Digital Commons Network^™

Recombinant Human Alpha-N-Acetylglucosamine-6-Sulfatase Delivered To Sanfilippo D Mice With Repeated Intracerebroventricular Injections Corrects Cns Pathology, Grant L. Austin, Feng Wang, Steven Q. Le, Alexander Sorensen, Shan Li, Lai C. Foong, Srikanth Singamsetty, Jill Wood, Tsui-Fen Chou, Patricia I. Dickson

2020-Current year OA Pubs

Mucopolysaccharidosis type IIID (MPS IIID; Sanfilippo D) is caused by biallelic pathogenic variants in N-acetylglucosamine-6-sulfatase (GNS), which participates in catabolism of heparan sulfate (HS) glycosaminoglycans. Characterization of MPS IIID disease at a cellular level has not been robustly achieved. We used unbiased quantitative proteomics to establish a cellular phenotype for MPS IIID mice. Recombinant human GNS (rhGNS), a variant of which previously demonstrated single dose efficacy in MPS IIID human fibroblasts and in MPS IIID neonatal mice, was used to establish a repeat dosing schedule to treat MPS IIID mice. Adult Gns KO mice or heterozygous carriers were treated via …

Preliminary Investigation Of Obstructive Sleep Apnea And Alzheimer's Disease In Down Syndrome, Dasoo M Yoon, David T Plante, Victoria Fleming, Benjamin Handen, Patrick Lao, Jamie Peven, Bradley Christian, Ozioma Okonkwo, Charles Laymon, Beau Ances, Christy Hom, Brian Helsel, Sigan L Hartley

2020-Current year OA Pubs

This study provided a preliminary examination of indices of obstructive sleep apsnea (OSA) and sleep disruptions in adults with Down syndrome (DS), and their associations with Alzheimer's disease (AD) pathology and symptomatology. A total of 93 adults with DS (aged 25-61 years) from the Alzheimer Biomarker Consortium-DS completed cognitive assessments, MRI and positron emission tomography (PET) scans (assessing amyloid-beta [Aβ] and tau), and a one-night home sleep study using the WatchPAT-300 device. Study partners also reported on depressive symptoms and diagnoses. Correlational analyses examined relationships between sleep variables, PET biomarkers, and AD symptomatology (cognitive functioning and depressive mood), controlling for …

The Paradoxical Effect Of Job Autonomy On Communication Overload: A Pathway To Innovative Work Behaviour, Oualid Abidi, Rupali Bhagat, Hamsa Sarhan, Mirna Safi

Research outputs 2022 to 2026

Purpose: This research aims to propose autonomy as a job characteristic that alleviates communication overload, thereby fostering the innovative work behaviours exhibited by knowledge workers. Design/methodology/approach – An empirical survey was administered to 277 knowledge workers from diverse sectors in Kuwait. To analyse the interrelations among the three constructs of the research model, i.e. job autonomy, innovative work behaviours and communication overload, the structural equation modelling method was applied, and mediation analysis was conducted using a bootstrapping method to examine the indirect effect of communication overload. Findings – The findings indicate that the level of job autonomy has a significant …

Thalamic And Visual Network Dysfunction Relates To Tremor Response In Thalamic Deep Brain Stimulation, Aimee E Morris, Babatunde Adeyemo, Meghan C Campbell, Abraham Z Snyder, Joel S Perlmutter, Jonathan W Mink, Scott A Norris

2020-Current year OA Pubs

BACKGROUND: Essential tremor (ET) is the most common movement disorder in adults, but its pathogenesis is incompletely understood. Deep brain stimulation of the ventral intermediate thalamic nucleus (VIM DBS) provides effective treatment for medically-refractory cases. We aimed to determine how pre-surgical resting-state functional connectivity (FC) in medically-refractory ET relates to VIM DBS clinical response.

