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ObjectiveDry eye disease (DED) is a condition associated with a myriad of systemic disorders. According to recent preliminary data, axial spondylarthritis (axial-SpA) could represent a new entity associated with DED. Therefore, this study aimed to assess DED in patients with axial SpA by performing quantitative and qualitative specific tests to investigate the potential association between DED and ocular surface damage in patients with axial-SpA and to identify potential variables associated with DED.MethodsA total of 71 patients with axial-SpA who fulfilled the Assessment of SpondyloArthritis International Society (ASAS) classification criteria and 19 healthy controls were enrolled in this study. Both the patients and the controls underwent a complete ocular assessment aimed at evaluating the tear film and ocular surface, which included the Schirmer test, tear break-up time (TBUT), fluorescein staining, and lissamine green staining. The Ocular Surface Disease Index (OSDI) questionnaire was administered to all patients.ResultsDED symptoms were reported in 46 (64.8%) patients and three (15.8%) healthy controls (p = 0.0004). The odds ratio for receiving a diagnosis of axial-SpA based on the presence of dry-eye-related symptoms was 9.2 (95% C.I. 2.72–42.52, p = 0.001). The Schirmer test values of < 6 mm/5 min were observed in 31 (43.7%) patients with axial-SpA and two (10.5%) healthy controls (p = 0.013); a TBUT of

Abstract Introduction This study aims to characterize ocular manifestations of juvenile Behçet’s disease (jBD). Methods This was a registry-based observational prospective study. All subjects with jBD from the Autoinflammatory Diseases Alliance (AIDA) Network BD Registry showing ocular manifestations before 18 years were enrolled. Results We included 27 of 1000 subjects enrolled in the registry (66.7% male patients, 45 affected eyes). The median (interquartile range [IQR]) age at ocular involvement was 14.2 (4.7) years. Uveitis affected 91.1% of eyes (anterior 11.1%, posterior 40.0%, panuveitis 40.0%), retinal vasculitis 37.8% and other manifestations 19.8%. Later onset (p = 0.01) and male predominance (p = 0.04) characterized posterior involvement. Ocular complications occurred in 51.1% of eyes. Patients with complications had earlier onset (p

IntroductionNon-infectious inflammatory ocular diseases pose significant challenges in diagnosis and management, often requiring systemic immunosuppressive therapy. Since Janus kinase (JAK) inhibitors may represent a novel therapeutic option for these disorders, the present study aimed to expand current knowledge about their efficacy and safety in patients with these conditions.MethodsThis prospective cohort study included 12 adult patients from the international AutoInflammatory Disease Alliance (AIDA) Network registries dedicated to non-infectious ocular inflammatory conditions. We assessed ocular flares, visual acuity, disease course, and complications before and after initiating JAK inhibitor therapy.ResultsOcular inflammation was related to a systemic disease in 8 (66.7%) patients as follows: spondyloarthritis (n = 3), peripheral psoriatic arthritis (n = 1), rheumatoid arthritis (n = 1), antinuclear antibodies (ANA) positive juvenile idiopathic arthritis (n = 1), Behçet’s syndrome (n = 1), Vogt-Koyanagi-Harada syndrome (n = 1). In total, 4 patients received baricitinib, 1 patient received tofacitinib, and 7 patients underwent upadacitinib treatment. The overall average duration of JAK inhibitors treatment was 8.6 ± 5.5 months (ranging from 3 to 20 months). At the last assessment, ocular disease control was complete in 12/12 patients. One patient discontinued baricitinib due to poor compliance after a 12-month relapse-free period. The incidence of ocular flares was 125 episodes/1.000 person-months prior to the initiation of JAK inhibitors and 28.6 episodes/1.000 person-months thereafter. The incidence rate ratio for experiencing a relapse before starting a JAK inhibitor compared to the following period was 4.37 (95% CI 1.3–14.7, p-value: 0.02).ConclusionJAK inhibitors demonstrate efficacy and safety in controlling ocular inflammatory relapses, confirming that they represent a valuable treatment option for patients with non-infectious inflammatory ocular diseases resistant to conventional treatments.

ObjectiveInflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF.MethodsThe risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still’s disease patients and Behçet’s disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression.Results580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet’s disease patients and 497 Still’s disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p=0.10) in FMF compared to Still’s disease and 0.60 (95% CI. 0.29-1.28, p=0.18) in FMF compared to Behçet’s disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (β1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (β1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (β1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (β1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (β1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (β1 = 2.089, 95% CI. 0.7-3.5, p=0.002).ConclusionsThe risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.

