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Background and Aims: Phenylalanine (Phe) restricted diet, combined with Phe-free l-amino acid supplementation, is the mainstay of treatment for phenylketonuria (PKU). Being the diet a key factor modulating gut microbiota composition, the aim of the present paper was to compare dietary intakes, gut microbiota biodiversity and short chain fatty acids (SCFAs) production in children with PKU, on low-Phe diet, and in children with mild hyperphenylalaninemia (MHP), on unrestricted diet.Methods and Results: We enrolled 21 PKU and 21 MHP children matched for gender, age and body mass index z-score. Dietary intakes, including glycemic index (GI) and glycemic load (GL), and fecal microbiota analyses, by means of denaturing gradient gel electrophoresis (DGGE) and Real-time PCR were assessed. Fecal SCFAs were quantified by gas chromatographic analysis.Results: We observed an increased carbohydrate (% of total energy), fiber and vegetables intakes (g/day) in PKU compared with MHP children (p = 0.047), as well a higher daily GI and GL (maximum p < 0.001). Compared with MHP, PKU showed a lower degree of microbial diversity and a decrease in fecal butyrate content (p = 0.02). Accordingly, two of the most abundant butyrate-producing genera, Faecalibacterium spp. and Roseburia spp., were found significantly depleted in PKU children (p = 0.02 and p = 0.03, respectively).Conclusion: The low-Phe diet, characterized by a higher carbohydrate intake, increases GI and GL, resulting in a different quality of substrates for microbial fermentation. Further analyses, thoroughly evaluating microbial species altered by PKU diet are needed to better investigate gut microbiota in PKU children and to eventually pave the way for pre/probiotic supplementations. [ABSTRACT FROM AUTHOR]

Background and Aims: No data exist in the current literature on the glycemic index (GI) and glycemic load (GL) of the diet of phenylketonuric (PKU) children. The aims of this study were to examine the dietary GI and GL in PKU children on a low-phenylalanine (Phe)-diet and to evaluate whether an association may exist between the carbohydrate quality and the metabolic profile.Methods: Twenty-one PKU children (age 5-11 years) and 21 healthy children, gender and age matched, were enrolled. Dietary (including GI and GL) and blood biochemical assessments were performed.Results: No difference was observed for daily energy intake between PKU and healthy children. Compared to healthy controls, PKU children consumed less protein (p = 0.001) and fat (p = 0.028), and more carbohydrate (% of total energy, p = 0.004) and fiber (p = 0.009). PKU children had higher daily GI than healthy children (mean difference (95% confidence interval), 13.7 (9.3-18.3)) and higher GL (31.7 (10.1-53.2)). PKU children exhibited lower blood total and low density lipoprotein cholesterol (LDL) levels (p < 0.01) and higher triglyceride level (p = 0.014) than healthy children, while glucose and insulin concentrations did not differ. In PKU children the dietary GL was associated with triglyceride glucose index (Spearman's correlation coefficient = 0.515, p = 0.034).Conclusion: In PKU children a relationship of the dietary treatment with GI and GL, blood triglycerides and triglyceride glucose index may exist. Improvement towards an optimal diet for PKU children could include additional attention to the management of dietary carbohydrate quality. [ABSTRACT FROM AUTHOR]

Aims: The aim of this paper is to review the possible relationship of restricted phenylalanine (Phe) diet, a diet primarily comprising low-protein foods and Phe-free protein substitutes, with major cardiovascular risk factors (overweight/obesity, blood lipid profile, plasma levels of homocysteine, adiponectin and free asymmetric dimethylarginine (ADMA), oxidative stress and blood pressure) in PKU children.Data Synthesis: In PKU children compliant with diet, blood total cholesterol, low-density lipoprotein cholesterol (LDL-C), plasma ADMA levels and diastolic pressure were reported to be lower and plasma adiponectin levels to be higher compared to healthy controls. No difference was observed in overweight prevalence and in high-density lipoprotein cholesterol (HDL-C) levels. Inconsistent results were found for plasma homocysteine levels and antioxidant status.Conclusions: PKU children compliant with diet seem to display non-different cardiovascular risks compared with the healthy population. Well-designed longitudinal studies are required to clarify the potential underlying mechanisms associated with PKU and cardiovascular risk factors. [ABSTRACT FROM AUTHOR]

In the building energy efficiency field, developing automatic and accurate fault detection and diagnosis methods is necessary in order to ensure optimal operations of systems and to save energy. In this paper first, fault detection analysis based on statistical methods where anomalies are detected through a comparison with neighborhood and averaged fault-free values and through a clustering technique is performed. Following the fault detection step, a fault diagnosis analysis based on fuzzy sets and fuzzy logic is implemented. Experimentation is carried out over a one day monitoring data set in December 2013 for the fan coil electric consumption of an actual office building located at ENEA ‘Casaccia’ Research Centre. Results show the effectiveness of proposed approaches in automatic detection and diagnosis of abnormal building fan coil electric consumption. [ABSTRACT FROM AUTHOR]