METHODS: We analyzed resting-state FC MRI in 21 participants with medically-refractory ET who subsequently underwent VIM DBS and 34 matched controls. We applied rigorous quality assurance to minimize motion artifact. Whole-brain correlation matrices were computed across 300 cortical, subcortical, and cerebellar regions and compared across groups …

Improving Hpv Vaccine Rates For Aboriginal Young People Through A Social Media Campaign: An Interrupted Time Series, Natalie A. Strobel, Jocelyn Jones, Kim Gates, Simon L. Turner, Joanne E. Mckenzie, Daniel R. Mcaullay

Research outputs 2022 to 2026

Human papillomavirus (HPV) is a common sexually transmitted infection that can lead to HPV-related cancers such as cervical cancer and other cancers such as anal, vaginal, and penile cancer. HPV rarely produces symptoms and cannot be cured or treated; therefore, vaccination is essential to protect against HPV and HPV-related diseases. However, young Aboriginal and Torres Strait Islander (hereafter, respectfully, ‘Aboriginal’) people are not receiving their HPV vaccine dose, resulting in missed opportunities to be protected from HPV-related cancers. Health promotion is a critical way to empower people to take ownership over and control their health and is a core function …

Professional Experience In Initial Teacher Education: Integrating Standards, Supervisors And Mentor Teachers, Madlen Griffiths, Mandie Shean

Research outputs 2022 to 2026

Professional experience is the training ground for preservice teachers, where they apply what they have learned at university, in the reality of the classroom setting. Our article investigates the contribution of university supervisors to preservice teacher learning, from the perspective of both groups. This qualitative study gathered data from interviews with 28 preservice teachers and supervisors. Findings highlighted the complexity of the task assigned to supervisors, and the distinctive role that they play in the preservice teachers’ learning. This study offers a model of supervision drawn from the voices of these two key stakeholders that is underpinned by theory and …

Sustainability Assessment: The State Of The Art In 2026, Alan Bond, Angus Morrison-Saunders, A. John Sinclair

Research outputs 2022 to 2026

Sustainability assessment (SA) has developed over the past three decades as a decision-support process explicitly directing choices towards sustainability. This paper provides an updated state-of-the-art review of SA in 2025, building on the 2012 assessment by Bond, Morrison-Saunders and Pope. We examine progress in theory, practice and process across selected jurisdictions, considering how SA has evolved conceptually and in application. While the volume of academic research on SA continues to grow, practical uptake remains uneven and context-dependent. Key innovations shaping the field include the embedding of sustainability provisions within Canada’s next-generation impact assessment framework, the incorporation of holistic assessment principles …

To Discard Or Not To Discard 1pns? A Systematic Review And Meta-Analysis On 291,474 Embryos, T. Lee, F. Qi, K. Peirce, J. Natalwala, V. Chapple, P. J. Mark, K. Sanders, Y. Liu

Research outputs 2022 to 2026

Unlike embryos that display two pronuclei (2PN), monopronucleated (1PN) embryos are assumed to be haploid and can be routinely discarded. Although there are reports of live births after 1PN embryo transfer, the developmental and clinical prognosis of 1PN embryos has not been systematically evaluated. The aim of this review was to elucidate the developmental, clinical and neonatal outcomes of 1PN embryos compared with 2PN embryos. Twenty-four studies met the inclusion criteria. The blastulation rate was lower in 1PN embryos compared with 2PN embryos [risk ratio (RR) 0.50, 95% CI 0.48–0.51]. The live birth rate was lower in 1PN blastocysts when …

What Makes An Airbnb Experience Memorable? A Text Analysis Using Leximancer, Eunjung Kim, Songshan Huang

Research outputs 2022 to 2026

This study explores the factors that contribute to memorable experiences in the context of Airbnb. An open-ended question, ‘Please describe your most memorable experience with Airbnb’, was asked in the survey, and a total of 824 valid responses were obtained. The qualitative analysis of the data was conducted using Leximancer. The findings highlight the significance of the ‘Stay’, ‘Home’, ‘Family’, ‘Host’, ‘Beach’, and ‘Use’ themes, each playing a vital role in creating memorable experiences. In particular, the present study extends these findings by offering a more nuanced understanding of these dimensions, with specific themes emerging in relation to different demographic …

A Clinical Practice Review Of Optimizing The Approach For Correction Of Thoracolumbar Deformity— When And How To Perform Anterior Releases And Fusion, Brian M. Phelps, Munish C. Gupta

2020-Current year OA Pubs

No abstract provided.

