Colour and Colorimetry Multidisciplinary Contributions Vol. XIb

It is well known that the subject of colour has an impact on a range of disciplines. Colour has been studied in depth for many centuries, and as well as contributing to theoretical and scientific knowledge, there have been significant developments in applied colour research, which has many implications for the wider socio-economic community. At the 7th Convention of Colorimetry in Parma, on the 1st October 2004, as an evolution of the previous SIOF Group of Colorimetry and Reflectoscopy founded in 1995, the "Gruppo del Colore" was established. The objective was to encourage multi and interdisciplinary collaboration and networking between people in Italy that addresses problems and issues on colour and illumination from a professional, cultural and scientific point of view. On the 16th of September 2011 in Rome, in occasion of the VII Color Conference, the members assembly decided to vote for the autonomy of the group. The autonomy of the Association has been achieved in early 2012. These are the proceedings of the English sessions of the XI Conferenza del Colore

Assessment of Lake Orta sediments phytotoxicity after the liming treatment

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Assessment-driven selection and adaptation of exercise difficulty in robot-assisted therapy: a pilot study with a hand rehabilitation robot

Background Selecting and maintaining an engaging and challenging training difficulty level in robot-assisted stroke rehabilitation remains an open challenge. Despite the ability of robotic systems to provide objective and accurate measures of function and performance, the selection and adaptation of exercise difficulty levels is typically left to the experience of the supervising therapist. Methods We introduce a patient-tailored and adaptive robot-assisted therapy concept to optimally challenge patients from the very first session and throughout therapy progress. The concept is evaluated within a four-week pilot study in six subacute stroke patients performing robot-assisted rehabilitation of hand function. Robotic assessments of both motor and sensory impairments of hand function conducted prior to the therapy are used to adjust exercise parameters and customize difficulty levels. During therapy progression, an automated routine adapts difficulty levels from session to session to maintain patients’ performance around a target level of 70%, to optimally balance motivation and challenge. Results Robotic assessments suggested large differences in patients’ sensorimotor abilities that are not captured by clinical assessments. Exercise customization based on these assessments resulted in an average initial exercise performance around 70% (62% ± 20%, mean ± std), which was maintained throughout the course of the therapy (64% ± 21%). Patients showed reduction in both motor and sensory impairments compared to baseline as measured by clinical and robotic assessments. The progress in difficulty levels correlated with improvements in a clinical impairment scale (Fugl-Meyer Assessment) (r s = 0.70), suggesting that the proposed therapy was effective at reducing sensorimotor impairment. Conclusions Initial robotic assessments combined with progressive difficulty adaptation have the potential to automatically tailor robot-assisted rehabilitation to the individual patient. This results in optimal challenge and engagement of the patient, may facilitate sensorimotor recovery after neurological injury, and has implications for unsupervised robot-assisted therapy in the clinic and home environment.ISSN:1743-000

A new methodology for probabilistic flood displacement risk assessment: the case of Fiji and Vanuatu

This paper presents an enhanced probabilistic flood displacement risk assessment methodology. Several techniques have been proposed to estimate the number of people at risk of being displaced triggered due to climatic extremes. Among these methods, the probabilistic approach is promising for its quantitative nature and versatility at different scales. However, it has so far been limited to assessing loss of housing as the sole cause of displacement. The proposed methodology addresses this limitation by considering two additional elements beyond the traditional evaluation of housing loss: the likelihood of losing means of livelihood, directly included in the computation, and the likelihood of losing access to essential services, such as schools and health centers, provided as a factor to increase the propensity to displace. This new methodology is applied to assess flood disaster displacement risk in Fiji and Vanuatu, where climate change, coupled with the vulnerability of exposed assets, poses an existential threat to these Pacific islands, potentially leading to internal and cross-border population movements. Different climate scenarios were considered: current climate conditions (1979–2016 period), medium-term projected climate conditions (2016–2060), and long-term projected climate conditions (2061–2100). The average annual displacement increases in Fiji and Vanuatu by a factor of 3 and 4, respectively, in the projected long-term pessimistic climate scenario compared to current conditions. Depending on the country and climate change scenario, 20 to 40% of these displacements stem from loss of livelihoods as a dominant factor, highlighting the importance of considering this aspect in the vulnerability approach. The outcomes of these scenarios serve as the foundation for implementing displacement risk adaptation and management measures. This novel quantitative methodology holds significant potential for applications in larger domains and even globally

Prognostic significance of normalized FDG-PET parameters in patients with multiple myeloma undergoing induction chemotherapy and autologous hematopoietic stem cell transplantation: a retrospective single-center evaluation

