Multicenter validation of secondary hemophagocytic lymphohistiocytosis diagnostic criteria.

Background: Five fulfilled hemophagocytic lymphohistiocytosis (HLH)-2004 criteria, and the HScore are widely used and recommended by international expert consensus to diagnose secondary HLH. Both diagnostic scores have never been validated in heterogeneous patient cohorts of secondary HLH patients. We aimed to systematically optimize and validate diagnostic criteria of secondary HLH using a multicenter approach.

Comorbidities and Healthcare Utilization Among Young Adults With Congenital Heart Defects by Down Syndrome Status-Congenital Heart Survey to Recognize Outcomes, Needs, and wellbeinG, 2016-2019.

Background: Almost half of individuals born with Down syndrome (DS) have congenital heart defects (CHDs). Yet, little is known about the health and healthcare needs of adults with CHDs and DS. Therefore, we examined comorbidities and healthcare utilization of this population.

The Varying Histology of Hepatic Sarcoidosis and the Relation of Bile Duct Damage and Loss to the Presence of Portal Hypertension and Cirrhosis.

Background And Aims: Sarcoidosis is a multisystem disorder characterized by nonnecrotizing granulomas. Studies suggest 20%-70% of patients with sarcoidosis have abnormal liver chemistries or abdominal imaging. Hepatic sarcoidosis may be complicated by portal hypertension (portal HTN) with or without cirrhosis.

Clin-STAR Corner: Rehabilitation for Older Adults With Heart Failure.

Heart failure is a major contributor to morbidity, mortality, and healthcare costs, especially among older adults. Despite a large body of evidence supporting the benefits of cardiac rehabilitation, less than 30% of eligible Medicare beneficiaries participate in cardiac rehabilitation. Thus, it is essential to examine alternatives, such as physical rehabilitation, a rehabilitation approach that focuses on physical activity and function.

Exploring the Clinical Diversity of Castleman Disease and TAFRO Syndrome: A Japanese Multicenter Study on Lymph Node Distribution Patterns.

Individuals diagnosed with Castleman disease (CD) and TAFRO syndrome (characterized by thrombocytopenia, anasarca, fever, bone marrow fibrosis, and organomegaly) displays a wide range of clinical symptoms, including varying patterns of lymph node enlargement, systemic inflammation, and impaired organ function. Some patients may present with both CD and TAFRO syndrome concurrently. A retrospective study conducted across multiple centers in Japan examined 321 cases to determine if the quantity and position of swollen lymph nodes could forecast the clinical progression and intensity of these conditions.

Sex-specific effects on the heart from combined exposure to simulated galactic cosmic radiation and hindlimb unloading.

Future long duration space missions will expose astronauts to higher doses of galactic cosmic radiation (GCR) than those experienced on the international space station. Recent studies have demonstrated astronauts may be at risk for cardiovascular complications due to increased radiation exposure and fluid shift from microgravity. However, there is a lack of direct evidence on how the cardiovascular system is affected by GCR and microgravity since no astronauts have been exposed to exploratory mission relevant GCR doses.

Global metabolomic alterations associated with endocrine-disrupting chemicals among pregnant individuals and newborns.

Background: Gestational exposure to non-persistent endocrine-disrupting chemicals (EDCs) may be associated with adverse pregnancy outcomes. While many EDCs affect the endocrine system, their effects on endocrine-related metabolic pathways remain unclear. This study aims to explore the global metabolome changes associated with EDC biomarkers at delivery.

Placental PFAS concentrations are associated with perturbations of placental DNA methylation.

The placenta is crucial for fetal development, is affected by PFAS toxicity, and evidence is accumulating that gestational PFAS perturb the epigenetic activity of the placenta. Gestational PFAS exposure can adversely affect offspring, yet individual and cumulative impacts of PFAS on the placental epigenome remain underexplored. Here, we conducted an epigenome-wide association study (EWAS) to examine the relationships between placental PFAS levels and DNA methylation in a cohort of mother-infant dyads in Arkansas (N=151).

Setting standards for brain collection procedures in metabolomic studies.

