Microbiological Safety and Functional Properties of a Fermented Nut-Based Product.

Fermented nut-based products, obtained after soaking and fermentation, are gaining increasing interest as animal food substitutes because of ethical, environmental and health reasons. In these products, Lactic Acid Bacteria (LAB) perform the fermentation, leading to matrix acidification and contributing to controlling spoilage and pathogenic microbiota. In this work, LAB strains isolated from an artisanal product and combined with a commercial strain were added as starter cultures during nut soaking to produce a cheese-like fermented plant-based product.

Vitamin B12 deficiency in newborns: impact on individual's health status and healthcare costs.

Objectives: The identification of vitamin B12 (B12) deficiency in the newborn may prevent neurological damage and a delay in the normal growth. In this study we characterized the incidence of B12 deficiency in newborns, the costs associated to the clinical diagnosis and management, and the relevance to optimize the use of cobalamin biomarkers during treatment follow-up.

Methods: Starting from a continuous case series of 146,470 screened newborns (November, 1st 2021- December, 3rd 2023), the Regional Reference Laboratory for Neonatal Screening identified 87 newborns having altered levels of biomarkers of cobalamin metabolism measured by Newborn Screening.

Telemedicine in nutritional management of children with severe neurological impairment: implication for quality of life.

Children with severe neurological impairment (SNI) frequently present feeding problems requiring a close monitoring of their nutritional status. In addition to constant clinical monitoring of body composition and nutritional indexes in these patients, frequent reports of dietary intake and weight gain variations are useful to ensure proper nutritional management. Furthermore, non-oral feeding is often needed to avoid malnutrition or aspiration pneumonia, constantly necessitating medical assistance.

Chlorpyrifos induces cytotoxicity via oxidative stress and mitochondrial dysfunction in HepG2 cells.

Chlorpyrifos (CPF), a widely used broad-spectrum organophosphate pesticide, has been associated with various adverse health effects in animals and humans. While its primary mechanism of action involves the irreversible inhibition of acetylcholinesterase, secondary mechanisms have also been suggested. The aim of the present study was to explore the secondary mechanisms of action involved in CPF-induced acute cytotoxicity using human hepatocarcinoma HepG2 cells.

Desensitization of olipudase alfa-induced anaphylaxis in a child with chronic neurovisceral acid sphingomyelinase deficiency.

Unlabelled: 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.

Long-COVID symptom monitoring: Insights from a two-year telemedicine study.

Background: The diverse manifestations of Long-COVID have become increasingly important due to their significant impact on patients' lives. Telemedicine has emerged as an important tool for post COVID-19 follow-up. This study is part of a large cohort study involving COVID-positive patients monitored by the COD19 telemedicine platform operations center.

Novel selective proline-based peptidomimetics for human cathepsin K inhibition.

Human cathepsin K (CatK) stands out as a promising target for the treatment of osteoporosis, considering its role in degrading the bone matrix. Given the small and shallow S2 subsite of CatK and considering its preference for proline or hydroxyproline, we now propose the rigidification of the leucine fragment found at the P2 position in a dipeptidyl-based inhibitor, generating rigid proline-based analogs. Accordingly, with these new proline-based peptidomimetics inhibitors, we selectively inhibited CatK against other human cathepsins (B, L and S).

Lung Diseases and Rare Disorders: Is It a Lysosomal Storage Disease? Differential Diagnosis, Pathogenetic Mechanisms and Management.

Pulmonologists may be involved in managing pulmonary diseases in children with complex clinical pictures without a diagnosis. Moreover, they are routinely involved in the multidisciplinary care of children with rare diseases, at baseline and during follow-up, for lung function monitoring. Lysosomal storage diseases (LSDs) are a group of genetic diseases characterised by a specific lysosomal enzyme deficiency.

Glycomacropeptide-Based Protein Substitutes for Children with Phenylketonuria in Italy: A Nutritional Comparison.

Advancements in food science technology have allowed the development of new products for the therapeutic management of inherited metabolic diseases such as phenylketonuria (PKU). Glycomacropeptide (GMP), a peptide derived from casein, is naturally low in phenylalanine (Phe) and, thus, adequate for protein substitutes (PSs) for the management of PKU in children. This review aims primarily to analyse the differences in the nutritional composition of GMP-based protein substitutes in different formulations (ready to drink, powdered, and bars), and secondarily to assess the quality of these products, comparing their nutritional composition with that of standard amino acid (L-AA) mixtures.

Gaucher Disease or Acid Sphingomyelinase Deficiency? The Importance of Differential Diagnosis.

Gaucher disease is a lysosomal storage disorder caused by functional glucocerebrosidase enzyme deficiency. Hepatosplenomegaly and hematological complications are found in both Gaucher disease and Acid Sphingomyelinase Deficiency, which is caused by acid sphingomyelinase dysfunction. The possible overlap in clinical presentation can cause diagnostic errors in differential diagnosis.