Abstract: Corrosion-fatigue is one of the key failure modes of railway axles. We show the results of a series of corrosion-fatigue experiments under rainwater with a Q&T steel adopted for manufacturing railway axles. The process can be divided into the following phases by: i) an early formation of pits followed by ii) a pit-to-crack transition at approximately 30μm diameter and iii) the growth of small cracks with a pronounced scatter. The variability of the different phases needs to be accurately described for achieving a realistic life prediction and a good comparison with full-scale experiments. [Copyright &y& Elsevier]

OBJECTIVE: To review the literature on the communicative behaviours primary care patients want from a 'good' physician. METHODS: An electronic search used the key words doctor-patient relation AND patient desires OR patient expectations OR patient preferences (from now on referred to as expectations). The qualitative and quantitative articles meeting the selection criteria were analysed separately, comparing methods, definitions, measures and outcomes. The physician behaviours desirable from a patient perspective were grouped by linking them to the communicative functions of an effective medical encounter as defined from a professional perspective. RESULTS: Twenty-seven studies were included. Critical issues were the heterogeneity of definitions and measures and the lack of integration between quantitative and qualitative findings. Most of the expectations in qualitative studies were related to the function 'Fostering the relationship'. Similar expectations arose less often in quantitative studies. CONCLUSIONS: Patients do have concrete expectations regarding each of the functions to be met in the medical encounters. The research approach tends to bias the results. PRACTICE IMPLICATIONS: The collected expectations suggest how physicians may perform each of their tasks according to the patient perspective. Future research on patients' communicative expectations needs to overcome the gap between qualitative and quantitative findings. [ABSTRACT FROM AUTHOR]

OBJECTIVE: The objective of this study was to assess whether a minimally invasive protocol can be effective in the long-term control of necrotic areas and pain in patients suffering osteonecrosis of the jaw associated with the use of bisphosphonate drugs (BRONJ). STUDY DESIGN: Thirty-four consecutive patients (14 male, 20 female) with BRONJ under treatment with zoledronate, pamidronate, or alendronate were enrolled. All of the patients received professional oral hygiene treatment and antiseptic oral rinses, and if in pain they assumed an antibiotic therapy with amoxicillin/clavulanate potassium and metronidazole for ten days. At the baseline visit, as well as at each 3-month recall, the size of the osteonecrotic lesions were measured and the pain level assessed with a visual analog scale. RESULTS: The results from the general linear model showed a statistically significant (F = 16.1; P < .01; r(2) = 0.95) time-related decrease in the size of exposed bone areas during the nonsurgical therapy (from 12.5 ± 12.0 mm to 8.8 ± 10.3 mm). CONCLUSIONS: This conservative protocol seems to provide successful treatment in the vast majority of patients. [ABSTRACT FROM AUTHOR]

Abstract: We present a study of the optical properties of Gd-doped sol–gel silica glasses densified at different temperatures (from 450 up to 1050°C) by means of optical absorption (OA) and radio luminescence (RL). The effect of a post-densification rapid thermal treatment (RTT) at approximately 1800°C is also considered. Room temperature OA and RL measurements have revealed a slight low-energy shift of Gd3+ absorption/emission lines by densification temperature increasing accompanied by a parallel increase of Gd3+ RL intensities, especially strong after RTT. These effects are interpreted on the basis of structural modifications of the silica matrix and of the removal of non-radiative channels competing with Gd3+ emission. Moreover, RL spectra of fully densified samples display high-energy shoulders on the 6P7/2–8S Gd3+ emission possibly related to crystal field splitting of the 6P7/2 state. This interpretation is supported by the temperature dependence of RL spectra, investigated in the 10–320K temperature interval: an increase of the intensity of high-energy components vs. temperature has been observed, which can be interpreted as due to thermally assisted excitation of electrons belonging to the lower 6P7/2 state to higher crystal field states and their subsequent radiative recombination. [Copyright &y& Elsevier]

Abstract: The optical properties of Yb-doped sol–gel silica glasses were studied by optical absorption and radio-luminescence (RL) measurements, that revealed the typical absorption and emission pattern of Yb3+ ions. Moreover, RL bands in the 1.5–3.5eV interval were also observed, and related to defects of the silica matrix. The RL intensity temperature dependence, investigated in the 10–320K interval, evidenced the presence of the SiO2 self-trapped exciton emission at 2.2eV, whose intensity was rapidly quenched by temperature increasing. At variance, the Yb3+ emission intensity increased markedly by temperature increasing. This phenomenon is interpreted by considering a competitive role of point defects in free carrier trapping, evidenced by parallel wavelength resolved thermally stimulated luminescence measurements. [Copyright &y& Elsevier]