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Large neutral amino acids (LNAA) have been used on a limited number of patients with phenylketonuria (PKU) with the purpose of decreasing the influx of phenylalanine (Phe) to the brain. In an open-label study using LNAA, a surprising decline of blood Phe concentration was found in patients with PKU in metabolic treatment centres in Russia, the Ukraine, and the United States. To validate the data obtained from this trial, a short-term double-blind placebo control study was done using LNAA in patients with PKU, with the participation of three additional metabolic centres – Milan, Padua and Rio de Janeiro. The results of the short trial showed significant lowering of blood Phe concentration by an average of 39% from baseline. The data from the double-blind placebo control are encouraging, establishing proof of principle of the role of orally administered LNAA in lowering blood Phe concentrations in patients with PKU. Long-term studies will be needed to validate the acceptability, efficacy and safety of such treatment. [ABSTRACT FROM AUTHOR]

Optical fiber sensors are by now broadly accepted as an innovative and reliable device for structural health monitoring, to be used either embedded into or bonded on structures. The accuracy of the strain measurement achievable by optical fiber sensors is critically dependent on the characteristics of the bonding of the various interface layers involved in the sensor bonding/embedding (structure material and gluing agent, fiber coating and gluing agent, fiber coating and fiber core). In fact, the signal of the bonded/embedded optical fiber sensor must correspond to the strain experienced by the monitored structure, but the quality of each involved interface can affect the strain transfer. This paper faces the characterization, carried on by both mechanical tests and morphological analysis, of the strain transfer function resulting with epoxidic and vinylester gluing agent on polyimide and acrylate coated optical fibers. [ABSTRACT FROM AUTHOR]

Unlabelled: The aim of this study was to determine whether any relationship exists between the severity of mutation of the phenylalanine hydroxylase (PAH) gene and the plasma concentrations of phenylalanine (Phe) and tyrosine (Tyr) under fasting and semifasting conditions among heterozygotes in a matched case-control study. Parents of patients affected by PAH deficiency (n = 25) detected through the Italian Neonatal Screening Program and referred from January 1994 to June 2000, and parents of healthy children were investigated. In total, 68 subjects without any disease, 34 hyperphenylalaninaemia (HPA) heterozygous parents and 34 age- and gender-matched controls, were recruited. Plasma concentrations of Phe and Tyr in fasting and semifasting (1600 mg Phe oral load) conditions were the main outcome measures. DNA analysis for PAH mutations was performed in all 68 subjects. Compared with controls, heterozygotes showed higher fasting and semifasting Phe concentrations (p < 0.0001), lower semifasting Tyr concentrations (p = 0.015), lower Tyr variations (p = 0.003) and a higher Phe/Tyr ratio (p < 0.0001) in switching from fasting to semifasting conditions. Heterozygotes carrying a severe mutation showed semifasting plasma Tyr concentrations lower than controls (p = 0.019) but not significantly different from Tyr levels found in non-severe carriers (p = 0.197). The Tyr variations were minor in severe carriers than controls (p < 0.001) and non-severe carriers too, although with lower significance (p = 0.089). In six carriers of A403V mutation, parents of mild hyperphenylalaninaemics on an unrestricted diet, significant differences in variations from fasting to semifasting conditions were found compared with parents of patients on a diet.Conclusion: Although the great heterogeneity of PAH mutations limits any general conclusion, the results suggest that monitoring plasma Tyr variations may be more sensitive than plasma Phe in assessing the severity of PAH mutations in HPA heterozygotes. [ABSTRACT FROM AUTHOR]

