Lysosomal dysfunction and inflammatory sterol metabolism in pulmonary arterial hypertension.

Vascular inflammation regulates endothelial pathophenotypes, particularly in pulmonary arterial hypertension (PAH). Dysregulated lysosomal activity and cholesterol metabolism activate pathogenic inflammation, but their relevance to PAH is unclear. Nuclear receptor coactivator 7 () deficiency in endothelium produced an oxysterol and bile acid signature through lysosomal dysregulation, promoting endothelial pathophenotypes.

Third Proceedings of The North American Airway Collaborative (NoAAC): Consensus Statement on Trial Design for Airway Stenosis.

Importance: Airway stenosis is a rare but debilitating disorder that significantly degrades the quality of life in affected patients. Treatments are primarily surgical, and disease management lacks established medical therapies. The North American Airway Collaborative held its third symposium at The Johns Hopkins Hospital in Baltimore, Maryland, on April 15, 2024, focused on strategies to advance the care of these patients.

Prescriptions (Rx) for Prevention: Clinical Tools for Integrating Environmental Health into Pediatric Clinical Care.

The integration of environmental health (EH) into routine clinical care for children is in its early stages. The vision of pediatric EH is that all clinicians caring for children are aware of and able to help connect families to needed resources to reduce harmful environmental exposures and increase health-enhancing ones. Environmental exposures include air pollution, substandard housing, lead, mercury, pesticides, consumer products chemicals, drinking water contaminants, industrial facility emissions and, increasingly, climate change-related extreme weather and heat events.

Arthur (Andy) M. Horne (1942-2024).

Memorializes Arthur (Andy) M. Horne (1942-2024), esteemed counseling psychologist, lauded for his contributions to group counseling, bullying prevention, violence reduction, and prevention. Andy served the University of Georgia in the Department of Counseling and Human Development Services from 1989 to 2012 as training director, department chair, distinguished research professor, and dean (2008-2012).

Bullying Behaviors in Clinical Settings: Potentially Harmful Distractions.

Nursing students exposed to bullying behaviors are at risk for making medication errors. For a quasi-experimental study, 15 prelicensure nursing students at a Midwestern university were exposed to simulated bullying behaviors or common distractions while administering medications in a laboratory setting. Data were analyzed using Kruskal-Wallis tests.

Genomic sequencing in diverse and underserved pediatric populations: parent perspectives on understanding, uncertainty, psychosocial impact, and personal utility of results.

Purpose: Limited evidence evaluates parents' perceptions of their child's clinical genomic sequencing (GS) results, particularly among individuals from medically underserved groups. Five Clinical Sequencing Evidence-Generating Research (CSER) consortium studies performed GS in children with suspected genetic conditions with high proportions of individuals from underserved groups to address this evidence gap.

Methods: Parents completed surveys of perceived understanding, personal utility, and test-related distress after GS result disclosure.

Utility of serum biomarkers in real-world practice for predicting response to omalizumab therapy in patients with chronic spontaneous urticaria.

Background: Omalizumab (OMA), a recombinant humanized IgG monoclonal anti-IgE antibody, is approved for treatment for chronic spontaneous urticaria (CSU) refractory to second-generation H-antihistamine (SGAH) therapy. However, currently, there are no validated serum biomarkers to reliably predict response to OMA treatment.

Objective: We explored the real-world clinical utility of using serum biomarkers for predicting response to OMA for CSU patients with disease refractory to high-dose SGAH therapy.

The 2022 ADEA Climate Study in U.S. and Canadian Dental Schools and Allied Dental Programs: Methodological Considerations.

Objectives: From January 2020 to the end of August 2020, preliminary research gathered data about the need for and the feasibility of an ADEA-led joint Climate Study of dental schools and allied dental programs in the United States and Canada. Informed by these findings, the first ever ADEA-led joint Climate Study took place in 2022. The objectives of this manuscript were to describe the timeline of this climate study and provide information about its methodology, specifically about (a) who participated in this research, (b) what was assessed, (c) how the study was conducted, and (d) how the results were communicated.

Lymph Node Staging and Treatment in Pediatric Patients With Soft Tissue Sarcomas: A Consensus Opinion from the Children's Oncology Group, European paediatric Soft tissue sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe.

Accurate staging of nodal involvement in pediatric sarcoma patients is important to determine correct systemic and local therapy, with the goal to reduce subsequent recurrences. However, differences in lymph node staging strategies, definitions, and treatment protocols between the Children's Oncology Group (COG), European paediatric Soft tissue sarcoma Study Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS) complicate comparisons. In this article, we aim to establish internationally recognized recommendations for lymph node assessment and treatment of children and adolescents diagnosed with rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) according to the Consensus Conference Standard Operating Procedure methodology.

A two-stage HDR reconstruction pipeline for extreme dark-light RGGB images.

RGGB sensor arrays are commonly used in digital cameras and mobile photography. However, images of extreme dark-light conditions often suffer from insufficient exposure because the sensor receives insufficient light. The existing methods mainly employ U-Net variants, multi-stage camera parameter simulation, or image parameter processing to address this issue.

Granulomatous Variant of Giant Centrifugal Miliaria Profunda in an 8-Month-Old Boy: Expanding the Dermatologic Phenotype of Cardiofaciocutaneous Syndrome.

Miliarias are a group of eccrine disorders characterized by sweat retention due to the occlusion of eccrine ducts. Miliaria profunda is the rarest form of miliaria and occurs when eccrine ducts are obstructed at the dermal-epidermal junction. A granulomatous variant of centrifugal miliaria profunda classic findings of granulomatous infiltrate on histology and centrifugal expansion of deep nodules or plaques.