Despite societal assumptions, women in ancient Rome actively participated in public discussions and policy-making, taking on leadership roles that contributed to developing educational and employment opportunities for themselves, their families, and their communities. The leadership characteristics of Roman women in civic institutions, which differed from those of Roman men or non-Roman women, have yet to be studied in academic literature. The article explores what we have identified as dialogical traits in the leadership of Roman social and political action. We define these traits as ‘dialogical’ because they are built upon and have emerged from equal and intersubjective dialogue between Roman women and their communities. These traits involve leadership assumed relationally, seen as a collective responsibility, developed with others. The focus is on shared values and identity, with a profound understanding of the necessity for intergenerational solidarity to drive organisational action. Competenze umane e donne romane. Proposte per l’educazione umanistica. A prescindere da ciò che la società tradizionale ritiene, le donne romane sono coinvolte nel dibattito pubblico e nella definizione delle politiche, assumendo un ruolo di leadership nella creazione di opportunità di istruzione e di lavoro per loro stesse, per le loro famiglie e per le loro comunità. I tratti specifici del tipo di leadership sviluppata ed esercitata dalle donne romane nelle istituzioni civiche, rispetto al tipo di leadership esercitata dagli uomini romani o dalle donne non romane, sono ancora poco studiati nella letteratura accademica. L’articolo introduce e discute quelli che abbiamo identificato come tratti dialogici nella leadership delle donne romane quando sono impegnate nell’azione sociale e politica. Definiamo questi tratti “dialogici”, in quanto costruiti ed emersi sulla base di un dialogo paritario e intersoggettivo tra le donne romane e le loro comunità. La leadership relazionale, come responsabilità collettiva costruita con gli altri, incentrata sui valori e sull’identità condivisa dei Romani con una forte intuizione della necessità di solidarietà intergenerazionale tra loro come motore dell’azione organizzativa.

IntroductionThe effectiveness of canakinumab may change according to the different times it is used after Still’s disease onset. This study aimed to investigate whether canakinumab (CAN) shows differences in short- and long-term therapeutic outcomes, according to its use as different lines of biologic treatment.MethodsPatients included in this study were retrospectively enrolled from the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Still’s disease. Seventy-seven (51 females and 26 males) patients with Still’s disease were included in the present study. In total, 39 (50.6%) patients underwent CAN as a first-line biologic agent, and the remaining 38 (49.4%) patients were treated with CAN as a second-line biologic agent or subsequent biologic agent.ResultsNo statistically significant differences were found between patients treated with CAN as a first-line biologic agent and those previously treated with other biologic agents in terms of the frequency of complete response (p =0.62), partial response (p =0.61), treatment failure (p >0.99), and frequency of patients discontinuing CAN due to lack or loss of efficacy (p =0.2). Of all the patients, 18 (23.4%) patients experienced disease relapse during canakinumab treatment, 9 patients were treated with canakinumab as a first-line biologic agent, and nine patients were treated with a second-line or subsequent biologic agent. No differences were found in the frequency of glucocorticoid use (p =0.34), daily glucocorticoid dosage (p =0.47), or concomitant methotrexate dosage (p =0.43) at the last assessment during CAN treatment.ConclusionCanakinumab has proved to be effective in patients with Still’s disease, regardless of its line of biologic treatment.

Abstract Introduction Scientific evidence of the effectiveness of the tumor necrosis factor inhibitor adalimumab (ADA) in pediatric patients with non-infectious non-anterior uveitis is still limited. The aim of this study is to investigate the therapeutic role of ADA in a cohort of pediatric patients with non-anterior uveitis. Methods This is an international multicenter study analyzing real-life data referred to pediatric patients treated with ADA for intermediate uveitis/pars planitis, posterior uveitis and panuveitis. Data were drawn from the AutoInflammatory Disease Alliance (AIDA) registry for patients with uveitis. Results Twenty-one patients (36 affected eyes) were enrolled, and all patients benefited from ADA administration. In detail, 11 patients (19 affected eyes) did not experience further ocular inflammation after ADA introduction; 10 cases (17 affected eyes) showed a significant clinical improvement consisting of a decrease in severity and/or frequency of ocular relapses. The number of ocular flares dropped from 3.91 to 1.1 events/patient/year after ADA introduction (p = 0.0009); macular edema and retinal vasculitis were respectively observed in 18 eyes and 20 eyes at the start of ADA and in 4 eyes and 2 eyes at the last assessment. The mean daily glucocorticoid dosage significantly decreased from 26.8 ± 16.8 mg/day at the start of ADA to 6.25 ± 6.35 mg/day at the last assessment (p = 0.002). Intermediate uveitis/pars planitis (p = 0.01) and posterior uveitis (p = 0.03) were more frequently observed in patients with full response to ADA; panuveitis (p = 0.001) was significantly more frequent among patients continuing to experience uveitic flares. This could be related to a higher use of systemic glucocorticoids (p = 0.002) and conventional immunosuppressants (p = 0.007) at the start of ADA when treating intermediate uveitis/pars planitis. Regarding the safety profile, only one adverse event was reported during ADA treatment, consisting of the development of generalized adenopathy. Conclusions ADA proved to have an effective therapeutic role in all pediatric patients with non-anterior uveitis enrolled in the study. An overall glucocorticoid-sparing effect was observed despite the severity of cases enrolled. A more aggressive treatment of panuveitis and posterior uveitis at start of ADA could increase the likelihood of full response to therapy.