The new Industry 4.0 scenarios offer opportunity for both the prevention of biomechanical overload risks and the implementation of extra return-to-work initiatives for people with disabilities. Wearable assistive devices (e.g., an industrial exoskeleton) can help to lower the risk of acquiring work-related musculoskeletal diseases (WMSDs) caused by manual material handling (MMH) tasks, among other things [1]. The most prevalent and expensive musculoskeletal diseases are WLBDs, which are primarily caused by manual lifting jobs [1]. This study aims to investigate the influence of TRUExo, a passive exoskeleton for the trunk composed of a textile and a mechanical component for energy restitution, on trunk coactivation in lifting activities at various risk levels (more details cannot currently be described because the device is under patent). Twelve healthy volunteers (age: 36.33±7.66 years; BMI: 22.34±2.21 kg/m2; 6 females and 6 males) completed the lifting tasks designed using the revised NIOSH lifting equation, [2], at progressively increasing lifting indexes (from 1 to 3). The tasks were performed with and without the TRUExo exoskeleton, and the activity of the erector spinae longissimus (RESL) and rectus abdominis superior (RAS) was recorded bilaterally using a 16-channel Wi-Fi transmission surface electromyograph (Mini Wave Infinity, Cometa, Milan, Italy) system at a sampling rate of 2000 Hz. Before beginning the lifting tasks, participants completed three repetitions of a specific exercise to record the isometric maximal voluntary contractions (iMVCs) for the muscles under investigation. Then, after sEMG processing and normalization respect to iMVC values, we computed the time-varying multi-muscle co-activation function (TMCf), [3]. Our findings reveal that the mean TMCf of all patients is lower with the passive exoskeleton than without it at each LI. The mean of the TMCf reduces significantly with LI when wearing the exoskeleton, according to two-way repeated-measures ANOVA and post-hoc analysis (Fig. 1B). The results reveal that the TMCf increases in relation to the risk levels both with and without the exoskeleton, but the TMCf is lower at each LI when the subject wears the exoskeleton (Fig. 1, B). These findings could lend credence to the notion that a passive exoskeleton worn during a working activity could alleviate biomechanical load and, as a result, lower the likelihood of WMSD occurrence. Furthermore, our findings highlight the TMCf's sensitivity to differentiate different risk levels even when wearing an exoskeleton, implying that it might be used as an operational tool to assess biomechanical risk in situations where traditional approaches (e.g., NIOSH's method) cannot be used. [ABSTRACT FROM AUTHOR]

Abstract: Combining the economics and the environmental and social aspects of bioenergy chain implementation is a difficult task, particularly for wood fuel production. Multiple aspects, such as financial analysis, technology development, natural resource exploitation and avoided environmental pollution, must be considered simultaneously. Thus, decision support models seem to be the proper tools for the overall definition of strategies in the wood energy sector. The objective of this study has been to develop a partial equilibrium model able to quantify the socio-economic and environmental effects of policy, technology and best biomass allocation scenarios on the forest residue chain. The model, based on multi-objective linear programming and spatial analysis, considers the financial benefits/losses and the potential trends of three compartments: sawmills, forest enterprises and energy plants. In addition, the model computes avoided emissions for bioheat and bioelectricity production and introduces an impact indicator for the road transport of biomass. Model outputs are defined using a multi criteria approach. The main results stress the importance of both environmental parameters and the implementation of organic Rankine cycle technology for the optimization of the entire bioenergy chain. The model was tested in an Italian Alpine region (province of Trento). [Copyright &y& Elsevier]

