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1.
Front Nutr ; 11: 1452880, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39224181

RESUMO

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. Despite their necessity for frequent hospital accesses, these patients' disabilities represent an important obstacle to accessing care, generating anxiety and concern in children and their families. Telemedicine has proven to be a promising instrument for improving pediatric patients' healthcare in several fields. By breaking down geographical and temporal barriers, telehealth may represent a valuable tool to implement in clinical practice, in order to improve patients' outcomes and quality of life. The aim of this narrative review is to provide an overview of the main nutritional issues in children with SNI, the potential implications of telemedicine in their management and the available evidence regarding the effects and benefits of telehealth.

2.
Chemistry ; : e202402294, 2024 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-39101875

RESUMO

In this work, we synthesize a series of push-pull compounds bearing naphthalimide as the electron acceptor and tetraphenylethylene (TPE)/triphenylamine (TPA)/phenothiazine (PTZ) as the electron rich/electron donor units. These moieties are arranged in highly conjugated quadrupolar structures. The structure-property relationships are investigated through a joint experimental time-resolved spectroscopic and computational TD-DFT study. The femtosecond transient absorption and fluorescence up-conversion experiments reveal ultrafast photoinduced intramolecular charge transfer. This is likely the key factor leading to efficient spin-orbit CT-induced intersystem crossing for the TPA- and PTZ-derivatives as well as to small singlet-to-triplet energy gap. Consequently, evidence for a delayed fluorescence component is found together with the main prompt emission in the fluorescence kinetics both in solution and in thin film. The weight of the Thermally Activated Delayed Fluorescence (TADF) is greatly enhanced when these fluorophores are used as guests in solid-state host matrices. TADF is interestingly revealed in the orange-red region of the visible. Such long wavelength emission is here observed with surprisingly large fluorescence quantum yields, thanks to the conjugation enhancement achieved in these newly synthesized structures relative to previous studies. Our findings may be thus promising for the future development of efficient third generation TADF-based OLEDs.

3.
Front Nutr ; 11: 1417981, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39070252

RESUMO

Neurodegenerative disorders are a group of diseases characterized by progressive degeneration of the nervous system, leading to a gradual loss of previously acquired motor, sensory and/or cognitive functions. Leukodystrophies are amongst the most frequent childhood-onset neurodegenerative diseases and primarily affect the white matter of the brain, often resulting in neuro-motor disability. Notably, gastrointestinal (GI) symptoms and complications, such as gastroesophageal reflux disease (GERD) and dysphagia, significantly impact patients' quality of life, highlighting the need for comprehensive management strategies. Gut dysbiosis, characterized by microbial imbalance, has been implicated in various GI disorders and neurodegenerative diseases. This narrative review explores the intricate relationship between GI symptoms, Gut Microbiota (GM), and neurodegeneration. Emerging evidence underscores the profound influence of GM on neurological functions via the microbiota gut-brain axis. Animal models have demonstrated alterations in GM composition associated with neuroinflammation and neurodegeneration. Our single-centre experience reveals a high prevalence of GI symptoms in leukodystrophy population, emphasizing the importance of gastroenterological assessment and nutritional intervention in affected children. The bidirectional relationship between GI disorders and neurodegeneration suggests a potential role of gut dysbiosis in disease progression. Prospective studies investigating the GM in leukodystrophies are essential to understand the role of gut-brain axis dysfunction in disease progression and identify novel therapeutic targets. In conclusion, elucidating the interplay between GI disorders, GM, and neurodegeneration holds promise for precision treatments aimed at improving patient outcomes and quality of life.

4.
PLoS One ; 19(7): e0307834, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39058743

RESUMO

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. We recontacted patients who were initially monitored from February 2020 to May 2020 to assess the presence of Long-COVID symptoms at a 2-year follow-up. METHODS: We conducted interviews to evaluate Long-COVID symptoms at the 2-year mark and investigated whether patients had contracted a second COVID-19 infection between the 1-year and 2-year follow-ups, and recorded their vaccination status. RESULTS: Out of 165 patients, 139 (84%) reported symptoms at the 1-year follow-up, while only 101 (61%) reported symptoms at the 2-year follow-up. Among patients with Long-COVID symptoms at the 2-year follow-up, the majority (80, 49%) had experienced Long-COVID at the 1-year follow-up, received the SARS-CoV-2 vaccine, and had not experienced a second infection between the two follow-ups. Both having Long-COVID at the 1-year follow-up and contracting a second infection were significant risk factors for presenting with Long-COVID at the 2-year follow-up. CONCLUSIONS: To the best of our knowledge, this study stands out as one of the few that includes a 2-year follow-up on Long-COVID symptoms using telemedicine. Telemedicine has proven to be an effective and innovative tool for long-term patient monitoring, early diagnosis, and treatment. Telemedicine represents a significant future challenge for healthcare.


