University of Palermo

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    Unfolding dermatological spectrum of Still’s disease: a cohort study from the International AIDA Network Still’s Disease Registry

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    Objectives To investigate cutaneous manifestations in Still's disease patients, evaluating any correlation with ethnic origin, age at disease onset, disease patterns, occurrence of macrophage activation syndrome (MAS) and systemic activity scores.Methods Data were retrospectively drawn from the International AutoInflammatory Disease Alliance (AIDA) Network Registry dedicated to Still's disease.Results A total of 518 patients (41.3% males) were enrolled. Salmon-coloured evanescent skin rash (n=304, 63.9%), macules (n=40, 7.7%), urticarial eruptions (n=31, 5.9%), erythema (n=27, 5.2%) and persistent pruritic papules and plaques (PPPP) (n=25, 4.8%) accounted for the most frequent skin manifestations observed in Still's disease. Overall, atypical skin rash were described in 110 (21.2%) patients. Salmon-coloured evanescent skin rash and pruritus were more common among patients aged < 16years compared with patients aged 16-60 (P=0.002 and P=0.008, respectively). Pruritus was significantly more frequent among White than among Arab patients (P=0.008) and in polycyclic vs monocyclic course (P=0.049). Hispanics showed a significantly higher rate of atypical skin manifestations compared with Arabs (P=0.036) and White (P=0.036). Also, macules were more frequent among Hispanics than White (P=0.027), while PPPP was more frequent among Hispanics than Arabs (P=0.023) and White (P=0.002). Salmon-coloured evanescent skin rash was significantly more frequent among patients with a systemic activity score >= 7 (P < 0.001).Conclusion The present study enhances dermatologists' awareness of the diverse cutaneous lesions that may represent heterogeneous manifestations of Still's disease, shedding new light on the difference related to the age at disease onset, the patients' ethnic origin and the severity of the disease

    Assessing Energy Poverty in Renewable Energy Communities (CER): Indicators between Adaptability and Restriction in the Italian regulations

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    The energy transition of the European Union (EU) to renewable sources implies the formation of a new energy-scape, which will cause radical changes in the urban and rural landscape. Instead, the transformations produced in the social system are likely to be less manifest if the phenomenon of energy poverty is not properly addressed. In this context, the widespread localization of photovoltaic electricity production systems in the territory through Renewable Energy Communities (RECs) contributes to the reduction of climate-changing emissions and promotion of energy self-sufficiency, and fosters social cohesion. The RECs are also seen by the EU as a tool to tackle energy poverty, according to the “leaving no one behind” principle. In fact, some EU, national or local regulations suggest, or establish as a constraint, the inclusion of energy-poor households in the RECs. However, defining energy poverty through socio-economic indicators as well as identifying households in such conditions are still challenging. This study, therefore, proposes a critical review of the main indicators used to measure energy poverty, with the aim of testing their applicability in the context of Italian REC regulations. The results of our study show that the economic and social indicators constructed to be applied on a national scale are not as effective in measuring fuel poverty at the micro-territory or building level, impacting the effectiveness of targeted social policy actions. The main critical issue lies in the lack of detailed data (e.g. energy consumption and expenditure by household type and micro area) which are essential for selecting vulnerable households to be involved in RECs. Therefore, our study emphasizes the demand to develop new indicators or adapt current ones to meet the legislative and regulatory requirements of RECs in providing a more equitable social-energy-scape

    The Risk of Developing Ocular Involvement Among Behçet’s Disease Patients Presenting with Mucocutaneous Involvement at Disease Onset: Data from the International AIDA Network Behçet’s Disease Registry