Leuzzi V, Carducci Ca, Carducci Cl, Pozzessere S, Burlina A, Cerone R, Concolino D, Donati MA, Fiori L, Meli C, Ponzone A, Porta F, Strisciuglio P, Antonozzi I, Blau N. Phenotypic variability, neurological outcome and genetics background of 6-pyruvoyl-tetrahydropterin synthase deficiency. This study aimed to investigate the clinical variability and factors implied in the outcome of 6-pyruvoyl-tetrahydropterin synthase deficiency (PTPSd). Biochemical and clinical phenotype, treatment variables, and 6-pyruvoyl-tetrahydropterin synthase ( PTS) genotype, were explored retrospectively in 19 Italian patients (12 males and 7 females, aged 4 months to 33 years). According to the level of biogenic amines in cerebrospinal fluid (CSF) at the diagnosis, the patients were classified as mild (6) (normal level) or severe (13) (abnormal low level) form (MF and SF, respectively). Blood Phe ranged from 151 to 1053 µmol/l in MF (mean ± SD: 698 ± 403) and 342–2120 µmol/l in SF (mean ± SD: 1175 ± 517) (p = 0.063). Patients with MF showed a normal neurological development (a transient dystonia was detected in one), while all SF patients except one presented with severe neurological impairment and only four had a normal neurological development. The outcome of the SF was influenced by the precocity of the treatment. Serial CSF examinations revealed a decline of 5-hydroxyindolacetic acid in MFs and an incomplete restoration of neurotransmitters in SFs: neither obviously affected the prognosis. PTS gene analysis detected 17 different mutations (seven so far unreported) (only one affected allele was identified in three subjects). A good correlation was found between genotype and clinical and biochemical phenotype. The occurrence of brain neurotransmitter deficiency and its early correction (by the therapy) are the main prognostic factors in PTPSd. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor about the prevention and treatment of cow's milk allergy, published in the journal "Archives of Disease in Childhood" as of January 2001.

This study focuses on the extraction of phenolic compounds from spent coffee grounds by coupling hydrothermal delignification with ultrasound assisted extraction. The hydrothermal treatment was carried out at three different temperatures (200 °C, 230 °C and 260 °C) and two residence times (1 h and 3 h). The ultrasound assisted extractions were performed onto dry hydrochars and slurry fraction, prior treated with three solvents: water, methanol and a water-methanol mixture. The results show that ultra sound assisted extraction applied to the hydrochars using methanol as a solvent lead to the highest extraction of total phenolic content (11.7–20.3 mg GAE/mg of dry sample). The hydrothermal process conditions strongly affect the following extraction: the mildest the delignification process conditions, the highest the extraction of phenols from hydrochar. The hydrochars show also good energy properties in terms of fuel ratio (0.3–0.7), energy yield (83.0–95.2%) and HHV values (28.5–33.2 MJ/kg) and this is promising for their usage as solid biofuels. • Hydrothermal delignification (HDL) converts coffee spent grounds into hydrochars. • The Higher Heating Values up to 28.5 MJ/kg were found for the hydrochars. • At 260 °C and 3 h, FC content is 2.4 times greater than in initial feedstock. • Ultrasound assisted extraction is an ecologic and fast method for phenol extraction. [ABSTRACT FROM AUTHOR]

Blood fatty acid status and visual function of 20 treated hyperphenylalaninemic (HPA) children, randomly allocated into two groups to receive supplementation of either long chain polyunsaturated fatty acids (LCPUFA), including docosahexaenoic acid (DHA), or placebo for 12 months, have been investigated three years after the end of the treatment. Although in the LCPUFA group blood DHA levels and P100 wave latency improved at the end of supplementation, they had returned to baseline after three years. [ABSTRACT FROM AUTHOR]

Bioplastics possess the potential to foster a sustainable circular plastic economy, but their end-of-life is still challenging. To sustainably overcome this problem, this work proposes the hydrothermal carbonization (HTC) of residual bioplastics as an alternative green path. The focus is on cellulose acetate - a bioplastic used for eyewear, cigarette filters and other applications - showing the proof of concept and the chemistry behind the conversion, including a reaction kinetics model. HTC of pure and commercial cellulose acetates was assessed under various operating conditions (180-250 °C and 0-6 h), with analyses on the solid and liquid products. Results show the peculiar behavior of these substrates under HTC. At 190-210 °C, the materials almost completely dissolve into the liquid phase, forming 5-hydroxymethylfurfural and organic acids. Above 220 °C, intermediates repolymerize into carbon-rich microspheres (secondary char), achieving solid yields up to 23 %, while itaconic and citric acid form. A comparison with pure substrates and additives demonstrates that the amounts of acetyl groups and derivatives of the plasticizers are crucial in catalyzing HTC reactions, creating a unique environment capable of leading to a total rearrangement of cellulose acetates. HTC can thus represent a cornerstone in establishing a biorefinery for residual cellulose acetate., (© 2024 The Authors. ChemSusChem published by Wiley-VCH GmbH.)