Current metabolomics technologies can measure hundreds of chemical entities in tissue extracts with good reliability. However, long-recognized requirements to halt enzyme activities during the initial moments of sample preparation are usually overlooked, allowing marked postmortem shifts in levels of labile metabolites representing diverse pathways. In brain many such changes occur in a matter of seconds.

Perinatal Outcomes and Management of Umbilical Vein Varix: A Comprehensive Review of 392 Cases.

Case reports and case series have linked umbilical vein varices (UVVs) with adverse pregnancy outcomes. Newer case reports and series suggest better perinatal outcomes in cases with an isolated UVV. The purpose of this literature review is to determine if there is commonality in management, outcomes, and association in pregnancy with UVV and fetal aneuploidy, growth restriction, demise, thrombosis, and turbulent flow.

Proteins Implicated in the Reduced Virulence of and Mutants in Osteomyelitis.

Using a murine osteomyelitis model, we recently demonstrated that and mutants generated in the USA300 strain LAC are attenuated to a greater extent than an isogenic mutant and that this can be attributed to a significant extent to the increased production of extracellular proteases in both mutants. Based on this, we used a mass-based proteomics approach to compare the proteomes of LAC, its isogenic , , and mutants, and isogenic derivatives of all four of these strains unable to produce the extracellular proteases aureolysin, SspA, SspB, ScpA, or SplA-F. This allowed us to identify proteins that were present in reduced amounts in , and / mutants owing to the increased production of extracellular proteases.

The Clinical Utility of a Next-Generation Sequencing-Based Approach to Detecting Circulating HPV DNA in Patients with Advanced Anal Cancer.

Background: To extend the practicality of liquid biopsy beyond the historical HPV circulating tumor DNA (ctDNA) assays, we evaluated the clinical relevance of a novel next-generation sequencing HPV ctDNA assay in patients with locally advanced and metastatic squamous cell cancer of the anal canal (mSCCA).

Methods: ctDNA isolated from the plasma of patients with mSCCA was sequenced using a 1.4 Mb hybrid-capture target-enrichment panel covering the whole genome sequences of all 193 HPV types.

Activity Performance Priorities for Adults Experiencing Homelessness: Insights from Management and Staff at a Transitional Housing Facility.

For people experiencing homelessness (PEH), the provision of affordable housing has been recognized as the most crucial intervention for improving housing stability and facilitating substance abuse treatment. However, evidence indicates that providing housing does not significantly improve substance abuse, mental health, or physical health outcomes. Optimal participation in essential daily activities has been shown to improve health outcomes and support independent living, but there is limited research that identifies activity performance priorities among PEH living in transitional housing.

A Qualitative Exploration of Relations Between Salutogenesis and Persistent Music Performance Among Refugees Resettled in the United States.

In its broadest meaning, salutogenesis denotes an orientation toward the origins and assets for positive health, as opposed to the origins and risk factors associated with disease (i.e., pathogenesis).

Distinct Urinary Metabolite Signatures Mirror In Vivo Oxidative Stress-Related Radiation Responses in Mice.

Exposure to ionizing radiation disrupts metabolic pathways and causes oxidative stress, which can lead to organ damage. In this study, urinary metabolites from mice exposed to high-dose and low-dose whole-body irradiation (WBI HDR, WBI LDR) or partial-body irradiation (PBI BM2.5) were analyzed using targeted and untargeted metabolomics approaches.

Single-cell analysis of neoplastic plasma cells identifies myeloma pathobiology mediators and potential targets.

Multiple myeloma is a clonal plasma cell (PC) dyscrasia that arises from precursors and has been studied utilizing approaches focused on CD138 cells. By combining single-cell RNA sequencing (scRNA-seq) with scB-cell receptor sequencing (scBCR-seq), we differentiate monoclonal/neoplastic from polyclonal/normal PCs and find more dysregulated genes, especially in precursor patients, than we would have by analyzing bulk PCs. To determine whether this approach can identify oncogenes that contribute to disease pathobiology, mitotic arrest deficient-2 like-1 (MAD2L1) and S-adenosylmethionine synthase isoform type-2 (MAT2A) are validated as targets with drug-like molecules that suppress myeloma growth in preclinical models.

