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Celiac Disease (CD) continues to require a strict lifetime gluten-free diet (GFD) to maintain healthy status. Many studies have assessed the GFD nutritional adequacy in their cohorts, but an overall picture in adults and children would offer a lifetime vision to identify actionable areas of change. We aimed at assessing the nutrient intakes of adult and pediatric CD patients following a GFD diet and identifying potential areas of improvement. Systematic review was carried out across PubMed, Scopus and Scholar up to October 2022, including full-text studies that assessed the nutrient intakes of CD patients on GFD, in terms of macro- and/or micronutrients (absolute or percentage daily average). Random-effect meta-analysis and univariable meta-regression were applied to obtain pooled estimates for proportions and influencing variables on the outcome, respectively. Thirty-eight studies with a total of 2114 patients were included. Overall, the daily energy intake was 1995 (CI 1884–2106) Kcal with 47.8% (CI 45.7–49.8%) from carbohydrates, 15.5% (CI 14.8–16.2%) from proteins, and 35.8% (CI 34.5–37.0%) from fats. Of total fats, 13.2% (CI 12.4–14.0%) were saturated fats. Teenagers had the highest consumption of fats (94.9, CI 54.8–134.9 g/day), and adults presented insufficient dietary fiber intake (18.9 g, CI 16.5–21.4 g). Calcium, magnesium, and iron intakes were particularly insufficient in adolescence, whereas vitamin D was insufficient in all age groups. In conclusion, GFD may expose CD patients to high fat and low essential micronutrient intakes. Given GFD is a lifelong therapy, to prevent the occurrence of diseases (e.g. cardiovascular or bone disorders) dietary intakes need to be assessed on long-term follow-ups. [ABSTRACT FROM AUTHOR]

The gut microbiome (GM) has shown to influence hematopoietic stem cell transplantation (HSCT) outcome. Evidence on levofloxacin (LVX) prophylaxis usefulness before HSCT in pediatric patients is controversial and its impact on GM is poorly characterized. Post-HSCT parenteral nutrition (PN) is oftentimes the first-line nutritional support in the neutropenic phase, despite the emerging benefits of enteral nutrition (EN). In this exploratory work, we used a global-to-local networking approach to obtain a high-resolution longitudinal characterization of the GM in 30 pediatric HSCT patients receiving PN combined with LVX prophylaxis or PN alone or EN alone. By evaluating the network topology, we found that PN, especially preceded by LVX prophylaxis, resulted in a detrimental effect over the GM, with low modularity, poor cohesion, a shift in keystone species and the emergence of modules comprising several pathobionts, such as Klebsiella spp., [Ruminococcus] gnavus, Flavonifractor plautii and Enterococcus faecium. Our pilot findings on LVX prophylaxis and PN-related disruption of GM networks should be considered in patient management, to possibly facilitate prompt recovery/maintenance of a healthy and well-wired GM. However, the impact of LVX prophylaxis and nutritional support on short- to long-term post-HSCT clinical outcomes has yet to be elucidated. A longitudinal characterization of the gut microbiome in 30 pediatric hematopoietic stem cell transplantation patients reveals that the combination of levofloxacin prophylaxis and parental nutrition disrupts gut microbiome networks. [ABSTRACT FROM AUTHOR]

To obtain information on characteristics, management, current objective nutritional status and perception of nutritional status of children with cerebral palsy (CP) from healthcare professionals (HCPs) and caregivers. A detailed survey of several items on eight main topics (general characteristics, motor function, comorbidities, therapies, anthropometry, feeding mode and problems and perceived nutritional status) was developed and tested for the study. Correlation between nutritional status and Gross Motor Function Classification System (GMFCS) levels was assessed using continuous variables (Z-scores for weight-for-age, height-for-age, weight-for-height, and body mass index-for-age), and categorical variables (being malnourished, stunted, or wasted). HCP and caregiver perceptions of the child's nutritional status as well as agreement between perceived and objective nutritional status and agreement between perceived nutritional status and concerns about the nutritional status were analyzed. Data were available for 497 participants from eight European countries. Poorer nutritional status was associated with higher (more severe) GMFCS levels. There was minimal agreement between perceived and objective nutritional status, both for HCPs and caregivers. Agreement between HCP and caregiver perceptions of the child's nutritional status was weak (weighted kappa 0.56). However, the concerns about the nutritional status of the child were in line with the perceived nutritional status. The risk of poor nutritional status is associated with more severe disability in children and adolescents with CP. There is a mismatch between HCP and caregiver perceptions of participants' nutritional status as well as between subjective and objective nutritional status. Our data warrant the use of a simple and objective screening tool in daily practice to determine nutritional status in children and adolescents with CP. Clinical trial registration: ClinicalTrials.gov Identifier: NCT03499288 (). Use of the ESPGHAN recommendations and simple screening tools in daily practice is needed to improve nutritional care for individuals with CP. Attention should be paid to the differences in the perception of nutritional status of individuals with CP between professionals and caregivers to improve appropriate referral for nutritional support. Objective measures rather than the professional's perception need to be used to define the nutritional status of individuals with CP. [ABSTRACT FROM AUTHOR]

