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Background: Neonatal cerebral stroke includes a range of focal and multifocal ischemic and hemorrhagic brain lesions, occurring in about one of 3000 live births. More than 50% of children with neonatal stroke develop adverse outcomes, mainly unilateral cerebral palsy. Asymmetries in segmental movements at three months have been proven to be an early sign of CP in infants with unilateral brain damage. Recognition of additional early signs could enhance prognostic assessment and enable an early and targeted intervention. Aim: The aim of the study was to assess early signs of CP in infants with arterial cerebral stroke through the General Movements Assessment and the Motor Optimality Score—Revised (MOS-R). Method: Twenty-four infants born at term (12 females and 12 males) diagnosed with ACS, and 24 healthy infants (16 females and 8 males) were assessed. The GMs (fidgety movements) and MOS-R were assessed from videos recorded at 11–14 weeks of post-term age. Cognitive and motor outcomes were assessed at 24 months using the Griffiths III developmental quotient and Amiel-Tison neurological examination. The gross motor function classification system expanded and revised (GMFCS-E&R) was adopted to categorize CP. Results: Among infants with ACS, 21 (87.5%) developed unilateral CP. Most of them showed non-disabling CP (14 had GMFCS-E&R grade 1 [66.6%], 6 grade 2 [28.6%], and 1 grade 5 [4.8%]). Fidgety movements (FMs) were absent in 17 (70.8%), sporadic in 4 (16.7%) infants, and normal in 3 (12.5%). Segmental movement asymmetry was found in 22/24 (91.7%). According to the MOS-R, motor items (kicking, mouth movements), postural patterns (midline centered head, finger posture variability), and movement character (monotonous and stiff) were statistically different among infants with ACS and healthy infants. The MOS-R median global score was lower in the group with ACS compared to the control group (6 vs 26; p < 0.01). FMs, segmental movement asymmetry, and MOS-R global score were significantly correlated with abnormal outcome. MOS-R global scores less than or equal to 13 had 100% specificity and sensitivity in predicting GMFCS-E&R grade ≥ 2 CP in infants with ACS. Conclusions: The rate of CP was high among infants with ACS, but in most cases it showed low GMFCS-E&R grades. The study highlighted a significant correlation between MOS-R, together with absent FMs and unilateral CP in infants with ACS. Moreover, the MOS-R showed high sensitivity and specificity in the prediction of CP. Combined assessment of FMs and MOS-R could help to better identify infants at high risk of developing UCP in a population of infants with ACS. Early identification of precocious signs of unilateral CP is fundamental to providing an early individualized intervention. [ABSTRACT FROM AUTHOR]

Despite its high importance for crop yield prediction and monitoring, early-season crop mapping is severely hampered by the absence of timely ground truth. To cope with this issue, this study aims at evaluating the capability of PRISMA hyperspectral satellite images compared with Sentinel-2 multispectral imagery to produce early- and in-season crop maps using consolidated machine and deep learning algorithms. Results show that the accuracy of crop type classification using Sentinel-2 images is meaningfully poor compared with PRISMA (14% in overall accuracy (OA)). The 1D-CNN algorithm, with 89%, 91%, and 92% OA for winter, summer, and perennial cultivations, respectively, shows for the PRISMA images the highest accuracy in the in-season crop mapping and the fastest algorithm that achieves acceptable accuracy (OA 80%) for the winter, summer, and perennial cultivations early-season mapping using PRISMA images. Moreover, the 1D-CNN algorithm shows a limited reduction (6%) in performance, appearing to be the best algorithm for crop mapping within operational use in cross-farm applications. Machine/deep learning classification algorithms applied on the test fields cross-scene demonstrate that PRISMA hyperspectral time series images can provide good results for early- and in-season crop mapping. [ABSTRACT FROM AUTHOR]

Food allergy (FA) has shown an increasing prevalence in the last decades, becoming a major public health problem. However, data on the prevalence of FA across the world are heterogeneous because they are influenced by several factors. Among IgE-mediated FA, an important role is played by FA related to plant-derived food which can result from the sensitization to a single protein (specific FA) or to homologous proteins present in different foods (cross-reactive FA) including non-specific lipid transfer proteins (nsLTPs), profilins, and pathogenesis-related class 10 (PR-10). In addition, the clinical presentation of FA is widely heterogeneous ranging from mild symptoms to severe reactions up to anaphylaxis, most frequently associated with nsLTP-related FA (LTP syndrome). Considering the potential life-threatening nature of nsLTP-related FA, the patient's geographical setting should always be taken into account; thereby, it is highly recommended to build a personalized approach for managing FA across the world in the precision medicine era. For this reason, in this review, we aim to provide an overview of the prevalence of nsLTP-mediated allergies in the Mediterranean area and to point out the potential reasons for the different geographical significance of LTP-driven allergies with a particular focus on the allergenic properties of food allergens and their cross reactivity. [ABSTRACT FROM AUTHOR]

