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Thirty four plum local varieties and accessions obtained from different growing area of the Sicilian island were analyzed for their qualitative and nutraceutical properties and three commercialcultivar were used as references. These properties included the fruit fresh weight (g), the pulp firmness (FFF), the total soluble solids (TSS), the titratable acidity (TA), the total anthocyanins,the phenolics content and the antioxidant activity.This preliminary study showed significantly differeces among the plums; Zuccarato giallo and Prunu Niuru presented TSS higher than the commercial cultivars (24.9 and 21.6 °Brix respectively)and interesting data obtained on the nutraceutical compounds values suggested these local cultivars as sources of polyphenols (Zuccarato giallo with 663 mg GA/100 gFW) and naturalantioxidants (Pruno Regina with 47.46 Fe2+/100 gFW). The characterization of these plums could represent also an important resource for the international activity in the genetic improving andthe collection of the more interesting quality traits could be useful for improving the Prunus database actually in use.

The improvement of the storage solutions and the infrastructure is a significant opportunity to the management of the post - harvest fruit losses and the maintenance of the quality in the supply chain. The environmental issue and the sustainability of the applied processes are the key of the succesful for the innovation of the food system; the sanification of the storage environment could represent a good opportunity to achieve this objective. The focus of this work was to evaluate the efficacy of the ozone (O3) such environmental sanitization system to limit both the quantitative and the qualitative losses of the raspberry fruits. Fruits exposed to the O3 constant concentration of 500 ppb (S1) and to the O3 concentration between 200 and 50 ppb (S2) were compared with fruits maintained in the normal atmosphere (control). The efficacy of the S1 and S2 treatments were evaluated measuring along the storage time the fruits weight losses (%), the total soluble solids content (SSC), the pulp firmness, the acidity content, the skin colour and the most important nutraceutical compounds. The fruits were stored at 1±1°C in a cold room held at 90–95% RH for 3, 6, 9 and 13 days. After each time at low temperature the same berries were held in shelf life for different additional days (respectively +6, +3, +9 and +2) at 20±1°C to simulate retailer conditions. All the qualitative and nutraceutical traits were maintained to the exposure of O3. Fruits stored with the S2 treatment showed the highest sensorial profile.

Background: In children, laparoscopic cholecystectomy (LC) is now considered the gold standard for gallbladder (GB) removal. In the past, hemolytic disorders associated with cholelithiasis represented the most frequent conditions requiring LC; this is being overtaken by cholelithiasis and biliary conditions in overweight or ex-premature children., Aims: This study aims to describe current indications and timing for LC in pediatric patients., Methods: Retrospective study. Data on previous medical therapy, ultrasound, pre- and intraoperative aspects, and histology were collected for patients treated in 2020-2023., Results: In total, 45 patients were enrolled: 15 who underwent urgent surgery and 30 electives. Groups differed in terms of obesity rate, symptoms, ultrasound features, and intraoperative status. The most relevant risk factors for surgical complexity were age and pubertal stage, elevated cholestasis indexes, and gallbladder wall thickness > 3 mm at ultrasound. GB wall thickening ≥3 mm, US Murphy sign, fluid collections, and gallbladder distention on ultrasound correlated with high surgical scores., Conclusions: Indications for laparoscopic cholecystectomy in children seem to evolve caused by changing characteristics of the pediatric population. Patients with overweight/obesity may develop more complex GB diseases. Asymptomatic patients should be considered for surgery after observation, considering age and/or pubertal maturation when other risk factors are absent.

Background: Patients with anorectal malformation (ARM) need long-term follow-up, in order to evaluate fecal continence; the main predictors of longer-term success are the type of ARM, associated anomalies and sacral integrity. Three-Dimensional High Resolution Anorectal Manometry (3D-HRAM) gives detailed information on pressure on the anal complex profile. Our objective was to analyze anal sphincter activity in ARM patients with 3D-HRAM establishing the correlation between manometric and clinical data., Methods: Forty ARM patients were submitted to 3D-HRAM: manometric, anatomical and clinical scores were correlated with each other and with the bowel management response (BM)., Results: A positive correlation between all scores and types of ARM was found: in high ARM and in patients with spinal anomalies (regardless to ARM type) lower scores were reported and even after BM they did not achieve good continence., Conclusions: 3D-HRAM gives detailed data on the functional activity of the anal sphincter complex. Our study revealed a correlation between manometric parameters and clinical outcomes, confirming spinal malformations and ARM type as the most important prognostic risk factors for a bad outcome. Specific sphincteric defects can also be explored with manometry, allowing for tailored bowel management strategies.