Enteral Nutrition (EN) is recommended as first line nutritional support for patients undergoing Allogeneic Hematopoietic Stem Cell Transplantation (allo-HSCT), but only few studies exist in the literature which compare EN to Parenteral Nutrition (PN) in the paediatric population.Forty-two consecutive paediatric patients undergoing allo-HSCT at our referral centre between January 2016 and July 2019 were evaluated. Post-transplant and nutritional outcomes of patients receiving EN for more than 7 days (EN group, n = 14) were compared with those of patients receiving EN for fewer than 7 days or receiving only PN (PN group, n = 28). In the EN group, a reduced incidence of Blood Stream Infections (BSI) was observed (p = 0.02) (n = 2 vs. n = 15; 14.3% vs. 53.6%). The type of nutritional support was also the only variable independently associated with BSI in the multivariate analysis (p = 0.03). Platelet engraftment was shorter in the PN group than in the EN group for a threshold of > 20*109/L (p = 0.04) (23.1 vs 35.7 days), but this correlation was not confirmed with a threshold of > 50*109/L. The Body Mass Index (BMI) and the BMI Z-score were no different in the two groups from admission to discharge.Our results highlight that EN is a feasible and nutritionally adequate method of nutritional support for children undergoing allo-HSCT in line with the present literature. Future functional studies are needed to better address the hypothesis that greater intestinal eubyosis maintained with EN may explain the observed reduction in BSI. [ABSTRACT FROM AUTHOR]

Background: Biosynthetic human recombinant short-acting insulin is added to parenteral nutrition (PN) admixtures to nourish glucose-intolerant patients. Insulin, however, is electrostatically attracted and inactivated by ethyl-vinyl-acetate (EVA) bags and filling system tubes. Our aim was to verify and quantify the presence of insulin in PN with and without intravenous lipid emulsion (ILE), just after addition (T0) until the infusion's end (T24).Methods: Four undiluted samples of 12 different PN complete admixtures (6 with ILE and 6 without), each containing 250 g of glucose in a 2000 mL volume, were taken and analyzed at T0 and T24 by an automated electrochemiluminescence immunoassay after the addition of biosynthetic human recombinant short-acting insulin at increasing doses (from 6 to 72 IU/bag) by an automated compounding device. Assay sensitivity was set at 2 μIU/mL. Admixtures with and without ILE were compared in terms of insulin-detected amounts at T0 and T24.Results: Regardless of the amount initially provided, insulin was missing in PN without ILE. In admixtures with ILE, the greater the insulin and ILE doses initially included, the higher the insulin availability at T0 and T24, both in absolute terms and as a percentage of the initial amount (from 3 to 81% at T0 and from 2.5 to 72.5% at T24). ILE may prevent insulin attraction to plastic surfaces.Conclusions: Insulin is recovered in the presence of ILE in PN even though considerable amounts are untraceable. This aspect needs verification. Until then, insulin should safely be injected in a different manner in uncontrolled situations. [ABSTRACT FROM AUTHOR]

Background: Intravenous fat emulsions (IVFE) with different fatty acid compositions contain vitamin E as a by-product of vegetable and animal oil during the refining processes. Likewise, other lipid-soluble vitamins may be present in IVFE. No data, however, exist about phytonadione (vitamin K1) concentration in IVFE information leaflets. Therefore, our aim was to evaluate the phytonadione content in different IVFE.Materials and Methods: Analyses were carried out in triplicate on 6 branded IVFE as follows: 30% soybean oil (100%), 20% olive-soybean oil (80%-20%), 20% soybean-medium-chain triglycerides (MCT) coconut oil (50%-50%), 20% soybean-olive-MCT-fish oil (30%-25%-30%-15%), 20% soybean-MCT-fish oil (40%-50%-10%), and 10% pure fish oil (100%). Phytonadione was analyzed and quantified by a quali-quantitative liquid chromatography-mass spectrometry (LC-MS) method after its extraction from the IVFE by an isopropyl alcohol-hexane mixture, reverse phase-liquid chromatography, and specific multiple-reaction monitoring for phytonadione and vitamin d3 (as internal standard). This method was validated through specificity, linearity, and accuracy.Results: Average vitamin K1 content was 500, 100, 90, 100, 95, and 70 µg/L in soybean oil, olive-soybean oil, soybean-MCT coconut oil, soybean-olive-MCT-fish oil, soybean-MCT-fish oil, and pure fish oil intravenous lipid emulsions (ILEs), respectively. The analytical LC-MS method was extremely effective in terms of specificity, linearity ( r = 0.99), and accuracy (coefficient of variation <5%).Conclusions: Phytonadione is present in IVFE, and its intake varies according to IVFE type and the volume administered. It can contribute to daily requirements and become clinically relevant when simultaneously infused with multivitamins during long-term parenteral nutrition. LC-MS seems adequate in assessing vitamin K1 intake in IVFE. [ABSTRACT FROM AUTHOR]