Family acceptance or rejection has mediating effects on the mental health of LGBTQ + young adults. Restrictive measures imposed during the COVID-19 pandemic forced many of these individuals to exclusively interface with their family of origin, with significant consequences on their psychological well-being. This study aims to explore, from a qualitative perspective, the subjective experience of Italian LGBTQ + young adults during the COVID-19-related home isolation. An online semi-structured interview with 13 questions was administered to a sample of 25 participants. Inclusion criteria consisted of being aged 18 years or older and having spent at least one of the three pandemic waves with the family of origin in Italy. The interviews have been processed using the Thematic Analysis methodology. The findings of the present work showed that Italian LGBTQ + young adults perceived increased distress due to a lack of privacy and acceptance from their family members, which led to forced coming-out and repression of free expression. However, some participants perceived greater psychological well-being because they experienced the family environment as safe and supportive to explore their minority identities. The presence of coping strategies also emerged, such as resilience and avoidance. [ABSTRACT FROM AUTHOR]

Background: The security measures implemented in response to the COVID-19 emergency have caused complex consequences. The aim of the present study is to examine the repercussions of the pandemic on individuals belonging to gender identity minority groups, who have experienced heightened levels of stress in comparison to the general population. Methods: Online interviews with 12 transgender participants who resided in Italy during the pandemic were conducted and subsequently analyzed following the thematic analysis methodology. Results: The majority of the participants reported an increase in stress levels primarily attributed to the lack of acceptance and support within their familial environments, obstacles encountered in accessing specialized healthcare services, and a lack of support from the broader LGBTQ+ community. Despite these challenges, several participants developed effective coping strategies and a subset of them also benefited from multiple resilience factors, including familial support and assistance from mental health professionals. Conclusions: The outcomes of the present study indicate that the COVID-19 pandemic, while fostering certain protective factors within this population, has also given rise to new and critical mental health concerns. These findings hold significant implications for professionals working with transgender populations, highlighting the necessity of addressing these emerging mental health issues. [ABSTRACT FROM AUTHOR]

Background: Improvements in perinatal care have substantially decreased mortality rates among preterm infants, yet their neurodevelopmental outcomes and quality of life persist as a pertinent public health concern. Family-centered care has emerged as a holistic philosophy that promotes effective alliances among patients, families, and healthcare providers to improve the quality of care. Aims: This longitudinal prospective study aims to evaluate the neurodevelopmental outcomes and brain MRI findings in a cohort of preterm newborns admitted to a neonatal intensive care unit (NICU) adopting a family-centered care model. Methods: Very low birth weight (VLBW) infants admitted to the NICU of Modena between 2015 and 2020 were enrolled. Infants who underwent conventional brain magnetic resonance imaging (MRI) at term-equivalent age were included. Neurodevelopmental follow-up was performed until the age of 24 months by a multidisciplinary team using the Amiel-Tison neurological assessment and the Griffiths Mental Developmental Scales (GMDS-R). Neurodevelopmental outcomes were classified as major sequelae (cerebral palsy, DQ ≤ 70, severe sensory impairment), minor sequelae (minor neurological signs such as clumsiness or DQ between 71 and 85), and normal outcomes (no neurological signs and DQ > 85). Risk factors for severe outcomes were assessed. Results: In total, 49 of the 356 infants (13.8%) died before hospital discharge, and 2 were excluded because of congenital disorders. Of the remaining 305 infants, 222 (72.8%) completed the 24 month follow-up and were included in the study. Neurodevelopmental outcomes were classified as normal (n = 173, 77.9%), minor (n = 34, 15.3%), and major sequelae (n = 15, 6.8%). Among 221 infants undergoing brain MRI, 76 (34.4%) had major lesions (intraventricular hemorrhage, hemorrhagic parenchymal infarction, periventricular leukomalacia, and large cerebellar hemorrhage). In the multivariate regression model, the retinopathy of prematurity (OR 1.8; p value 0.016) and periventricular–intraventricular hemorrhage (OR 5.6; p value < 0.004) were associated with major sequelae. Conclusions: We reported low rates of severe neurodevelopmental outcomes in VLBW infants born in an Italian NICU with FCC. Identifying the risk factors for severe outcomes can assist in tailoring and optimizing early interventions on an individual basis, both within the NICU and after discharge. [ABSTRACT FROM AUTHOR]

Sudden unexpected postnatal collapse (SUPC) is a rare event, potentially associated with catastrophic consequences. Since the beginning of the 2000s, therapeutic hypothermia (TH) has been proposed as a treatment for asphyxiated neonates after SUPC. However, only a few studies have reported the outcome of SUPC after TH. The current study presents the long-term neurodevelopmental outcome of four cases of SUPC treated with TH in a single Italian center. Furthermore, we reviewed the previous literature concerning 49 cases of SUPC treated with TH. Among 53 total cases (of whom four occurred in our center), 15 (28.3%) died before discharge from the NICU. A neurodevelopmental follow-up was available only for 21 (55.3%) out of the 38 surviving cases, and seven infants developed neurodevelopmental sequelae. TH should be considered in neonates with asphyxia after SUPC. However, SUPC is a rare event, and there is a lack of comparative clinical data to establish the risk/benefit of TH after SUPC with different degrees of asphyxia. Analysis of large cohorts of newborns with SUPC, whether treated with TH or untreated, are needed in order to better identify infants who should undergo TH. [ABSTRACT FROM AUTHOR]

