Bone mass and quality in patients with juvenile idiopathic arthritis: longitudinal evaluation of bone mass determinants using dual energy X-ray absorptiometry, peripheral quantitative computed tomography, and quantitative ultrasonography.

INTRODUCTION: Our objective was to evaluate longitudinally the main bone-mass and quality predictors in young juvenile idiopathic arthritis (JIA) patients by using lumbar spine dual-energy X-ray absorptiometry (DXA) scan, radius peripheral quantitative computed tomography (pQCT), and phalangeal quantitative ultrasonography (QUS) at the same time. METHODS: In total, 245 patients (172 females, 73 males; median age, 15.6 years: 148 oligoarticular, 55 polyarticular, 20 systemic, and 22 enthesitis-related-arthritis (ERA) onset) entered the study. Of these, 166 patients were evaluated longitudinally. Data were compared with two age- and sex-matched control groups. RESULTS: In comparison with controls, JIA patients, but not with ERA, had a reduced spine bone-mineral apparent density (BMAD) standard deviation score (P < 0.001) and musculoskeletal deficits, with significantly lower levels of trabecular bone mineral density (TrabBMD) (P < 0.0001), muscle cross-sectional area (CSA) (P < 0.005), and density-weighted polar section modulus (SSIp) (P < 0.05). In contrast, JIA showed fat CSA significantly higher than controls (P < 0.0001). Finally, JIA patients had a significant reduced amplitude-dependent speed of sound (AD-SoS) (P < 0.001), and QUS z score (P < 0.005). Longitudinally, we did not find any difference in all JIA patients in comparison with baseline, except for the SSIp value that normalized. Analyzing the treatments, a significant negative correlation among spine BMAD values, TrabBMD, AD-SoS, and systemic and/or intraarticular corticosteroids, and a positive correlation among TNF-α-blocking agents and spine BMAD, TrabBMD, and AD-SoS were observed. CONCLUSIONS: JIA patients have a low bone mass that, after a first increase due to the therapy, does not reach the normal condition over time. The pronounced bone deficits in JIA are greater than would be expected because of reduction in muscle cross-sectional area. Thus, bone alterations in JIA likely represent a mixed defect of bone accrual and lower muscle forces

Monitoring and Evaluation as a Way to Complete and to Implement a Policy : The Case of Brazilian National Innovation Policy

The present National Innovation Policy (henceforth called PNI) was instituted in Brazil by Decree No. 10.537, of October 28, 2020, within the federal public administration. The policy's objective is to guide, coordinate and articulate strategies, programs, and actions across several public entities to foster innovation in the productive sector. Thus, it aims to increase the productivity and competitiveness of companies and other institutions throughout the innovation process. The policy is structured around six axes: funding innovation, innovation culture, technological background, market development, educational systems, and intellectual property. Objectives and guidelines are defined for each of these Axes. However, neither a previous Theory of Change, a Logical Framework, nor a monitoring and evaluation system was established to guide the implementation. Operationally speaking, the policy was implemented by gathering ongoing actions from eighteen different federal ministries and agencies and then assigning them to the axes above. It was an ad hoc measure to put the policy into practice. As can be inferred, the selected actions did not emerge as a breakdown from axes. In 2021 the design and implementation of a monitoring and evaluation model (M&E) were commissioned to an independent evaluation group. The objective was to develop an original methodology and indicators for monitoring and evaluating outputs, outcomes and impacts of the PNI. The challenge of the model is twofold: to adjust the matching amongst actions, axes, and the policy's objectives, and to implement a common framework for M&E able to coordinate the involved agencies. A Theory of Change and a Logical Framework were ex-post designed and validated, and based on these references, the model was conceived. It defined a classical template for monitoring the ongoing actions (using flags and lights according to execution), adding indicators of outcomes and impacts. It is an integrated M&E model that, once implemented, will require that new actions be justified not only in terms of their outputs but also on the expected outcomes and impacts. Standard indicators of science, technology and innovation outcomes and impacts from different sources as for OECD, UNESCO, Global Innovation Index and Sustainable Development Goals (SDG/UN) have been gathered and organized to be employed in the M&E process. Non-standard indicators are also foreseen so that the model can measure internationally comparable and specific indicators. The whole model is now under development and validation among stakeholders. We expect this process to go beyond the domain of M&E and influences the way priorities are defined, and governance is established and run. By involving different actors, incorporating the SDGs into the policy's core, the evaluation process facilitated the policy's implementation and coordination. Furthermore, the articulation between the different stakeholders, promoted by the M&E, minimized fragmentation and uncoordinated actions within the scope of the policy's formulation. It is in this sense that the evaluation process is a way to transform the policy itsel

