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• A bottom-up methodology to develop cooling load profiles in wineries is proposed. • The use of seasonal performance indicators to assess energy savings is discussed. • The proposed approaches are applied to a medium-scale winery located in Italy. • Annual distribution of process cooling loads is very different than in buildings. • Customized seasonal efficiency indicators allow for reliable estimation of energy saving. The food sector is a major consumer of energy and growing efforts are being made in the search for solutions that will guarantee the efficient and sustainable use of energy resources. Among the different sectors, wineries are attracting particular interest due to the continuous growth of the global market and production. Surveys conducted in the winemaking sector have highlighted the importance of performing accurate energy audits and have identified the installation of efficient refrigeration systems as a promising solution in a variety of cases. Unfortunately, the savings achievable by efficient cooling technologies are often estimated using simplified approaches which do not take into consideration the actual operating conditions of the equipment typically variable on seasonal and daily bases. In this paper a novel bottom-up procedure is presented, aimed at developing reliable profiles for refrigeration and air-conditioning loads and at assessing the extent to which more efficient chilling units could contribute to reducing electricity consumption. The use of standard Seasonal Energy Efficiency Ratios is critically discussed and a novel customized indicator is proposed. The method is applied to a medium-scale winery producing still red and white wines and sparkling wines, for which only aggregated energy consumption data are available. After deriving detailed load profiles, it is proven that the use of standard seasonal indicators leads to 56.85% and 83.87% overestimation of potential energy savings, respectively, for low and medium temperature cooling energy uses, confirming the importance of adopting seasonal indicators customized on the actual operating conditions of chillers. [ABSTRACT FROM AUTHOR]

Background: To review the descriptive epidemiological data on neuronal ceroid lipofuscinoses (NCLs) in Italy, identify the spectrum of mutations in the causative genes, and analyze possible genotype-phenotype relations., Methods: A cohort of NCL patients was recruited through CLNet, a nationwide network of child neurology units. Diagnosis was based on clinical and pathological criteria following ultrastructural investigation of peripheral tissues. Molecular confirmation was obtained during the diagnostic procedure or, when possible, retrospectively., Results: One hundred eighty-three NCL patients from 156 families were recruited between 1966 and 2010; 124 of these patients (from 88 families) were tested for known NCL genes, with 9.7% of the patients in this sample having not a genetic diagnosis. Late infantile onset NCL (LINCL) accounted for 75.8% of molecularly confirmed cases, the most frequent form being secondary to mutations in CLN2 (23.5%). Juvenile onset NCL patients accounted for 17.7% of this cohort, a smaller proportion than found in other European countries. Gene mutations predicted severe protein alterations in 65.5% of the CLN2 and 78.6% of the CLN7 cases. An incidence rate of 0.98/100,000 live births was found in 69 NCL patients born between 1992 and 2004, predicting 5 new cases a year. Prevalence was 1.2/1,000,000., Conclusions: Descriptive epidemiology data indicate a lower incidence of NCLs in Italy as compared to other European countries. A relatively high number of private mutations affecting all NCL genes might explain the genetic heterogeneity. Specific gene mutations were associated with severe clinical courses in selected NCL forms only.

Background: Gilles de la Tourette syndrome (GTS) is a chronic childhood-onset neuropsychiatric disorder with a significant impact on patients' health-related quality of life (HR-QOL). Cavanna et al. (Neurology 2008; 71: 1410-1416) developed and validated the first disease-specific HR-QOL assessment tool for adults with GTS (Gilles de la Tourette Syndrome-Quality of Life Scale, GTS-QOL). This paper presents the translation, adaptation and validation of the GTS-QOL for young Italian patients with GTS., Methods: A three-stage process involving 75 patients with GTS recruited through three Departments of Child and Adolescent Neuropsychiatry in Italy led to the development of a 27-item instrument (Gilles de la Tourette Syndrome-Quality of Life Scale in children and adolescents, C&A-GTS-QOL) for the assessment of HR-QOL through a clinician-rated interview for 6-12 year-olds and a self-report questionnaire for 13-18 year-olds., Results: The C&A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability. Internal consistency reliability was high (Cronbach's alpha > 0.7) and validity was supported by interscale correlations (range 0.4-0.7), principal-component factor analysis and correlations with other rating scales and clinical variables., Conclusions: The present version of the C&A-GTS-QOL is the first disease-specific HR-QOL tool for Italian young patients with GTS, satisfying criteria for acceptability, reliability and validity.

To establish the incidence of neuronal ceroid-lipofuscinoses (NCL) in Italy, we sent a questionnaire to all Neuropediatric and Child Neuropsychiatric Departments (answer rate 15/34 = 44%). Diagnoses were accepted only when based on firm clinical and/or electron microscopic criteria. We collected 58 cases born between 1966-1991 (2 infantile NCL, 37 late infantile NCL, and 19 juvenile NCL). The incidence was calculated only on patients born between 1974-1984. In this period, the incidence of overall NCL in the Italian population was calculated to be 0.56 per 100,000 live births (0.36 for late infantile NCL, and 0.20 for juvenile NCL). Our data show that infantile NCL is very rare in Italy, and that late infantile seems to be the most frequent form of NCL.