NIR spectrometers based on micro-electromechanical systems (MEMS) have become available in the market, with lower prices and smaller dimensions than traditional spectrometers. MEMS technology allows for miniaturizing and reduces the cost of the spectrometers, allowing a wider use for agricultural consultants, technicians, and scientific researchers. The aim of this work was to evaluate an innovative FT-NIR MEMS spectrometer, namely the Neospectra Scanner (NS), covering the range from 1350 to 2500 nm. The assessment was performed by comparing the accuracy of prediction of soil organic carbon, texture fractions, and total calcium carbonate, obtained with NS, with that of a standard full VIS-NIR spectrometer, namely the ASD-Fieldspec Fr Pro (AF). A dataset of 182 soil samples, dried and sieved at 2 mm, collected from 4 different agricultural areas of Italy were scanned with both devices. AF showed slightly higher R2 and lower prediction error (RMSEP) than NS for all soil features, but the accuracy of the two instruments can be considered comparable. Removing the 350–1350 nm range from VIS-NIR spectra of AF, i.e., as to have the same spectral range of NS, made the prediction accuracy of AF reduced spectra (1350–2500 nm) slightly lower than that of NS. This demonstrates that the lower accuracy of the NS in soil features prediction is not due to the lower resolution of the spectra, but probably due to the lack of visible and beginning of the NIR range (350–1300 nm). [ABSTRACT FROM AUTHOR]

In March 2019, the PRISMA (PRecursore IperSpettrale della Missione Applicativa) hyperspectral satellite was launched by the Italian Space Agency (ASI), and it is currently operational on a global basis. The mission includes the hyperspectral imager PRISMA working in the 400–2500 nm spectral range with 237 bands and a panchromatic (PAN) camera (400–750 nm). This paper presents an evaluation of the PRISMA top-of-atmosphere (TOA) L1 products using different in situ measurements acquired over a fragmented rural area in Southern Italy (Pignola) between October 2019 and July 2021. L1 radiance values were compared with the TOA radiances simulated with a radiative transfer code configured using measurements of the atmospheric profile and the surface spectral characteristics. The L2 reflectance products were also compared with the data obtained by using the ImACor code atmospheric correction tool. A preliminary assessment to identify PRISMA noise characteristics was also conducted. The results showed that: (i) the PRISMA performance, as measured at the Pignola site over different seasons, is characterized by relative mean absolute differences (RMAD) of about 5–7% up to 1800 nm, while a decrease in accuracy was observed in the SWIR; (ii) a coherent noise could be observed in all the analyzed images below the 630th scan line, with a frequency of about 0.3–0.4 cycles/pixel; (iii) the most recent version of the standard reflectance L2 product (i.e., Version 2.05) matched well the reflectance values obtained by using the ImACor atmospheric correction tool. All these preliminary results confirm that PRISMA imagery is suitable for an accurate retrieval of the bio-geochemical variables pertaining to a complex fragmented ecosystem such as that of the Southern Apennines. Further studies are needed to confirm and monitor PRISMA data performance on different land-cover areas and on the Radiometric Calibration Network (RadCalNet) targets. [ABSTRACT FROM AUTHOR]

Background: Classic infantile onset of Pompe disease (c-IOPD) leads to hypotonia and hypertrophic cardiomyopathy within the first days to weeks of life and, without treatment, patients die of cardiorespiratory failure in their first 1–2 years of life. Enzymatic replacement therapy (ERT) with alglucosidase alfa is the only available treatment, but adverse immune reactions can reduce ERT's effectiveness and safety. It is therefore very important to identify strategies to prevent and manage these complications. Several articles have been written on this disease over the last 10 years, but no univocal indications have been established. Methods: Our study presents a review of the current literature on management of immune responses to ERT in c-IOPD as considered by an Italian study group of pediatric metabolists and immunologists in light of our shared patient experience. Results: We summarize the protocols for the management of adverse reactions to ERT, analyzing their advantages and disadvantages, and provide expert recommendations for their optimal management, to the best of current knowledge. However, further studies are needed to improve actual management protocols, which still have several limitations. [ABSTRACT FROM AUTHOR]