Objective Still’s disease is more frequently observed in the paediatric context, but a delayed onset is not exceptional both in the adulthood and in the elderly. However, whether paediatric-onset, adult-onset and elderly-onset Still’s disease represent expressions of the same disease continuum or different clinical entities is still a matter of controversy. The aim of this study is to search for any differences in demographic, clinical features and response to treatment between pediatric-onset, adult-onset and elderly-onset Still’s disease.Methods Subjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still’s disease.Results A total of 411 patients suffering from Still’s disease were enrolled; the disease occurred in the childhood in 65 (15.8%) patients, in the adult 314 (76.4%) patients and in the elderly in 32 (7.8%) patients. No statistically significant differences at post-hoc analysis were observed in demographic features of the disease between pediatric-onset, adult-onset and elderly-onset Still’s disease. The salmon-coloured skin rash (p=0.004), arthritis (p=0.009) and abdominal pain (p=0.007) resulted significantly more frequent among paediatric patients than in adult cases, while pleuritis (p=0.015) and arthralgia (p

Background Different patient clusters were preliminarily suggested to dissect the clinical heterogeneity in Still’s disease. Thus, we aimed at deriving and validating disease clusters in a multicentre, observational, prospective study to stratify these patients.Methods Patients included in GIRRCS AOSD-study group and AIDA Network Still Disease Registry were assessed if variables for cluster analysis were available (age, systemic score, erythrocyte sedimentation rate (ESR), C reactive protein (CRP) and ferritin). K-means algorithm with Euclidean metric and Elbow plot were used to derive an adequate number of clusters.Results K-means clustering assessment provided four clusters based on means standardised according to z-scores on 349 patients. All clusters mainly presented fever, skin rash and joint involvement. Cluster 1 was composed by 115 patients distinguished by lower values of age and characterised by skin rash myalgia, sore throat and splenomegaly. Cluster 2 included 128 patients identified by lower levels of ESR, ferritin and systemic score; multiorgan manifestations were less frequently observed. Cluster 3 comprised 31 patients categorised by higher levels of CRP and ferritin, they were characterised by fever and joint involvement. Cluster 4 contained 75 patients derived by higher values of age and systemic score. Myalgia, sore throat, liver involvement and life-threatening complications, leading to a high mortality rate, were observed in these patients.Conclusions Four patient clusters in Still’s disease may be recognised by a multidimensional characterisation (‘Juvenile/Transitional’, ‘Uncomplicated’, ‘Hyperferritinemic’ and ‘Catastrophic’). Of interest, cluster 4 was burdened by an increased rate of life-threatening complications and mortality, suggesting a more severe patient group.

IntroductionThis paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet’s disease (BD).MethodsThe project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry.ResultsRespondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behçet’s Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0–30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1–50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 ± 1.1 (range – 1.8–4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status (p

BeckgroundDespite the recent advances in the field of autoinflammatory diseases, most patients with recurrent fever episodes do not have any defined diagnosis. The present study aims at describing a cohort of patients suffering from apparently unexplained recurrent fever, in whom non-radiographic axial spondylarthritis (SpA) represented the unique diagnosis identified after a complete clinical and radiologic assessment.Materials and methodsPatients’ data were obtained from the international registry on Undifferentiated Systemic AutoInflammatory Diseases (USAIDs) developed by the AutoInflammatory Disease Alliance (AIDA) network.ResultsA total of 54 patients with recurrent fever episodes were also affected by non-radiographic axial SpA according to the international classification criteria. SpA was diagnosed after the start of fever episodes in all cases; the mean age at the diagnosis of axial SpA was 39.9 ± 14.8 years with a diagnostic delay of 9.3 years. The highest body temperature reached during flares was 42°C, with a mean temperature of 38.8 ± 1.1°C. The most frequent manifestations associated to fever were: arthralgia in 33 (61.1%) cases, myalgia in 24 (44.4%) cases, arthritis in 22 (40.7%) cases, headache in 15 (27.8%) cases, diarrhea in 14 (25.9%) cases, abdominal pain in 13 (24.1%) cases, and skin rash in 12 (22.1%) cases. Twenty-four (44.4%) patients have taken daily or on-demand non-steroidal anti-inflammatory drugs (NSAIDs) and 31 (57.4%) patients have been treated with daily or on demand oral glucocorticoids. Colchicine was used in 28 (51.8%) patients, while other conventional disease modifying anti-rheumatic drugs (cDMARDs) were employed in 28 (51.8%) patients. Forty (74.1%) patients underwent anti-tumor necrosis factor (TNF) agents and 11 (20.4%) were treated with interleukin (IL)-1 inhibitors. The response to TNF inhibitors on recurrent fever episodes appeared more effective than that observed with anti-IL-1 agents; colchicine and other cDMARDs were more useful when combined with biotechnological agents.ConclusionSigns and symptoms referring to axial SpA should be inquired in patients with apparently unexplained recurrent fever episodes. The specific treatment for axial SpA may lead to a remarkable improvement in the severity and/or frequency of fever episodes in patients with unexplained fevers and concomitant axial SpA.

The objectives of this research were to establish the differences between Spain and Italy regarding the presence of bullying in primary and secondary schools, as well as to determine whether there are differences between experiencing or perpetrating bullying and gender and age in the practice of school bullying. To assess the EBIPQ scores in terms of country and gender, the chi-squared test was used, and ANOVA was applied for age. A total of 1536 students from primary and secondary schools in Spain and Italy participated in the study. Their ages ranged from 10 to 19 years (mean = 13.01, standard deviation = 2.19). The results revealed statistically significant differences in terms of bullying categories concerning the country of origin and gender, with a higher number of Italian participants in the role of “no victim aggress” and Spanish participants in the roles of “victim” and “victim and aggress”. Additionally, there were more boys in the role of “victim and aggress” and girls in the role of “no victim aggress”. Regarding age, statistically significant differences were found, with older students taking on the role of “aggress” on average, while younger students assumed the role of “victim”.

