Toward a precision, complexity-informed cultural policy design: Structural bottlenecks to culture-led development in Skaraborg, Sweden

We analyze the spatial-temporal dynamics of cultural vibrancy in the Swedish sub-region of Skaraborg. Our database consists of 4170 geo-localized cultural activities and facilities, mapped between October 2013 and March 2014. We make use of the TWC methodology for the dynamic simulation of the evolution of geo-localized activity starting from an observed distribution of events, and of the AutoCM ANN architecture to understand how cultural variables are related to the rest of the Skaraborg socio-economy. We find that cultural vibrancy in Skaraborg is likely characterized by a 'flaring' pattern of initial, widespread activity followed by a re-concentration into the main local urban hubs. The deep reason behind this unsuccessful developmental trajectory is the lack of centrality of cultural production in the local socio-economy, and of integration across cultural production sectors. This is in turn due also to structural bottlenecks of a non-cultural nature such as insufficient access of women to higher education. We make a case for the necessity to develop a new precision cultural policy design approach founded upon the science of complexity for both policy design and assessment, and we provide and illustrate a first technical toolkit to this purpose.(c) 2022 Elsevier B.V. All rights reserved

A nonlinear, data-driven, ANNs-based approach to culture-led development policies in rural areas: The case of Gjakove and Pee districts, Western Kosovo

We develop a computational approach to the analysis of cultural vibrancy and to the role of the cultural and cre-ative sectors in the socio-economic organization of two districts of Western Kosovo, Gjakove and Pee. Our anal-ysis is built on a geolocalized mapping of the cultural activities and facilities, and on the main socio-economic variables for the two districts, and makes use of innovative data analysis techniques: Theory of Impossible Words (TIW), the Topological Weighted Centroid (TWC), and the AutoCM ANN. We find that the dynamics of cul-tural vibrancy of the territory is mainly driven by the competing attraction pulls of the nearby countries of Serbia and Albania, that also form the region's main and often conflicting ethnicities, and that such dynamics are likely to further polarize in the future. We also find that the cultural system plays a marginal role in the territory's socio-economic organization. This situation makes a case for a more active role of cultural policy in shaping future local developmental models in rural areas and in acting as an agent of social cohesion.(c) 2022 Elsevier Ltd. All rights reserved

Thoracic Endovascular Aortic Repair for Type B Acute Aortic Dissection Complicated by Descending Thoracic Aneurysm

OBJECTIVES: To analyse the results and review the literature about thoracic aortic endovascular repair (TEVAR) for type B acute aortic dissection (TBAAD) complicated by descending thoracic aortic aneurysm (DTA) in the hyperacute or acute phases. METHODS: This was a multicentre, observational descriptive study. Inclusion criteria were TBAAD with a DTA of 6550 mm, TBAAD on an already known aneurysmal descending thoracic aorta, and TBAAD presenting with an enlarged aorta with a total diameter <50 mm, but with >50% diameter increase compared with a previous computed tomography angiography (CTA) showing a non-dissected aorta with normal sizing. Primary endpoints were early and long-term survival, freedom from TEVAR and aortic related mortality (ARM), and freedom from re-intervention. RESULTS: Twenty-two patients were included in the analysis. The mean aortic diameter was 66 \ub1 26 mm (range 42-130; IQR 51-64). The in hospital TEVAR related mortality was 14% (n = 3). The mean radiological follow-up was 56 \ub1 45 months (range 6-149; IQR 12-82), and the follow-up index 0.97 \ub1 0.1. All surviving patients were available for follow-up. During follow-up the cumulative mortality was 26% (n = 5) and TEVAR related mortality was 5% (n = 1). Overall the estimate of survival was 82% (95%CI: 61.5-93) at 1 year, and 64% at 5 years. Ongoing primary clinical success was 79% (re-intervention n = 4). Freedom from aortic related mortality was 86% (95%CI: 66-95) at 1 and 5 year, while freedom from re-intervention was 95% (95%CI: 75.5-95) at 1 year, and 77% (95%CI: 50-92) at 5 years. CONCLUSIONS: In our experience, DTA is a frequent complication from the very beginning of the clinical onset of TBAAD. In this high-risk cohort, TEVAR showed satisfactory results, better than those predicted by the risk score for open repair, with favourable stability of the aortic diameter and no aortic related adverse events during follow-up

Early Immunotherapy and Longer Corticosteroid Treatment Are Associated With Lower Risk of Relapsing Disease Course in Pediatric MOGAD

