Defying Expectations: Stylistically Unconventional Anger in a Contemporary Dutch Literary Novel

Abstract and presentation for the DHBenelux 2022 Conference

Physical activity and physical fitness in children with heritable connective tissue disorders

ObjectivesHealth problems in patients with heritable connective tissue disorders (HCTD) are diverse and complex and might lead to lower physical activity (PA) and physical fitness (PF). This study aimed to investigate the PA and PF of children with heritable connective tissue disorders (HCTD).MethodsPA was assessed using an accelerometer-based activity monitor (ActivPAL) and the mobility subscale of the Pediatric Evaluation of Disability Inventory Computer Adaptive Test (PEDI-CAT). PF was measured in terms of cardiovascular endurance using the Fitkids Treadmill Test (FTT); maximal hand grip strength, using hand grip dynamometry (HGD) as an indicator of muscle strength; and motor proficiency, using the Bruininks-Oseretsky Test of Motor Proficiency-2 (BOTMP-2).ResultsA total of 56 children, with a median age of 11.6 (interquartile range [IQR], 8.8–15.8) years, diagnosed with Marfan syndrome (MFS), n = 37, Loeys-Dietz syndrome (LDS), n = 6, and genetically confirmed Ehlers-Danlos (EDS) syndromes, n = 13 (including classical EDS n = 10, vascular EDS n = 1, dermatosparaxis EDS n = 1, arthrochalasia EDS n = 1), participated. Regarding PA, children with HCTD were active for 4.5 (IQR 3.5–5.2) hours/day, spent 9.2 (IQR 7.6–10.4) hours/day sedentary, slept 11.2 (IQR 9.5–11.5) hours/day, and performed 8,351.7 (IQR 6,456.9–1,0484.6) steps/day. They scored below average (mean (standard deviation [SD]) z-score −1.4 (1.6)) on the PEDI-CAT mobility subscale. Regarding PF, children with HCTD scored well below average on the FFT (mean (SD) z-score −3.3 (3.2)) and below average on the HGD (mean (SD) z-score −1.1 (1.2)) compared to normative data. Contradictory, the BOTMP-2 score was classified as average (mean (SD) z-score.02 (.98)). Moderate positive correlations were found between PA and PF (r(39) = .378, p < .001). Moderately sized negative correlations were found between pain intensity and fatigue and time spent actively (r(35) = .408, p < .001 and r(24) = .395 p < .001, respectively).ConclusionThis study is the first to demonstrate reduced PA and PF in children with HCTD. PF was moderately positively correlated with PA and negatively correlated with pain intensity and fatigue. Reduced cardiovascular endurance, muscle strength, and deconditioning, combined with disorder-specific cardiovascular and musculoskeletal features, are hypothesized to be causal. Identifying the limitations in PA and PF provides a starting point for tailor-made interventions

Physical activity and physical fitness in children with heritable connective tissue disorders

ObjectivesHealth problems in patients with heritable connective tissue disorders (HCTD) are diverse and complex and might lead to lower physical activity (PA) and physical fitness (PF). This study aimed to investigate the PA and PF of children with heritable connective tissue disorders (HCTD).MethodsPA was assessed using an accelerometer-based activity monitor (ActivPAL) and the mobility subscale of the Pediatric Evaluation of Disability Inventory Computer Adaptive Test (PEDI-CAT). PF was measured in terms of cardiovascular endurance using the Fitkids Treadmill Test (FTT); maximal hand grip strength, using hand grip dynamometry (HGD) as an indicator of muscle strength; and motor proficiency, using the Bruininks-Oseretsky Test of Motor Proficiency-2 (BOTMP-2).ResultsA total of 56 children, with a median age of 11.6 (interquartile range [IQR], 8.8-15.8) years, diagnosed with Marfan syndrome (MFS), n = 37, Loeys-Dietz syndrome (LDS), n = 6, and genetically confirmed Ehlers-Danlos (EDS) syndromes, n = 13 (including classical EDS n = 10, vascular EDS n = 1, dermatosparaxis EDS n = 1, arthrochalasia EDS n = 1), participated. Regarding PA, children with HCTD were active for 4.5 (IQR 3.5-5.2) hours/day, spent 9.2 (IQR 7.6-10.4) hours/day sedentary, slept 11.2 (IQR 9.5-11.5) hours/day, and performed 8,351.7 (IQR 6,456.9-1,0484.6) steps/day. They scored below average (mean (standard deviation [SD]) z-score -1.4 (1.6)) on the PEDI-CAT mobility subscale. Regarding PF, children with HCTD scored well below average on the FFT (mean (SD) z-score -3.3 (3.2)) and below average on the HGD (mean (SD) z-score -1.1 (1.2)) compared to normative data. Contradictory, the BOTMP-2 score was classified as average (mean (SD) z-score.02 (.98)). Moderate positive correlations were found between PA and PF (r(39) = .378, p < .001). Moderately sized negative correlations were found between pain intensity and fatigue and time spent actively (r(35) = .408, p < .001 and r(24) = .395 p < .001, respectively).ConclusionThis study is the first to demonstrate reduced PA and PF in children with HCTD. PF was moderately positively correlated with PA and negatively correlated with pain intensity and fatigue. Reduced cardiovascular endurance, muscle strength, and deconditioning, combined with disorder-specific cardiovascular and musculoskeletal features, are hypothesized to be causal. Identifying the limitations in PA and PF provides a starting point for tailor-made interventions