Background: Dysphagia in neurologically impaired children is associated with feeding difficulties, malnutrition and aspiration pneumonia. Esophageal high-resolution manometry (HRM) has been used in the diagnosis of motility disorders affecting the swallowing process. The aim of this study was to analyze swallowing functions in NI children by using HRM in order to establish swallow parameters identifying inhalation risk., Methods: Twenty-five NI children with cerebral palsy were submitted to esophageal HRM with UES analysis, comparing the results with non-NI children. The following parameters were evaluated: maximum pressure and duration of contraction of the velopharynx (VP) and tongue base (TB), and maximal, minimal, resting pressure and relaxation duration of the upper esophageal sphincter (UES)., Results: pVP max, pTB max, pUES max and resting pressure were lower, while p UES minimal was higher and relaxation duration was shorter in NI children vs. the control group. Predictive values of inhalation risk were evaluated., Conclusions: This study evaluates inhalation risk in NI children using HRM to study UES function. Our results confirm the alterations described in NI children: insufficient contraction and clearing force for bolus transmission through the pharynx and incomplete UES relaxation can predispose to pharyngeal residues and inhalation independently of swallowing because of lower values of UES resting.

The public catering sector has important responsibilities in seeking a change toward more sustainable choices for many aspects related to the environmental impacts of their services. The environmental impact of production processes can be studied through life cycle assessment (LCA), which allows a greater awareness of choices and has rarely been applied to catering. In this work, we studied the impacts of two dishes (braised meat and cauliflower meatballs) in a school canteen, their impacts were studied using the daily energy requirement (expressed in kcal) as a functional unit. Global warming potential (GWP) and nonrenewable energy (NRE) were calculated starting from the supply of raw materials up to distribution. Electricity and the act of cooking the meatballs accounted for more than 60% of the measured impact in terms of GWP, whereas, less markedly, they dominated in terms of nonrenewable energy used. In the case of braised meat, the total impact was, however, attributable to the life cycle of the meat (between 60% and 76%) and the consumption of electricity (between 19% and 27%), whereas for all other factors, the contribution was never particularly high. Additionally, a discussion on the correct functional unit to be used proposed the environmental impact of different recipes as an additional criterion for nutritionists during the composition of the menu. An integrated system appears important for changing policies and behaviors and the application of LCA can be a tool capable of contributing to the construction of a holistic instrument of sustainability.

Background: Transanal irrigation (TAI) is employed for children with fecal incontinence, but it can present several problems which require a study of their outcomes among different pathologies and without a tailored work up. The aim of our study was to evaluate the effectiveness of an advanced protocol in order to tailor TAI, prevent complications, and evaluate outcomes., Methods: We included 70 patients (14 anorectal malformation, 12 Hirschsprung's disease, 24 neurological impairment, 20 functional incontinence) submitted to a comprehensive protocol with Peristeen ® : fecal score, volumetric enema, rectal ultrasound, anorectal 3D manometry, and diary for testing and parameter adjustment., Results: Among the patients, 62.9% needed adaptations to the parameters, mainly volume of irrigated water and number of puffs of balloon. These adaptations were positively correlated with pre-treatment manometric and enema data. In each group, the improvement of score was statistically significant in all cases ( p 0.000); the main factor influencing the efficacy was the rate of sphincter anomalies. The ARM group had slower improvement than other groups, whereas functional patients had the best response., Conclusions: Our results showed that TAI should not be standardized for all patients, because each one has different peculiarities; evaluation of patients before TAI with rectal ultrasound, enema, and manometry allowed us to tailor the treatment, highlighting different outcomes among various pathologies, thus improving the efficacy.