Long-term effects of SARS-CoV-2 infection in hospitalized children: findings from an Italian single-center study.

Background: Limited evidence exists regarding the association between COVID-19 and Long COVID manifestations in children, particularly concerning variants of concern (VOCs). We aimed to characterize a cohort of pediatric patients hospitalized with confirmed acute SARS-CoV-2 and monitor them for Long COVID symptoms. Additionally, it seeks to explore any potential correlations between VOCs and clinical symptoms.

Are Phe-Free Protein Substitutes Available in Italy for Infants with PKU All the Same?

Breastfeeding or standard infant formulas, alongside phenylalanine (Phe)-free protein substitutes, constitute the dietary management for infants with PKU to guarantee protein requirements are met in compliance with metabolic tolerance. This work aims to analyse the nutritional composition of Phe-free infant protein substitutes, in terms of macronutrients, micronutrients and functional components, available for PKU dietary management in Italy. A total of seven infant Phe-free protein substitutes were included in this review, six powder and one liquid.

Dietary Inflammatory Potential in Pediatric Diseases: A Narrative Review.

Inflammatory status is one of the main drivers in the development of non-communicable diseases (NCDs). Specific unhealthy dietary patterns and the growing consumption of ultra-processed foods (UPFs) may influence the inflammation process, which negatively modulates the gut microbiota and increases the risk of NCDs. Moreover, several chronic health conditions require special long-term dietary treatment, characterized by altered ratios of the intake of nutrients or by the consumption of disease-specific foods.

Coloration and Fire Retardancy of Transparent Wood Composites by Metal Ions.

Transparent wood composites (TWs) offer the possibility of unique coloration effects. A colored transparent wood composite (C-TW) with enhanced fire retardancy was impregnated by metal ion solutions, followed by methyl methacrylate (MMA) impregnation and polymerization. Bleached birch wood with a preserved hierarchical structure acted as a host for metal ions.

Possible role of tryptophan metabolism along the microbiota-gut-brain axis on cognitive & behavioral aspects in Phenylketonuria.

Cognitive and psychiatric disorders are well documented across the lifetime of patients with inborn errors of metabolism (IEMs). Gut microbiota impacts behavior and cognitive functions through the gut-brain axis (GBA). According to recent research, a broad spectrum of GBA disorders may be influenced by a perturbed Tryptophan (Trp) metabolism and are associated with alterations in composition or function of the gut microbiota.

Microbial Spoilage of Traditional Goose Sausages Produced in a Northern Region of Italy.

Recently, during the ripening of goose sausage, a defect consisting of ammonia and vinegar smell was noticed. The producer of the craft facility, located in Lombardia, a Northern region of Italy, asked us to identify the cause of that defect. Therefore, this study aimed to identify the potential responsible agents for the spoilage of this lot of goose sausages.

Bio-Based Binder Development for Lithium-Ion Batteries.

The development of rechargeable lithium-ion battery (LIB) technology has facilitated the shift toward electric vehicles and grid storage solutions. This technology is currently undergoing significant development to meet industrial applications for portable electronics and provide our society with "greener" electricity. The large increase in LIB production following the growing demand from the automotive sector has led to the establishment of gigafactories worldwide, thus increasing the substantial consumption of fossil-based and non-sustainable materials, such as polyvinylidene fluoride and/or styrene-butadiene rubber as binders in cathode and anode formulations.

Performance analysis of indicators of chaos for nonlinear dynamical systems.

The efficient detection of chaotic behavior in orbits of a complex dynamical system is an active domain of research. Several indicators have been proposed, and new ones have recently been developed in view of improving the performance of chaos detection by means of numerical simulations. The challenge is to predict chaotic behavior based on the analysis of orbits of limited length.

ICARUS at the Fermilab Short-Baseline Neutrino program: initial operation.

The ICARUS collaboration employed the 760-ton T600 detector in a successful 3-year physics run at the underground LNGS laboratory, performing a sensitive search for LSND-like anomalous appearance in the CERN Neutrino to Gran Sasso beam, which contributed to the constraints on the allowed neutrino oscillation parameters to a narrow region around 1 eV. After a significant overhaul at CERN, the T600 detector has been installed at Fermilab. In 2020 the cryogenic commissioning began with detector cool down, liquid argon filling and recirculation.

Fat-Soluble Vitamins Deficiency in Pediatric Cholestasis: A Scoping Review.

Background: This review aims to identify the current indications and gaps in the management of fat-soluble vitamins in pediatric patients with cholestasis.

Methods: A comprehensive review of the literature using PubMed, Scopus, Web of Science and Embase was performed. Two authors independently identified the most relevant studies published over the past 20 years up to February 2022, including original papers, narrative reviews, observational studies, clinical trials, systematic reviews and meta-analyses.