Bow-riding occurs when dolphins swim in the pressure waves at the front of a vessel. Bow-riding is hypothesized to be "fun" for dolphins or to save them energy although the energetics have not been explored. An UAS (Unoccupied Aerial System) was used to follow and video-record adult dusky dolphins (Lagenorhynchus obscurus) bow-riding in front of a research vessel or free-swimming off Kaikoura, New Zealand. Videos of individual dolphins swimming in a linear direction at consistent speeds were analyzed with respiration rate used as a proxy for energy expenditure (bow riding n = 51; free-swimming n = 62). The respiration rates of bow-riding dolphins remained relatively constant across swimming speeds and were 45% lower than free-swimming dolphins at speeds exceeding 4 m/s, indicating substantial energetic savings. The respiration rates of free-swimming dolphins increased exponentially with speed, suggesting that dolphins incur comparatively high energetic expenditures from swimming rapidly. This research advances understanding of the biological function of bow-riding behavior and supports the energy saving hypothesis. Swimming energetics can be used to assess the impacts of anthropogenic disturbances to dolphin energy budgets., Competing Interests: Declarations. Competing interests: The authors declare no competing interests., (© 2024. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Background: Multisystem inflammatory disease in children (MIS-C) is a post-infectious condition following coronavirus disease-19 infection. Long-term follow-up data suggests that initial clinical severity does not necessarily correlate with long-term outcomes. The long-term immunological response in children with MIS-C remains poorly understood. We analyzed cytokine profiles at diagnosis and during follow-up, in pediatric patients with MIS-C, exploring correlations among cytokine expressions and standard biochemical and hormonal test results., Methods: Twenty-five MIS-C patients (mean 9.4 ± 3.9) with complete test results at diagnosis and at 6- and 12-months follow-up were included in the study. Selected cytokines, such as IL-9, eotaxin, IP-10, MIP-1β, RANTES, MCP-1(MCAF), TNF-α, PDGF-B, IL-4, and MIP-1α, were included in the analysis., Results: IP-10, MCP-1 (MCAF), and MIP-1α levels normalized or nearly normalized at 6-12 months, the remaining cytokines, including IL-9, eotaxin, MIP-1β, RANTES, TNF-α, PDGF-B, IL-4, remained higher in MIS-C than in controls at our last follow-up time. At 6 months post-diagnosis, a mild negative correlation between triglycerides and HOMA-IR with MCP-1 (MCAF), IL-4, and Eotaxin was noted. At the 12-month follow-up we found a mild positive correlation of cortisol and ACTH levels with PDGF-B, MIP-1α, and TNF-α. Conversely, a negative correlation between these cytokines with fasting glucose and HOMA-IR was observed., Conclusions: Our study findings highlight a notable cytokine-mediated inflammatory response in pediatric patients with MIS-C, characterized by sustained elevated levels over a 12-month monitoring period compared to the control group. We have identified various interrelationships among different cytokines, as well as correlations between heightened cytokine levels and metabolic and hormonal patterns. The pronounced inflammatory response underscores its involvement in acute organ damage, while its persistence suggests potential implications for long-term metabolic disorders., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

The lifestyle of the population has undergone significant changes due to the COVID-19 pandemic, which could have influenced alterations in dietary habits and overall well-being among workers. This study aimed to evaluate healthy eating practices and their relationship with the workers' quality of life and physical activity during the COVID-19 pandemic. This was a cross-sectional investigation involving workers in the city of Curitiba, southern Brazil. The study was conducted through the application of an online questionnaire. The data were evaluated using non-parametric tests and fitting a logistic regression model. A total of 123 workers participated in the study, most of them male (53.2%), aged between 31 and 40 years (42.2%), with a predominance of workers with postgraduate degrees (62.6%, n = 77), and the majority of workers (68.2%, n = 84) were performing their professional activities remotely for at least one day during the week, and 73.2% ( n = 90). It was observed that 52.8% had excellent healthy eating practices, and the older their age and the greater the practice of physical activity (time and frequency), the better the workers' healthy eating practices. When assessing quality of life, the lowest average score for healthy eating practices was in the domain of social relationships. A direct relationship of older age, social relationships, and the practice of physical activity with the best individuals' healthy eating practices was detected. Considering that remote work continues to be adopted post-pandemic, evaluating the dietary practices, physical activity, and quality of life of workers is necessary to understand this new labour phenomenon., Competing Interests: The authors declare no conflict of interest., (© The Author(s) 2024.)