Work-Integrated Learning As A Landscape Of Practice, Denise Jackson, Karsten E. Zegwaard, David Drewery

Research outputs 2022 to 2026

This article proposes that work-integrated learning (WIL) is a Landscape of Practice (LoP) within the wider higher education ecosystem. It applies concepts from the LoP literature to present a new perspective on WIL programs and ecosystems. It further emphasizes that students are central within the WIL LoP, developing knowledge as they traverse individual communities including industry partners, professional accrediting bodies, careers services, academics, curriculum, and support staff connected to WIL. Additionally, the article highlights the role of higher education institutions in addressing tensions of difference between communities comprising the WIL LoP. The article contributes to the literature on WIL and …

Accuracy And Reliability Of Video-Based Range-Of-Motion Assessments In Postreconstruction Brachial Plexus Patients, Christopher J. Dy, Stephen J. Demartini, Eshan Sane, David M. Brogan, Planet Study Group

2020-Current year OA Pubs

PURPOSE: Following surgical reconstructions considered successful, many patients with brachial plexus injuries (BPI) have limited limb motion. In addition to manual muscle testing, clinicians typically measure active range of motion (AROM). AROM measurement relies on in-person examination and manual goniometers or visual estimation, both of which are subject to interobserver variability. The purpose of this proof-of-concept study was to evaluate reliability of video-based AROM assessments. We hypothesized that video-based assessment of AROM would have high inter-rater reliability (IRR) among surgeons who perform BPI reconstruction.

METHODS: We video recorded a standardized examination in a convenience sample of 8 postreconstruction BPI patients …

Caregiver Worry About Covid-19 As A Predictor Of Social Mitigation Behaviours And Sars-Cov-2 Infection In A 12-City U.S. Surveillance Study Of Households With Children, Steven M Brunwasser, Katherine Rivera-Spoljaric, Et Al.

2020-Current year OA Pubs

OBJECTIVE: Understanding compliance with COVID-19 mitigation recommendations is critical for informing efforts to contain future infectious disease outbreaks. This study tested the hypothesis that higher levels of worry about COVID-19 illness among household caregivers would predict lower (a) levels of overall and discretionary social exposure activities and (b) rates of household SARS-CoV-2 infections.

METHODS: Data were drawn from a surveillance study of households with children (

RESULTS: Caregivers with high enrollment levels of worry about COVID-19 illness were more likely to reduce direct social contact outside the household, particularly during the U.S.'s most deadly pandemic wave. Households of caregivers with …

Disclosing Disabilities: Barriers For Medical School Applicants, Sarah Leah Solomon, Aitan E. Magence, Harry Haran, Fern R. Juster, Kristina H. Petersen

2020-Current year OA Pubs

This study investigated the availability of accommodation request procedures for medical school admissions interviews and the accessibility of Technical Standards (TS) for candidates with disabilities (CWD) across Liaison Committee on Medical Education (LCME)-accredited institutions in the United States and Canada. Utilizing a cross-sectional study methodology, surveys were distributed to Deans of Admissions and Disabilities Resource Professionals (DRP) at all LCME-accredited US and Canadian MD programs. Surveys gathered data about interview formats, interview accommodation procedures, and TS accessibility during the 2018-2019 academic year. We received responses from 71 institutions (41.3%), with 56.3% survey completion rate (n = 40). Among respondents, interview …

A Climate Justice Analysis Of Western Australian Government Funding For Collie’S Transition From Coal, 2019-2024, Emma O’Donovan, Naomi Joy Godden

Research outputs 2022 to 2026

The WA Government will close its State owned coal-fired power stations in Collie by 2030, and has committed AU$692 million for Collie’s energy transition. At the request of Wilman and Kaneang Elders and community members, Edith Cowan University’s Centre for People, Place and Planet conducted desktop research about how Collie’s transition funding was allocated between January 2019 and July 2024. This report is a climate justice analysis of publicly-available funding announcements for Collie’s transition during this period to assess whether the distribution of transition resources is equitable and just.

Comparison And Clinical Applicability Analysis Of All-Posterior, Combined Anterior-Posterior, And Combined Lateral-Posterior Approaches In Adult Spinal Deformities: A Narrative Review, Garrison P. Bentz, Christopher Diaz, Nicholas A. Pollatta, Mark J. Lambrechts

2020-Current year OA Pubs

No abstract provided.