Glioblastoma multiforme (GBM) is considered the most malignant form of primary brain tumor. Despite multimodal treatment, prognosis remains poor. Ketogenic diet (KD) has been suggested for the treatment of GBM. In this study, the syngenic, orthotopic GL261 mouse glioma model was used to evaluate the effects of KD on the metabolic responses of the tumor using 7T magnetic resonance imaging/spectroscopy. GL261 cells were injected into the caudate nucleus of mice. Following implantation, animals were fed with standard chow or underwent a KD. 18 days after initiating the diet, mice fed with KD displayed significantly higher plasmatic levels of ketone bodies and survived longer than those fed with the standard diet. Decreased concentrations of gamma-aminobutyric acid, N-Acetyl-Aspartate and N-acetylaspartylglutamate were found in tumor tissue after 9 days into the KD, while a huge increase in beta-hydroxybutyrate (bHB) was detected in tumor tissue as compared to normal brain. The accumulation of bHB in the tumor tissue in mice undergoing the KD, may suggest either elevated uptake/release of bHB by tumor cells, or the inability of tumor cells in this context to use it for mitochondrial metabolism.

Background: High resolution manometry (HRM), has been recently introduced in clinical practice to detect esophageal intraluminal pressure and esophageal motor function. We evaluated the feasibility and usefulness of intraoperative esophageal HRM during antireflux laparoscopic procedures in pediatric cases with neurological impairment (NI) or esophageal atresia (EA)., Methods: From January to November 2019, seven children (5 NI, 2 EA) with gastroesophageal reflux (GER) were enrolled. Data on intraoperative pressure changes of the esophagogastric junction (EGJ) and postoperative follow-up data were collected., Results: Average preoperative LES pressures were not significantly different from postoperative pressures. A sliding hernia was detected in all patients as evidenced by EGJ double peak pressures. Hernia correction after esophageal traction was complete in 71.4% of the patients, and residual hernia (<2 cm) was detected in 28.6%. Postoperative EGJ pressures were higher compared to preoperative sphincteric pressures ( p < 0.001); in NI patients, higher postoperative values were noted compared to EA ( p = 0.05). No sliding hernia and/or GER relapses were recorded. Two patients reported dysphagia postoperatively., Conclusions: Intraoperative HRM may optimize esophageal pressure changes during laparoscopic fundoplication. Further studies are needed to confirm the usefulness of a tailored surgical approach to reduce postoperative complications.

Background: Laparoscopic cholecystectomy (LC) is the standard of care for gallbladder (GB) pathologies. We evaluated clinical, ultrasonographic (US) data as well as histopathological findings in children affected with symptomatic cholecystic disease (SCD) who underwent LC, with the aim of defining surgical timing. Methods: We reviewed our cases who underwent elective LC (ELC) or urgent LC (ULC). Clinical, US, surgical and histological features were used to create different risk scores. Results: We considered 26 children (17 ELC/9 ULC). US signs were not different in the two groups ( p > 0.05). Operating times were longer in ELC than in ULC ( p = 0.01). Histopathological evaluation revealed fibrosis and atrophy in both ELC and ULC. The clinical risk score was higher in ELC compared to ULC ( p < 0.001). An increased operative risk score was noted in patients with systemic inflammatory signs (OR1.98), lithotherapy (OR1.4.3) and wall thickening ≥3 mm (OR2.6). An increased histopathological risk score was detected in children with symptom duration >7 days (OR3.61), concomitant hematological disease (OR1.23) and lithotherapy (OR3.61). Conclusion: Criteria adopted in adults cannot be adopted to detect the severity of GB damage in children. A dedicated clinical and US score is mandatory to define the most appropriate surgical timing., (Copyright © 2020 Pelizzo, Bussani, De Silvestri, Di Mitri, Rosone, Amoroso, Milazzo, Girgenti, Mura, Unti, Rozze, Shafiei and Calcaterra.)

Dehydration is common and frequently under-diagnosed in chronic malnourished children, leading to life-threatening conditions. In this pilot study we applied bioimpedance vector analysis (BIVA) to determine hydration status in 52 neurologically impaired (NI) paediatric patients (14.08 ± 5.32). Clinical and biochemical data were used to define malnutrition and dehydration. Body composition analysis and hydration were also assessed by BIVA and we considered 143 normal-weight healthy subjects (15.0 ± 1.7), as controls for hydration status assessment. BIVA revealed a pathological hydration status in NI children, showing higher resistance (p < 0.001) and reactance values (p = 0.001) compared to controls. No differences in reactance and resistance were detected between well-nourished and under-nourished subjects. Four patients out of 52 showed mild signs of dehydration; no severe dehydration was detected. Laboratory data, suggestive for dehydration, were similar in well-nourished and under-nourished NI subjects. In conclusion, in our sample of NI paediatrics, dehydration according to clinical signs and laboratory data was under-diagnosed. BIVA showed specific bioelectrical characteristics that could be compatible with impaired hydration status. Further studies are necessary to confirm that BIVA may an applicable tool for defining dehydration status and guiding rehydration in NI children.