The identification of zones within an agricultural field that respond differently to environmental factors and agronomic management is a key requirement for the adoption of more precise and sustainable agricultural practices. Several approaches based on spatial clustering methods applied to different data sources, e.g. yield maps, proximal sensors and soil surveys, have been proposed in the last decades. The current availability of a huge amount of free remote sensing data allows to apply these approaches to agricultural areas where ground or proximal data are not available. However, in order to provide useful agronomic management information, it is essential that the zoning obtained by clustering is linked to the underlying spatial variability of soil properties. In this work we explore the hypothesis that the response of crop vigor to temporal climate variability, assessed by remote sensing data time series, selected to correspond to specific growth phases and seasonal climate patterns, provides indications on the variability of soil properties within agricultural fields, for both herbaceous and tree crops. NDVI time-series for 38 years (1984–2021) were obtained for fourteen non-irrigated herbaceous and tree crop fields in Central Italy, from multispectral satellites data (Landsat 5/7/8, Sentinel 2). The Standardized Precipitation-Evapotranspiration Index (SPEI) was used to classify time series into three climatic classes (dry/normal/wet) for five different periods of the growth season, covering the main phenological phases. K-means clustering was used to identify patterns of crop growth from climatically classified image sets, as well as for all the bulked images for comparison (bulk clustering). Clustering results were compared with soil maps obtained from spatialized ground data, for soil texture (clay, silt and sand), soil organic matter and available soil water (ASW). The agreement between the different clustering results and soil maps was assessed by the Adjusted Rand Index. Agreement with soil maps varied depending on the field, the phenological phase considered and the soil property considered. Climate driven clustering from long, late growth season periods best matched soil properties, both for herbaceous and tree crops, despite being based on a limited number of images. The clustering from images spanning a longer growth period for dry years systematically outpaced the bulk clustering for silt, sand and ASW, while the clustering for normal climatic conditions was the best for organic matter. The performance of the matching between clustering and soil maps increased with soil variability significantly more (P < 0.05) than in the bulk clustering (mean slopes respectively 0.468 ± 0.167; 0.113 ± 0.270). The integration of the SPEI climatic index into the clustering procedure systematically improved the identification of zones with homogeneous soil properties, highlighting that a greater attention should be posed to the climate-crop-field interactions when using remotely sensed images. • Field soil zoning is essential for precision agronomic management. • A clustering method integrating climate criteria is proposed for non-irrigated fields. • Clustering results are compared to ground truth soil texture, organic matter and available soil water. • Climatic clusterings, based on images from a few dry years, best matched soil maps. • Soil maps matching improved with increasing soil hetereogenity [ABSTRACT FROM AUTHOR]

Background: Children are the most vulnerable population exposed to the use of antibiotics often incorrectly prescribed for the treatment of infections really due to viruses rather than to bacteria. We designed the MAREA study which consisted of two different studies: i) a surveillance study to monitor the safety/efficacy of the antibiotics for the treatment of pneumonia (CAP), pharyngotonsillitis and acute otitis media in children younger than 14 yrs old, living in Liguria, North-West Italy and ii) a pre-/post-interventional study to evaluate the appropriateness of antibiotic prescription for the treatment these infections. In this paper, we show only results of the appropriateness study about the antibiotic prescription for the treatment of pneumonia. Methods: Patients included in this study met the following inclusion criteria: i) admission to the Emergency/ Inpatient Dpt/outpatient clinic of primary care pediatricians for pneumonia requiring antibiotics, ii) informed written consent. The practice of prescribing antibiotics was evaluated before-and-after a 1 day-educational intervention on International/National recommendations. Results: Global adherence to guidelines was fulfilled in 45%: main reason for discordance was duration (shorter than recommended). Macrolide monotherapy and cephalosporins were highly prescribed; ampicillin/amoxicillin use was limited. 61% of patients received >1 antibiotic; parenteral route was used in 33%. After intervention, i) in all CAP, cephalosporin prescription decreased (-23%) and the inappropriate macrolide prescriptions was halved and, ii) in not hospitalized CAP (notH-CAP), macrolides were prescribed less frequently (-25%) and global adherence to guidelines improved (+39%); and iii) in H-CAP antibiotic choice appropriateness increase. Conclusion: Prescribing practices were sufficiently appropriate but widespread preference for multidrug empirical regimens or macrolide in monotherapy deserve closer investigation. [ABSTRACT FROM AUTHOR]

Knowledge about the association between alcohol and Barrett’s oesophagus and reflux oesophagitis is conflicting. In this case–control study we evaluated the role of specific alcoholic beverages (red and white wine, beer and liquors) in 339 Barrett’s oesophagus and 462 oesophagitis patients compared with 619 endoscopic controls with other disorders, recruited in twelve Italian endoscopic units. Data on alcohol and other individual characteristics were obtained from structured questionnaires. No clear, monotonic significant dose–response relationship was pointed out for red wine. However, a generalised U-shaped trend of Barrett’s oesophagus/oesophagitis risk due to red wine consumption particularly among current drinkers was found. Similar results were also found for white wine. Liquor/spirit consumption seemed to bring about a 1·14–2·30 risk excess, although statistically non-significant, for current Barrett’s oesophagus/oesophagitis drinkers. Statistically significant decreasing dose–response relationships were found in Barrett’s oesophagus for frequency and duration of beer consumption. Similar, but less clear downward tendencies were also found for oesophagitis patients. In conclusion, although often not statistically significant, our data suggested a reduced risk of Barrett’s oesophagus and oesophagitis with a low/moderate intake of wine and beer consumption. A non-significant increased risk of Barrett’s oesophagus/oesophagitis was observed with a higher intake of any type of heavy alcohol consumption, but no conclusion can be drawn owing to the high number of non-spirit drinkers and to the small number of drinkers at higher alcohol intake levels. [ABSTRACT FROM PUBLISHER]

