Evaluation of Lyso-Gb1 as a biomarker for Gaucher disease treatment outcomes using data from the Gaucher Outcome Survey.

Background: Patients with Gaucher disease (GD) require continual monitoring; however, lack of specific disease biomarkers was a significant challenge in the past. Glucosylsphingosine (lyso-Gb1) has been shown to be a reliable, key, specific, and sensitive biomarker for diagnosis, prognosis, and treatment response in clinical studies of patients with GD. We evaluated the change in lyso-Gb1 concentration over time following enzyme replacement therapy in patients with confirmed GD using real-world data from the Gaucher Outcome Survey disease registry.

Academic Achievement, Externalizing Problems, and Close Friends in Middle School: Testing a Developmental Cascade Model Leading to Educational Attainment in the Late Twenties.

Because educational attainment is associated with well-being in the long term, it is important to understand the developmental processes that enhance academic outcomes during adolescence. Also, although the importance of friends is well documented in adolescence, little is known about how close friends' characteristics work together with youth's own characteristics to shape adolescents' educational trajectories. This study fills an important gap in knowledge by focusing on how middle school students' academic achievement and externalizing problems are associated with their friends' achievement and externalizing problems over time, and how these variables predict educational attainment in adulthood.

Accuracy of remote, video-based supraventricular tachycardia detection in patients undergoing elective electrical cardioversion: a prospective cohort.

Unobtrusive pulse rate monitoring by continuous video recording, based on remote photoplethysmography (rPPG), might enable early detection of perioperative arrhythmias in general ward patients. However, the accuracy of an rPPG-based machine learning model to monitor the pulse rate during sinus rhythm and arrhythmias is unknown. We conducted a prospective, observational diagnostic study in a cohort with a high prevalence of arrhythmias (patients undergoing elective electrical cardioversion).

Photon-counting detector CTA to assess intracranial stents and flow diverters: an in vivo study with ultrahigh-resolution spectral reconstructions.

Background: To define optimal parameters for the evaluation of vessel visibility in intracranial stents (ICS) and flow diverters (FD) using photon-counting detector computed tomography angiography (PCD-CTA) with spectral reconstructions.

Methods: We retrospectively analyzed consecutive patients with implanted ICS or FD, who received a PCD-CTA between April 2023 and March 2024. Polyenergetic, virtual monoenergetic, pure lumen, and iodine reconstructions with different keV levels (40, 60, and 80) and reconstruction kernels (body vascular [Bv]48, Bv56, Bv64, Bv72, and Bv76) were evaluated by two radiologists with regions of interests and Likert scales.

An evaporite sequence from ancient brine recorded in Bennu samples.

Evaporation or freezing of water-rich fluids with dilute concentrations of dissolved salts can produce brines, as observed in closed basins on Earth and detected by remote sensing on icy bodies in the outer Solar System. The mineralogical evolution of these brines is well understood in regard to terrestrial environments, but poorly constrained for extraterrestrial systems owing to a lack of direct sampling. Here we report the occurrence of salt minerals in samples of the asteroid (101955) Bennu returned by the OSIRIS-REx mission.

fragSMILES as a chemical string notation for advanced fragment and chirality representation.

Generative models have revolutionized de novo drug design, allowing to produce molecules on-demand with desired physicochemical and pharmacological properties. String based molecular representations, such as SMILES (Simplified Molecular Input Line Entry System) and SELFIES (Self-Referencing Embedded Strings), have played a pivotal role in the success of generative approaches, thanks to their capacity to encode atom- and bond- information and ease-of-generation. However, such 'atom-level' string representations could have certain limitations, in terms of capturing information on chirality, and synthetic accessibility of the corresponding designs.

The Society of Thoracic Surgeons General Thoracic Surgery Database: 2024 Update on Outcomes and Research.

The Society of Thoracic Surgeons General Thoracic Surgery Database (STS GTSD) remains the largest and most comprehensive audited thoracic surgical database in the world. As the STS GTSD grows to nearly 1 million cases, the pulmonary resection for cancer and esophagectomy short-term risk models have been refined to provide participants with benchmarked performance reports to facilitate quality improvement efforts. New for 2025 will be the development of long-term risk models and the online release of both short- and long-term risk calculators.

National Survey of Lung Cancer Screening Eligibility in United States Veterans.