Background: Insulin resistance (IR) plays a key role in the pathogenesis of type 2 diabetes (T2D). In neurologically impaired (NI) children unfavorable cardio-metabolic risk profile with high prevalence of IR has been reported. We evaluated the prevalence of T2D in NI children and adolescents, in order to define if a dedicated glucose monitoring may be recommended in these subjects. Methods: We retrospectively evaluated 63 patients (11.4 ± 4.0 years) with severe disabilities. Auxological parameters were recorded. Metabolic blood assays included fasting blood glucose (FBG), fasting insulin, triglycerides (TG). IR was detected with the homeostasis model assessment for insulin resistance (HOMA-IR > 97.5th percentile for age and sex) and triglyceride-glucose index (TyG index > 7.88). Elevated FBG was defined with values >100 mg/dl. T2D was defined according to American Diabetes Association criteria. Results: Impaired insulin sensitivity, pathological TyG index and elevated FBG were observed, respectively, in 41.3, 63.5, and 11.1% patients. T2D was diagnosed in 3.2% asymptomatic patients. The prevalence of diabetes was higher in pre-pubertal compared to pubertal subjects ( p = 0.03). Conclusions: T2D in NI children and adolescents without obesity could represent a new emerging entity. IR and/or surrogate markers of IR index may be useful for the primary screening of this at-risk disabled population so as to prevent diabetes.

A limited number of sclerosing Angiomatoid Nodular Transformation (SANT) have been reported in pediatric age. We describe the first case of SANT occurring in a nine-week-old female infant that was admitted to our unit for severe abdominal distension and rectal bleeding. Enlarged spleen was detected on physical examination. Laboratory investigations revealed severe anemia and coagulation abnormalities. Abdominal ultrasound and computed tomography revealed ascites and splenomegaly with a large mass at the lower medial splenic pole. A diagnosis of intraabdominal hemorrhage was presumed and an exploratory laparotomy was performed. A complete transformation of the giant splenomegaly to bossellated masses and multiple bleeding capsular ruptures without subcapsular hematoma were found and an urgent splenectomy was performed. At histology, a SANT was diagnosed (CD34, CD31, CD8 positivity). The postoperative follow up was uneventful. SANT may also occur in infancy with a potentially lifethreatening presentation. Splenectomy may represent the only treatment in severe cases.

Introduction: Emphysematous pyelonephritis (EPN) is a rare, life-threatening necrotizing infection of the kidney. To date, very few cases of EPN have been described in the pediatric age. The first case of EPN in a toddler occurring after ureterovesical reimplantation for congenital obstructive megaureter is reported with a literature review. Case Report: A 23-month-old male, with a prenatal diagnosis of obstructive megaureter and incomplete duplication of the left urinary tract, was admitted to our Unit where he underwent surgery to treat increased dilatation of the renal pelvis and appearance of an obstructive curve. The latter was revealed at renal scintigraphy, the exam highlighted the radiographic aspect of the cortical renal parenchymal sufferance. At admission preoperative exams were normal, and no recurrent urinary tract infections were documented. Surgical removal of the left stenotic ureteral common tract of the incomplete duplex collecting system was accomplished; ureterovesical reimplantation was performed without ureteral recalibration. No intraoperative complications were recorded. In the immediate postoperative period, urosepsis and the patient's lethargic condition led to life-threatening conditions requiring urgent admission to the intensive care unit. Biochemical analysis showed leukocytosis, anemia, increased C-reactive protein, prolonged prothrombin time, pancytopenia, hyponatremia. Abdominal sonographic evaluation revealed the presence of gas in the left kidney. Unilateral EPN (Class 2) was confirmed by CT- scan. Escherichia coli was cultured from peripheral blood and antimicrobial therapy was started. No additional interventions were required. The child was discharged 14 days postoperatively with normal renal function. Conclusion: EPN is a serious condition that can occur after surgical treatment for urinary tract obstruction. Early detection of air in the kidney should be considered a sign of complicated urinary tract infection. Immediate aggressive resuscitation and antimicrobial therapy are effective and curative with a positive outcome.