Background and Objectives We sought to identify early factors associated with relapse and outcome in paediatric-onset myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD). Methods In a multicenter retrospective cohort of pediatric MOGAD (≤18 years), onset features and treatment were compared in patients with monophasic vs relapsing disease (including cases with follow-up ≥12 months after onset or relapse at any time) and in patients with final Expanded Disability Status Scale (EDSS) 0 vs ≥1 at last follow-up (including cases with followup >3 months after last event or EDSS0 at any time). Multivariable logistic regression models were used to evaluate factors associated with relapsing disease course and EDSS ≥ 1 at final follow-up. Results Seventy-five children were included (median onset age 7 years; median 30 months of follow-up). Presentation with acute disseminated encephalomyelitis was more frequent in children aged 8 years or younger (66.7%, 28/42) than in older patients (30.3%, 10/33) (p = 0.002), whereas presentation with optic neuritis was more common in children older than 8 years (57.6%, 19/33) than in younger patients (21.4%, 9/42) (p = 0.001). 40.0% (26/65) of patients relapsed. Time to first relapse was longer in children aged 8 years or younger than in older patients (median 18 vs 4 months) (p = 0.013). Factors at first event independently associated with lower risk of relapsing disease course were immunotherapy <7 days from onset (6.7-fold reduced odds of relapsing course, OR 0.15, 95% CI 0.03–0.61, p = 0.009), corticosteroid treatment for ≥5 weeks (6.7-fold reduced odds of relapse, OR 0.15, 95% CI 0.03–0.80, p = 0.026), and abnormal optic nerves on onset MRI (12.5-fold reduced odds of relapse, OR 0.08, 95% CI 0.01–0.50, p = 0.007). 21.1% (15/71) had EDSS ≥ 1 at final follow-up. Patients with a relapsing course had a higher proportion of final EDSS ≥ 1 (37.5%, 9/24) than children with monophasic disease (12.8%, 5/39) (p = 0.022, univariate analysis). Each 1-point increment in worst EDSS at onset was independently associated with 6.7-fold increased odds of final EDSS ≥ 1 (OR 6.65, 95% CI 1.33–33.26, p = 0.021). Discussion At first attack of pediatric MOGAD, early immunotherapy, longer duration of corticosteroid treatment, and abnormal optic nerves on MRI seem associated with lower risk of relapse, whereas higher disease severity is associated with greater risk of final disability (EDSS ≥ 1)

Predictive Process Monitoring Methods: Which One Suits Me Best?

Predictive process monitoring has recently gained traction in academia and is maturing also in companies. However, with the growing body of research, it might be daunting for companies to navigate in this domain in order to find, provided certain data, what can be predicted and what methods to use. The main objective of this paper is developing a value-driven framework for classifying existing work on predictive process monitoring. This objective is achieved by systematically identifying, categorizing, and analyzing existing approaches for predictive process monitoring. The review is then used to develop a value-driven framework that can support organizations to navigate in the predictive process monitoring field and help them to find value and exploit the opportunities enabled by these analysis techniques

Cyclic vomiting syndrome in children: a nationwide survey of current practice on behalf of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP)

Background: Cyclic Vomiting Syndrome (CVS) is a rare functional gastrointestinal disorder, which has a considerable burden on quality of life of both children and their family. Aim of the study was to evaluate the diagnostic modalities and therapeutic approach to CVS among Italian tertiary care centers and the differences according to subspecialties, as well as to explore whether potential predictive factors associated with either a poor outcome or a response to a specific treatment. Methods: Cross-sectional multicenter web-based survey involving members of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP). Results: A total of 67 responses were received and analyzed. Most of the respondent units cared for less than 20 patients. More than half of the patients were referred after 3 to 5 episodes, and a quarter after 5 attacks. We report different diagnostic approaches among Italian clinicians, which was particularly evident when comparing gastroenterologists and neurologists. Moreover, our survey demonstrated a predilection of certain drugs during emetic phase according to specific clinic, which reflects the cultural background of physicians. Conclusion: In conclusion, our survey highlights poor consensus amongst clinicians in our country in the diagnosis and the management of children with CVS, raising the need for a national consensus guideline in order to standardize the practice

Adult-onset KMT2B-related dystonia

KMT2B-related dystonia (DYT-KMT2B, also known as DYT28) is an autosomal dominant neurological disorder characterized by varying combinations of generalized dystonia, psychomotor developmental delay, mild-to-moderate intellectual disability and short stature. Disease onset occurs typically before 10 years of age. We report the clinical and genetic findings of a series of subjects affected by adult-onset dystonia, hearing loss or intellectual disability carrying rare heterozygous KMT2B variants. Twelve cases from five unrelated families carrying four rare KMT2B missense variants predicted to impact protein function are described. Seven affected subjects presented with adult-onset focal or segmental dystonia, three developed isolated progressive hearing loss, and one displayed intellectual disability and short stature. Genome-wide DNA methylation profiling allowed to discriminate these adult-onset dystonia cases from controls and early-onset DYT-KMT2B patients. These findings document the relevance of KMT2B variants as a potential genetic determinant of adult-onset dystonia and prompt to further characterize KMT2B carriers investigating non-dystonic features