Can The Ability To Infer Relevance Account For Dimensional Psychoticism? An Exploration Of A Representative Community Sample, Adam Schwarz, Frank D. Baughman, Evan Dauer, Ruby Koyllor Alarcon Gleeson, Jennifer Haywood, Bo Huei Huang, Karen H. Heslop, Barbara A. Mullan, Susan L. Rossell, Nicolas H. Hart, Darren Haywood

Research outputs 2022 to 2026

Purpose: Psychoticism is a common feature of psychological disorders. Deficits in perceptual inference have been associated with the development of psychotic symptoms. Traditional testing of perceptual inference has had low ecological validity, limiting the ability to infer functional relationships. The aims of this study were to investigate the relationship between the capacity to infer relevance in dynamic environments and dimensional psychoticism, as well as to investigate the association between inferring relevance and dimensional psychoticism. Method: Four hundred participants, representative of the general population of the USA, completed an online questionnaire consisting of the Brief Symptom Inventory, as well as demographic …

Compulsory Service-Learning Programs In Secondary Schools: The Lasting Impact Beyond The Classroom, Linda Cranley, Gregory Hine

Research outputs 2022 to 2026

The purpose of this research project was to investigate the extent to which a compulsory service-learning program (SLP) offered in one secondary school inspired students to extend their service to the community beyond their schooling and make the connection between real-life social inequities and the SLP. The project involved interviewing current staff and past students from a co-educational Catholic secondary school in Perth, Western Australia, about their experiences of participating in the school’s SLP. Staff and student participants highlighted key elements of the SLP, including the types of community involvement encouraged within the program, and provided insight into how the …

Efficacy And Safety Of Dostarlimab In Combination With Chemotherapy In Patients With Dmmr/Msi-H Primary Advanced Or Recurrent Endometrial Cancer In A Phase 3, Randomized, Placebo-Controlled Trial (Engot-En6-Nsgo/Gog-3031/Ruby), Matthew A Powell, Carolyn Mccourt, Et Al.

2020-Current year OA Pubs

OBJECTIVES: Part 1 of the RUBY trial (NCT03981796) demonstrated improved survival in patients with primary advanced or recurrent endometrial cancer (EC) treated with dostarlimab plus carboplatin-paclitaxel versus placebo plus carboplatin-paclitaxel. Here, we examine additional efficacy and safety data from patients with mismatch repair deficient/microsatellite instability-high (dMMR/MSI-H) EC in the RUBY trial.

METHODS: Patients were randomized 1:1 to dostarlimab 500 mg or placebo plus carboplatin-paclitaxel every 3 weeks for 6 cycles followed by dostarlimab or placebo every 6 weeks for up to 3 years. In the dMMR/MSI-H population of RUBY Part 1, analysis of progression-free survival by investigator assessment compared with …

Early Changes In Spatiotemporal Dynamics Of Remapped Circuits And Global Networks Predict Functional Recovery After Stroke In Mice, Ryan M Bowen, Jake Lee, Brendon Wang, Keith R Lohse, Hanyang Miao, Jonah A Padawer-Curry, Asher J Albertson, Eric C Landsness, Adam Q Bauer, Jin-Moo Lee

2020-Current year OA Pubs

SIGNIFICANCE: Stroke is the leading cause of chronic disability in the United States. How stroke size affects post-stroke repair and recovery is poorly understood.

AIM: We aim to investigate the effects of stroke size on early repair patterns and determine how early changes in neuronal circuits and networks predict functional outcomes after stroke.

APPROACH: We used wide-field optical imaging, photothrombosis, and the cylinder-rearing assay to examine changes in neuronal circuit and network activity in the context of functional recovery after stroke.