ObjectiveTo evaluate the potential role of Streptococcus salivarius K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction.Patients and methodsThe medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.00 ± 7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00 ± 28.00 months.ResultsThe number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), p < 0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [p < 0.001]. Similarly, the highest temperature in°C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), p < 0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, p < 0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis (p < 0.001), oral aphthae (p < 0.001) and cervical lymphadenopathy (p < 0.001) significantly decreased following SSK12.ConclusionSSK12 prophylaxis given for at least 6.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the duration of the single febrile episode, lowered body temperature by 1°C in the febrile flare, provided a steroid-sparing effect, and significantly reduced the accompanying symptoms related to the syndrome.

IntroductionOne of the biggest challenges facing students today is procrastination, which is closely related to stress, anxiety and, in the most severe cases, depression. The aim of the present study is to analyze the influence of academic self-regulation on procrastination, academic anxiety and stress, academic resilience and academic performance.MethodThe sample consisted of 991 high school students aged between 16 and 19 years (M = 17.25; SD = 3.45). A structural equation model was used to analyze the relationship between the study variables.ResultsAnalyzes showed that academic self-regulation negatively predicted procrastination. In turn, procrastination positively predicted academic stress and anxiety. However, resilience exerted a protective influence by being positively related to academic stress and anxiety. Finally, resilience positively predicted academic performance, whereas stress and anxiety negatively predicted academic performance.ConclusionThus, the importance of generating strategies for students to learn to self-regulate in academic contexts, manage emotions, foster motivation and develop strategies to help them overcome the vicissitudes they face is emphasized.

ObjectiveInterleukin (IL)-1 inhibitors are largely employed in patients with Still’s disease; in cases with refractory arthritis, IL-6 inhibitors have shown to be effective on articular inflammatory involvement. The aim of the present study is to assess any difference in the effectiveness of the IL-1β antagonist canakinumab prescribed as first-line biologic agent between the systemic and the chronic-articular Still’s disease.MethodsData were drawn from the retrospective phase of the AutoInflammatory Disease Alliance (AIDA) international registry dedicated to Still’s disease. Patients with Still’s disease classified according to internationally accepted criteria (Yamaguchi criteria and/or Fautrel criteria) and treated with canakinumab as first-line biologic agent were enrolled.ResultsA total of 26 patients (17 females, 9 males; 18 patients developing Still’s disease after the age of 16 years) were enrolled; 16 (61.5%) patients suffered from the systemic pattern of the disease; 10 (38.5%) patients suffered from the chronic-articular type. No differences were observed between the systemic and the chronic-articular Still’s disease in the frequency of complete response, of flares after the start of canakinumab (p = 0.701) and in the persistence in therapy (p = 0.62). No statistical differences were observed between the two groups after 3 months, 12 months and at the last assessment in the decrease of: the systemic activity score (p = 0.06, p = 0.17, p = 0.17, respectively); the disease activity score on 28 joints (p = 0.54, p = 0.77, p = 0.98, respectively); the glucocorticoid dosage (p = 0.15, p = 0.50, and p = 0.50, respectively); the use of concomitant disease modifying anti-rheumatic drugs (p = 0.10, p = 1.00, and p = 1.00, respectively). No statistically significant differences were observed in the decrease of erythrocyte sedimentation rate (p = 0.34), C reactive protein (p = 0.48), and serum ferritin levels (p = 0.34) after the start of canakinumab.ConclusionCanakinumab used for Still’s disease has been effective in controlling both clinical and laboratory manifestations disregarding the type of disease course when used as first-line biotechnological agent. These excellent results might have been further enhanced by the early start of IL-1 inhibition.

The article proposes to highlight the link between leadership and emotions starting from the Ignatian lesson and Nussbaum's lesson. The leadership of discernment, characterised by Saint Ignatius' pedagogy, is proposed in its formative practices and in the possibility of coexisting in the practices of working and professional life. Relational leadership, suggested by the reading of capabilities and the factors that regulate emotions, also becomes an engine of social and work transformation. The training practices that have the Humanistic Approach as a method find in the proposed Lessons a solid tool to deal with a liquid theme.

ObjectiveThe present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network.MethodsThis is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform.ResultsAIDA project will share knowledge and expertise on mAIDs. Since its start, 118 centers from 24 countries and 4 continents have joined the AIDA project. Fifty-nine centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 337 users (122 Principal Investigators, 210 Site Investigators, 2 Lead Investigators, and 3 data managers). The Registry collects baseline and follow-up data using 3,748 fields organized into 21 instruments, which include demographics, patient history, symptoms, trigger/risk factors, therapies, and healthcare information for mAIDs patients.ConclusionsThe AIDA mAIDs Registry, acts both as a research tool for future collaborative real-life studies on mAIDs and as a service to connect all the figures called to participate. On this basis, the registry is expected to play a pivotal role in generating new scientific evidence on this group of rare diseases, substantially improving the management of patients, and optimizing the impact on the healthcare system. NCT 05200715 available at https://clinicaltrials.gov.