Introduction: Lung cancer screening is underutilized, especially in rural areas where lung cancer mortality is high. Approximately 11.2% of the United States (US) population over age 50 meet the United States Preventive Services Task Force (USPSTF) 2021 lung cancer screening eligibility criteria; the proportion of eligible Veterans is unknown.

Pediatric ECMO Outcomes in Children With Severe Infections: Does Infectious Source Matter?

Background: Although Extracorporeal Membrane Oxygenation (ECMO) utilization in pediatric patients with cardiopulmonary failure due to infection improves mortality, it is unclear whether the infectious etiology impacts outcomes. The aim of this study is to compare ECMO outcomes in children with sepsis and severe acute lung injury secondary to infections based on culture data.

Methods: A retrospective review was done of patients aged <18 with severe infections whose management included ECMO from 2013 to 2022 at a quaternary children's hospital.

Efficacy, immunogenicity, and safety of an investigational maternal respiratory syncytial virus prefusion F protein-based vaccine.

Background: In this phase 3 trial of an investigational maternal respiratory syncytial virus prefusion F protein-based vaccine (RSVPreF3-Mat), a higher rate of preterm birth was observed in the vaccine (6.8%) versus the placebo group (4.9%).

Helping authors produce FAIR taxonomic data: evaluation of an author-driven phenotype data production prototype.

It is well-known that the use of vocabulary in phenotype treatments is often inconsistent. An earlier survey of biologists who create or use phenotypic characters revealed that this lack of standardization leads to ambiguities, frustrating both the consumers and producers of phenotypic data. Such ambiguities are challenging for biologists, and more so for Artificial Intelligence, to resolve.

Heterogeneity analysis provides evidence for a genetically homogeneous subtype of bipolar-disorder.

Background: Bipolar Disorder (BD) is a complex disease. It is heterogeneous, both at the phenotypic and genetic level, although the extent and impact of this heterogeneity is not fully understood. One way to assess this heterogeneity is to look for patterns in the subphenotype data.

A head-to-head comparison of the adult EQ-5D-5L and youth EQ-5D-Y-5L in adolescents with idiopathic scoliosis.

Background: Multiple diseases, such as Adolescent Idiopathic Scoliosis (AIS), present at adolescent age and the impact on quality of life (QoL) prolongs into adulthood. For the EQ-5D, a commonly used instrument to measure QoL, the current guideline is ambiguous whether the youth or adult version is to be preferred at adolescent age. To assess which is most suitable, this study tested for equivalence along predefined criteria of the youth (EQ-5D-5L) and adult (EQ-5D-Y-5L) version in an adolescent population receiving bracing therapy for AIS.

Family Size across the Life Course and Cognitive Decline in Older Mexican Adults.

Objectives: A growing body of research has identified associations between family size and cognition in older adults. These studies largely focus on older adults' own fertility history instead of sibship size, defined as one's number of siblings. Sibship size may impact cognitive development during early childhood, creating differences that may persist into late-life.

Updated Adjustment of the HCAHPS Survey for New Modes of Survey Administration and Patient Mix.

Background: Web-first multimode survey protocols increase HCAHPS survey response rates and representativeness but may result in different HCAHPS scores because of survey mode effects and selective email address availability. A variable absent from many patient-mix adjustment models that may result in more positive patient experiences is whether the hospital admission was planned; adjustment for planned stays may better measure hospital performance.

Objectives: Develop adjustments for new Web-first survey protocols and planned admissions to facilitate comparisons across hospitals.

Basket study of oral progesterone antagonist onapristone extended-release in combination with anastrozole in progesterone receptor-positive recurrent adult-type granulosa cell tumor of the ovary.

Objective: We sought to determine the safety and efficacy of the oral progesterone antagonist onapristone in combination with anastrozole in patients with recurrent progesterone receptor-positive adult-type granulosa cell tumor of the ovary.

Methods: This was a single-institution phase II study of patients with progesterone receptor-positive adult-type granulosa cell tumor who received at least 1 prior line of chemotherapy. Patients were enrolled from November 2021 to August 2022 and tissue was evaluated for progesterone receptor status via immunohistochemistry.

[Can Coronavirus HCoV-229E be Used as a Model Virus Instead of SARS-CoV-2 in Antiviral Efficacy Studies?].

