This plain language summary shares results from a clinical study called INTEGRIS-IPF that was published in the in 2024. This study looked at a medicine called (beck-so-teh-grast) as a possible treatment for (i-dee-uh-pa-thick pul-muh-ner-ee fie-bro-sis; IPF). is an investigational medicine, which means that it is being studied and has not yet been approved by the US Food and Drug Administration (FDA), for people with IPF to take as a treatment.
View Article and Find Full Text PDF: Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive disease characterized by dyspnea and loss of lung function. Transforming growth factor-beta (TGF-β) activation mediated by α integrins is central to the pathogenesis of IPF. Bexotegrast (PLN 74809) is an oral, once-daily, dual-selective inhibitor of αβ and αβ integrins under investigation for the treatment of IPF.
View Article and Find Full Text PDFDisabil Rehabil Assist Technol
January 2025
The purpose of this study was to evaluate the effectiveness of custom seating and mobility services provided an international service learning (ISL) model on the occupational performance of individuals with disabilities in Guatemala. A one-group prospective pretest-posttest design was implemented using the standardized Wheelchair Outcome Measure (WhOM - Spanish) administered on the day of wheelchair fitting, and at 3- and 6-month intervals after receiving seating and mobility services. A two-tailed -test demonstrated a statistically significant ( < 0.
View Article and Find Full Text PDFAm J Respir Crit Care Med
August 2024
Idiopathic pulmonary fibrosis (IPF) is a rare and progressive disease that causes progressive cough, exertional dyspnea, impaired quality of life, and death. Bexotegrast (PLN-74809) is an oral, once-daily, investigational drug in development for the treatment of IPF. This Phase-2a multicenter, clinical trial randomized participants with IPF to receive, orally and once daily, bexotegrast at 40 mg, 80 mg, 160 mg, or 320 mg, or placebo, with or without background IPF therapy (pirfenidone or nintedanib), in an approximately 3:1 ratio in each bexotegrast dose cohort, for at least 12 weeks.
View Article and Find Full Text PDFMaterials (Basel)
May 2024
In this article, the practical issues connected with guided wave measurement are studied: (1) the influence of gluing of PZT plate actuators (NAC2013) on generated elastic wave propagation, (2) the repeatability of PZT transducers attachment, and (3) the assessment of the possibility of comparing the results of Laser Doppler Vibrometry (LDV) measurement performed on different 2D samples. The consideration of these questions is crucial in the context of the assessment of the possibility of the application of the guided wave phenomenon to structural health-monitoring systems, e.g.
View Article and Find Full Text PDFMutations of the gene, which encodes the voltage-dependent Na channel's α subunit, are associated with diverse epileptic syndromes ranging in severity, even intra-family, from febrile seizures to epileptic encephalopathy. The underlying cause of this variability is unknown, suggesting the involvement of additional factors. The aim of our study was to describe the properties of mutated channels and investigate genetic causes for clinical syndromes' variability in the family of five gene p.
View Article and Find Full Text PDFJ R Stat Soc Ser C Appl Stat
January 2024
We formulate a statistical flight-pause model (FPM) for human mobility, represented by a collection of random objects, called motions, appropriate for mobile phone tracking (MPT) data. We develop the statistical machinery for parameter inference and trajectory imputation under various forms of missing data. We show that common assumptions about the missing data mechanism for MPT are not valid for the mechanism governing the random motions underlying the FPM, representing an understudied missing data phenomenon.
View Article and Find Full Text PDFCoenzyme Q5 (COQ5), a C-methyltransferase, modifies coenzyme Q10 (COQ10) during biosynthesis and interacts with polyA-tail regulating zinc-finger protein ZC3H14 in neural development. Here, we present a fifth patient (a third family) worldwide with neurodevelopmental and physiological symptoms including COQ10 deficiency. Our patient harbors one novel c.
View Article and Find Full Text PDFMaterials (Basel)
November 2022
The aim of the presented examination is condition-monitoring of GFRP-reinforced concrete structural members using elastic wave propagation. As an example, a deck slab is selected. The deck slab is made of concrete of the targeted C30/37 class under three-point bending.
View Article and Find Full Text PDFThis paper introduces a machine vision-based system promising low-cost solution for detecting a fatigue crack propagation caused by alternating mechanical stresses. The fatigue crack in technical components usually starts on surfaces at stress concentration points. The presented system was designed to substitute a strain gauge sensor-based measurement using an industrial camera in cooperation with branding software.
View Article and Find Full Text PDFMicrocephaly presents in neurodevelopmental disorders with multiple aetiologies, including bi-allelic mutation in , a component of the biologically fundamental and conserved microtubule-nucleation complex, γ-TuRC. Elucidating underlying principles driving microcephaly requires clear phenotype recapitulation and assay reproducibility, areas where go-to experimental models fall short. We present an alternative simple vertebrate/invertebrate dual system to investigate fundamental -related processes driving human microcephaly and associated developmental traits.
View Article and Find Full Text PDFThe transfer of graphics to a product's surface is a widely known technology. Printing, engraving, and etching are used every day in production processes with countless types of materials. This paper deals with quality control for laser engraving on surfaces with variable dimensions via optical sensors.