Assuntos
COVID-19 , SARS-CoV-2 , Telemedicina , Humanos , COVID-19/epidemiologia , COVID-19/virologia , COVID-19/diagnóstico , Feminino , Masculino , Pessoa de Meia-Idade , SARS-CoV-2/isolamento & purificação , Idoso , Adulto , Seguimentos , Estudos de Coortes , Síndrome de COVID-19 Pós-Aguda , Vacinas contra COVID-19
5.
Mol Genet Metab Rep ; 40: 101120, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39081552

RESUMO

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.

6.
Children (Basel) ; 11(6)2024 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-38929247

RESUMO

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. Despite varying pathogen and organ involvement, they are linked by the pathological accumulation of exceeding substrates, leading to cellular toxicity and subsequent organ damage. Less severe forms of LSDs can manifest during childhood or later in life, sometimes being underdiagnosed. Respiratory impairment may stem from different pathogenetic mechanisms, depending on substrate storage in bones, with skeletal deformity and restrictive pattern, in bronchi, with obstructive pattern, in lung interstitium, with altered alveolar gas exchange, and in muscles, with hypotonia. This narrative review aims to outline different pulmonary clinical findings and a diagnostic approach based on key elements for differential diagnosis in some treatable LSDs like Gaucher disease, Acid Sphingomyelinase deficiency, Pompe disease and Mucopolysaccharidosis. Alongside their respiratory clinical aspects, which might overlap, we will describe radiological findings, lung functional patterns and associated symptoms to guide pediatric pulmonologists in differential diagnosis. The second part of the paper will address follow-up and management specifics. Recent evidence suggests that new therapeutic strategies play a substantial role in preventing lung involvement in early-treated patients and enhancing lung function and radiological signs in others. Timely diagnosis, driven by clinical suspicion and diagnostic workup, can help in treating LSDs effectively.

7.
J Clin Med ; 13(5)2024 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-38592326

RESUMO

Background: 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. For this reason, in patients with an initial clinical suspicion of Gaucher disease, we aimed to carry out a parallel screening of acid sphingomyelinase and glucocerebrosidase. Methods: Peripheral blood samples of 627 patients were collected, and enzymatic activity analysis was performed on both glucocerebrosidase and acid sphingomyelinase. The specific gene was studied in samples with null or reduced enzymatic activity. Specific molecular biomarkers helped to achieve the correct diagnosis. Results: In 98.7% of patients, normal values of glucocerebrosidase activity excluded Gaucher disease. In 8 of 627 patients (1.3%), the glucocerebrosidase enzymatic activity assay was below the normal range, so genetic GBA1 analysis confirmed the enzymatic defect. Three patients (0.5%) had normal glucocerebrosidase activity, so they were not affected by Gaucher disease, and showed decreased acid sphingomyelinase activity. SMPD1 gene mutations responsible for Acid Sphingomyelinase Deficiency were found. The levels of specific biomarkers found in these patients further strengthened the genetic data. Conclusions: Our results suggest that in the presence of typical signs and symptoms of Gaucher disease, Acid Sphingomyelinase Deficiency should be considered. For this reason, the presence of hepatosplenomegaly, thrombocytopenia, leukocytopenia, and anemia should alert clinicians to analyze both enzymes by a combined screening. Today, enzyme replacement therapy is available for the treatment of both pathologies; therefore, prompt diagnosis is essential for patients to start accurate treatment and to avoid diagnostic delay.

8.
Nutrients ; 16(7)2024 Mar 27.
Artigo em Inglês | MEDLINE | ID: mdl-38612990

RESUMO

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. Thirty-five GMP-based PSs produced by six different companies were included in this review: twenty-one powdered PSs, eight ready to drink, and six bars. The analysis revealed great heterogeneity not only among the different formulations (powdered, ready to drink, and bars) but also within the same group, in terms of energy content and nutritional composition. GMP-based PSs were shown to have higher contents of sugars and saturated fatty acids compared to L-AA PSs, especially in ready-to-drink formulations and bars. The latter also provided the highest amounts of energy among the GMP-based products. This finding may be related to a higher risk of developing overweight and obesity. The greater palatability of these GMP-based PSs, combined with improved nutritional quality, could not only improve adherence to diet therapy but also reduce the incidence of obesity-related comorbidities in PKU.


