Integration strategy and tool between formal ontology and graph database technology

Ontologies, and especially formal ones, have traditionally been investigated as a means to formalize an application domain so as to carry out automated reasoning on it. The union of the terminological part of an ontology and the corresponding assertional part is known as a Knowledge Graph. On the other hand, database technology has often focused on the optimal organization of data so as to boost efficiency in their storage, management and retrieval. Graph databases are a recent technology specifically focusing on element-driven data browsing rather than on batch processing. While the complementarity and connections between these technologies are patent and intuitive, little exists to bring them to full integration and cooperation. This paper aims at bridging this gap, by proposing an intermediate format that can be easily mapped onto the formal ontology on one hand, so as to allow complex reasoning, and onto the graph database on the other, so as to benefit from efficient data handling

Neuropsychological performances, quality of life, and psychological issues in pediatric onset multiple sclerosis: a narrative review

Multiple sclerosis (MS) is primarily a disease diagnosed in young and middle-aged adults. Although MS is a rare condition in pediatric age, an increasing rate of patients is diagnosed under the age of 18. The disabling nature of the disease cannot be reduced only to physical symptoms. Several additional symptoms such as cognitive impairment, fatigue, and psychological symptoms are common features of pediatric MS. The reviewed literature suggests that, despite the lower physical disability, children and adolescents diagnosed with MS are vulnerable to cognitive impairment even in the early stage of the disease. The neuropsychological profile of pediatric MS may resemble that of adult MS, including an impairment in attention/information processing speed, learning, verbal, and visuospatial memory. However, cognitive difficulties in children and adolescents are more likely to involve also general intelligence and linguistic abilities, presumably due to patients’ younger age and cognitive growth stage. Cognitive difficulties, beyond physical disability and relapses, may have a considerable impact on learning and school achievement. Depression and fatigue are other highly prevalent disturbances in pediatric MS and may contribute to patients’ low functional outcomes. Overall, these manifestations may cause considerable functional impairment on daily activities and quality of life that may require individualized rehabilitative treatment and extensive psychosocial care. Additional neuropsychological research evaluating larger samples, using more homogenous methods, and exploring the role of MS treatment on cognitive and psychological development is required

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

Safety of SARS-CoV2 vaccination and COVID-19 short-term outcome in pediatric acquired demyelinating disorders of central nervous system: A single center experience

IntroductionConcern of a correlation between disease relapse in patients with acquired demyelinating disorders of central nervous system (CNS) and SARS-CoV2 vaccines has been raised. In this single center study, we retrospectively evaluated safety of SARS-CoV2 vaccination and COVID-19 short-term outcome in pediatric acquired demyelinating disorders of CNS. Materials and methodsPatients with multiple sclerosis (MS), myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) and neuromyelitis optica spectrum disorder (NMOSD) with disease onset before 18 years of age were included. Demographic and clinical data, and information regarding previous SARS-CoV-2 infection and vaccination were collected. ResultsWe included nine patients with MOGAD. Six patients received SARS-CoV2 vaccination and complained pain at injection site while only one had fever and fatigue. Median follow-up was 28 weeks (range 20-48). Seven patients had COVID-19 occurring with mild flu-like symptoms and median follow-up was 28 weeks (range 24-34). Nobody had disease relapse. Five patients with NMOSD were included. All patients received SARS-CoV2 vaccination (BNT162b2-Pfizer-BioNTech). The median follow-up was 20 weeks (range 14-24) and only two patients complained pain at injection site, fever and fatigue. Three patients had also COVID-19 with mild flu-like symptoms, despite two of them being under immunosuppressive treatment. Lastly, forty-three patients with MS were included. 35 out of 43 received SARS-CoV2 vaccination with a median follow-up of 24 weeks (range 8-36). Fourteen patients had no side effects, while 21 complained mild side effects (mainly pain at injection site) and one experienced a disease relapse with complete recovery after steroid therapy. At vaccination, all but one were under treatment. Sixteen patients had COVID-19 occurring with mild symptoms. DiscussionCOVID-19 outcome was good although many patients were under immunosuppressive treatment. Vaccine-related side effects were frequent but were mild and self-limited. Only one MS patient had a post-vaccination relapse with complete recovery after steroid therapy. In conclusion, our data support the safety of SARS-CoV-2 vaccines in pediatric MS, MOGAD and NMOSD

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

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