RESULTS: Larger strokes ablating

CONCLUSIONS: These results suggest that diffuse patterns of remapping, and desynchronization and hyperconnectivity of cortical …

Piezo1-Mediated Calcium Influx Transiently Alters Nuclear Mechanical Properties Via Actin Remodeling In Chondrocytes, Jaquelin M Garcia-Castorena, Rosa Riester, Miranda Gamino-Ornelas, Nikitha Ada, Farshid Guilak, Marina Danalache

2020-Current year OA Pubs

Mechanosensation allows cells to generate intracellular signals in response to mechanical cues from their environment. Previous research has demonstrated that mechanical stress can alter the mechanical properties of the nucleus, affecting gene transcription, chromatin methylation, and nuclear mechanoprotection during mechanical loading. PIEZO1, a mechanically gated Ca

Treatment Of Hereditary Hemorrhagic Telangiectasias With Sclerotherapy: A Case Series, Jenny Ji, Andrew M Peterson, Jay F Piccirillo

2020-Current year OA Pubs

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is characterized by abnormal blood vessel formation. One treatment for HHT-related arteriovenous malformations (AVMs) is sclerotherapy, which collapses the blood vessels by irritating the endothelial lining.

METHODS: This case series describes two HHT patients undergoing in-office sodium tetradecyl sulfate sclerotherapy for non-nasal telangiectasias and AVMs. The first patient had AVMs on the red lip while the second patient had an AVM and several telangiectasias on the tongue.

RESULTS: Both patients' AVMs required only one treatment visit and were no longer noticeable within a week. Neither of the patients had any side effects from treatment.

CONCLUSION: …

Expert Consensus On The Diagnosis And Management Of Digoxin Toxicity, Jason B Hack, Sue Wingate, Ron Zolty, Michael W Rich, Paul J Hauptman

2020-Current year OA Pubs

While there has been a decline in the use of digoxin in patients with heart failure and atrial fibrillation, acute and chronic digoxin toxicity remains a significant clinical problem. Digoxin's narrow therapeutic window and nonspecific signs and symptoms of toxicity create clinical challenges and uncertainty around the diagnostic criteria of toxicity and responsive treatment choices for the bedside clinician. A systematic review of published literature on digoxin toxicity (34,587 publications over 6 decades, with 114 meeting inclusion criteria) was performed to develop 33 consensus statements on diagnostic and therapeutic approaches which were then evaluated through a modified Delphi process involving …

Metadata Makes Big Data Useful For Ai/Ml-Ready, Equitable Pandemic Response, Sudarshan Anand, Miae Lee, Seshadri S. Vasan

Research outputs 2022 to 2026

While genomic data helps understand the pathogen and its evolution, patient metadata is critical to get the host side of the picture, so that the pandemic response is effective and equitable.

Clinical Relevance: We highlight the importance of patient stratification and metadata to help develop diagnostics, therapies and vaccines as the pathogen (virus) evolves.

Surviving And Thriving In Educational Leadership: Challenges And Coping Strategies Of High School Principals In Nepal, Shankar Dhakal

Research outputs 2022 to 2026

School leadership in Nepal is shaped by sociocultural, institutional, and resource-related challenges. This study fills a gap in the literature by examining leadership dynamics within this unique and diverse educational context. Guided by resilient leadership theory as a referent, this qualitative case study, grounded in a constructivist paradigm, involved semi-structured interviews with public high school principals to investigate their workplace challenges and coping strategies. The findings reveal challenges including sociocultural and gender biases, teachers’ resistance to modern pedagogical practices, and resource limitations. They navigate these challenges through strategies of humility, intrinsic motivation, and collaborative partnerships. The findings highlight the need …

Acquired Ductopenia: An Insight Into Imaging Findings, Rachita Khot, Nathan R Shelman, Daniel R Ludwig, Rashmi T Nair, Mark A Anderson, Sudhakar K Venkatesh, Raj Mohan Paspulati, Rex A Parker, Christine O Menias

2020-Current year OA Pubs

Hepatic ductopenia is a pathologic diagnosis characterized by a decrease in the number of intrahepatic bile ducts as a consequence of various underlying etiologies. Some etiologies, such as primary sclerosing cholangitis, primary biliary cholangitis, and ischemic cholangitis, often have distinctive imaging findings. In contrast, other causes such as chronic rejection following liver transplantation, drug-induced biliary injury, infection, malignancy such as lymphoma, and graft-versus-host disease may only have ancillary or non-specific imaging findings. Thus, diagnosing ductopenia in conditions with nonspecific imaging findings requires a multidimensional approach, including clinical evaluation, serological testing, imaging, and liver histology to identify the underlying cause. These …

Validating A Novel Measure For Assessing Patient Openness And Concerns About Using Artificial Intelligence In Healthcare, Bryan A Sisk, Alison L Antes, Sunny C Lin, Paige Nong, James M Dubois

2020-Current year OA Pubs

OBJECTIVES: Patient engagement is critical for the effective development and use of artificial intelligence (AI)-enabled tools in learning health systems (LHSs). We adapted a previously validated measure from pediatrics to assess adults' openness and concerns about the use of AI in their healthcare.