Purpose: The purpose of this study was to determine retrospectively, through a single-center evaluation, whether FDG PET-CT normalized semi-quantitative parameters may predict response to induction chemotherapy (iChT) and hematopoietic stem cell transplantation (HSCT), as well as disease progression and progression-free survival in multiple myeloma (MM) patients, thus becoming a tool of personalized medicine. Methods: Patients undergoing iChT and HSCT with baseline and post-treatment FDG PET-CTs from January 2008 to July 2015 were included. The following baseline and post-treatment parameters were obtained: SUVmax, SUVmean, SUVpeak, MTVsum, TLGsum, rPET (lesion SUVmax/liver SUVmax) and qPET (lesion SUVpeak/liver SUVmean). Baseline-to-post-treatment changes (Δ) were also calculated. Metabolic and clinical laboratory progression or response at follow-up were noted; time-to-metabolic-progression (TMP) was defined as the interval from post-treatment scan to eventual progression at follow-up FDG PET-CTs. Possible association between each functional parameter and metabolic/clinical-laboratory progression or response was determined. Kaplan-Meier curves allowed to depict the TMP trend according to FDG PET-CT parameters. Results: Twenty-eight patients were included. Significantly higher ΔrPET and ΔqPET values were observed in ten patients with “metabolic response”, with respect to 18 patients having “metabolic progression” (median 0.62 [IQR 0.32 – 1.34] vs median 0.00 [IQR -0.25 – 0.49] for ΔrPET; P = 0.045; median 0.51 [IQR 0.32 – 1.13] vs median 0.00 [IQR -0.31 – 0.67] for ΔqPET; P = 0.035). Neither normalized nor non normalized parameters differed significantly between the 20 patients with “clinical-laboratory response” and the eight patients with “clinical-laboratory progression”. ΔrPET value lower than 0.38 and ΔqPET value lower than 0.27 predicted a significantly shorter TMP (P = 0.003 and P = 0.005, respectively). Conclusions: Normalized semi-quantitative parameters are effective in predicting persistent response to treatment and shorter TMP in patients with MM undergoing iChT and HSCT

Partial T cell defects and expanded CD56bright NK cells in an SCID patient carrying hypomorphic mutation in the IL2RG gene

X-linked severe combined immunodeficiency (X-SCID) caused by full mutation of the IL2RG gene leads to T- B+ NK- phenotype and is usually associated with severe opportunistic infections, diarrhea, and failure to thrive. When IL2RG hypomorphic mutation occurs, diagnosis could be delayed and challenging since only moderate reduction of T and NK cells may be present. Here, we explored phenotypic insights and the impact of the p.R222C hypomorphic mutation (IL2RGR222C ) in distinct cell subsets in an 8-month-old patient with atypical X-SCID. We found reduced CD4+ T cell counts, a decreased frequency of naïve CD4+ and CD8+ T cells, and an expansion of B cells. Ex vivo STAT5 phosphorylation was impaired in CD4+ CD45RO+ T cells, yet compensated by supraphysiological doses of IL-2. Sanger sequencing on purified cell subsets showed a partial reversion of the mutation in total CD3+ cells, specifically in recent thymic emigrants (RTE), effector memory (EM), and CD45RA+ terminally differentiated EM (EMRA) CD4+ T cells. Of note, patient's NK cells had a normal frequency compared to age-matched healthy subjects, but displayed an expansion of CD56bright cells with higher perforin content and cytotoxic potential, associated with accumulation of NK-cell stimulatory cytokines (IL-2, IL-7, IL-15). Overall, this report highlights an alteration in the NK-cell compartment that, together with the high disease-phenotype variability, should be considered in the suspicion of X-SCID with hypomorphic IL2RG mutation

Baseline Demographics, Comorbidities, Treatment Patterns and Burden of Atopic Dermatitis in Adults and Adolescents from the GLOBOSTAD Long-Term Observational Study

Introduction - Insights into real-world treatment of atopic dermatitis (AD) are relevant to clinical decision making. The aim of this analysis was to characterize patients who receive dupilumab for AD in a real-world setting. Methods - The GLOBOSTAD registry is an ongoing, longitudinal, prospective, observational study of patients with AD who receive dupilumab according to country-specific prescribing information. We report baseline characteristics, comorbidities and treatment patterns for patients enrolled from July 11, 2019 to March 31, 2022. Analyses are descriptive; no formal statistical comparisons were performed. Results - Nine hundred fifty-two adults and adolescents were enrolled in GLOBOSTAD. Patients had a high disease burden before starting dupilumab: (mean [standard deviation]) percent body surface area affected (44.8 [24.42]), Eczema Area and Severity Index total score (24.8 [12.95]), SCORing Atopic Dermatitis total score (60.5 [16.34]), Patient-Oriented Eczema Measure total score (19.7 [6.37]) and Dermatology Life Quality Index total score (13.7 [7.02]). Overall, 741 (77.8%) patients reported ≥ 1 type 2 inflammatory comorbidities, most frequently allergic rhinitis (492 [51.7%]), asthma (323 [33.9%]), food allergy (294 [30.9%]) or another allergy (274 [28.8%]). In the previous 12 months, 310 (32.6%) patients had received systemic non-steroidal immunosuppressants and 169 (17.8%) systemic corticosteroids; 449 (47.2%) had received topical corticosteroids, most commonly potent topical corticosteroids; 141 (14.8%) had received topical calcineurin inhibitors and 32 (3.4%) ultraviolet therapy. Most (713 [74.9%]) patients started dupilumab because of prior treatment failure. Conclusion - Patients enrolled in GLOBOSTAD demonstrated considerable multidimensional burden of disease across AD signs, symptoms and quality of life despite previous use of systemic and non-systemic AD treatments