Quantifying bed roughness beneath contemporary and palaeo-ice streams

Bed roughness is an important control on ice-stream location and dynamics. The majority of previous bed roughness studies have been based on data derived from radio echo sounding (RES) transects across Antarctica and Greenland. However, the wide spacing of RES transects means that the links between roughness and flow are poorly constrained. Here, we use Digital Terrain Model (DTM)/bathymetry data from a well-preserved palaeo-ice stream to investigate basal controls on the behaviour of contemporary ice streams. Artificial transects were set up across the Minch Palaeo-Ice Stream (NW Scotland) to mimic RES flight lines over Institute and Möller Ice Streams (Antarctica). We then explored how different data-resolution, transect orientation and spacing, and different methods, impact upon roughness measurements. Our results show that fast palaeo-ice flow can occur over a rough, hard bed, not just a smooth, soft bed, which much previous work has suggested. Smooth areas of the bed occur over both bedrock and sediment covered regions. Similar trends in bed roughness values were found using Fast Fourier Transform analysis and standard deviation methods. Smoothing of bed roughness results can hide important details. We propose that the typical spacing of RES transects is too wide to capture different landform assemblages, and that transect orientation influences bed roughness measurements in both contemporary and palaeo-ice-stream setting

Tumor-Infiltrating Lymphocytes (TILs) and Risk of a Second Breast Event After a Ductal Carcinoma in situ

Women with a diagnosis of ductal carcinomain situ(DCIS) have a high risk of developing a second breast event (SBE). The immune system might play a role in trying to prevent a SBE. Patients diagnosed with DCIS were identified in the population-based cancer registry of Area Vasta Romagna from 1997 to 2010. Median follow-up is 8.5 years. Tumor-infiltrating lymphocytes (TILs) were evaluated both in index DCIS and in SBE. The main endpoint was to assess the association between TILs' levels in index DCIS and risk of a SBE. Out of 496 DCIS patients, 100 SBEs (20.2%) were identified: 55 ipsilateral (11.1%) and 43 contralateral (8.7%). The distribution of TILs was heterogeneous, but significantly associated with grade, necrosis, screen detection and type of surgery. Patients stratified according to TILs percentage (5%) did not show a statistically significant difference in the 5-year cumulative incidence of SBEs: 14.9% (95% CI 11.3-19.1) and 11.0% (95% CI, 6.9-16.2), respectively (p= 0.147). In the subgroup of patients who did not receive radiotherapy, TILs >5% were associated with a reduced risk of SBE (HR 0.34, 95% CI 0.14-0.82,p= 0.016). Although we did not find any significant association between TILs and SBE, further studies evaluating their role according to radiotherapy are warranted

The still under-investigated role of cognitive deficits in PML diagnosis

Background: Despite cognitive deficits frequently represent the first clinical manifestations of Progressive Multifocal Leukoencephalopathy (PML) in Natalizumab-treated MS patients, the importance of cognitive deficits in PML diagnosis is still under-investigated. The aim of the current study is to investigate the cognitive deficits at PML diagnosis in a group of Italian patients with PML. Methods: Thirty-four PML patients were included in the study. The demographic and clinical data, the lesion load and localization, and the longitudinal clinical course was compared between patients with (n = 13) and without (n = 15) cognitive deficit upon PML suspicion (the remaining six patients were asymptomatic). Clinical presentation of cognitive symptoms was described in detail. Result: After symptoms detection, the time to diagnosis resulted to be shorter for patients presenting with cognitive than for patients with non cognitive onset (p = 0.03). Within patients with cognitive onset, six patients were presenting with language and/or reading difficulties (46.15%); five patients with memory difficulties (38.4%); three patients with apraxia (23.1%); two patients with disorientation (15.3%); two patients with neglect (15.3%); one patients with object agnosia (7.7%), one patient with perseveration (7.7%) and one patient with dementia (7.7%). Frontal lesions were less frequent (p = 0.03), whereas temporal lesions were slightly more frequent (p = 0.06) in patients with cognitive deficits. The longitudinal PML course seemed to be more severe in cognitive than in non cognitive patients (F = 2.73, p = 0.03), but differences disappeared (F = 1.24, p = 0.29) when balancing for the incidence of immune reconstitution syndrome and for other treatments for PML (steroids, plasma exchange (PLEX) and other therapies (Mefloquine, Mirtazapine, Maraviroc). Conclusion: Cognitive deficits at PML onset manifest with symptoms which are absolutely rare in MS. Their appearance in MS patients should strongly suggest PML. Clinicians should be sensitive to the importance of formal neuropsychological evaluation, with particular focus on executive function, which are not easily detected without a formal assessment

