Correction to: Emergency endovascular treatments for delayed hemorrhage after pancreaticobiliary surgery: indications, outcomes, and follow‑up of a retrospective cohort

n/

BeWeB: un giovane progetto che compie vent'anni

Il portale BeWeB rappresenta il racconto delle comunità di eredità che esprimono il valore identitario del patrimonio culturale nelle sue diverse tipologie, materiale e immateriale, come riflesso ed espressione dei loro valori, delle conoscenze e tradizioni, in continua evoluzione. I beni ecclesiastici, oltre ad avere un'accezione culturale, storica, artistica, bibliografica, archivistica e patrimoniale, rappresentano anche uno dei luoghi in cui si compie la missione della chiesa. Le diocesi e le parrocchie, nel catalogare i propri beni attraverso un progetto locale di carattere nazionale, hanno investito risorse culturali, umane ed economiche motivate da una prospettiva principalmente legata alla conoscenza, certamente patrimoniale e di tutela, svolgendo l'attività con crescente consapevolezza. Ultimamente questa consapevolezza del dono da custodire e da trasmettere, da usare oggi, è cresciuta, e ormai in quei beni le comunità identificano i motivi della propria identità, un valore di vita e di annuncio

On the orbital and internal evolution of cluster galaxies

Galaxies inhabiting a cluster environment experience significant evolution in their orbital motions throughout time; this is accompanied by changes in the anisotropy parameter, measuring the relative importance of radial and tangential motions for a given class of objects. Along with orbital changes, galaxies in clusters are well known to undergo severe alteration in their hot/cold gas content and star formation properties. Understanding the link between the changes in the internal properties of galaxies and their orbital motion is of crucial importance in the study of galaxy evolution, as it could unveil the primary mechanism responsible for its environmental dependence. Do the changes in the internal properties happen in parallel with those in the orbital motion? Or are the orbital features at the time of infall what determines the fate of the member galaxies? Alternatively: are the properties of galaxies at a given time related to the coeval orbital anisotropy or are they better related to the anisotropy at infall? In order to answer these questions, we studied the orbital evolution of different galaxy populations in the semi-analytic models of Guo et al. (2011) applied on to the Millennium Simulation. For each class of objects, characterised by different internal properties (such as age, star formation rate and colour), we studied the anisotropy profile at redshift zero and its evolution by tracing the progenitors back in time. We conclude that the orbital properties at infall strongly influence the subsequent evolution of the internal features of galaxies and that the overall anisotropy of the galaxy population tends to increase with time.Comment: 11 pages, 9 figures. Accepted for publication in MNRA

Gross Motor Function in Pediatric Onset TUBB4A-Related Leukodystrophy: GMFM-88 Performance and Validation of GMFC-MLD in TUBB4A

TUBB4A pathogenic variants are associated with a spectrum of neurologic impairments including movement disorders and leukodystrophy. With the development of targeted therapies, there is an urgent unmet need for validated tools to measure mobility impairment. Our aim is to explore gross motor function in a pediatric-onset TUBB4A-related leukodystrophy cohort with existing gross motor outcome tools. Gross Motor Function Measure-88 (GMFM-88), Gross Motor Function Classification System (GMFCS-ER), and Gross Motor Function Classification-Metachromatic Leukodystrophy (GMFC-MLD) were selected through face validity. Subjects with a confirmed clinical and molecular diagnosis of TUBB4A-related leukodystrophy were enrolled. Participants' sex, age, genotype, and age at disease onset were collected, together with GMFM-88 and concurrent GMFCS-ER and GMFC-MLD. Performances on each measure were compared. GMFM-88 floor effect was defined as total score below 20%. A total of 35 subjects participated. Median performance by GMFM-88 was 16.24% (range 0-97.31), with 42.9% (n = 15) of individuals performing above the floor. GMFM-88 Dimension A (Lying and Rolling) was the best-performing dimension in the GMFM-88 (n = 29 above the floor). All levels of the Classification Scales were represented, with the exception of the GMFC-MLD level 0. Evaluation by GMFM-88 was strongly correlated with the Classification Scales (Spearman correlations: GMFCS-ER:GMFM-88 r = 0.90; GMFC-MLD:GMFM-88 r = 0.88; GMFCS-ER:GMFC-MLD: r = 0.92). Despite overall observation of a floor effect, the GMFM-88 is able to accurately capture the performance of individuals with attenuated phenotypes. GMFM-88 Dimension A shows no floor effect. GMFC-MLD shows a strong correlation with GMFCS-ER and GMFM-88, supporting its use as an age-independent functional score in TUBB4A-related leukodystrophy

Newborn screening in metachromatic leukodystrophy – European consensus-based recommendations on clinical management