Context: The safety of enteral formulas is important to restore and maintain the health of patients., Objective: A systematic review of the literature was conducted to assess the microbiological contamination present in enteral tube feeding prepared in hospitals and/or at home., Data Sources: A systematic search was conducted of the Medline, Scopus, BVS, CAPES/MEC, Embase, Science Direct, and SciELO databases and gray literature., Data Extraction: Eligible studies that analyzed the contamination of enteral formulas manipulated in hospitals and/or at home were selected; a quality assessment tool was used., Data Analysis: Twenty-three studies evaluated 1099 enteral formulations. Of these, 44.67% of enteral formulas (n = 491) exceeded the acceptable bacterial count. Samples of homemade enteral formulation preparations (86.03%; n = 191) had the highest bacterial counts, followed by mixed preparations (79.72%; n = 59), and commercial formulas (30.01%; n = 241). The number of samples of enteral formulations that exceeded the bacterial count at home was 70.79% (n = 160 at the hospital was 37.91% (n = 331). Total coliforms (82.68%; n = 406) and mesophilic aerobes (79.22%; n = 389) were the most common microorganisms. Samples with bacterial pathogens were also identified, with Bacillus cereus (4.07%; n = 20) and Listeria monocytogenes (3.66%; n = 18) being the most prevalent., Conclusions: A high number of samples of enteral formulations exceeded the bacterial count, but the risk to patient's health when consuming enteral tube feeding prepared in hospitals or at home may be low. This is because the bacteria present in the samples are not considered potential causes of disease but rather indicators of hygiene conditions., Systematic Review Registration: PROSPERO registration no. CRD42022367573., (© The Author(s) 2023. Published by Oxford University Press on behalf of the International Life Sciences Institute. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

For 88 years, biologists and engineers have sought to understand the hydrodynamics enabling dolphins to swim at speeds seemingly beyond their energetic capabilities, a phenomenon known as Gray's paradox. Hydromechanical models calculating the drag of swimming dolphins estimated power requirements for sustained high-speed swimming, which were physiologically impossible. Using an uncrewed aerial vehicle, we calculated the total power of free-ranging dusky dolphins ( Lagenorhynchus obscurus ) at speeds from 0.9 to 6.9 m s -1 , deriving drag coefficients ( Cd ) and drag. Our results showed that the Cd decreased exponentially with speed, reducing drag by up to 89% at speeds >2 m s -1 , with an additional 17% reduction during porpoising (>4.0 m s -1 ). At 6.9 m s -1 , drag was 32 N, with a power of 15.8 W kg -1 , nearly identical to the mass-specific allometric prediction for the maximum aerobic capacity of other mammals and physiologically possible. The Cd at speeds >2.5 m s -1 indicated reduced turbulence in the boundary layer around the dolphin's body, thereby reducing drag. The ability of dusky dolphins to swim at sustained high speeds resulted from an exponential decrease in Cd , which was further reduced by porpoising, thereby explaining the low drag and locomotory power that resolved Gray's paradox.