GEN-O-MA project: an Italian network studying clinical course and pathogenic pathways of moyamoya disease—study protocol and preliminary results

Background: GENetics of mOyaMoyA (GEN-O-MA) project is a multicenter observational study implemented in Italy aimed at creating a network of centers involved in moyamoya angiopathy (MA) care and research and at collecting a large series and bio-repository of MA patients, finally aimed at describing the disease phenotype and clinical course as well as at identifying biological or cellular markers for disease progression. The present paper resumes the most important study methodological issues and preliminary results. Methods: Nineteen centers are participating to the study. Patients with both bilateral and unilateral radiologically defined MA are included in the study. For each patient, detailed demographic and clinical as well as neuroimaging data are being collected. When available, biological samples (blood, DNA, CSF, middle cerebral artery samples) are being also collected for biological and cellular studies. Results: Ninety-eight patients (age of onset mean ± SD 35.5 ± 19.6 years; 68.4% females) have been collected so far. 65.3% of patients presented ischemic (50%) and haemorrhagic (15.3%) stroke. A higher female predominance concomitantly with a similar age of onset and clinical features to what was reported in previous studies on Western patients has been confirmed. Conclusion: An accurate and detailed clinical and neuroimaging classification represents the best strategy to provide the characterization of the disease phenotype and clinical course. The collection of a large number of biological samples will permit the identification of biological markers and genetic factors associated with the disease susceptibility in Italy

Cross sections for the γp→K*+Λ and γp→K*+Σ0 reactions measured at CLAS

The first high-statistics cross sections for the reactions γp→K*+Λ and γp→K*+Σ0 were measured using the CLAS detector at photon energies between threshold and 3.9 GeV at the Thomas Jefferson National Accelerator Facility. Differential cross sections are presented over the full range of the center-of-mass angles, and then fitted to Legendre polynomials to extract the total cross section. Results for the K*+Λ final state are compared with two different calculations in an isobar and a Regge model, respectively. Theoretical calculations significantly underestimate the K*+Λ total cross sections between 2.1 and 2.6 GeV, but are in better agreement with present data at higher photon energies

Zebrafish Tric-b is required for skeletal development and bone cells differentiation

IntroductionTrimeric intracellular potassium channels TRIC-A and -B are endoplasmic reticulum (ER) integral membrane proteins, involved in the regulation of calcium release mediated by ryanodine (RyRs) and inositol 1,4,5-trisphosphate (IP3Rs) receptors, respectively. While TRIC-A is mainly expressed in excitable cells, TRIC-B is ubiquitously distributed at moderate level. TRIC-B deficiency causes a dysregulation of calcium flux from the ER, which impacts on multiple collagen specific chaperones and modifying enzymatic activity, leading to a rare form of osteogenesis imperfecta (OI Type XIV). The relevance of TRIC-B on cell homeostasis and the molecular mechanism behind the disease are still unknown.ResultsIn this study, we exploited zebrafish to elucidate the role of TRIC-B in skeletal tissue. We demonstrated, for the first time, that tmem38a and tmem38b genes encoding Tric-a and -b, respectively are expressed at early developmental stages in zebrafish, but only the latter has a maternal expression. Two zebrafish mutants for tmem38b were generated by CRISPR/Cas9, one carrying an out of frame mutation introducing a premature stop codon (tmem38b-/-) and one with an in frame deletion that removes the highly conserved KEV domain (tmem38bΔ120-7/Δ120-7). In both models collagen type I is under-modified and partially intracellularly retained in the endoplasmic reticulum, as described in individuals affected by OI type XIV. Tmem38b-/- showed a mild skeletal phenotype at the late larval and juvenile stages of development whereas tmem38bΔ120-7/Δ120-7 bone outcome was limited to a reduced vertebral length at 21 dpf. A caudal fin regeneration study pointed towards impaired activity of osteoblasts and osteoclasts associated with mineralization impairment.DiscussionOur data support the requirement of Tric-b during early development and for bone cell differentiation