In this research the importance of several choice attributes of beef for Piedmontese consumers was examined. The survey was conducted on a sample of consumers in sixteen meat stores in Piedmont, Northwest Italy. A choice experiment (Best-Worst scaling methodology) was used to identify consumer preferences and five clusters of purchaser. The responses were also analyzed on the basis of two variables, the frequency of meat consumption and the place of purchase. Piedmontese consumers considered "price" as the most important factor in meat purchasing, but "animal welfare" considerations played some part too., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

The presence of redundant copy number variants (CNVs) in groups of patients with neurological diseases suggests that these variants could have pathogenic effect. We have collected array comparative genomic hybridization (CGH) data of about 2,500 patients affected by neurocognitive disorders and we observed that CNVs in 2p16.3 locus were as frequent as those in 15q11.2, being both the most frequent unbalances in our cohort of patients. Focusing to 2p16.3 region, unbalances involving NRXN1 coding region have been already associated with neuropsychiatric disorders, although with incomplete penetrance, but little is known about CNVs located proximal to the gene, in the long noncoding RNA AK127244. We found that, in our cohort of patients with neuropsychiatric disorders, the frequency of CNVs involving AK127244 was comparable to that of NRXN1 gene. Patients carrying 2p16.3 unbalances shared some common clinical characteristics regardless NRXN1 and AK127244 CNVs localization, suggesting that the AK127244 long noncoding RNA could be involved in neurocognitive disease with the same effect of NRXN1 unbalances. AK127244 as well as NRXN1 unbalances seem to have a particular influence on language development, behavior or mood, according with the topographic correlation between NRXN1 expression and prefrontal cortex functions., (© 2018 Wiley Periodicals, Inc.)

Background: Epilepsy is a frequent symptom in patients with glioma. Although treatment with antiepileptic drugs is generally effective in controlling seizures, drug-resistant patients are not uncommon. Multidrug resistance proteins (MRPs) and P-gp are over-represented in brain tissue of patients with drug-resistant epilepsy, suggesting their involvement in the clearance of antiepileptic medications. In addition to their anticonvulsant action, some drugs have been documented for cytotoxic effects. Aim of this study was to evaluate possible in vitro cytotoxic effects of two new-generation antiepileptic drugs on a human glioma cell line U87MG., Methods: Cytotoxicity of brivaracetam and lacosamide was tested on U87MG, SW1783 and T98G by MTS assay. Expression of chemoresistance molecules was evaluated using flow cytometry in U87MG and human umbilical vein endothelial cells (HUVECs). To investigate the putative anti-proliferative effect, apoptosis assay, microRNA expression profile and study of cell cycle were performed., Results: Brivaracetam and lacosamide showed a dose-dependent cytotoxic and anti-migratory effects. Cytotoxicity was not related to apoptosis. The exposure of glioma cells to brivaracetam and lacosamide resulted in the modulation of several microRNAs; particularly, the effect of miR-195-5p modulation seemed to affect cell cycle, while miR-107 seemed to be implicated in the inhibition of cells migration. Moreover, brivaracetam and lacosamide treatment did not modulate the expression of chemoresistance-related molecules MRPs1-3-5, GSTπ, P-gp on U87MG and HUVECs., Conclusion: Based on antineoplastic effect of brivaracetam and lacosamide on glioma cells, we assume that patients with glioma could benefit by the treatment with these two molecules, in addition to standard therapeutic options.