The quality of preparations offered in the workplace can vary according to the different segments of food services and may impact the health of the workers. This study aimed to qualitatively assess the food preparation offered to workers in from different food services. A total of 384 preparations were offered to workers in Curitiba City, Brazil. The preparations from three different segments of food services were evaluated: commercial (pilot study), non-commercial, and outsourced, selected for convenience. To identify the preparations, the nutritionist was interviewed, and the production process was monitored. The Score for Qualitative Assessment of Preparations (EAQP) was applied to evaluate the preparations, and they were classified according to their quality: high, intermediate, low, and very low quality. The chi-square and Kruskal-Wallis tests with post-hoc Least Significant Difference (LSD) Test were used. Most of the preparations were of high quality (72.9%), using mainly the unprocessed or minimally processed ingredients. The preparations offered by the non-commercial food service provider had a better mean quality score when compared to other food services ( p  < 0.01). This study outcome is essential to help food service professionals to decide and choose the ingredients used in the preparations., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Dohms, Stangarlin-Fiori and Medeiros.)

Olipudase alfa is indicated for the non-central nervous system manifestations of Acid sphingomyelinase deficiency (ASMD). Anaphylaxis is a very rare and life-threatening adverse reaction described for this drug. Here, we report the case of a 2-year-old boy affected by chronic neurovisceral ASMD who experienced signs of hypersensitivity reactions to olipudase alfa since the administered dose of 1 mg/kg during dose escalation and a proper anaphylactic reaction during the second administration of the target therapeutic dose of 3 mg/kg. The treatment was stopped for 15 weeks and then a 7-step desensitization protocol with the infused dose of 0.03 mg/kg was applied. Subsequent gradual dose escalation was resumed, successfully reaching the dose of 0.3 mg/kg. Moreover, biochemical, and radiological disease indexes, which were increased during treatment discontinuation, have gradually improved since the restart of treatment. However, at the second administration of the dose of 0.6 mg/kg, the patient experienced another adverse drug reaction with facial urticarial rash and bronchospasm, requiring the administration of adrenaline, methylprednisolone, and inhaled salbutamol. This case report highlights the need to customize the olipudase alfa desensitization protocol according to individual tolerance and raises the issue of achieving the established therapeutic target in the most sensitive children., Synopsis: We report a case of anaphylaxis to olipudase alfa in a child affected by chronic neurovisceral Acid sphingomyelinase deficiency (ASMD) and describe a 7-step desensitization procedure. This procedure, with the total administered dose of 0.03 mg/kg, followed by gradual dose escalation, allowed to reach the dose of 0.3 mg/kg without adverse reactions; however, at the second administration of the dose of 0.6 mg/kg our patient presented another adverse reaction suggesting the need of a different desensitization strategy., Competing Interests: The authors declare no conflict of interest., (© 2024 The Authors.)

Background: Mobile technology is increasingly prevalent in healthcare, serving various purposes, including remote health monitoring and patient self-management, which could prove beneficial to early hospital discharges., Aims: This study investigates the transitional care program experience facilitating early discharges in a pediatric setting through the use of an easy-to-use mobile medical device (TytoCare™, TytoCare Ltd., Natanya, Israel)., Outcomes: This study aims to assess the effectiveness of telehomecare in achieving complete resolution of diseases without readmission, compare the length of stay between intervention and standard care groups, and gather user and professional experiences., Methods: A randomized open-label, controlled pilot study enrolled 102 children, randomly assigned to the telehomecare (TELE) group ( n = 51, adopting early hospital discharge with continued home monitoring) or the standard-of-care (STAND) group ( n = 51). Primary outcomes include complete disease resolution without readmission. Secondary objectives include recording a shorter length of stay in the intervention group. Surveys on user and professional experiences were conducted. A group of 51 children declining telemedicine services (NO-TELE) was also included., Results: In the TELE group, 100% of children achieved complete disease resolution without readmission, with a median duration of stay of 4 days, significantly shorter than the 7 days in the STAND group ( p = 0.01). The telemedicine system demonstrated efficient performance and high satisfaction levels. The NO-TELE group showed no significant differences in demographics or digital technology competence. Perceived benefits of telemedicine included time and cost savings, reduced hospital stays, and technology utility and usability., Conclusions: This study demonstrates that user-friendly mobile medical devices effectively facilitate early hospital discharges in a pediatric setting. These devices serve as a bridge between home and hospital, optimizing care pathways.