The Kinesin Kar3 is Required for Endoplasmic Reticulum-Associated Degradation.

Degradation of aberrant, excess, and regulatory proteins at the endoplasmic reticulum (ER) is a conserved feature of eukaryotic cells, disruption of which contributes to disease. While remarkable progress has been made in recent years, mechanisms and genetic requirements for ER-Associated Degradation (ERAD) remain incompletely understood. We recently conducted a screen for genes required for turnover of a model ER translocon-associated substrate of the Hrd1 ubiquitin ligase in .

Spleen stiffness in a healthy pediatric population undergoing liver magnetic resonance elastography.

Background: Splenic stiffness is a potential imaging marker of portal hypertension. Normative spleen stiffness values are needed to define diagnostic thresholds.

Objective: To report stiffness measurements of the spleen in healthy children undergoing liver magnetic resonance (MR) elastography across MRI vendors and field strengths.

Intercellular mRNA transfer alters the human pluripotent stem cell state.

Intercellular transmission of messenger RNA (mRNA) is being explored in mammalian species using immortal cell lines. Here, we uncover an intercellular mRNA transfer phenomenon that allows for the adaptation and reprogramming of human primed pluripotent stem cells (hPSCs). This process is induced by the direct cell contact-mediated coculture with mouse embryonic stem cells under the condition impermissible for primed hPSC culture.

Closed-loop ventilation and oxygenation with decision support fluid resuscitation to treat major burn injury with smoke induced acute respiratory distress syndrome.

Introduction: The understanding of the interaction of closed-loop control of ventilation and oxygenation, specifically fraction of inspired oxygen (FiO2) and positive end-expiratory pressure (PEEP), and fluid resuscitation after burn injury and acute lung injury from smoke inhalation is limited. We compared the effectiveness of FiO2, PEEP, and ventilation adjusted automatically using adaptive support ventilation (ASV) and decision support fluid resuscitation based on urine output in a clinically relevant conscious ovine model of lung injury secondary to combined smoke inhalation and major burn injury.

Methods: Sheep were subjected to burn and smoke inhalation injury under deep anesthesia and analgesia.

Early and Late Outcomes of Transcatheter Aortic Valve Replacement in Patients With Prior Chest Radiation: A Systematic Review and Meta-Analysis.

Background: Patients with prior history of chest or mediastinal radiation are deemed high risk for surgical AVR. Transcatheter aortic valve replacement (TAVR) has emerged as a promising alternative for these patients, however, this patient population was underrepresented in prior TAVR trials.

Aims: To compare the outcomes of TAVR in patients with versus without a history of prior chest or mediastinal radiation.

A Case Study of a Female Infant With Primary Hypertrophic Osteoarthropathy Demonstrates That Early Initiation of Celecoxib Slows but Does Not Prevent Symptom Progression.

Primary Hypertrophic Osteoarthropathy (PHOAR1) is characterized by autosomal recessive loss of function variants in 15-hydroxyprostaglandin dehydrogenase (HPGD) leading to digital clubbing, periostosis, pachydermia, and severe hyperhidrosis. HPGD catalyzes the first step of prostaglandin E2 (PGE2) degradation. Selective COX-2 inhibitors have proved beneficial in adults, though it is unknown if early initiation of COX-2 inhibitors can alter the natural history of PHOAR1.

Prepancreatic postduodenal portal vein discovered in a pediatric patient undergoing total pancreatectomy with islet autotransplantation: a case report and review of literature.

Background: Prepancreatic postduodenal portal vein (PPPV) is a rare anatomic variant where the portal vein (PV) runs anterior to the pancreas and posterior to the duodenum. Only 20 cases of PPPV, all in adults, have been reported in literature. We report the first case of PPPV in a pediatric patient discovered intraoperatively during total pancreatectomy with islet autotransplantation (TPIAT) and the third known case in which the PPPV could be isolated intraoperatively.

Salivary cardiac troponin does not correlate with serum levels.

Introduction: Several studies suggest a potential correlation between troponin levels detected in serum and saliva. However, prior investigations have not adequately addressed the critical aspect of collecting samples upon admission, which is essential for timely troponin level determination. This study aimed to evaluate the relationship between troponin levels in serum and saliva among patients admitted for chest pain evaluation.

Wellbeing and quality of life among parents of individuals with Fontan physiology.

Purpose: To examine global and health-related quality of life (QOL) among parents of individuals with Fontan physiology and determine associations with sociodemographic, parent and child-related health, psychological, and relational factors.

Methods: Parents participating in the Australian and New Zealand Fontan Registry (ANZFR) QOL Study (N = 151, Parent Mean age = 47.9 ± 10.

Pediatric Predictive Artificial Intelligence Implemented in Clinical Practice from 2010-2021: A Systematic Review.

Objective: To review pediatric artificial intelligence (AI) implementation studies from 2010-2021 and analyze reported performance measures.

Methods: We searched PubMed/Medline, Embase CINHAL, Cochrane Library CENTRAL, IEEE and Web of Science with controlled vocabulary.

Inclusion Criteria: AI intervention in a pediatric clinical setting that learns from data (i.

Opportunities to Improve Care Processes for Ventilator-Dependent Children: A Single-Center Study.

Objective: Children with chronic ventilator dependence are a complex and heterogeneous population with unique needs. We sought input from parents, clinicians, and hospital staff to identify opportunities for process improvement during hospitalization and discharge preparation.

Methods: We conducted a qualitative study at a large referral center for children with chronic ventilator dependence.