" special collection frame the invited papers by envisioning a next era in assessment of medical education, based on ideas developed during a summit that convened professional and educational leaders and scholars. The authors posit that the next era of assessment will focus unambiguously on serving patients and the health of society, reflect its sociocultural context, and support learners' longitudinal growth and development. As such, assessment will be characterized as transformational, development-oriented and socially accountable.

The potential of antibody-drug conjugates for effective therapy in diffuse large B-cell lymphoma.

Introduction: Antibody-drug conjugates (ADCs) are a rapidly evolving class of anti-cancer drugs with a significant impact on management of hematological malignancies including diffuse large B-cell lymphoma (DLBCL). ADCs combine a cytotoxic drug (a.k.

Long-term neurobehavioral and neuroimaging outcomes in athletes with prior concussion(s) and head impact exposure.

The long-term health of former athletes with a history of multiple concussions and/or repetitive head impact (RHI) exposure has been of growing interest among the public. The true proportion of dementia cases attributable to neurotrauma and the neurobehavioral profile/sequelae of multiple concussion and RHI exposure among athletes has been difficult to determine. Across three exposure paradigms (i.

Advances in Glioblastoma Diagnosis: Integrating Genetics, Noninvasive Sampling, and Advanced Imaging.

Glioblastoma is the most common primary brain tumor in adult patients, and despite standard-of-care treatment, median survival has remained less than two years. Advances in our understanding of molecular mutations have led to changes in the diagnostic criteria of glioblastoma, with the WHO classification integrating important mutations into the grading system in 2021. We sought to review the basics of the important genetic mutations associated with glioblastoma, including known mechanisms and roles in disease pathogenesis/treatment.

Chiropractic international research collaborative (CIRCuit): the development of a new practice-based research network, including the demographics, practice, and clinical management characteristics of clinician participants.

Objectives: To describe the structure and development of a new international, chiropractic, practice-based research network (PBRN), the Chiropractic International Research Collaborative (CIRCuit), as well as the demographic, practice, and clinical management characteristics of its clinician participants. An electronic survey was used to collect information on their demographics, practice, and clinical management characteristics from clinicians from 17 October through 28 November 2022. Descriptive statistics were used to report the results.

Lentivirus-mediated gene therapy for Fabry disease: 5-year End-of-Study results from the Canadian FACTs trial.

Background: Fabry disease is an X-linked lysosomal storage disorder due to a deficiency of α-galactosidase A (α-gal A) activity. Our goal was to correct the enzyme deficiency in Fabry patients by transferring the cDNA for α-gal A into their CD34+ hematopoietic stem/progenitor cells (HSPCs). Overexpression of α-gal A leads to secretion of the hydrolase; which can be taken up and used by uncorrected bystander cells.

Results of a Global Survey on the State of Interventional Radiology 2024.

Purpose: To understand the state of IR practice and education worldwide and identify factors impacting access to IR services.

Methods And Materials: A Society of Interventional Radiology (SIR) approved anonymous survey was created to gather demographics, regional IR practice and education characteristics, and challenges impacting local IR development. The survey was distributed to members of global IR societies and shared on social media from 6/2022 to 9/2023.

Hydroxychloroquine prevents resistance and potentiates the antitumor effect of SHP2 inhibition in NF1-associated malignant peripheral nerve sheath tumors.

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas and the primary cause of mortality in patients with neurofibromatosis type 1 (NF1). These malignancies develop within preexisting benign lesions called plexiform neurofibromas (PNs). PNs are solely driven by biallelic loss eliciting RAS pathway activation, and they respond favorably to MEK inhibitor therapy.

Multiomics dissection of human RAG deficiency reveals distinctive patterns of immune dysregulation but a common inflammatory signature.

Human recombination-activating gene (RAG) deficiency can manifest with distinct clinical and immunological phenotypes. By applying a multiomics approach to a large group of -mutated patients, we aimed at characterizing the immunopathology associated with each phenotype. Although defective T and B cell development is common to all phenotypes, patients with hypomorphic variants can generate T and B cells with signatures of immune dysregulation and produce autoantibodies to a broad range of self-antigens, including type I interferons.

The Gut in Critical Illness.

Purpose Of Review: The purpose of this narrative review is to describe the mechanisms for gut dysfunction during critical illness, outline hypotheses of gut-derived inflammation, and identify nutrition and non-nutritional therapies that have direct and indirect effects on preserving both epithelial barrier function and gut microbiota during critical illness.

Recent Findings: Clinical and animal model studies have demonstrated that critical illness pathophysiology and interventions breach epithelial barrier function and convert a normally commensal gut microbiome into a pathobiome. As a result, the gut has been postulated to be the "motor" of critical illness and numerous hypotheses have been put forward to explain how it contributes to systemic inflammation and drives multiple organ failure.

Metabolomics-Driven Biomarker Discovery for Breast Cancer Prognosis and Diagnosis.

Breast cancer is a cancer with global prevalence and a surge in the number of cases with each passing year. With the advancement in science and technology, significant progress has been achieved in the prevention and treatment of breast cancer to make ends meet. The scientific intradisciplinary subject of "metabolomics" examines every metabolite found in a cell, tissue, system, or organism from different sources of samples.

Exploring Perceptions of Hospice Care in the American Muslim Community: A Qualitative Descriptive Study.

Objectives: To explore American Muslims' perceptions and experiences regarding hospice care within the United States.

Methods: A qualitative descriptive study of 11 participants, including one patient and ten family caregivers. Data was collected through semi-structured interviews and analyzed using a framework approach to identify key themes related to perceptions, ethical concerns, and experiences with hospice care.

Competing risks regression for clustered data with covariate-dependent censoring.

Competing risks data in clinical trial or observational studies often suffer from cluster effects such as center effects and matched pairs design. The proportional subdistribution hazards (PSH) model is one of the most widely used methods for competing risks data analyses. However, the current literature on the PSH model for clustered competing risks data is limited to covariate-independent censoring and the unstratified model.

Adjusted curves for clustered survival and competing risks data.

Observational studies with right-censored data often have clustered data due to matched pairs or a study center effect. In such data, there may be an imbalance in patient characteristics between treatment groups, where Kaplan-Meier curves or unadjusted cumulative incidence curves can be misleading and may not represent the average patient on a given treatment arm. Adjusted curves are desirable to appropriately display survival or cumulative incidence curves in this case.