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    Introduction Behcet's disease (BD) frequently arises with exclusively mucocutaneous involvement, but some patients will develop major organ involvement, including ocular inflammation. This study aims to assess the patients' demographic and clinical characteristics that may be associated with the development of ocular involvement in patients with BD with exclusively mucocutaneous involvement in the early stages.Methods Patients' data were collected in the International AutoInflammatory Disease Alliance (AIDA) Network registry dedicated to BD.Results A total of 328 patients with BD were enrolled, 36 (11%) of whom developed ocular involvement over time. The following variables were significantly associated with the development of ocular inflammation at binary logistic regression: positive family history for BD (OR 4.32, 95% CI 1.16-14.72, p = 0.02), Arab ethnicity (OR 3.6, 95% CI 1.3-9.9, p = 0.01), presence of major oral aphthous ulceration (OR 3.26, 95% CI 1.18-8.99, p = 0.02), pseudofolliculitis plus erythema nodosum (OR 4.58, 95% CI 1.33-15.7, p = 0.016); conversely, white patients/patients of European descent (OR 0.33, 95% CI 0.12-0.91, p = 0.03) and the presence of a low number (one to two) ulcers at genital aphthous flares (OR 0.002, 95% CI similar to 0-0.07, p = 0.0006) were protective against the occurrence of ocular inflammatory manifestations. Genital aphthosis with more than five concurrent ulcers was associated with eye involvement in Arab patients (OR 209.2, 95% CI 5.8-7497, p = 0.003).Conclusions Major oral aphthosis, genital aphthous attacks with more than five concurrent ulcers, erythema nodosum coexisting with pseudofolliculitis, a positive family history of BD, and Arab ethnicity are associated with a higher risk of ocular involvement when BD arises with the sole mucocutaneous involvement

    Prefazione alla monografia: Criptoattività nell’economia digitale. Tecnologia, diritto ed implicazioni aziendali

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    Le criptoattività sfidano i framework contabili, giuridici e fiscali costruiti per gli asset tradizionali. Il libro propone un’architettura interpretativa che intreccia la tecnologia, il diritto e le implicazioni aziendali, dalla Distributed Ledger Technology al Regolamento MiCA, dalla rappresentazione contabile secondo i principi internazionali e nazionali al conseguente regime fiscale. L’analisi procede dalle fondamenta tecnologiche alle questioni operative che i professionisti incontrano nella classificazione degli asset, nelle metodologie di valutazione, nel trattamento tributario e negli obblighi dichiarativi. Il metaverso emerge come frontiera dove blockchain e realtà virtuale generano nuovi paradigmi economici, ponendo interrogativi sulla governance di ecosistemi digitali che sfidano la prassi professionale consolidata

    Dynamic driving pressure and clinical outcomes in mechanically ventilated patients with acute brain injury: a secondary analysis of the VENTIBRAIN study

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    Purpose: To investigate the association between dynamic driving pressure (ΔPdyn) and mortality in mechanically ventilated patients with acute brain injury (ABI), and to evaluate whether neurological and pulmonary injury severity modify this relationship. Methods: This prespecified secondary analysis of the VENTIBRAIN prospective study (NCT04459884) included mechanically ventilated adult patients with ABI (traumatic brain injury, subarachnoid hemorrhage, intracranial hemorrhage, or ischemic stroke). ΔPdyn was calculated daily over the first 14 days as peak inspiratory pressure minus positive end-expiratory pressure (PEEP). Bayesian joint models evaluated the time-varying association between ΔPdyn and mortality at ICU discharge, hospital discharge, and 6 months. Secondary analyses evaluated effect modification by baseline Glasgow Coma Scale (GCS), PaO2/FiO2, and ABI subtype. Results: Among 1,555 patients (median age 59 years, 34.5% female), higher time-varying ΔPdyn was associated with increased ICU mortality (hazard ratio [HR], 1.057 per daily 1cmH2O; 95% credible interval, 1.037-1.078; posterior probability of HR > 1, 99.9%). Findings were consistent across all ABI subtypes and were similar for static ΔP (plateau pressure minus PEEP). The association was strongest in patients with severe ABI (GCS ≤ 8) and severe hypoxemia (PaO2/FiO2 ≤ 100). Results were robust across all outcome timepoints and multiple sensitivity analyses. Conclusion: Higher time-varying ΔPdyn was associated with increased mortality in this cohort of patients with ABI. Neurological injury severity independently modified the harm from ΔPdyn with a magnitude comparable to severe hypoxemia. Measurement of ΔPdyn may aid risk stratification and ventilation strategies in ABI; future trials should evaluate the effect of reducing ΔPdyn in this population