Background Mucopolysaccharidoses ( MPS) are inherited lysosomal storage disorders caused by deficiency of required glycosaminoglycans breakdown enzymes, inducing cardiac involvement. Little is known about myocardial deformation involvement in MPS. Our aim was to assess biventricular structure and function in asymptomatic children with MPS using standard echo Doppler and 2D speckle tracking ( STE). Methods Fifteen MPS children (one type I, six type II, three type III A, one III B, three IV A, one VI), asymptomatic for cardiac symptoms, and 15 age and sex-matched healthy controls underwent echo Doppler and STE. Left ventricular ( LV) wall thicknesses, diameters, and mass were normalized by z-score. LV global longitudinal strain ( GLS), global circumferential strain ( GCS), global radial strain ( GRS) at papillary muscles, LV twisting, and right ventricular ( RV) GLS were measured. Results The two groups were comparable for body mass index, heart rate, and blood pressure. LV mass index and relative wall thickness were higher in MPS. Ejection fraction (EF), and s′ velocity did not differ between the two groups. E/A ratio was lower and E/e′ higher in MPS. Tricuspid annular plane systolic excursion, RV s′ and e′ were lower in MPS. LV GLS did not differ between the two groups, but GCS ( P=.014), GRS ( P=.023), twisting ( P=.012), and RV GLS ( P<.001) were lower in the MPS group. Conclusions LV strain abnormalities are detectable in MPS pediatric patients, independently of MPS type, when EF is still normal. RV GLS is also involved consensually with TAPSE reduction. STE can be useful for detection of subclinical myocardial damage in MPS. [ABSTRACT FROM AUTHOR]

Objective. Thymus alterations have been related to several autoimmune disorders. In particular, previous studies identified a significant frequency of gland abnormalities by chest high-resolution CT (HRCT) in SSc patients. In this study we aimed to investigate the prevalence of radiological thymic alterations and their correlation with clinical and serological features in a large SSc series.Methods. We retrospectively evaluated thymic shape on CT scans of 200 consecutive, unselected SSc patients aged over 30 years The presence of radiological abnormalities, i.e. enlarged gland >13 mm or nodular lesions >7 mm, was correlated with SSc clinico-serological features. Moreover, the patients were also classified using a second thickness cut-off of 7 mm in order to identify incomplete thymic involution.Results. Twenty-four of 200 (12%) SSc patients presented an abnormal thymus at HRCT, including hyperplasic (19/24) and nodular (5/24) glands. Otherwise, using the cut-off of 7 mm for gland thickness and excluding subjects with nodular thymus, 50/195 (25.6%) patients presented an incomplete thymic involution. Thymic radiological alterations are significantly correlated with younger age and diffuse cutaneous SSc. Moreover, an abnormally enlarged thymus tended to be more common in patients with shorter disease duration.Conclusion. The present report on a large series of SSc patients further reinforces previous data present in the literature that includes other cohort studies and a number of anecdotal observations. Even though the actual role of thymus radiological abnormalities remains unclear, possible involvement of the gland in the early phase of immune-mediated SSc pathogenesis might be supposed. [ABSTRACT FROM PUBLISHER]

Despite possible agronomic and environmental benefits, the diffusion of soil conservation tillage systems in Italy is currently rather low. The aim of this study was to compare the performance of different soil tillage techniques, in an effort to identify suitable soil management options for irrigated crops in Central Italy. An experiment was carried out on maize and soybean from April to October in two consecutive years (1993 and 1994) in Maccarese (a coastal location near Rome). The systems compared were: conventional mouldboard ploughing (CT), minimum tillage, ridge tillage and no-tillage (NT). In 1993, actual crop evapotranspiration was measured throughout the growing season on NT and CT soybean, using a micrometeorological technique. No significant differences due to soil tillage were found for grain yield and yield irrigation water use efficiency (IWUEy), except for soybean in 1994, in which yields and IWUEy were 59 % higher on conservation tillage treatments compared with CT. In 1994 soybean yield water use efficiency was 10.1 and 9.5 kg ha−1 mm−1 for NT and CT respectively. The results suggest that the adoption of soil conservation tillage is feasible, for the specific cropping system, with equivalent or better performances as conventional tillage. [ABSTRACT FROM AUTHOR]

Abstract: A non-conventional approach for the estimation of leaf area index (LAI) and leaf angle distribution (LAD), based on the use of information contained in multiangular images and the inversion of a canopy ray tracing model, is proposed in this work. As an alternative to the use of overall image reflectance data, the image fraction components, i.e. sunlit and shaded leaves and soil, are obtained by supervised classification of ground-based multiangular images acquired using an inexpensive colour infrared camera, the Dycam ADC. These data are used for the inversion of a numerical model of a vegetation canopy in which the latter is described as composed of randomly distributed disks (leaves). The model was developed using the POV-ray ray tracer. Model inversion is carried out using the look-up-table approach. The proposed methodology was tested using an extensive data set gathered on the potato crop during experimental trials carried out at Viterbo (Italy) for 3 years. The results show that LAI was successfully estimated with a RMSE varying from 0.29 to 0.75 in the different years. The potential sources of error in both estimated and measured LAI values are extensively discussed. [Copyright &y& Elsevier]