In the challenging world of territorial transformations within the agriculture, there is an increasing need for an integrated methodological framework of assessment that is able to reconcile the demand for solutions that are both economically sustainable and contribute to environmental and social improvement. This study aims to assess the introduction of innovation into agro-food systems by combining an environmental life cycle (LCA) assessment and a social life cycle assessment (s-LCA) to support the decision making process of a fruit growers co-op for the adoption of mulching and covering in raspberry farming. LCA and s-LCA have been applied independently under specific consistency requirements, selecting two scenarios to compare the impact with (1) and without (2) the innovation and then combined within a cause-effect chain. The interactions between the environment and socioeconomic components were considered within a nested frameset of business and territorial features. The total emissions from raspberry production in Scenario 1, according to the Global Warming Potential (GWP) Impact Category amounted to 2.2840kg of CO2 eq. In Scenario 2, the impact of production was associated with a GWP of 0.1682kg of CO2 eq. Social repercussions analysis from Scenario 1 compared to Scenario 2 indicate more satisfaction for working conditions and the management of climate risks. The mulching and covering, implemented within a given framework of farm activity, created conditions for the preservation of a model in which raspberry production contributes to landscape protection, the business sustainability of farms and the creation of employment. The combined use of the two methods contributes to the development of a strategy planning due to its ability to deliver, as well as specific analysis at a functional level, a wider framework for assessing the consistency of the impacts related to innovation in raspberry production., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Mature cystic teratoma is the most frequent benign ovarian neoplastic lesion in adolescents and is generally composed of fully differentiated tissue arising from multipotential three germinal layers. It accounts for approximately 50% of benign ovarian tumors in childhood. Rarely, a bilateral, synchronous, or metachronous presentation can be observed, supporting a conservative approach as the first surgical approach. We report a case of an ovarian mature cystic incarcerated in indirect inguinal hernia in a 15-year-old girl undergoing ovary-sparing surgery. To our knowledge this is the first case of such lesion incarcerated in an inguinal hernia reported in literature.

Background: No studies are available in the literature on the distribution of different melanoma features and risk factors in the Italian geographical areas., Objective: To identify the differences in clinical-pathological features of melanoma, the distribution of risk factors and sun exposure in various Italian macro-areas., Methods: Multicentric-observational study involving 1,472 melanoma cases (713 north, 345 centre, 414 south) from 26 referral centres belonging to the Italian Multidisciplinary Group for Melanoma., Results: Melanoma patients in northern regions are younger, with thinner melanoma, multiple primaries, lower-intermediate phototype and higher counts of naevi with respect to southern patients; detection of a primary was mostly connected with a physician examination, while relatives were more involved in the south. Northern patients reported a more frequent use of sunbeds and occurrence of sunburns before melanoma despite sunscreen use and a lower sun exposure during the central hours of the day., Conclusions: The understanding of differences in risk factors distribution could represent the basis for tailored prevention programmes.

We developed an in vitro contact through-feet blood brain barrier (BBB) model built using type IV collagen, rat astrocytes, and human umbilical vein endothelial cells (HUVECs) cocultured through Transwell porous polycarbonate membrane. The contact between astrocytes and HUVECs was demonstrated by electron microscopy: astrocytes endfeet pass through the 8.0 μm pores inducing HUVECs to assume a cerebral phenotype. Using this model we evaluated transmigration of melanoma cells from two different patients (M1 and M2) selected among seven melanoma primary cultures. M2 cells showed a statistically significant higher capability to pass across the in vitro BBB model, compared to M1. Expression of adhesion molecules was evaluated by flow cytometry: a statistically significant increased expression of MCAM, αvβ3, and CD49b was detected in M1. PCR array data showed that M2 had a higher expression of several matrix metalloproteinase proteins (MMPs) compared to M1. Specifically, data suggest that MMP2 and MMP9 could be directly involved in BBB permeability and that brain invasion by melanoma cells could be related to the overexpression of many MMPs. Future studies will be necessary to deepen the mechanisms of central nervous system invasion.

In this study, we analysed the environmental profile of the strawberry industry in Northern Italy. The analysis was conducted using two scenarios as reference systems: strawberry crops grown in unheated plastic tunnels using currently existing cultivation techniques, post-harvest management practices and consumption patterns (scenario 1) and the same strawberry cultivation chain in which some of the materials used were replaced with bio-based materials (scenario 2). In numerous studies, biodegradable polymers have been shown to be environmentally friendly, thus potentially reducing environmental impacts. These materials can be recycled into carbon dioxide and water through composting. Many materials, such as Mater-BI® and PLA®, are also derived from renewable resources. The methodology chosen for the environmental analysis was a life cycle assessment (LCA) based on a consequential approach developed to assess a product's overall environmental impact from the production system to its usage and disposal. In the field stage, a traditional mulching film (non-biodegradable) could be replaced with a biodegradable product. This change would result in waste production of 0 kg/ha for the bio-based product compared to 260 kg/ha of waste for polyethylene (PE). In the post-harvest stage, the issue addressed was the use and disposal of packaging materials. The innovative scenario evaluated herein pertains to the use of new packaging materials that increase the shelf life of strawberries, thereby decreasing product losses while increasing waste management efficiency at the level of a distribution platform and/or sales outlet. In the event of product deterioration or non-sale of the product, the packaging and its contents could be collected together as organic waste without any additional processes because the packaging is compostable according to EN13432. Scenario 2 would achieve reductions of 20% in the global warming potential and non-renewable energy impact categories., (Copyright © 2013 Elsevier B.V. All rights reserved.)