    Enhancing frequency management in renewable-powered smart grids with metaheuristic optimization of cascaded controller

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    The growing integration of Renewable Energy Sources (RES) needs advanced approaches to ensure power grid stability and reliability. This study improves frequency regulation by using metaheuristic optimization to fine-tune a cascaded Proportional Integral Derivative with Derivative Filter and Proportional Integral (PIDn-PI) controller, addressing issues such as load variability, communication delays, incorporation of RES, Electric Vehicles (EVs), Redox Flow Batteries (RFBs) and thermal power. Simulations are conducted across various load scenarios featuring fixed, variable and realistic disturbances. Sine Cosine Algorithm (SCA), Grey Wolf Optimization (GWO), Particle Swarm Optimization-Grey Wolf Optimization (PSO-GWO), Salp Swarm Algorithm (SSA), Tunicate Swarm Algorithm (TSA), Particle Swarm Optimization (PSO) and Chaos Game Optimization (CGO) are used for this purpose. SCA consistently performs best and ensures the fastest stabilization with minimal frequency fluctuations, overshoot and undershoot. GWO, TSA, and PSO-GWO work well but take longer to reach steady-state. PSO balances performance and computational time, but performs worse than SCA, whereas SSA and CGO have the worst control performance. The performance of SCA: PIDn-PI demonstrates that when there is 1 % load change in both areas, the frequency response in zone 1 and zone 2 settles in 1.64 s and 1.13 s, respectively, whereas it settles in 1.22 s and 1.60 s in area 1 and area 2, respectively, when the load changes by 5 %. Moreover, the robustness of SCA: PIDn-PI is tested under various RES combinations and penetration levels, Denial-of-Service (DoS) cyberattacks, and sensitivity analysis is carried out