Assuntos
Caseínas , Fragmentos de Peptídeos , Fenilcetonúrias , Criança , Humanos , Itália , Obesidade
9.
Ital J Pediatr ; 50(1): 27, 2024 Feb 14.
Artigo em Inglês | MEDLINE | ID: mdl-38355648

RESUMO

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. METHODS: We conducted a prospective study involving children hospitalized from November 2021 to March 2023, with confirmed acute SARS-CoV-2 infection. A telephone survey was conducted at 3-6-12 months after discharge. RESULTS: We included 167 patients (77 F/90 M). Upon hospital admission, 95.5% of patients presented as symptomatic. Regarding patients for whom it was feasible to determine the SARS-CoV-2 variant (n = 51), the Delta variant was identified in 11 children (21.6%) and Omicron variant in the remaining 40 patients (78.4%: 27.5% BA.1 variant; 15% BA.2 variant; 57.5% BA.5 variant). 19 patients (16.5%) reported experiencing at least one symptom indicative of Long COVID (weight loss 31.6%, inappetence 26.3%, chronic cough 21.1%, fatigue 21.1%, and sleep disturbances, wheezing, abdominal pain and mood disorders 15.8%). In only 4 patients with Long COVID we could identified a specific SARS-CoV-2 variant (3 Omicron: 2 BA.1 and 1 BA.2; 1 Delta). CONCLUSIONS: this study underscores that long COVID is a significant concern in the pediatric population. Our data reinforce the importance of continuously monitoring the impact of long-COVID in infants, children, and adolescents. A follow-up following SARS-CoV-2 infection is therefore advisable, with symptom investigation tailored to the patient's age.


Assuntos
COVID-19 , Criança Hospitalizada , Adolescente , Lactente , Humanos , Criança , SARS-CoV-2 , COVID-19/epidemiologia , Síndrome de COVID-19 Pós-Aguda , Estudos Prospectivos , Itália/epidemiologia
10.
Int J Food Microbiol ; 410: 110489, 2024 Jan 30.
Artigo em Inglês | MEDLINE | ID: mdl-38039926

RESUMO

Companilactobacillus alimentarius is a facultatively heterofermentative lactic acid bacterium (LAB) that is a significant constituent within the microbiota of various traditional fermented foods exerting several functions in fermentative or ripening processes. This species has been isolated from Spanish fermented sausages, where its frequency of isolation was comparable to those of Latilactobacillus sakei and Latilactobacillus curvatus. Despite to its presence in several niches, ecological information on this species is still scarce and only few publications report information about its safety features (i.e. antibiotic resistance). Since studies on C. alimentarius concern the analysis of a few individual traits regarding this species, a more extensive work on a larger number of isolates from the same matrix have been performed to allow a clearer interpretation of their phenotypic and technological characteristics. Specifically, 14 strains of C. alimentarius isolated from Mediterranean spontaneously fermented sausages, have been screened for their safety and technological characteristics (such as antibiotic resistance, biogenic amine production, inhibiting potential, growth at different temperatures and NaCl concentrations) and with phenotype microarrays with the aim to elucidate their potential role and contribution to sausage fermentation and ripening. In general, a wide variability was observed in relation to the parameters considered. Several of the tested strains were able to produce histamine, tyramine and putrescine while the antibiotic resistance greatly varied according to the strains, with the exception of vancomycin. In addition, C. alimentarius strains showed a relevant potential to grow in conditions of salt and temperature mimicking those found in fermented foods. In particular, the growth at 10 °C and in the presence of salt can explain the presence of C. alimentarius in sausages and its adaptation to fermented meat environment in which low temperature can be applied during ripening. The differentiation of the phenotypic profile reflected the environmental conditions that influenced the isolation source, including those derived by the raw materials. Given the species frequent association with spontaneous fermentations or the ripening microbiota of various products, despite not being intentionally used as starter cultures, the data presented in this study contribute to a deeper comprehension of their role, both advantageous and detrimental, in numerous significant fermented foods.