The NF-kB family of transcription factors is up-regulated in inflammation and different cancers. Recent data described heterozygous deletions of the NF-kB Inhibitor alpha gene (NFKBIA) in about 20% of glioblastomas (GBM): deletions were mutually exclusive with epidermal growth factor receptor (EGFR) amplification, a frequent event in GBM. We assessed the status of NFKBIA and EGFR in 69 primary GBMs and in corresponding neurospheres (NS). NFKBIA deletion was investigated by the copy number variation assay (CNV); EGFR amplification by CNV ratio with HGF; expression of EGFR and EGFRvIII by quantitative PCR or ReverseTranscriptase PCR. Heterozygous deletions of NFKBIA were present in 3 of 69 primary GBMs and, surprisingly, in 30 of 69 NS. EGFR amplification was detected in 36 GBMs: in corresponding NS, amplification was lost in 13 cases and reduced in 23 (10 vs 47 folds in NS vs primary tumors; p < 0.001). The CNV assay was validated investigating HPRT1 on chromosome X in females and males. Results of array-CGH performed on 3 primary GBMs and 1 NS line were compatible with the CNV assay. NS cells with NFKBIA deletion had increased nuclear activity of p65 (RelA) and increased expression of the NF-kB target IL-6. In absence of EGF in the medium, EGFR amplification was more conserved and NFKBIA deletion less frequent point to a low frequency of NFKBIA deletions in GBM and suggest that EGF in the culture medium of NS may affect frequency not only of EGFR amplifications but also of NFKBIA deletions.

This study examined the emissions produced during the pre-farm, farm and post-farm phases of the production cycle of raspberries and giant American whortleberries (blueberries) cultivated in one of the best-adapted areas in northern Italy. The pre-farm phase included the greenhouse gas emissions from the production of plants in the nursery and the transportation of the plants to the production farms. The farm phase involved the emissions of greenhouse gases from chemical products, the water used for irrigation, the generation of waste, and the consumption of electricity and other energy. The post-farm phase comprised the transportation of the products to the distribution centre (DC) and their storage in the DC. The use phase is not included in the system, nor is transportation from the supermarket to the home of the final consumer, but the disposal of the packaging is nevertheless taken into account. Indeed, the use of traditional plastic materials during both the field phase (nursery and cultivation) and the post-harvesting phase (packaging) produced the greatest estimated impact., (Copyright © 2013 Elsevier B.V. All rights reserved.)

5q14.3 deletions including the MEF2C gene have been identified to date using genomic arrays in patients with severe developmental delay or intellectual disability, stereotypic behavior, epilepsy, cerebral malformations and a facial gestalt not really distinctive though characterized by broad and/or high, bulging forehead, upslanting palpebral fissures, flat nasal root and bridge, small, upturned nose, hypotonic small mouth resulting in cupid bow/tented upper lip. MEF2C mutations have been also identified in patients with overlapping phenotype so that it is considered the gene responsible for the 5q14.3 deletion syndrome. To date, one single duplication including MEF2C has been reported in a patient with intellectual disability but its clinical significance remains uncertain also because of the large size of the imbalance. Here we present two further patients with 5q14.3 duplications including MEF2C. Their phenotype indeed suggest the pathogenic effect of the MEF2C duplication although other duplicated genes also brain expressed might contribute to the clinical features. In none of them a clear-cut syndrome can be identified. A comparison between MEF2C deleted/mutated and duplicated patients is also presented., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Extranodal natural killer/T-cell (ENK/T) lymphoma is a rare neoplasm, subcategorized into ENK/T-nasal (ENK/T-N) and ENK/T-nasal type (ENK/T-NT) lymphomas. ENK/T-NT lymphoma with initial presentation in the skin is known as primary cutaneous ENK/T-NT (PC-ENK/T-NT) lymphoma. The aim of this study was to investigate pathogenesis, genomic alterations, and prognosis of cutaneous ENK/T lymphomas to provide further insights into clinicopathologic features and genetic mechanism of lymphomagenesis. A retrospective case study of 5 white patients affected by ENK/T lymphoma (4 PC-ENK/T-NT and 1 ENK/T-N with cutaneous involvement) was performed. Most of the cases presented with multiple nodules and ulcerations localized on the extremities. A considerable percentage had disease in advanced stage with a 12-month survival rate of 40%. Genomic alterations were detected by array-based comparative genomic hybridization that showed gains of 1q, 7q and loss of 17p in the cases of PC-ENK/T-NT lymphomas and gain of 7q and loss of 9p, 12p, 12q in the case of ENK/T-N lymphoma. In conclusion, ENK/T lymphoma is a very aggressive entity, and, in our cases, the exclusively cutaneous presentation was not associated with a better prognosis. The results of our array comparative genomic hybridization analysis could be useful to better define the different ENK/T lymphoma subgroups with cutaneous involvement.