Introduction: The aim of this paper is to describe personalized pediatric parenteral admixture compounding in a specialized University Hospital. Materials and methods: Pediatric formulations require small volumes, high concentrations of nutrients and electrolyte micro-additions, thus they must be carefully evaluated by pharmacists and compounded by means of an automatic filling system in a “clean room”. Different kinds of lipid emulsions are used according to the patients’ needs: composite lipids or long and medium chain triglycerides with or without fish oil-based omega-3 fatty acid addition. Admixture stability is defined by determining particle diameter, which must be in the range of 0.4-1.0 µm, like chylomicra, for a safe infusion to patients. Analyses were carried out by means of a laser diffraction technique. Results: In the last 3 years 21,500 personalized pediatric admixtures were compounded (15,480 for children and 6,020 for neonates); the total number of treated patients was 1,700, including 550 neonates. Stability studies pointed out that, in all admixtures, the physicochemical stability did not change between t=0 and t=96 hours and particle diameter was in the expected range of 0.4-1.0 µm provided calcium concentration remained below 9 mEq/L. When calcium exceeded this level, as it is often required for neonates, in the admixtures containing fish oil-based emulsion, 12% of the particles had a diameter larger than 1.0 µm and 2% exceeded 5.0 µm immediately after compounding. Conclusions: Pediatric admixture compounding in a centralized service, and stability studies allowed to prepare any required formulation, providing patients with a safe and effective product. In particular, our analyses pointed out that admixtures containing fish oil-based emulsion and calcium concentrations higher than 9 mEq/L cannot be safely infused to pediatric patients; in these cases it is advisable to infuse the fish oil-based emulsion alone through a second intravenous line. [ABSTRACT FROM AUTHOR]

Introduction: Phytosterols may lead to parenteral nutrition-associated cholestasis. We planned this study to quantify plasma and liver phytosterols and relate them to liver histology in rats receiving different lipid emulsions. Materials and methods: Plasma phytosterols were quantified by gas chromatography-mass spectrometry in 30 inbred rats divided into 6 groups receiving total parenteral nutrition with soybean oil, soy+glutamine, olive oil, structured triglycerides, soy enteral formula, or chow. Hepatic phytosterols and cholesterol oxidation products were determined by gas chromatography-flame ionization. Data were related to liver histology. Results: Groups on total parenteral nutrition with soy and olive oil had the highest plasma sitosterol levels, while those receiving soy or soy+glutamine had more stigmasterol (p=0.0013). These last 2 groups stored high quantities of hepatic campesterol, sitosterol, and stigmasterol. Hepatic cholesterol oxidation products, similarly represented across groups, tripled in rats receiving structured triglycerides. Across groups, liver specimens were normal. Conclusions: Phytosterols seem to accumulate more in the liver, particularly in rats on total parenteral nutrition with soy or soy+glutamine. However, the liver was not damaged by short-term infusion of different phytosterols. It may be that free movement and the lack of lipophilic drugs protected the animals. [ABSTRACT FROM AUTHOR]

Introduction: A healthy diet is characterized by a variety of food and a balanced energy intake, which should accompany every human being since early childhood. Unfortunately, excessive consumption of protein, fat, and lately sugar are very common in developed countries. Sugar intakes are not easily quantifiable and comparable among subjects. Therefore, we decide to analyze dietary patterns in children of different ages and diets (with and without gluten) using a food and nutrient database and a new application called the "Zuccherometro". Patients and methods: This is a descriptive observational study conducted among children that are recruited consecutively either during a pediatric evaluation or through a school survey. Sociodemographic, nutritional and anthropometric data, degree of physical activity, and presence of medical conditions are collected. Dietary intake data are obtained by a 24 h recall diet. Results: The study analyzes 400 children: 213 girls and 187 boys. The majority of children (70.7%) are in normal weight range with similar extreme values (6.5% obese and 6.7% underweight). Celiac disease is diagnosed in 186 children. Caloric intakes are in line with the recommendations in all age-distributed groups with the exception of adolescents (11–17 years old), whose caloric intake is lower than recommendations. Protein intakes, on the contrary, are always exceeding recommendations and are significantly elevated in preschool children, (more than three times the population reference intakes). As for sugar intakes, all the children except the 11–17 years adolescents exceed the recommended cut off of 15% of daily calories. The same trend is obtained using the "Zuccherometro" that shows different percentages of age-stratified children exceeding the reference values: 1–3 years, 59% of children; 4–6 years, 68%; 7–10 years, 39.8%; 11–14 years, 25.5%; 15–17 years, 24.5%. The sugar load consists of both natural or added sugars (fructose and lactose) in food or beverages. Sugar intakes are more generously consumed by all age-stratified controls than by celiac children with the exception of the youngest ones (1–3 years old) and male adolescents. Conclusion: Since high sugar intakes are constantly accompanying children during their growth, important dietary education and coordination between families and institutions are mandatory. [ABSTRACT FROM AUTHOR]