    Study of molecular mechanism regulated by CD271 in colon cancer progression

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    La progressione metastatica rappresenta la principale causa di morte nel carcinoma del colon-retto (CRC) e i meccanismi attraverso i quali le cellule tumorali disseminanti sopravvivono, rimangono dormienti e successivamente si riattivano sono ancora poco compresi. Al centro di questo processo vi sono le cellule staminali del carcinoma del colon-retto (CR-CSC), una piccola sottopopolazione capace di passare dinamicamente tra stati di dormienza e proliferazione, guidando la recidiva tumorale e la metastatizzazione. In questo studio l’asse di segnalazione NGF/NGFR è stato identificato come un regolatore chiave della dormienza, dell’invasività e della riattivazione delle CR-CSC. L’analisi dei dati TCGA e di tessuti derivati da pazienti ha rivelato che l’espressione di NGF, NGFR e NTRK1 è ridotta nei tumori primitivi ma risulta aumentata nelle metastasi rispetto alla mucosa sana, ed inoltre è arricchita nel sottotipo aggressivo CMS4, correlando con una prognosi sfavorevole. Studi funzionali hanno dimostrato che un’elevata espressione di NGFR identifica una sottopopolazione di CR-CSC dormienti e a basso rate proliferativo, caratterizzata da aumento delle caratteristiche staminali, dell'invasività associata all’EMT e maggiore rilascio di lattato. L' over-espressione di NGFR induce un programma di dormienza mediato da NR2F1, mentre la stimolazione con NGF inverte questo stato, promuovendo il rientro nel ciclo cellulare, la proliferazione, l’attivazione della via Wnt/β-catenin e l’espansione della popolazione cellulare altamente tumorigenica CD44v6+/Ki-67+. Nel complesso, questi risultati forniscono un modello integrato in cui la via di segnalazione NGF/NGFR coordina dormienza, staminalità, metabolismo e interazioni con il microambiente tumorale per guidare il processo di metastatizzazione del CRC. Questo lavoro non solo approfondisce la comprensione della biologia delle CR-CSC, ma identifica anche l'asse NGF/NGFR come un promettente bersaglio terapeutico per prevenire la recidiva metastatica e migliorare gli esiti clinici dei pazienti.Metastatic progression is the leading cause of death in colorectal cancer (CRC), and the mechanisms by which disseminated tumor cells survive, remain dormant, and later reactivate remain poorly understood. Central to this process are colorectal cancer stem cells (CR-CSCs), a small subpopulation capable of dynamically transitioning between dormancy and proliferation, driving tumor recurrence and metastasis. Here, we identify the NGF/NGFR signaling axis as a key regulator of CR-CSC dormancy, invasiveness, and reawakening. Analysis of TCGA data and patient-derived tissues revealed that NGF, NGFR, and NTRK1 expression are reduced in primary tumors but elevated in metastases, compared to healthy mucosa, and enriched in the aggressive CMS4 subtype, correlating with poor patient survival. Functional studies demonstrated that high NGFR expression identifies a dormant, slow-cycling CR-CSC subpopulation characterized by enhanced stemness, EMT-associated invasiveness, and elevated lactate release. NGFR overexpression induced an NR2F1-driven dormancy program, whereas stimulation with NGF reversed this state, promoting cell cycle re-entry, proliferation, Wnt/β-catenin activation, andthe expansion of highly tumorigenic CD44v6+/Ki-67+ cells. Together, these findings provide a comprehensive model in which NGF/NGFR signaling coordinates dormancy, stemness, metabolism, and tumor-microenvironment interactions to drive CRC metastasis. This work not only advances our understanding of CR-CSC biology but also highlights NGF/NGFR as a promising therapeutic target to prevent metastatic relapse and improve patient outcomes

    Universally Wheeler Languages

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    The notion of Wheeler languages is rooted in the Burrows- Wheeler transform (BWT), one of the most central concepts in data com- pression and indexing. The BWT has been generalized to finite automata, the so-called Wheeler automata, by Gagie, Manzini, and Sirén. Wheeler languages have subsequently been defined as the class of regular lan- guages for which there exists a Wheeler automaton accepting them. Besides their advantages in data indexing, these Wheeler languages also satisfy many interesting properties from a language theoretic point of view. A characteristic yet unsatisfying feature of Wheeler languages how- ever is that their definition depends on a fixed order of the alphabet. In this paper we introduce the Universally Wheeler languages UW, i.e., the regular languages that are Wheeler with respect to all orders of a given alphabet. Our first main contribution is to relate UW to some very well known regular language classes. We first show that the Strictly Locally Testable languages are strictly included in UW. After noticing that UW is not closed under taking the complement, we prove that the class of languages for which both the language and its complement are in UW exactly coincides with those languages that are Definite or Reverse Def- inite. Secondly, we prove that deciding if a regular language given by a DFA is in UW can be done in quadratic time. We also show that this is optimal unless the Strong Exponential Time Hypothesis fails

    L’esclusione del beneficium excussionis in assenza di opposizione dei soci al decreto ingiuntivo

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    Con la pronuncia in commento, la Corte di cassazione esclude l’operatività del beneficio di preventiva escussione quando il decreto ingiuntivo emesso contestualmente ed incondizionatamente nei confronti di una società in nome collettivo e dei suoi soci venga opposto solo dalla società e non anche da questi ultimi, individuando nella formulazione testuale del titolo una peculiare fonte di responsabilità diretta dei soci medesimi. La nota intende ricostruire criticamente – senza pretese di esaustività – i principali profili di diritto sostanziale e processuale incisi dal ragionamento logico-giuridico seguito dalla Suprema Corte

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