ObjectiveAim of this paper is to illustrate the methodology, design, and development of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to patients with the Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome.MethodsThis is a physician-driven, non-population- and electronic-based registry proposed to gather real-world demographics, clinical, laboratory, instrumental and socioeconomic data from PFAPA patients. Data recruitment is realized through the on-line Research Electronic Data Capture (REDCap) tool. This registry is thought to collect standardized information for clinical research leading to solid real-life evidence. The international scope and the flexibility of the registry will facilitate the realization of cutting-edge study projects through the constant updating of variables and the possible merging and transfer of data between current and future PFAPA registries.ResultsA total of 112 centers have already been involved from 23 countries and 4 continents starting from August 24th, 2021, to April 6th, 2022. In total 56/112 have already obtained the formal approval from their local Ethics Committees. The platform counts 321 users (113 principal investigators, 203 site investigators, two lead investigators, and three data managers). The registry collects retrospective and prospective data using 3,856 fields organized into 25 instruments, including PFAPA patient's demographics, medical histories, symptoms, triggers/risk factors, therapies, and impact on the healthcare systems.ConclusionsThe development of the AIDA International Registry for PFAPA patients will enable the on-line collection of standardized data prompting real-life studies through the connection of worldwide groups of physicians and researchers. This project can be found on https://clinicaltrials.gov NCT 05200715.

ObjectiveThe aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) international Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination.MethodsThis Registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients' management. The Registry may potentially communicate with other on-line tools dedicated to VEXAS syndrome, thus enhancing international collaboration and data sharing for research purposes. The Registry is practical enough to be easily modified to meet future needs regarding VEXAS syndrome.ResultsTo date (April 22nd, 2022), 113 Centers from 23 Countries in 4 continents have been involved; 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) are currently able to access the registry for data entry (or data sharing) and collection. The Registry includes 4,952 fields organized into 18 instruments designed to fully describe patient's details about demographics, clinical manifestations, symptoms, histologic details about skin and bone marrow biopsies and aspirate, laboratory features, complications, comorbidities, therapies, and healthcare access.ConclusionThis international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease, with the final goal to obtain real-world evidence for daily clinical practice, especially in relation to the comprehension of this disease about the natural history and the possible therapeutic approaches. This Project can be found on https://clinicaltrials.gov NCT05200715.

ObjectiveThe present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome.MethodsThis is a clinical physician-driven, population- and electronic-based registry implemented for the retrospective and prospective collection of real-life data from patients with Schnitzler's syndrome; the registry is based on the Research Electronic Data Capture (REDCap) tool, which is designed to collect standardized information for clinical research, and has been realized to change over time according to future scientific acquisitions and potentially communicate with other existing or future similar registries.ResultsSince its launch, 113 centers from 23 countries in 4 continents have been involved. Fifty-seven have already obtained the approval from their local Ethics Committees. The platform counts 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) at current (April 28th, 2022). The registry collects baseline and follow-up data using 3,924 fields organized into 25 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, laboratory, instrumental exams, therapies, socioeconomic information, and healthcare access.ConclusionsThis International Registry for patients with Schnitzler's syndrome facilitates standardized data collection, enabling international collaborative projects through data sharing and dissemination of knowledge; in turn, it will shed light into many blind spots characterizing this complex autoinflammatory disorder.

ObjectiveThis paper points out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients affected by Undifferentiated Systemic AutoInflammatory Diseases (USAIDs).MethodsThis is an electronic registry employed for real-world data collection about demographics, clinical, laboratory, instrumental and socioeconomic data of USAIDs patients. Data recruitment, based on the Research Electronic Data Capture (REDCap) tool, is designed to obtain standardized information for real-life research. The instrument is endowed with flexibility, and it could change over time according to the scientific acquisitions and potentially communicate with other similar tools; this platform ensures security, data quality and data governance.ResultsThe focus of the AIDA project is connecting physicians and researchers from all over the world to shed a new light on heterogeneous rare diseases. Since its birth, 110 centers from 23 countries and 4 continents have joined the AIDA project. Fifty-four centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 179 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry is collecting baseline and follow-up data using 3,769 fields organized into 23 instruments, which include demographics, history, symptoms, trigger/risk factors, therapies, and healthcare information access for USAIDs patients.ConclusionsThe development of the AIDA International Registry for USAIDs patients will facilitate the online collection of real standardized data, connecting a worldwide group of researchers: the Registry constitutes an international multicentre observational groundwork aimed at increasing the patient cohort of USAIDs in order to improve our knowledge of this peculiar cluster of autoinflammatory diseases. NCT 05200715 available at https://clinicaltrials.gov/.