View Article and Find Full Text PDFMultiple myeloma (MM) and chronic lymphocytic leukemia (CLL) patients have increased morbidity and mortality rates of COVID-19 due to immunosuppression associated with the disease and ongoing therapy. The same immune impairment accompanying CLL and MM also affects suboptimal vaccine response. The study assessed the effectiveness of the humoral and T cell-mediated immunity following mRNA COVID-19 vaccination (using either BNT162b2 or mRNA-1273) in short-term (2-5 weeks after second dose) and long-term follow-up (12 weeks after vaccination).
View Article and Find Full Text PDFAlthough the outcome has improved over the past decades, due to improved supportive care, a better understanding of risk factors, and intensified chemotherapy, pediatric acute myeloid leukemia remains a life-threatening disease, and overall survival (OS) remains near 70%. According to French-American-British (FAB) classification, AML is divided into eight subtypes (M0-M7), and each is characterized by a different pathogenesis and response to treatment. However, the curability of AML is due to the intensification of standard chemotherapy, more precise risk classification, improvements in supportive care, and the use of minimal residual disease to monitor response to therapy.
View Article and Find Full Text PDFActin molecules are fundamental for embryonic structural and functional differentiation; γ-actin is specifically required for the maintenance and function of cytoskeletal structures in the ear, resulting in hearing. Baraitser-Winter Syndrome (B-WS, OMIM #243310, #614583) is a rare, multiple-anomaly genetic disorder caused by mutations in either cytoplasmically expressed actin gene, (β-actin) or (γ-actin). The resulting actinopathies cause characteristic cerebrofrontofacial and developmental traits, including progressive sensorineural deafness.
View Article and Find Full Text PDFThe apelinergic system, which includes the apelin receptor (APJ) as well as its two specific ligands, namely apelin and ELABELA (ELA/APELA/Toddler), have been the subject of many recent studies due to their pleiotropic effects in humans and other animals. Expression of these factors has been investigated in numerous tissues and organs-for example, the lungs, heart, uterus, and ovary. Moreover, a number of studies have been devoted to understanding the role of apelin and the entire apelinergic system in the most important processes in the body, starting from early stages of human life with regulation of placental function and the proper course of pregnancy.
View Article and Find Full Text PDFCongenital microcephaly causes smaller than average head circumference relative to age, sex and ethnicity and is most usually associated with a variety of neurodevelopmental disorders. The underlying etiology is highly heterogeneous and can be either environmental or genetic. Disruption of any one of multiple biological processes, such as those underlying neurogenesis, cell cycle and division, DNA repair or transcription regulation, can result in microcephaly.
View Article and Find Full Text PDFGlass fiber-reinforced polymer structures (GFRPS) are widely used in civil and mechanical fields due to their light weight and corrosion resistance. However, these structures are prone to damage with very-low-energy impacts. The reliability of such structures is of prime importance before their installation and usage.
View Article and Find Full Text PDFProper functioning of the body depends on hormonal homeostasis. White adipose tissue is now known as an endocrine organ due to the secretion of multiple molecules called adipokines. These proteins exert direct effects on whole body functions, including lipid metabolism, angiogenesis, inflammation, and reproduction, whereas changes in their level are linked with pathological events, such as infertility, diabetes, and increased food intake.
View Article and Find Full Text PDFResistin plays an important role in adipogenesis, obesity, insulin resistance, and reproduction. Previous studies showed resistin action on ovarian follicular cells; however, whether resistin regulates steroid secretion in luteal cells is still unknown. Our aim was first to determine the expression of resistin and its potential receptors (tyrosine kinase-like orphan receptor 1 (ROR1) and toll-like receptor 4 (TLR4)) in the porcine corpus luteum (CL), regulation of its expression, effect on kinases phosphorylation, and luteal steroidogenesis.
View Article and Find Full Text PDFIntroduction: Genetic forms of Parkinson's disease (PD) often cluster in different ethnic groups and may present with recognisable unique clinical manifestations. Our aim was to summarise the current state of knowledge regarding the genetic causes of PD and describe the first Polish patient with SNCA duplication.
Methodology: We searched the electronic database, PubMed, for studies between January 1995 and June 2020 that evaluated genetics in Polish patients with PD, using the search terms 'Parkinson's disease, 'Polish', 'genetics', 'mutations', and 'variants'.
Objectives: The study aimed to present a family with myoclonus dystonia (M-D) syndrome due to a mutation in the epsilon sarcoglycan gene (SGCE). Three members of the family suffered from treatment-refractory severe myoclonic jerks of the neck, trunk, and upper extremities. The mild dystonic symptoms recognized as cervical dystonia or truncal dystonia affected all individuals.
View Article and Find Full Text PDFThis study consists of nine case studies addressing thermal comfort in the public areas of city centres, with particular emphasis on the measurable effects of blue and green infrastructure on thermal exposure. Daytime on-site measurements were taken in summer in the paved areas of squares, in the proximity of water fountains, and in the shade of trees in order to evaluate levels of heat stress based on the universal thermal climate index (UTCI). The differences in UTCI values between the research points confirm substantial cooling associated with high vegetation (trees induced differences up to 10.
View Article and Find Full Text PDFThis paper presents a nondestructive analysis of debonds in an adhesively-bonded carbon-fibre reinforced composite structure under variable temperature conditions. Towards this, ultrasonic guided wave propagation based experimental analysis and numerical simulations are carried out for a sample composite structure to investigate the wave propagation characteristics and detect debonds under variable operating temperature conditions. The analysis revealed that the presence of debonds in the structure significantly reduces the wave mode amplitudes, and this effect further increases with the increase in ambient temperature and debond size.
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