Somatic symptoms, pain, catastrophizing and the association with disability among children with heritable connective tissue disorders

The aim of the present study was to investigate the nature and prevalence of nonspecific somatic symptoms, pain and catastrophizing in children with Heritable Connective Tissue Disorders (HCTD), and to determine their association with disability. This observational, multicenter study included 127 children, aged 4-18 years, with Marfan syndrome (MFS) (59%), Loeys-Dietz syndrome (LDS) (8%), Ehlers-Danlos syndromes (EDS) (12%) and hypermobile Ehlers-Danlos syndrome (hEDS) (23%). The assessments included the Children's Somatization Inventory or parent proxy (CSI, PCSI), pain visual-analogue scale (VAS), SUPERKIDZ body diagram, Pain Catastrophizing Scale Child or parent proxy (PCS-C, PCS-P) and Childhood Health Assessment Questionnaire (CHAQ-30). Data from children aged >= 8 years were compared to normative data. In children >= 8 years (n = 90), pain was present in 59%, with a median of 4 (IQR = 3-9) pain areas. Compared to normative data, the HCTD group reported significantly higher on the CSI (p <= 0.001, d = 0.85), VAS pain intensity (p <= 0.001, d = 1.22) and CHAQ-30 (p <= 0.001, d = 1.16) and lower on the PCS-C (p = 0.017, d = -0.82) and PCS-P (p <= 0.001, d = -0.49). The intensity of nonspecific somatic symptoms and pain explained 45% of the variance in disability (r(2) = 0.45 F(2,48) = 19.70, p <= 0.001). In children <= 7 years (n = 37), pain was present in 35% with a median of 5(IQR = 1-13) pain areas. The mean(SD) VAS scores for pain intensity was 1.5(2.9). Functional disability was moderately correlated to the number of pain areas (r = 0.56, p <= 0.001), intensity of nonspecific somatic symptoms (r = 0.63, p <= 0.001) and pain (r = 0.83, p <= 0.001). In conclusion, this study supports the need for comprehensive assessment of nonspecific somatic symptoms, pain, and disability in children with HCTD to allow tailored treatment

Computational Literary Studies Infrastructure (CLSINFRA): a H2020 Research Infrastructure Project that aids to connect researchers, data, and methods

The aim of this poster is to provide an overview of the principal objectives of the newly started H2020 Computational Literary Studies (CLS) project- https://www.clsinfra.io. CLS is a infrastructure project works to develop and bring together resources of high-quality data, tools and knowledge to aid new approaches to studying literature in the digital age. Conducting computational literary studies has a number of challenges and opportunities from multilingual and bringing together distributing information. At present, the landscape of literary data is diverse and fragmented. Even though many resources are currently available in digital libraries, archives, repositories, websites or catalogues, a lack of standardisation hinders how they are constructed, accessed and the extent to which they are reusable (Ciotti 2014). CLS project aims to federate these resources, with the tools needed to interrogate them, and with a widened base of users, in the spirit of the FAIR and CARE principles (Wilkinson et al. 2016). The resulting improvements will benefit researchers by bridging gaps between greater- and lesser- resourced communities in computational literary studies and beyond, ultimately offering opportunities to create new research and insight into our shared and varied European cultural heritage. Rather than building entirely new resources for literary studies, the project is committed to exploiting and connecting the already-existing efforts and initiatives, in order to acknowledge and utilize the immense human labour that has already been undertaken. Therefore, the project builds on recently- compiled high-quality literary corpora, such as DraCor and ELTeC (Fischer et al. 2019, Burnard et al. 2021, Schöch et al. in press), integrates existing tools for text analysis, e.g. TXM, stylo, multilingual NLP pipelines (Heiden 2010, Eder et al. 2016), and takes advantage of deep integration with two other infrastructural projects, namely the CLARIN and DARIAH ERICs. Consequently, the project aims at building a coherent ecosystem to foster the technical and intellectual findability and accessibility of relevant data. The ecosystem consists of (1) resources, i.e. text collections for drama, poetry and prose in several languages, (2) tools, (3) methodological and theoretical considerations, (4) a network of CLS scholars based at different European institutions, (5) a system of short-term research stays for both early career researchers and seasoned scholars, (6) a repository for training materials, as well as (7) an efficient dissemination strategy. This is achieved through a collaboration between participating institutions: Institute of Polish Language at the Polish Academy of Sciences, Poland; University of Potsdam, Germany; Austrian Academy of Sciences, Austria; National University of Distance Education, Spain; École Normale Supérieure de Lyon, France; Humboldt University of Berlin, German; Charles University, Czech Republic; Digital Research Infrastructure for the Arts and Humanities, France; Ghent Centre for Digital Humanities, Ghent University, Belgium; Belgrade Centre for Digital Humanities, Serbia; Huygens Institute for the History of the Netherlands (Royal Netherlands Academy of Arts and Sciences), Netherlands; Trier Center for Digital Humanities, Trier University, Germany; Moore Institute, National University of Ireland Galway, Ireland