Academic performance (AP) is a topic of particular interest in the academic context. Attributions for academic success (AAS) have been shown to have a significant impact on AP, and more specifically internal controllable attributions (ICA) are closely linked to academic success. Similarly, positive emotions (PE) have a significant influence on AP and may in turn be influenced by bullying. This study examines the connections between ICA of academic success and AP mediated through PE in late primary and early secondary school students and analyzes the relationships between PE and bullying categories. Students (N = 562, 49.46% female, Mage = 11.6 SD = 1.2) reported on their perceptions of ICA and PE in relation to exams and their relationship with bullying through validated questionnaires. The AP was obtained as the average mark of all subjects in the immediately preceding assessment. First, a multiple linear regression analysis considering ICA and PE as predictor variables was carried out, which showed a significantly positive link between ICA and PE, between ICA and AP, and between PE and AP. Subsequently, using the SPSS macro PROCESS, a simple mediation model was implemented to quantify the effect of ICA on AP through PE in exams, and finally an ANOVA between the categories of bullying and PE was performed. The results showed a significant indirect relationship with a positive predictive relationship for AP. The model shows that PE proves to be a significant mediator between ICA and AP, and it is shown that students disengaged from bullying score higher in PE.

ObjectiveAim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder.MethodsThis Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients' management. The collection of standardized data is thought to bring knowledge about real-life clinical research and potentially communicate with other existing and future Registries dedicated to Still's disease. Moreover, it has been conceived to be flexible enough to easily change according to future scientific acquisitions.ResultsStarting from June 30th to February 7th, 2022, 110 Centers from 23 Countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 4449 fields organized into 14 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access.ConclusionsThis international Registry for patients with Still's disease will allow a robust clinical research through collection of standardized data, international consultation, dissemination of knowledge, and implementation of observational studies based on wide cohorts of patients followed-up for very long periods. Solid evidence drawn from “real-life” data represents the ultimate goal of this Registry, which has been implemented to significantly improve the overall management of patients with Still's disease. NCT 05200715 available at https://clinicaltrials.gov/.

Soft skills are important for any career and are necessary to access and face the labor market. This research focuses on soft skills by exploring engineer profiles. It also determines how soft skills are developed through the study of a representative sample of 314 undergraduate engineering students from 15 different Italian universities. The instrument used is a questionnaire that investigates soft skills and is based on the Business-focused Inventory of Personality (BIP). Answers are grouped into four areas: intrapersonal, interpersonal, activity development, and impression management. Results show that these engineers have more self-confidence than the reference sample; they demonstrated a great commitment in setting job goals and pursuing projects, a good emotional adaptation to social situations, and enough attitudes in terms of problem solving and openness to change. Perception on the ability to work under pressure is in the average, and they seem ready to take on challenging tasks. The score shows that engineers from the sample are able to express positive and negative ideas and feelings in balance with the reference average, but sometimes they have difficulties in establishing personal relationships. Therefore, they are unable to understand the moods of those who around them and may also have difficulty in understanding their expectations. This results in some difficulties in teamwork. The general result underlines the opportunity of empowerment programs regarding soft skills.

Abstract. In this text, we develop reflections on some aspects of the work of the Brazilian educator Paulo Freire (1921-2021) in its original context, as well as relating it to the broader context of current educational scenarios. In a historical moment where the world is also wondering about the future of education, we find the opportunity to propose a critical reflection on the role of Freire’s pedagogy. The ideologies that had a great impact on Brazilian education throughout most of the 20th century are being renewed to adapt to new political and economic needs. And, for this renewal, Paulo Freire’s educational thought offers a very important and original contribution. The analysis of Freire’s pedagogical thinking allowed us to identify some aspects that we consider crucial to highlight the modernity and global reflection about Freire Method. The critical reflection expressed in the conclusions allows us to recognize the modernity in Freire’s pedagogy, basic assumptions for educators who interpret the challenges of a global community

Recent reports have suggested that many employees in the labour market today lack essential soft skills. Yet, the specific relevance of these skills for particular job occupations is still to explore. We investigated perceptions of skill requirements and their development potential reflected against cultural impacts, using close interviews of 64 managers at firms in different fields of industries in Italy and Finland. Managers reported that, due to heightened competitive pressure, specific soft skills, particularly Work in team and flexibility to develop, are becoming increasingly important. The study indicates that the perceived significance of soft skills seems to vary according to the working environment and occupation. Furthermore, some soft skills seem to have a higher potential for development and thus suggested to be implemented in the university curricula. The paper concludes that a country characterized by working environments conducive to the development of soft skills, must however promote research for the identification and relevance of the skills themselves.

Objective. To analyze the potential role of colchicine monotherapy in patients with tumor necrosis factor receptor associated periodic syndrome (TRAPS) in terms of control of clinical and laboratory manifestations. Methods. Patients with TRAPS treated with colchicine monotherapy were retrospectively enrolled; demographic, clinical and therapeutic data were collected and statistically analysed after having clustered patients according to different times at disease onset, penetrance of mutations, dosage of colchicine, and different disease manifestations. Results. 24 patients (14 males; 15 with pediatric disease onset) treated with colchicine monotherapy were enrolled. Colchicine resulted in a complete response in 3 (12.5%) cases, partial response in 14 (58.3%) patients, and lack of response in 7 (29.2%) patients. There were not significant differences in colchicine response between pediatric and adult disease onset (p=0.42), between low- and high-penetrance mutations (p=0.62), and according to different dosages (p=0.66). No significant differences were identified in the frequency of specific disease manifestations between patients experiencing any response to colchicine and patients with lack of response. Conclusions. Colchicine monotherapy is useful in a low percentage of TRAPS patients; nevertheless, it could be attempted in patients with milder phenotypes and at a lower risk of developing reactive amyloidosis.