Unlabelled: We studied the genotype/phenotype correlation in a cohort of glycogen storage disease type (GSD) 1b patients. A total of 25 GSD1b patients, 13 females and 12 males, age range: 4.3-28.4 years, mean:14.6+/-6.8 years; median: 15 years, representing the entire case load of Italian GSD1b patients, were enrolled in the study. Molecular analysis of the glucose 6-phosphate translocase (G6PT1) gene was performed in all patients. We analysed the presence of a correlation among both the clinical features associated with GSD1b (neutropenia, frequency of admission to the hospital for severe infections) and the presence of systemic complications (liver adenomas, nephropathy, bone mineral density defect, polycystic ovaries, short stature, inflammatory bowel disease) and the mutations detected in each patient. Nine patients were homozygous or compound heterozygous for mutations causing stop codons. In particular, three patients were homozygous for the same mutation (400X); of these patients, one showed chronic neutropenia with severe and frequent infections and severe inflammatory bowel disease, another patient cyclic neutropenia associated with rare bacterial infections and mild bowel involvement and the last one normal neutrophil count. Two patients were homozygous for the mutation 128X; one of these patients did not show neutropenia, whereas the other one had severe neutropenia needing frequent hospital admission and was under granulocyte-colony stimulating factor treatment. In three patients no mutations were detected.Conclusion: No correlation was found between individual mutations and the presence of neutropenia, bacterial infections and systemic complications. These results suggest that different genes and proteins modulate neutrophil differentiation, maturation and apoptosis and thus the severity and frequency of infections. The absence of detectable mutations in three patients could suggest that a second protein plays a role in microsomal phosphate transport. [ABSTRACT FROM AUTHOR]

Abstract Triennial field trials were carried out at Viterbo, central Italy, to investigate the influence of the sowing time, nitrogen fertilization and harvest dates of the two fibre flax (Linum usitatissimum L.) varieties Belinka and Ariane. Our results showed that warm and dry weather conditions, prevailing from late spring onwards, caused excessive shortening of the growing cycle of spring crops, with negative consequences for seed and fibre yield and quality. However, autumn sowings gave interesting results. Stem dry weight ranged between 5.6 and 6.7 t ha-1 for autumn sowings and between 1.9 and 4.1 t ha-1 for spring sowings. In autumn sowings, plant height and seed yields were also increased. Seed germinability was good even for early harvest dates. Stem dry weight remained constant at harvest dates following yellow-green ripening, indicating that biomass was no longer allocated to the stems after that phase. [ABSTRACT FROM AUTHOR]

The aim of this research is to explore the analysis of methods allowing a synergetic use of information exchange between Earth Observation (EO) data and growth models in order to provide high spatial and temporal resolution actual evapotranspiration predictions. An assimilation method based on the Ensemble Kalman Filter algorithm allows for combining Sentinel-2 data with a new version of Simple Algorithm For Yield (SAFY_swb) that considers the effect of the water balance on yield and estimates the daily trend of evapotranspiration (ET). Our study is relevant in the context of demonstrating the effectiveness and necessity of satellite missions such as Land Surface Temperature Monitoring (LSTM), to provide high spatial and temporal resolution data for agriculture. The proposed method addresses the problem both from a spatial point of view, providing maps of the areas of interest of the main biophysical quantities of vegetation (LAI, biomass, yield and actual Evapotranspiration), and from a temporal point of view, providing a simulation on a daily basis of the aforementioned variables. The assimilation efficiency was initially evaluated with a synthetic, large and heterogeneous dataset, reaching values of 70% even for high measurement errors of the assimilated variable. Subsequently, the method was tested in a case study in central Italy, allowing estimates of the daily Actual Evapotranspiration with a relative RMSE of 18%. The novelty of this research is in proposing a solution that partially solves the main problems related to the synergistic use of EO data with crop growth models, such as the difficult calibration of initial parameters, the lack of frequent high-resolution data or the high computational cost of data assimilation methods. It opens the way to future developments, such as the use of simultaneous assimilation of multiple variables, to deeper investigations using more specific datasets and exploiting the advanced tools. [ABSTRACT FROM AUTHOR]