Assuntos
Latilactobacillus sakei , Produtos da Carne , Lactobacillus , Fermentação , Aminas Biogênicas , Produtos da Carne/microbiologia
11.
Nutrients ; 15(24)2023 Dec 13.
Artigo em Inglês | MEDLINE | ID: mdl-38140353

RESUMO

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. In this narrative review, we aimed to collect the latest evidence on the pro-inflammatory potential of dietary patterns, foods, and nutrients in children affected by multifactorial diseases but also on the dietetic approaches used as treatment for specific diseases. Considering multifactorial diet-related diseases, the triggering effect of pro-inflammatory diets has been addressed for metabolic syndrome and inflammatory bowel diseases, and the latter for adults only. Future research is required on multiple sclerosis, type 1 diabetes, and pediatric cancer, in which the role of inflammation is emerging. For diseases requiring special diets, the role of single or multiple foods, possibly associated with inflammation, was assessed, but more studies are needed. The evidence collected highlighted the need for health professionals to consider the entire dietary pattern, providing balanced and healthy diets not only to permit the metabolic control of the disease itself, but also to prevent the development of NCDs in adolescence and adulthood. Personalized nutritional approaches, in close collaboration between the hospital, country, and families, must always be promoted together with the development of new methods for the assessment of pro-inflammatory dietary habits in pediatric age and the implementation of telemedicine.


Assuntos
Dieta , Doenças Inflamatórias Intestinais , Adulto , Adolescente , Humanos , Criança , Nutrientes , Dieta Saudável , Doenças Inflamatórias Intestinais/etiologia , Inflamação
12.
Curr Res Food Sci ; 7: 100615, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37881335

RESUMO

In this work, two autochthonous LAB strains (Lactiplantibacillus paraplantarum BPF2 and Pediococcus acidilactici ST6), isolated from spontaneously fermented sausages produced in Spain, were tested to produce Spanish fermented sausages (salchichón) in pilot plants, due to their promising technological and anti-listerial activity. These products were compared with a sample obtained with a commercial starter (RAP) and a spontaneously fermented control sample. Physico-chemical parameters, microbial counts, metagenomic analysis, biogenic amines content and organoleptic profile of the obtained samples were studied to assess the performances of the native starters. In fact, traditional and artisanal products obtained through spontaneous fermentations can represent an important biodiversity reservoir of strains to be exploited as new potential starter cultures, to improve the safety, quality and local differentiation of traditional products. The data underlined that ST6 strain resulted in a final lower percentage if compared with the other LAB used as starter cultures. The use of starters reduced the BA concentration observed in the sausages obtained with spontaneous fermentation and the BPF2 and ST6 strains were able to decrease the level of products rancidity. Moreover, a challenge test against L. monocytogenes were performed. The data confirmed the effectiveness in the inhibition of L. monocytogenes by the two bacteriocinogenic strains tested, with respect to RAP and control samples, highlighting their ability to produce bacteriocins in real food systems. This work demonstrated the promising application in meat industry of these autochthonous strains as starter cultures to improve sensory differentiation and recognizability of typical fermented sausages.

13.
Pharmacol Res ; 197: 106952, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37804926

RESUMO

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. Furthermore, early-life diets may influence children's neurodevelopment and cognitive deficits in adulthood. In Phenylketonuria (PKU), since the main therapeutic intervention is based on a life-long restrictive diet, important alterations of gut microbiota have been observed. Studies on PKU highlight the impact of alterations of gut microbiota on the central nervous system (CNS), also investigating the involvement of metabolic pathways, such as Trp and kynurenine (KYN) metabolisms, involved in numerous neurodegenerative disorders. An alteration of Trp metabolism with an imbalance of the KYN pathway towards the production of neurotoxic metabolites implicated in numerous neurodegenerative and inflammatory diseases has been observed in PKU patients supplemented with Phe-free amino acid medical foods (AA-MF). The present review investigates the possible link between gut microbiota and the brain in IEMs, focusing on Trp metabolism in PKU. Considering the evidence collected, cognitive and behavioral well-being should always be monitored in routine IEMs clinical management. Further studies are required to evaluate the possible impact of Trp metabolism, through gut microbiota, on cognitive and behavioral functions in IEMs, to identify innovative dietetic strategies and improve quality of life and mental health of these patients.