Objectives. To examine demographic and clinical characteristics and long-term visual outcome in a cohort of Italian patients affected by Behçet’s uveitis (BU). Materials and Methods. Retrospective chart review of 47 patients with BU attending our unit between January 2018 and December 2019. Ophthalmologic manifestations, best-corrected visual acuity (BCVA), fluoroangiography and optical coherence tomography findings, and ocular complications were recorded. Predictive factors of a poor visual outcome and long-term complications were also investigated. Results. Forty-seven patients (23 males and 24 females) for a total of 84 eyes were enrolled. Uveitis was bilateral in 37 (78.7%) patients with panuveitis being the most frequent anatomical pattern (40 out of 84 eyes), whereas 27 eyes presented a posterior uveitis. Isolated anterior uveitis was detected in 16 eyes. A significant improvement of median BCVA between baseline and last follow-up values was detected (p=0.042). A higher risk of poor visual prognosis was observed in patients with uveitis duration greater than 15 years (p=0.019). A significant resolution of retinal vasculitis was detected between baseline and last follow-up evaluation (p

Il riconoscimento del ruolo delle competenze trasversali nella scuola rende necessario l’approfondimento di tale ambito di ricerca per rispondere alla necessità di una formazione di tali competenze per la professione docente. Dopo una rassegna di studi nazionali e internazionali, sia teorici sia empirici, si evidenzia il carattere ‘multidimensionale’ delle competenze trasversali di tipo sociale cercando di identificare i principali fattori comuni alle varie classificazioni proposte in letteratura. Le evidenze presentate contribuiscono a definire il significato delle competenze trasversali socio-relazionali e comunicative del docente come la capacità di mettersi in relazione con l’altro ascoltando le sue esigenze in modo empatico e collaborativo. Vengono riassunti gli esiti principali di un’indagine empirica volta ad esplorare alcune competenze socio-relazionali (Socievolezza, Capacità di stabilire contatti, Sensibilità, Orientamento al team, Assertività), rilevate attraverso la somministrazione a 80 docenti di scuole secondarie superiori, della scala sulle “Competenze Sociali” del Questionario BIP (Business-focused Inventory of Personality) di Hossiep, Paschen (2003), adattato alla popolazione italiana da Fossati e Ciancaleoni (2013). In sintesi, si rileva che i docenti esaminati necessitano di sviluppare ulteriormente le loro competenze trasversali di tipo socio-relazionale e comunicativo: ciò è possibile grazie specifici programmi di formazione.

A dynamic framework that is «clearly identifiable» in relation to values research (Super, 1995) is essential in order to identify the meaning of interests, motivations, attitudes and ethics (Bellotto, 2004), focused on young generations facing work world. This research aims to: identify work values and values’ orientation in relation to work; specify generational and cultural differences. The research topic consists of: a sample of Spanish, composed by 230 persons, that has a more materialistic and risk-taking orientation that, even if it decreases with age, show how young Spanish are more competitive; in the Italian sample there are 203 subjects, that shows differences in terms of old and young generations regarding value orientation; the group of people from 18 to 29 years old has a significant materialistic self-orientation, definitely high if compared to the other groups. The results show the importance of work values and contribute to studies about how various groups adopt different values depending on cultural and social needs.

Entrepreneurship education is a new phenomenon in higher education, as a field of inquiry it is one of the most rapidly growing areas of research (Katz, 2008).The paper includes the analyses of the different entrepreneurial attitudes of Nigerian students in comparison with that of the Italian students. The related entrepreneurship education literatures make references towards exploring entrepreneurial attitudes and the values perceptions of Nigerian and Italian students. The findings suggest a considerable socio-cultural impact: one that starts with values and religion support and extends to the attitudes itself and on entrepreneurs’ access to entrepreneurship education (Chen, 2005). Building from these analyses, a theoretical paradigm for the growth of entrepreneurship education is offered, using the applied research as the example. The contribution of this article suggests the necessity to design and implement a curriculum which provides a systematic teaching of the entrepreneurial attitudes and values in the institutions of learning as a whole. The article seeks to contribute to a growing need to find innovative ways of thinking and develop entrepreneurial approaches to education.

Telemedicine, defined by the World Health Organization as ‘integrative part of telecommunication systems in the promotion of public health’, generates different positive results: more efficiency, less costs, better patient’s quality of life. Thanks to the advances in diagnostic technologies, information technologies, remote monitoring and long-distance care, telemedicine have increased the viability of home-based care, even for patients with serious conditions. At the same time we observe the increase in the elderly population suffering from pluripatologyand chronic diseases, that causes an increasing recourse to medical and hospital care; but prolonged hospital stay can determine loss of function and psychophysical disorders. Telemedicine is 'the investigation, monitoring and management of patients and education of patients and staff using systems which allow ready access to expert advice and patient information, no matter where the patient or relevant information is located'. We are empowering in different areas new biomedical and information technology as a support to clinical practice. The goal is to make a case study and to use for future analysis. In the experimental phase 4 patients were followed on hemodialysis and 4 in peritoneal dialysis, previously evaluated for clinical, social, logistical and psychological conditions. It is estimated that today there are 50.000 patients on dialysis and about 10.000 new cases a year only in Italy. Naturally there are threats to solve because users face difficulties, in terms of interaction with robots, in their usual context of life.The right methodology needs to coordinate the entire hospital, different professionals, patients, providing tools for care givers that follow patients, training technicians, aligning data collection and communication.The project reached great results in relation to the indicators: efficiency in dialysis, compliance with health protocols, impact on patient quality of life. Telemedicine and telecardiology projects are active at the ‘Hospital at Home Service of Bari’.