A better comprehension of soil properties and processes permits a progress in agricultural management effectiveness, together with a diminution of environmental damage and more beneficial use of resources. This research investigated the usage of multispectral (Sentinel-2 MSI) satellite data at the farm/regional level, for the identification of agronomic bare soil presence, utilizing bands of the spectral range from visible to shortwave infrared. The research purpose was to assess the frequency of cloud-free bare soil time-series images available during the year in typical agricultural areas, needed for the development of digital soil mapping (DSM) approaches for agricultural applications, using hyperspectral satellite missions such as current PRISMA and the planned EnMAP or CHIME. The research exploited the Google Earth Engine platform, by processing all available cloud-free Sentinel-2 images throughout a time span of four years. Two main results were obtained: (i) bare soil frequency, indicating where and when a pixel (or an agricultural field) was detected as bare surface in three representative agricultural areas of Italy, and (ii) a temporal sensitivity analysis, providing the acquisition frequency of useful bare soil images applicable for the retrieval of soil variables of interest. It was shown that, in order to provide for an effective agricultural soil monitoring capability, a revisit frequency in the range of five to seven days is required, which is less than the planned specifications e.g., of PRISMA or CHIME hyperspectral missions, but could be addressed by combining data from the two sensors. [ABSTRACT FROM AUTHOR]

Chronic hepatitis B virus (HBV) infection may be reactivated by immunosuppressive drugs in patients with autoimmune inflammatory rheumatic diseases. This study evaluates HBV serum markers' prevalence in rheumatic outpatients belonging to Spondyloarthritis, Chronic Arthritis and Connective Tissue Disease diagnostic groups in Italy. The study enrolled 302 subjects, sex ratio (M/F) 0.6, mean age ± standard deviation 57 ± 15 years, 167 (55%) of whom were candidates for immunosuppressive therapy. The Spondyloarthritis group included 146 subjects, Chronic Arthritis 75 and Connective Tissue Disease 83 (two patients had two rheumatic diseases; thus, the sum is 304 instead of 302). Ten subjects (3%) reported previous anti-HBV vaccination and tested positive for anti-HBs alone with a titer still protective (>10 IU/mL). Among the remaining 292 subjects, the prevalence of positivity for HBsAg, isolated anti-HBc, anti-HBc/anti-HBs, and any HBV marker was 2%, 4%, 18%, and 24%, respectively. A total of 26/302 (9%) patients with γ-globulin levels ≤0.7 g/dL were more frequently (p = 0.03455) prescribed immunosuppressive therapy, suggesting a more severe rheumatic disease. A not negligible percentage of rheumatic patients in Italy are at potential risk of HBV reactivation related to immunosuppressive therapy. Before starting treatment, subjects should be tested for HBV markers. Those resulting positive should receive treatment or prophylaxis with Nucleos (t) ides analogue (NUCs) at high barrier of resistance, or pre-emptive therapy, according to the pattern of positive markers. HB vaccination is recommended for those who were never exposed to the virus. [ABSTRACT FROM AUTHOR]

The present work reports the global sensitivity analysis of the Aquacrop Open Source (AOS) model, which is the open-source version of the original Aquacrop model developed by the Food and Agriculture Organization (FAO). Analysis for identifying the most influential parameters was based on different strategies of global SA, density-based and variance-based, for the wheat crop in two different geographical locations and climates. The main objectives were to distinguish the model's influential and non-influential parameters and to examine the yield output sensitivity. We compared two different methods of global sensitivity analysis: the most commonly used variance-based method, EFAST, and the moment independent density-based PAWN method developed in recent years. We have also identified non-influential parameters using Morris screening method, so to provide an idea of the use of non-influential parameters with a dummy parameter approach. For both the study areas (located in Italy and in China) and climates, a similar set of influential parameters was found, although with varying sensitivity. When compared with different probability distribution functions, the probability distribution function of yield was found to be best approximated by a Generalized Extreme Values distribution with Kolmogorov–Smirnov statistic of 0.030 and lowest Anderson–Darling statistic of 0.164, as compared to normal distribution function with Kolmogorov–Smirnov statistic of 0.122 and Anderson–Darling statistic of 4.099. This indicates that yield output is not normally distributed but has a rather skewed distribution function. In this case, a variance-based approach was not the best choice, and the density-based method performed better. The dummy parameter approach avoids to use a threshold as it is a subjective question; it advances the approach to setting up a threshold and gives an optimal way to set up a threshold and use it to distinguish between influential and non-influential parameters. The highly sensitive parameters to crop yield were specifically canopy and phenological development parameters, parameters that govern biomass/yield production and temperature stress parameters rather than root development and water stress parameters. [ABSTRACT FROM AUTHOR]

Mitotane is the main option of treatment for advanced adrenocortical carcinoma (ACC). However, limited evidence is available regarding the impact of plasma mitotane levels on patient outcome. To address this question, we retrospectively analyzed patients with advanced ACC treated with mitotane for ≥3 months, with ≥3 measurements of plasma mitotane reported in the Lysosafe Online® database (HRA Pharma, France), followed at 12 tertiary centers in Italy from 2005 to 2017. We identified 80 patients, initially treated with mitotane alone (56.2%) or plus chemotherapy (43.8%). The preference toward combination therapy was given to de novo stage IV ACC and younger patients. After the first line of treatment, 25% of valid cases experienced clinical benefit (14.5% objective response, 10.5% stabilization of disease) and 75% progression, without differences between the groups of treatment. Patients with progression had a lower time in the target range (TTR) of plasma mitotane and an unfavorable outcome. Death occurred in 76.2% of cases and multivariate analysis showed that clinical benefit after first treatment and longer TTR were favorable predictors of overall survival (OS). In conclusion, the present findings support the importance of mitotane monitoring and strengthen the concept of a therapeutic window for mitotane. [ABSTRACT FROM AUTHOR]