Assuntos
Eixo Encéfalo-Intestino , Fenilcetonúrias , Criança , Humanos , Triptofano , Qualidade de Vida , Cognição
14.
Microorganisms ; 11(8)2023 Jul 29.
Artigo em Inglês | MEDLINE | ID: mdl-37630502

RESUMO

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. Spoilage was first detected by sensory analysis using the "needle probing" technique; however, the spoiled sausages were not marketable due to the high ammonia and vinegar smell. The added starter culture did not limit or inhibit the spoilage microorganisms, which were represented by Levilactobacillus brevis, the predominant species, and by Enterococcus faecalis and E. faecium. These microorganisms grew during ripening and produced a large amount of biogenic amines, which could represent a risk for consumers. Furthermore, Lev. brevis, being a heterofermentative lactic acid bacteria (LAB), also produced ethanol, acetic acid, and a variation in the sausage colour. The production of biogenic amines was confirmed in vitro. Furthermore, as observed in a previous study, the second cause of spoilage can be attributed to moulds which grew during ripening; both the isolated strains, Penicillium nalgiovense, added as a starter culture, and P. lanosocoeruleum, present as an environmental contaminant, grew between the meat and casing, producing a large amount of total volatile nitrogen, responsible for the ammonia smell perceived in the ripening area and in the sausages. This is the first description of Levilactobacillus brevis predominance in spoiled goose sausage.

15.
Nutrients ; 15(11)2023 May 26.
Artigo em Inglês | MEDLINE | ID: mdl-37299454

RESUMO

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. The literature was screened, and preclinical studies about pathogenetic mechanisms were also included. Keywords searched for each fat-soluble vitamin (A, D, E and K), alone or in combination, were "cholestasis", "chronic liver disease", "biliary atresia", "malnutrition" and "nutritional needs". Studies published prior to the selected time range were searched manually and, when considered relevant, included within the list of references. RESULTS: Eight hundred twenty-six articles were initially screened. From these, 48 studies were selected. A comparison of the recommended methods of supplementation for fat-soluble vitamins was then carried out. The causes of malabsorption were explained and current methods for defining deficiency and monitoring complications were summarized. CONCLUSIONS: According to the literature, children with cholestasis are at a higher risk of fat-soluble vitamin deficiency. Although there are general recommendations, the treatment for vitamin deficiency is not uniformly validated.


Assuntos
Deficiência de Vitaminas , Colestase , Criança , Humanos , Vitaminas/uso terapêutico , Colestase/complicações , Deficiência de Vitaminas/complicações , Deficiência de Vitaminas/tratamento farmacológico
16.
Nutrients ; 16(1)2023 Dec 21.
Artigo em Inglês | MEDLINE | ID: mdl-38201860

RESUMO

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. A second analysis was conducted to compare them to the composition of formulas intended for healthy infants, taking into consideration the Commission Delegated Regulation (EU) 2016/127 and Commission Delegated Regulation (EU) 2016/128 for micronutrients. The analysis revealed heterogeneity among protein substitutes suitable for infants with PKU. The energy and protein equivalents (P.Eq.) content are different; all of the substitutes contain docosahexaenoic acid (DHA) and arachidonic acid (ARA), while eicosapentaenoic acid (EPA), fructo-oligosaccharides (FOS), galacto-oligosaccharides (GOS), human milk oligosaccharides (HMOs) and nucleotides are not present in all the substitutes. More attention should be paid to these infant products to ensure metabolic control of PKU, and also promote proper growth, cognitive neurodevelopment, favourable gut microbiota composition, and immune system health, while reducing the risk for non-communicable diseases (NCDs).


Assuntos
Nutrientes , Fenilalanina , Lactente , Humanos , Itália , Micronutrientes , Oligossacarídeos
17.
Foods ; 11(18)2022 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-36140904

RESUMO

Fermented meat products represent an important industrial sector in Europe, particularly in the Mediterranean Countries (MC), where the presence of numerous local productions, still obtained through spontaneous fermentation, is recognized as a formidable treasure chest of unexplored microbial biodiversity. Lactobacillaceae naturally occurring in fifteen spontaneously fermented sausages from MC (Italy, Spain, Croatia, and Slovenia) were isolated and taxonomically characterized using molecular techniques. Additionally, a safety assessment for the presence of antibiotic resistances and biogenic amine (BA) production was performed to determine their suitability as autochthonous starter cultures. Molecular typing, performed using REP-PCR, discriminated 151 strains belonging to Latilactobacillus sakei (59.6%), Latilactobacillus curvatus (26.5%) and Companilactobacillus alimentarius (13.9%). The minimum inhibitory concentrations (MICs) of eight different antibiotics revealed a high resistance to streptomycin (27%), tetracycline (16%), followed by gentamycin (14%) and kanamycin (13%). Interestingly, the results showed a geographical distribution of resistant biotypes. tetM/tetS or ermB genes were identified in only six strains. The amino-biogenic potential of the strains was assessed, confirming the absence of this trait among L. sakei, while a high number of producer strains was found among L. curvatus. On the 151 analyzed strains, 45 demonstrated safety traits for their future use as starter food cultures. These results open the way to further studies on the technological properties of these promising autochthonous strains, strongly linked to the Mediterranean environment.

