Results 31 to 40 of about 17,267 (219)

Re‐Purposing Sapropterin (Kuvan) for ACTA2‐Related Multisystemic Smooth Muscle Dysfunction Syndrome: A Translational Mechanistic and First‐In‐Human Therapeutic Report

open access: yesAnnals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Multisystemic smooth muscle dysfunction syndrome (MSMDS) is an ultra‐rare, ACTA2‐related disorder characterized by severe cerebrovascular disease, aortic aneurysms, and smooth muscle dysfunction. Using molecular dynamics simulations and in silico drug screening, we identified that sapropterin dihydrochloride (Kuvan) is a candidate capable of ...
Moran Hausman‐Kedem   +9 more
wiley   +1 more source

Artificial Intelligence in Systemic Sclerosis: Clinical Applications, Challenges, and Future Directions

open access: yesArthritis Care &Research, EarlyView.
Systemic sclerosis (SSc) is a rare autoimmune disease defined by immune dysregulation, vasculopathy, and progressive fibrosis of the skin and internal organs. Despite advances in care, major complications such as interstitial lung disease (ILD) and myocardial involvement remain the leading causes of morbidity and mortality.
Cristiana Sieiro Santos   +2 more
wiley   +1 more source

Immunosuppressive Drug Use in Limited Systemic Sclerosis: An International Survey

open access: yesArthritis Care &Research, EarlyView.
Objective Current guidelines recommend immunosuppressive treatment for diffuse cutaneous systemic sclerosis but are less clear on their use in limited cutaneous systemic sclerosis (lcSSc) in the absence of internal organ complications. We conducted an international survey to understand current immunosuppressive drug prescribing patterns in lcSSc ...
Sabrina Hoa   +3 more
wiley   +1 more source

Anorectal Dysfunction in Systemic Sclerosis: Clinical Phenotypes and Functional Patterns

open access: yesArthritis Care &Research, EarlyView.
Objective The aim of this study was to characterize specific physiologic defects in anorectal dysfunction in systemic sclerosis (SSc) using anorectal manometry (ARM), evaluate associations with gastrointestinal (GI) and extraintestinal clinical phenotypes, and explore potential serologic markers for risk stratification.
Timothy Kaniecki   +6 more
wiley   +1 more source

Microvascular damage is associated with carotid wall structural changes in Systemic Sclerosis: a capillaroscopy and ultrasound‐based observational study

open access: yesArthritis Care &Research, Accepted Article.
Introduction Systemic sclerosis (SSc) is characterized by cardiovascular risk excess not fully explained by traditional factors. Whether the severity of microvascular damage correlates with structural subclinical atherosclerosis remains unclear. We investigated the relationship between nailfold videocapillaroscopy (NVC) abnormalities and carotid ...
Eugenio Capparelli   +13 more
wiley   +1 more source

Elevated liver enzymes is not an impediment to allopurinol dose escalation: an observational cohort study

open access: yesArthritis Care &Research, Accepted Article.
Objective Routine liver enzyme monitoring is advocated while on allopurinol but supporting guidance is lacking. We assessed the incidence and factors associated with elevated liver enzymes in gout patients commenced on allopurinol. Methods 150 patients with gout and normal baseline serum aminotransferases initiated on allopurinol were followed ...
May Shuen Tang   +4 more
wiley   +1 more source

Mechanochemical Synthesis and Characterization of Nanostructured ErB4 and NdB4 Rare‐Earth Tetraborides

open access: yesAdvanced Engineering Materials, Volume 27, Issue 6, March 2025.
ErB4 and NdB4 nanostructured powders are produced by mechanochemical synthesis. 5 h mechanical alloying and 4 M HCl acid leaching are used in the production. ErB4 and NdB4 powders exhibit maximum magnetization of 0.4726 emu g−1 accompanied with an antiferromagnetic‐to‐paramagnetic phase transition at about TN = 18 K and 0.132 emu g−1 with a maximum at ...
Burçak Boztemur   +5 more
wiley   +1 more source

Microstructural Evolution and Vacancy Defect Formation in Mn–Mo–Ni RPV Steel Under Low Cycle Fatigue: Insights From EBSD and PALS

open access: yesAdvanced Engineering Materials, EarlyView.
Low‐cycle fatigue damage in Mn–Mo–Ni reactor pressure vessel steel is examined using a combined electron backscatter diffraction and positron annihilation lifetime spectroscopy approach. The study correlates texture evolution, dislocation substructure development, and vacancy‐type defect formation across uniform, necked, and fracture regions, providing
Apu Sarkar   +2 more
wiley   +1 more source

Thermodynamic Pathways of Nonequilibrium Solidification in Wire‐Arc Additive Manufacturing Fe‐Based Multicomponent Alloy Structures

open access: yesAdvanced Engineering Materials, EarlyView.
Geometry‐driven thermal behavior in wire‐arc additive manufacturing (WAAM) influences microstructural evolution during nonequilibrium solidification of a chemically complex Fe–Cr–Nb–W–Mo–C nanocomposite system. By comparing different deposits configurations, distinct entropy–cooling rate correlations, segregation, and carbide evolution are revealed ...
Blanca Palacios   +5 more
wiley   +1 more source

Pyramidal Structures on Yttria‐Stabilized Zirconia after High Temperature Exposure at 1500°C: New Features on an Old Material

open access: yesAdvanced Engineering Materials, EarlyView.
New features on yttria‐stabilized zirconia after exposure at 1500°C: Newly discovered pyramidal structures on an old material. After exposure at 1550°C on the cross section of YSZ new features, namely pyramidal structures are discovered. These structures grow with time, increase in numbers, appear as singularities, are often arranged in strings, and ...
Doris Sebold   +2 more
wiley   +1 more source

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