Son yıllarda pandemi nedeniyle virüslerin tanı ve tedavisine yönelik terapötik yöntemlerin geliştirilmesi ve antivirallerin test edilmesi amacıyla çok sayıda in vitro çalışma yapılmaktadır. Literatürde SARS-CoV-2'nin modellenebilmesi için HCoV-229E'nin kullanımının güvenli ve yeterli olup olmadığını inceleyen çalışmalar sınırlıdır. Bu sebeple bu çalışmada, BSL-2 şartlarında gerçekleştirilebilen HCoV-229E kültürü ve kantitasyon çalışmalarının, BSL-3 şartları gerektiren SARS-CoV-2 deneylerinde bir ön çalışma modeli olup olamayacağının antiviral etkinlik analizleri üzerinden araştırılması amaçlanmıştır.

A Belgian physician-initiated trial investigating the LifeStream peripheral stent graft system for the treatment of complex TASC C and D iliac lesions.

Background: Due to the heterogeneity of literature findings, stent type selection for the endovascular treatment of complex aorto-iliac occlusive disease remains challenging. The BELSTREAM study, a physician-initiated, prospective, multicenter, single-arm study, aims to report the safety and efficacy of the balloon expandable LifeStream Peripheral Stent Graft System (BD, Tempe, Arizona, USA) for the treatment of complex TASC C and D aorto-iliac artery lesions.

Methods: Seventy patients and 133 lesions were included at six Belgian institutions.

Noninferiority of single-incision laparoscopy vs conventional laparoscopy in salpingectomy or salpingotomy for ectopic pregnancy: a meta-analysis.

Objective: Ectopic pregnancy is an emergency frequently requiring laparoscopic intervention. This study aimed to determine whether single-incision laparoscopic surgery is a safe and effective treatment method compared with conventional laparoscopic surgery with multiple ports.

Data Sources: This study searched 6 databases from their inception to May 15, 2024, for articles comparing the safety outcomes of single-incision laparoscopic surgery with conventional laparoscopic surgery in managing women with ectopic pregnancy.

hsa-miR-548d-3p: a potential microRNA to target nucleocapsid and/or capsid genes in multiple members of the Flaviviridae family.

Introduction: Flaviviridae comprise a group of enveloped, positive-stranded RNA viruses that are mainly transmitted through either mosquitoes or tick bites and/or contaminated blood, blood products, or other body secretions. These viruses cause diseases ranging from mild to severe and are considered important human pathogens. MicroRNAs (miRNAs) are non-coding molecules involved in growth, development, cell proliferation, protein synthesis, apoptosis, and pathogenesis.

Ultrasound imaging: Enhancing the diagnosis of carpal tunnel syndrome.

Broader adoption of ultrasound (US) imaging in carpal tunnel syndrome management enhances patient care and outcome. This case underscores the importance in diagnosing carpal tunnel syndrome, highlighting its capability to uncover hidden anomalies and assist in surgical planning.

Prognostic Nutritional Index (PNI) As a Preoperative Screening Tool in Predicting Clinical Outcomes of Postoperative Pediatric Cardiac Surgery Patients.

Objective: The authors sought to evaluate the role of nutritional indices such as Onodera's prognostic nutrition index (PNI), World Health Organization (WHO)-based anthropometric measurements such as weight for age (w/a), height for age, weight for height, and perioperative serum albumin levels in the determination of postoperative clinical outcomes in pediatric patients who undergo surgery for congenital cardiac defects and surgical complexity (risk-adjusted congenital heart surgery score) and its correlation with postoperative course.

Material And Methods: In this prospective observational study, 108 post-pediatric cardiac surgery patients under the age of 18 months were enrolled between January 2023 and August 2023. Through receiver operating characteristic curve analysis we have found the cutoff value for PNI is ≤66.

Social learning preserves both useful and useless theories by canalizing learners' exploration.

In many domains, learning from others is crucial for leveraging cumulative cultural knowledge, which encapsulates the efforts of successive generations of innovators. However, anecdotal and experimental evidence suggests that reliance on social information can reduce the exploration of the problem space. Here, we experimentally investigate the extent to which cultural transmission fosters the persistence of arbitrary solutions in a context where participants are incentivized to improve a physical system across multiple trials.

Building blocks for nanophotonic devices and metamaterials.

Nanophotonic devices control and manipulate light at the nanometer scale. Applications include biological imaging, integrated photonic circuits, and metamaterials. The design of these devices requires the accurate modeling of light-matter interactions at the nanoscale and the optimization of multiple design parameters, both of which can be computationally demanding and time intensive.