Heritable connective tissue disorders in childhood : increased fatigue, pain, disability and decreased general health

Heritable Connective Tissue Disorders (HCTD) show an overlap in the physical features that can evolve in childhood. It is unclear to what extent children with HCTD experience burden of disease. This study aims to quantify fatigue, pain, disability and general health with standardized validated questionnaires. Methods. This observational, multicenter study included 107 children, aged 4-18 years, with Marfan syndrome (MFS), 58%; Loeys-Dietz syndrome (LDS), 7%; Ehlers-Danlos syndromes (EDS), 8%; and hypermobile Ehlers-Danlos syndrome (hEDS), 27%. The assessments included PROMIS Fatigue Parent-Proxy and Pediatric self-report, pain and general health Visual-Analogue-Scales (VAS) and a Childhood Health Assessment Questionnaire (CHAQ). Results. Compared to normative data, the total HCTD-group showed significantly higher parent-rated fatigue T-scores (M = 53 (SD = 12), p = 0.004, d = 0.3), pain VAS scores (M = 2.8 (SD = 3.1), p < 0.001, d = 1.27), general health VAS scores (M = 2.5 (SD = 1.8), p < 0.001, d = 2.04) and CHAQ disability index scores (M = 0.9 (SD = 0.7), p < 0.001, d = 1.23). HCTD-subgroups showed similar results. The most adverse sequels were reported in children with hEDS, whereas the least were reported in those with MFS. Disability showed significant relationships with fatigue (p < 0.001, r(s) = 0.68), pain (p < 0.001, r(s) = 0.64) and general health (p < 0.001, r(s) = 0.59). Conclusions. Compared to normative data, children and adolescents with HCTD reported increased fatigue, pain, disability and decreased general health, with most differences translating into very large-sized effects. This new knowledge calls for systematic monitoring with standardized validated questionnaires, physical assessments and tailored interventions in clinical care

CLS Infra Computational Literary Studies Infrastructure

Computational Literary Studies Infrastructure, funded by the Horizon2020 grant scheme, is a four-year, pan-European project that aims to unify the diverse landscape of computational text analysis, in terms of available texts, tools, methods, practices and so forth, within its growing international user community. The project started out in February 2021, meaning that it has been underway for just over a year. In our poster we discuss the various deliverables and activities that have come out of the CLS INFRA project in its first quarter to give an idea of its impact in practice

Heritable connective tissue disorders in childhood : increased fatigue, pain, disability and decreased general health

Heritable Connective Tissue Disorders (HCTD) show an overlap in the physical features that can evolve in childhood. It is unclear to what extent children with HCTD experience burden of disease. This study aims to quantify fatigue, pain, disability and general health with standardized validated questionnaires. Methods. This observational, multicenter study included 107 children, aged 4-18 years, with Marfan syndrome (MFS), 58%; Loeys-Dietz syndrome (LDS), 7%; Ehlers-Danlos syndromes (EDS), 8%; and hypermobile Ehlers-Danlos syndrome (hEDS), 27%. The assessments included PROMIS Fatigue Parent-Proxy and Pediatric self-report, pain and general health Visual-Analogue-Scales (VAS) and a Childhood Health Assessment Questionnaire (CHAQ). Results. Compared to normative data, the total HCTD-group showed significantly higher parent-rated fatigue T-scores (M = 53 (SD = 12), p = 0.004, d = 0.3), pain VAS scores (M = 2.8 (SD = 3.1), p < 0.001, d = 1.27), general health VAS scores (M = 2.5 (SD = 1.8), p < 0.001, d = 2.04) and CHAQ disability index scores (M = 0.9 (SD = 0.7), p < 0.001, d = 1.23). HCTD-subgroups showed similar results. The most adverse sequels were reported in children with hEDS, whereas the least were reported in those with MFS. Disability showed significant relationships with fatigue (p < 0.001, r(s) = 0.68), pain (p < 0.001, r(s) = 0.64) and general health (p < 0.001, r(s) = 0.59). Conclusions. Compared to normative data, children and adolescents with HCTD reported increased fatigue, pain, disability and decreased general health, with most differences translating into very large-sized effects. This new knowledge calls for systematic monitoring with standardized validated questionnaires, physical assessments and tailored interventions in clinical care