Mitotane is used as a post-operative adjuvant treatment for patients with adrenocortical carcinoma. Monitoring of plasma mitotane concentrations is recommended, but we do not know what impact target concentrations have on patient outcome. To answer this question, we retrospectively analyzed patient records in the Lysosafe Online® database (HRA Pharma, France) for patients who were treated for ≥6 months and who had ≥3 measurements of plasma mitotane levels during follow-ups at 11 tertiary centers in Italy from 2005 to 2017. We identified 110 patients treated with adjuvant mitotane for a median of 46 months (IQR, interquartile range, 28–62) with a median maintenance dose of 2.0 g/day (IQR 1.5–2.5). Achievement of target mitotane concentrations (≥14 mg/L) required a median of 8 months (IQR 5–19). Female sex was associated inversely with the dose, while body mass index (BMI) was correlated positively. Multivariate analysis showed that the Ki67 index and time to achieve the target range of plasma mitotane were independent predictors of recurrence-free survival (RFS). In a separate multivariate model, considering only the maintenance phase (month 7 to month 36, M7–M36) of treatment, the time in the target range of plasma mitotane was associated with a significantly lower risk of recurrence (Hazard Ratio, HR = 0.93; 0.88–0.98, p < 0.01). The prognostic implications of the time in target range and the time needed to reach target mitotane concentrations support the use of mitotane monitoring and may inform practice. [ABSTRACT FROM AUTHOR]

According to the Italian laws, some categories of workers entrusted with duties possibly constituting a threat to security, physical safety, and health of third parties have to be screened to exclude the use/abuse of the following drugs of abuse: opiates, cocaine, cannabinoids, amphetamine, methamphetamine, 3,4-methylenedioxymethamphetamine, methadone, and buprenorphine. Toxicological tests can be performed with urinary on-site rapid screening devices, provided that sensitivities up to specified cutoffs are ensured. The present study reports performances, in terms of sensitivity, specificity, and accuracy, of an automatic on-site test and of an FPIA-based device, using gas chromatography/mass spectrometry (GC/MS) as a reference methodology. Three levels of concentration were tested, corresponding to the cutoff and to 2 and 3 times the limits, respectively. In terms of sensitivities, neither the on-site nor the benchtop instrumentations gave positive results, since values of zero percentage were obtained for concentrations up to 2-fold the limits. Even if good results were obtained in terms of specificity and accuracy by both devices, none of them seem to be adequate for the current application to the toxicological screening at workplaces. In fact, a rapid screening device can be used for drug tests provided that it ensures sensitivity at the prescribed cutoffs. Data showed that such is completely rejected and a more sensitive instrumentation should be preferred. [ABSTRACT FROM AUTHOR]

Abstract: A new AMS system has been installed at Center for Isotopic Research on Cultural and Environmental Heritage (CIRCE) in Caserta/Italy. It was built by National Electrostatics Corp. Middleton, WI/USA, and arrived at CIRCE in the middle of January 2005; the installation and the acceptance tests were completed at the end of February 2005. At first, the system is intended for Radiocarbon AMS; for future Al and Be AMS only minor upgrades are needed and the magnets are designed to bend heavy isotopes like Uranium. Concerning Radiocarbon AMS, the first measurements of unknown samples, prepared in the internal preparation laboratory, already yielded results of high precision and 14C dating is in full swing. [Copyright &y& Elsevier]

The ITER Neutral Beam Test Facility, in an advanced stage of construction in Padova, includes the installation, tests, and optimization of the full prototype of the ITER Heating Neutral Beams injector (HNBs), named MITICA. The MITICA Neutral Beam Injector will host its main components in a SS304L vacuum vessel composed of two modules, connected between them on site: the Beam Source Vessel (cubic shape of 5 m side and 67 tons weight) containing the Beam Source and the Beam Line Vessel (section 4.5 m x 4.5 m, length 11 m and 76 tons weight) containing the Beam Line Components and the Cryopumps. The manufacturing is described, going through the FE analyses performed to assess the structural integrity, the materials selection, the welding qualifications, the implementation of the double barrier sealings and the control of the deformations. The Factory Acceptance Tests of the individual vessels are presented, including their Helium Leak Tests. The two vessels have been assembled on-site inside the MITICA bio-shield. The main outcomes of on-site final assembly and Site Acceptance Tests are described. Both the vessels have been detail designed, manufactured, installed and tested by De Pretto Industried, from Schio, (VI) Italy, supporting fusion technology's applications from more than 50 years. The design and the technical support were provided by Consorzio RFXa, while the procurement was managed by Fusion For Energye. [ABSTRACT FROM AUTHOR]