Introduction: Metachromatic leukodystrophy (MLD) is a rare autosomal recessive lysosomal storage disorder resulting from arylsulfatase A enzyme deficiency, leading to toxic sulfatide accumulation. As a result affected individuals exhibit progressive neurodegeneration. Treatments such as hematopoietic stem cell transplantation (HSCT) and gene therapy are effective when administered pre-symptomatically. Newborn screening (NBS) for MLD has recently been shown to be technically feasible and is indicated because of available treatment options. However, there is a lack of guidance on how to monitor and manage identified cases. This study aims to establish consensus among international experts in MLD and patient advocates on clinical management for NBS-identified MLD cases. Methods: A real-time Delphi procedure using eDELPHI software with 22 experts in MLD was performed. Questions, based on a literature review and workshops, were answered during a seven-week period. Three levels of consensus were defined: A) 100%, B) 75–99%, and C) 50–74% or >75% but >25% neutral votes. Recommendations were categorized by agreement level, from strongly recommended to suggested. Patient advocates participated in discussions and were involved in the final consensus. Results: The study presents 57 statements guiding clinical management of NBS-identified MLD patients. Key recommendations include timely communication by MLD experts with identified families, treating early-onset MLD with gene therapy and late-onset MLD with HSCT, as well as pre-treatment monitoring schemes. Specific knowledge gaps were identified, urging prioritized research for future evidence-based guidelines. Discussion: Consensus-based recommendations for NBS in MLD will enhance harmonized management and facilitate integration in national screening programs. Structured data collection and monitoring of screening programs are crucial for evidence generation and future guideline development. Involving patient representatives in the development of recommendations seems essential for NBS programs

BPSDiary study protocol: a multi-center randomized controlled trial to compare the efficacy of a BPSD diary vs. standard care in reducing caregiver's burden

Behavioral and Psychological Symptoms of Dementia (BPSD) are a heterogeneous set of psychological and behavioral abnormalities seen in persons with dementia (PwD), significantly impacting their quality of life and that of their caregivers. Current assessment tools, such as the Neuropsychiatric Inventory (NPI), are limited by recall bias and lack of direct observation. This study aims to overcome this limitation by making caregiver reports more objective through the use of a novel instrument, referred to as the BPSDiary. This randomized controlled trial will involve 300 caregiver-PwD dyads. The objective is to evaluate whether the use of the BPSDiary could significantly reduce caregiver burden, assessed using the Zarit Burden Interview (ZBI), compared to usual care. The study will include adult PwD, caregivers living with or close to the patient, and BPSD related to the HIDA (hyperactivity, impulsivity, irritability, disinhibition, aggression, agitation) domain. Caregivers randomized to the intervention arm will use the BPSDiary to record specific BPSD, including insomnia, agitation/anxiety, aggression, purposeless motor behavior, and delusions/hallucinations, registering time of onset, severity, and potential triggers. The primary outcome will be the change in ZBI scores at 3 months, with secondary outcomes including changes in NPI scores, olanzapine equivalents, NPI-distress scores related to specific BPSD domains, and caregiver and physician satisfaction. The study will be conducted in 9 Italian centers, representing diverse geographic and sociocultural contexts. While potential limitations include the relatively short observation period and the focus on specific BPSD disturbances, the BPSDiary could provide physicians with objective data to tailor appropriate non-pharmacological and pharmacological interventions. Additionally, it may empower caregivers by encouraging reflection on BPSD triggers, with the potential to improve the quality of life for both PwD and their caregivers.Trial registryNCT05977855

The Remapping of Time by Active Tool-Use

Multiple, action-based space representations are each based on the extent to which action is possible toward a specific sector of space, such as near/reachable and far/unreachable. Studies on tool-use revealed how the boundaries between these representations are dynamic. Space is not only multidimensional and dynamic, but it is also known for interacting with other dimensions of magnitude, such as time. However, whether time operates on similar action-driven multiple representations and whether it can be modulated by tool-use is yet unknown. To address these issues, healthy participants performed a time bisection task in two spatial positions (near and far space) before and after an active tool-use training, which consisted of performing goal-directed actions holding a tool with their right hand (Experiment 1). Before training, perceived stimuli duration was influenced by their spatial position defined by action. Hence, a dissociation emerged between near/reachable and far/unreachable space. Strikingly, this dissociation disappeared after the active tool-use training since temporal stimuli were now perceived as nearer. The remapping was not found when a passive tool-training was executed (Experiment 2) or when the active tool-training was performed with participants’ left hand (Experiment 3). Moreover, no time remapping was observed following an equivalent active hand-training but without a tool (Experiment 4). Taken together, our findings reveal that time processing is based on action-driven multiple representations. The dynamic nature of these representations is demonstrated by the remapping of time, which is action- and effector-dependent