Aim: A gluten-free diet (GFD) can expose children to excessive calories and fat intake. The study is intended to verify whether and how food intake, laboratory parameters, and growth are modified by a year of GFD. Methods: In 79 CD (coeliac disease) children (mean age 7.9 ± 3.8 years, 52 females, 27 males) diagnosed over 24 months, 24-h food diaries, food-frequency patterns, anthropometric and laboratory parameters (mainly blood sugar, insulin, lipid profile, and homocysteine) were prospectively collected before and during the first year of GFD. Nutrient intakes were compared over time and with recommendations. They were also used as regressors to explain the levels and changes of metabolic and growth variables. p-values < 0.05 were considered statistically significant. Results: Average macronutrient intake did not change during the year. Caloric intake remained below 90% (p ≤ 0.0001) and protein intake above 200% (p ≤ 0.0001) of recommendations. Lipid intake was stable at 34% of overall energy intake. Unsaturated fats increased (less omega-6 and more omega-3 with a ratio improvement from 13.3 ± 5.5 to 8.8 ± 3.1) and so did fibers, while folate decreased. The children who experienced a containment in their caloric intake during the year, presented a slower catch-up growth. Some differences were found across gender and age groups. In particular, adolescents consumed less calories, and females more omega-3. Fiber and simple sugar intakes emerged as implicated in lipid profile shift: fibers negatively with triglycerides (TG) (p = 0.033), simple sugars negatively with high-density lipoprotein (HDL) (p = 0.056) and positively with TG (p = 0.004). Waist-to-height ratio was positively associated with homocysteine (p = 0.018) and Homeostasis Model Assessment (p = 0.001), negatively with fibers (p = 0.004). Conclusion: In the short run, GFD is nutritionally very similar to any diet with gluten, with some improvements in unsaturated fats and fiber intake. Along with simple sugars containment, this may offer CD patients the opportunity for a fresh start. Caloric intakes may shift and should be monitored, especially in adolescents. [ABSTRACT FROM AUTHOR]

The newborn infant leaves a germ-free intrauterine environment to enter a contaminated extrauterine world and must have adequate intestinal defences to prevent the expression of clinical gastrointestinal disease states. Although the intestinal mucosal immune system is fully developed after a full-term birth, the actual protective function of the gut requires the microbial stimulation of initial bacterial colonization. Breast milk contains prebiotic oligosaccharides, like inulin-type fructans, which are not digested in the small intestine but enter the colon as intact large carbohydrates that are then fermented by the resident bacteria to produce SCFA. The nature of this fermentation and the consequent pH of the intestinal contents dictate proliferation of specific resident bacteria. For example, breast milk-fed infants with prebiotics present in breast milk produce an increased proliferation of bifidobacteria and lactobacilli (probiotics), whereas formula-fed infants produce more enterococci and enterobacteria. Probiotics, stimulated by prebiotic fermentation, are important to the development and sustainment of intestinal defences. For example, probiotics can stimulate the synthesis and secretion of polymeric IgA, the antibody that coats and protects mucosal surfaces against harmful bacterial invasion. In addition, appropriate colonization with probiotics helps to produce a balanced T helper cell response (Th1 = Th2 = Th3/Tr1) and prevent an imbalance (Th1 > Th2 or Th2 > Th1) contributing in part to clinical disease (Th2 imbalance contributes to atopic disease and Th1 imbalance contributes to Crohn's disease and Helicobacter pylori-induced gastritis). Furthermore, a series of pattern recognition receptors, toll-like receptors on gut lymphoid and epithelial cells that interact with bacterial molecular patterns (e.g. endotoxin (lipopolysaccharide), flagellin, etc.), help modulate intestinal innate immunity and an appropriate adaptive immune response. Animal and clinical studies have shown that inulin-type fructans will stimulate an increase in probiotics (commensal bacteria) and these bacteria have been shown to modulate the development and persistence of appropriate mucosal immune responses. However, additional studies are needed to show that prebiotics can directly or indirectly stimulate intestinal host defences. If this can be demonstrated, then prebiotics can be used as a dietary supplement to stimulate a balanced and an appropriately effective mucosal immune system in newborns and infants. [ABSTRACT FROM PUBLISHER]