STUDY DESIGN: Cross-sectional survey.

METHODS: We adapted the 33-item "Attitudes toward Artificial Intelligence in Healthcare for Parents" measure for administration to adults in the general US population (AAIH-A), recruiting participants through Amazon's Mechanical Turk (MTurk) crowdsourcing platform. AAIH-A assesses openness to AI-driven technologies and includes 7 subscales assessing participants' openness and concerns about these technologies. The openness …

Head-To-Head Preclinical Treatment Design Prioritizes Promising Therapies For Neurofibromatosis Type 1 Optic Glioma Clinical Translation, Talia Eligator, Jit Chatterjee, Shintaro Yamada, Anthony Kirchner, Hareesh B Nair, Jason R Fangusaro, David H Gutmann

2020-Current year OA Pubs

BACKGROUND: Authenticated preclinical brain tumor models provide unprecedented opportunities to evaluate next-generation treatments. However, some therapies with robust anti-tumor activity in mice fail in human trials, highlighting the need to better prioritize candidates for clinical translation. Herein, we implemented a head-to-head preclinical strategy using a well-characterized murine model of NF1-optic pathway glioma (

METHODS:

RESULTS: While all agents restored normal tissue architecture, reduced ON proliferation, and decreased TME soluble mediator and tumor cell marker RNA expression, only lamotrigine and mirdametinib also reduced ON volume. Everolimus, lamotrigine, and HBS-101 restored RNFL thickness to wild-type levels, whereas carboplatin showed a trend towards …

Improving Outcomes Of Single-Sided Deaf Cochlear Implant Users By Reducing Interaural Frequency And Loudness Mismatches Through Device Programming, Laura K Holden, Rosalie M Uchanski, Noël Y Dwyer, Ruth M Reeder, Timothy A Holden, Jill B Firszt

2020-Current year OA Pubs

The study aimed to improve outcomes in Nucleus cochlear implant (CI) recipients with single-sided deafness (SSD) by reducing interaural frequency and loudness mismatches through device programming. In Experiment 1a, a modified frequency allocation table (FAT) was created to better match the tonotopicity of the contralateral ear and reduce interaural frequency mismatch. Twenty experienced SSD-CI users completed localization and speech recognition tests with their everyday FAT. Tests were repeated after 6 weeks' use of the modified FAT. Participants compared both FATs for 2 weeks before being tested again with each. For 10 newly implanted SSD-CI recipients (Experiment 1b), Group A was …

Carpal Tunnel Syndrome And Occupational Co-Exposure To Biomechanical Factors And Neurotoxic Chemicals Using Job-Exposure Matrices And Self-Reported Exposure: Findings From The Constances Cohort, Julie Bodin, Clémence Rapicault, Alexis Descatha, Marc Fadel, Fabien Gilbert, Bradley Evanoff, Nathalie Bonvallot, Marcel Goldberg, Marie Zins, Yves Roquelaure

2020-Current year OA Pubs

OBJECTIVE: To study the association between occupational co-exposure to biomechanical risk factors, potentially neurotoxic chemicals and carpal tunnel syndrome (CTS) in a large cohort of French workers, using two methods to estimate chemical exposure: job-exposure matrices (JEM) and self-reported exposure.

METHODS: A randomly selected sample of adults were included between 2012 and 2018 in the French cohort CONSTANCES. Self-reported CTS was assessed using the first self-administered follow-up questionnaire, sent out approximately one year after baseline. Occupational exposure to biomechanical risk factors was assessed using self-administered questionnaire completed at inclusion. Lifetime occupational exposure to chemicals was assessed using two different methods: …