Characteristics of people living in Italy after a cancer diagnosis in 2010 and projections to 2020

BACKGROUND: Estimates of cancer prevalence are widely based on limited duration, often including patients living after a cancer diagnosis made in the previous 5 years and less frequently on complete prevalence (i.e., including all patients regardless of the time elapsed since diagnosis). This study aims to provide estimates of complete cancer prevalence in Italy by sex, age, and time since diagnosis for all cancers combined, and for selected cancer types. Projections were made up to 2020, overall and by time since diagnosis. METHODS: Data were from 27 Italian population-based cancer registries, covering 32% of the Italian population, able to provide at least 7 years of registration as of December 2009 and follow-up of vital status as of December 2013. The data were used to compute the limited-duration prevalence, in order to estimate the complete prevalence by means of the COMPREV software. RESULTS: In 2010, 2,637,975 persons were estimated to live in Italy after a cancer diagnosis, 1.2 million men and 1.4 million women, or 4.6% of the Italian population. A quarter of male prevalent cases had prostate cancer (n\u2009=\u2009305,044), while 42% of prevalent women had breast cancer (n\u2009=\u2009604,841). More than 1.5 million people (2.7% of Italians) were alive since 5 or more years after diagnosis and 20% since 6515 years. It is projected that, in 2020 in Italy, there will be 3.6 million prevalent cancer cases (+\u200937% vs 2010). The largest 10-year increases are foreseen for prostate (+\u200985%) and for thyroid cancers (+\u200979%), and for long-term survivors diagnosed since 20 or more years (+\u200945%). Among the population aged 6575 years, 22% will have had a previous cancer diagnosis. CONCLUSIONS: The number of persons living after a cancer diagnosis is estimated to rise of approximately 3% per year in Italy. The availability of detailed estimates and projections of the complete prevalence are intended to help the implementation of guidelines aimed to enhance the long-term follow-up of cancer survivors and to contribute their rehabilitation need

The instrument control unit of the ARIEL payload: design evolution following the unit and payload subsystems SRR (system requirements review)

ARIEL (Atmospheric Remote-sensing InfraRed Large-survey) is a medium-class mission of the European Space Agency, part of the Cosmic Vision program, whose launch is foreseen by early 2029. ARIEL aims to study the composition of exoplanet atmospheres, their formation and evolution. The ARIEL’s target will be a sample of about 1000 planets observed with one or more of the following methods: transit, eclipse and phase-curve spectroscopy, at both visible and infrared wavelengths simultaneously. The scientific payload is composed by a reflective telescope having a 1m-class elliptical primary mirror, built in solid Aluminium, and two focal-plane instruments: FGS and AIRS. FGS (Fine Guidance System)1 has the double purpose, as suggested by its name, of performing photometry (0.50-0.55 µm) and low resolution spectrometry over three bands (from 0.8 to 1.95 µm) and, simultaneously, to provide data to the spacecraft AOCS (Attitude and Orbit Control System) with a cadence of 10 Hz and contributing to reach a 0.02 arcsec pointing accuracy for bright targets. AIRS (ARIEL InfraRed Spectrometer) instrument will perform IR spectrometry in two wavelength ranges: between 1.95 and 3.9 µm (with a spectral resolution R > 100) and between 3.9 and 7.8 µm with a spectral resolution R > 30. This paper provides the status of the ICU (Instrument Control Unit), an electronic box whose purpose is to command and supply power to AIRS (as well as acquire science data from its two channels) and to command and control the TCU (Telescope Control Unit)