The aim of this work is to analyse, by a mixed research method, the positive impact of innovative education actions, with a specific focus on the Salamanca Summer School case study. In additions to that, this contribution will further strengthen the literature which confirms the educational and professional impact of innovation-oriented practices, if placed at the core of research and didactics. We will show through different focus groups and questionnaires to a sample of students, teachers and entrepreneurs, how the Universities “third mission” entrepreneurial vocation and its relationship with didactics are well conveyed in the innovative educational experience held at University of Sala-manca. A deeper insight is achieved both by using the above said instruments - before and after the experience - and by commenting the action impact. The answers provided are discussed in light of the need for an entrepreneurial par-adigm implementation at the university level, as recommended by the “third mission”.

Primary School teachers carry out a crucial role in education and future adults development, therefore it turns out to be fundamental to study their educational styles to identify the necessary competencies and skills to provide an education as complete and balanced as possible. The present research, starting from the already undertaken Spanish studies using the ECAD-EP scale (Self-Percieved Competencies Evaluation Scale), aims to investigate theaching styles inside an italian sample of primary school’s theachers, to compare them with the Spanish datas. Beside that, it deepens the personal values topic, to highlight the latter’s influence on theachers’ theaching styles.

“Small opportunities are often the beginning of great enterprises”. (Demonstene) In the specific study reported here, we targeted the situation of redundant workers in Italy. Following the review of statistical data 30 workers, who were made redundant by their companies, were interviewed. These individuals, who received a wage compensation by the Government for their total or partial loss of work, have attended the new SPAL (Active Labor Policies System) courses carried out by the Province of Rome. The aim of this study was to identify the extent to which the 30 individuals who were interviewed, who had good probabilities of leaving the labor market in adult age, already showed some of the key entrepreneurial characteristics. At the end of this study, these variables helped us to understand if and to what extend those redundant workers over 45 years of age had been inclined to start a new business and therefore how motivated they had been to embrace a new way of living and working, based on the development and launch of a new business. This work will present the results and the conclusions that emerged from the survey that was carried out between July 2012 and July 2013.

EL CONSEJO EDITORIAL DE LA REVISTA INFAD NO ACEPTA MANTENER SU PUBLICACIÓN POR MOTIVOS DE NORMAS DE PUBLICACIÓN.

The theme of Entrepreneurial Education has acquired more and more importance in the last years as, in the present economic situation, there is a growth of the exigency, above all among young people, to reinvent themselves and to create their own personal business. This happens in every field of the labor market, even in the fashion world. The present research has, in fact, the goal to introduce a real course on entrepreneurial education inside fashion agencies, to offer young models the basics to become entrepreneurs and make the most of their skills and attitudes, to make their way in a world which, contrary to what it seems, is not only made of beauty. The sample is represented by a group of eighty five (85) models who are part of different fashion agencies. The focus of this study is represented by the subject and his intraentrepreneurial being inside the work context.

The distinctiveness of migrants’ entrepreneurial propensity can be explained by considering both internal characteristics of the individual migrant, as well as the external environment of the host economy he/she operates in (Kloosterman et al. 1999). Migrants can perceive and seek entrepreneurial opportunities through specific sets of resources and personality-traits (Kloosterman/Rath 2001, Kloosterman 2010). Despite of this, the topic is vastly under-studied compared to its economic importance. After an in-depth study of the literature status quo about it, the paper analyses the motivation, intention, self-efficacy, culture, entrepreneurial orientation and performance of immigrant entrepreneurs. The main aim is to verify to what extent perceptions and skills of these immigrant entrepreneurs are similar to entrepreneurial psychological constructs studied in the literature. The used research instrument was the semi-structured interview consisting of 49 open-ended questions directed at the sample under examination. The results of the study showed a significant correlation between motivation and business performance (a motivated entrepreneur is more likely to succeed in business than an unmotivated entrepreneur) and a significant positive correlation between culture and motivation to start a business (a culture that is supportive of entrepreneurial activities, lowuncertainty avoidance, high individualism and lowpower distance relates positively to a high level of entrepreneurial self-efficacy).

Primary School teachers carry out a crucial role in education and future adults development, therefore it turns out to be fundamental to study their educational styles to identify the necessary competencies and skills to provide an education as complete and balanced as possible. The present research, starting from the already undertaken Spanish studies using the ECAD-EP scale (Self- Percieved Competencies Evaluation Scale), aims to investigate theaching styles inside an italian sample of primary school’s theachers, to compare them with the Spanish datas. Beside that, it deepens the personal values topic, to highlight the latter’s influence on theachers’ theaching styles.