Cognitive and behavioral impact of antiseizure medications, neuromodulation, ketogenic diet, and surgery in lennox-gastaut syndrome: A comprehensive review.

Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy marked by drug-resistant seizures and profound cognitive and behavioral impairments, with nearly 95% of individuals affected by moderate to severe intellectual disability. This review comprehensively explores the cognitive and behavioral impacts of current treatment options for LGS, including antiseizure medications (ASMs), neuromodulation strategies, the ketogenic diet, and surgical interventions. Given the limited availability of LGS-specific data for several ASMs, the evidence base is supplemented with findings from general epilepsy populations and individuals with epilepsy and intellectual disabilities.

Antiseizure medications for Lennox-Gastaut Syndrome: Comprehensive review and proposed consensus treatment algorithm.

Lennox-Gastaut syndrome (LGS) is a severe, childhood-onset developmental and epileptic encephalopathy characterized by multiple drug-resistant seizure types, specific electroencephalogram (EEG) patterns, and significant cognitive and behavioral impairments. To date, eight anti-seizure medications (ASMs) have been specifically approved by the U.S.

Development and validation of a self-report adaptability measure for the military remote operations community.

Building on emerging literature, a new self-report inventory was developed to assess multiple psychological attributes relevant to adaptability in remote warriors. Literature search backed by surveys of military and psychological experts identified 32 attributes for self-report scale development. Items were sorted reliably into targeted dimensions (67.

Multiplex Polymerase Chain Reaction Versus Standard Bacterial Culture in Critically Ill Children With Suspected Pneumonia.

Background: Bacterial lower respiratory tract infection, particularly ventilator-associated pneumonia (VAP), is a significant cause of morbidity and mortality in children who require mechanical ventilation (MV). Microbiologic diagnosis has relied on bacterial culture, but reverse transcriptase polymerase chain reaction (RT-PCR) with bacterial targets is now available for clinical use. We compared the diagnostic performance of tracheal aspirate (TA) multiplex RT-PCR to culture in children requiring MV with suspected lower respiratory tract infection.

The Representational Challenge of Integration and Interoperability in Transformed Health Ecosystems.

: Health and social care systems around the globe are currently undergoing a transformation towards personalized, preventive, predictive, participative precision medicine (5PM), considering the individual health status, conditions, genetic and genomic dispositions, etc., in personal, social, occupational, environmental, and behavioral contexts. This transformation is strongly supported by technologies such as micro- and nanotechnologies, advanced computing, artificial intelligence, edge computing, etc.

Severe Respiratory Distress From Subcutaneous Emphysema After Tracheocutaneous Fistula Repair: A Case Report.

Subcutaneous emphysema results from air or gas being forced into the fascial spaces of subcutaneous tissue. Once the air or gas has entered the fascial spaces, it travels along connective tissue causing a mass effect and swelling. This rare complication usually presents with mild severity during the immediate postoperative period following surgical procedures of the head or neck regions and self-resolves with conservative treatment.

Association of neighborhood Child Opportunity Index with visual outcomes in children with unilateral pediatric cataract.

Purpose: To demonstrate the association between neighborhood quality, using the Child Opportunity Index (COI), and the visual outcomes in children with unilateral pediatric cataract.

Methods: We retrospectively reviewed the medical records of patients ≤18 years of age who presented at Boston Children's Hospital between 2000 and 2022 with unilateral cataracts and underwent cataract extraction. The collected data included patient demographics, residential addresses, the reason for the initial presentation, the age at presentation, the morphology of the cataract, and the final visual acuity.

Practice patterns for acquiring neuroimaging after pediatric in-hospital cardiac arrest.

Aims: To determine which patient and cardiac arrest factors were associated with obtaining neuroimaging after in-hospital cardiac arrest, and among those patients who had neuroimaging, factors associated with which neuroimaging modality was obtained.

Methods: Retrospective cohort study of patients who survived in-hospital cardiac arrest (IHCA) and were enrolled in the ICU-RESUS trial (NCT02837497).

Results: We tabulated ultrasound (US), CT, and MRI frequency within 7 days following IHCA and identified patient and cardiac arrest factors associated with neuroimaging modalities utilized.