18.
Microorganisms ; 10(9)2022 Sep 04.
Artigo em Inglês | MEDLINE | ID: mdl-36144388

RESUMO

Seafood products are one of the most perishable foods, and their shelf life is limited by enzymatic and microbial spoilage. Developing methods to extend the shelf life of fresh fish could reduce food waste in the fishery industry, retail stores, and private households. In recent decades, the application of lactic acid bacteria (LAB) as bioprotective cultures has become a promising tool. In this study, we evaluated the use of four starter cultures, previously selected for their properties as bioprotective agents, for sea bass and sea bream burgers biopreservation. Starter cultures impacted the microbial populations, biochemical parameters (pH, TVB-N), and sensory properties of fish burgers, during 10 days of storage at 4 °C and then 20 days at 8 °C in modified atmosphere packaging (MAP). Also, storage time influenced the microbial and physicochemical characteristics of all the tested samples, except for TVB-N values, which were significantly higher in the uninoculated burgers. The volatilome changed in the different treatments, and in particular, the samples supplemented with starter presented a profile that described their rapid growth and colonization, with the production of typical molecules derived from their metabolism. The addition of bioprotective cultures avoided bloating spoilage and improved the sensory parameters of the burgers. The shelf life of the fish burgers supplemented with starter cultures could be extended up to 12 days.

19.
Adv Food Nutr Res ; 102: 47-92, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36064296

RESUMO

Fermented foods are of great importance for their role in preserving nutrients and enriching the human diet. Fermentation ensures longer shelf life and microbiological safety of food. Natural bioactive compounds have been paid attention as nutraceuticals or functional ingredients, which have health-promoting components since polysaccharides, especially chitosan, chitin and their derivatives, are biocompatible and biodegradable, biorenewable, without toxic properties and environmentally friendly. They have been applied in several fields such as medicine, agriculture, and food industry. This chapter provides information on polysaccharides obtained from crustacean as bioactive compounds as well as their effects in fermented foods.


Assuntos
Alimentos Fermentados , Fermentação , Microbiologia de Alimentos , Humanos , Polissacarídeos , Alimentos Marinhos
20.
Food Res Int ; 160: 111685, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36076392

RESUMO

The aim of this work was to investigate the possibility to industrially produce fermented sausages without the addition of nitrate and nitrite. Indeed, despite their antimicrobial effect and multiple technological roles, an increasing pressure for their removal has recently raised. To achieve this goal while maintaining an acceptable final product quality, we deeply modified the whole process, that was carried out at 10-15 °C (i.e., temperatures lower than traditional Mediterranean products) and by using bioprotective starter cultures at high concentrations (7 log CFU/g) to lead the fermentation. Different glucose amounts (0.2 or 0.4 % w/w) were also tested to optimize the process. The results showed no significant differences between the control (with nitrate/nitrite) and the sausages without preservatives in terms of aw (value range 0.908-0.914), weight loss (about 38% in all samples), lactic acid bacteria (value range 8.1-8.3 log CFU/g) and coagulase negative cocci (value range 6.8-7.1 log CFU/g). The amount of sugar affected the final characteristics of sausages. Indeed, in the absence of curing salts, lower sugar concentration resulted in better textural features (reduced hardness and gumminess) and lower oxidation (TBARS values 0.80 vs. 1.10 mg MDA/kg of meat product in samples with 0.2% or 0.4% of glucose, respectively). Finally, challenge tests evidenced the inability of selected strains of Listeria innocua, Salmonella enterica sub. enterica and Clostridium botulinum to grow, under the adopted conditions, in fermented sausages. This research highlighted that nitrate/nitrite removal from these meat products requires accurate technological changes to guarantee the final quality.


Assuntos
Produtos da Carne , Nitritos , Glucose , Produtos da Carne/análise , Nitratos , Açúcares
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