We report a case of erysipeloid in a 73-year-old woman. The disease was characterized clinically by erythematous and erosive lesions on three fingers of the left hand. Diagnosis was made based on the patient's occupation (housewife), history of previous traumatic contact with a scorpion fish, typical inflammatory lesions located on one hand, lack of severe systemic features, mild laboratory abnormalities and rapid remission after specific treatment.

Syringoma is a benign neoplasm of eccrine origin. Clinically, it manifests as small skin-colored to yellowish soft papules usually localized around the eyes and on the upper cheeks of middle-aged women. Familial cases have rarely been reported and may be inherited as an autosomal dominant trait or result from either germ line or somatic mutations. Syringoma can coexist with various conditions, notably Down syndrome. Herein, we report a family with multiple syringomas affecting members of three following generations and describe in detail a 36-year-old woman and her 17-year-old son. In the latter, steatocystoma multiplex, which is regarded as a benign cystic neoplasm of the folliculosebaceous unit or a nevoid malformation differentiated in the direction of the sebaceous duct, was associated. Acral distribution of steatocystoma multiplex and its presentation as subcutaneous nodules in this patient were unique.

Lymphogranuloma venereum (LGV) is a sexually transmitted infection endemic in Central Africa, South-East Asia and in some countries of Central and South America. In Italy LGV has been sporadically reported in patients coming from abroad. The etiological agent of LGV is Chlamydia trachomatis serovars L1, L2, L3, differentiating by pathogenetic action. The clinical course of LGV can be divided into three stages with a initial small papule, which may ulcerate, at the site of inoculation, followed by massive lymphadenopathy, which is usually unilateral, and eventually by lymphatic obstruction, causing elephantiasis. During 2004 a LGV ano-rectal clinical variant has been described as an erosive proctitis among homosexual HIV-positive men in some countries of Western Europe, not coming from endemic areas. Until now this syndrome has been often explained as chronic intestinal inflammatory disease. This report describes a case of proctitis caused by CT serovar L2; to Authors' knowledge this is the first case reported in Italy.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered to be severity variants of the same disease, which is almost always associated with drug intake. In contrast, erythema multiforme (EM) is a disorder regarded as only rarely caused by drugs. Keratinocyte apoptosis has been shown to play an important part in the pathogenesis of SJS and TEN, whilst its role in EM remains controversial. To determine the expression of apoptosis-associated molecules Fas, Fas ligand (FasL), Bcl-2 and Bax in the above disorders, an immunohistochemical analysis was performed. We studied both lesional skin from thirty patients having drug-induced EM and 5 cases classified within the SJS/TEN spec nottrum and normal skin samples. We found a keratinocyte overexpression of Fas antigen, an important molecule mediating apoptosis, not only in SJS and TEN but also in EM. Another noteworthy finding was the strong expression of Bcl-2, a protein known as blocking apoptosis, along the basal layer and in the dermal infiltrate both in SJS/TEN and in EM. Taken together, these findings suggest that Fas-dependent keratinocyte apoptosis may play a part in the pathogenesis of both SJS/TEN and EM. Fas-mediated cell death may be partially suppressed by the Bcl-2 protein.