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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 (https://clinicaltrials.gov/ct2/show/NCT03499288). IMPLICATIONS FOR REHABILITATIONUse 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. 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.

Additional file 1. Composition of the enteral nutrition formula.

The n-6 or omega 6 series [i.e. arachidonic acid (AA)] and the n-3 or omega-3 polyunsaturated fatty acids (n-3 PUFA), and particularly the long chain highly unsaturated ones (LC PUFA) [i.e. eicosapentaenoic and docosahexaenoic acid (EPA and DHA)], are essential for humans and work sometimes in synergy and sometimes in competition. Today, their role has been recognized as not only mere caloric, but with effects in cell differentiation and communication through a deep modulation to the cell core involved in gene regulation. These effects occur in all the body systems and organs, but with different outcomes depending on local or systemic inputs. Therefore, during fetal and neonatal development, LC PUFA contribute to growth and evolution, while, during inflammation, they orchestrate various functions of cells. Many disease conditions develop from inflammation. Obesity and metabolic syndrome are two examples in which diet and the hormonal milieu may disrupt multiple cells with release of cytokines. In the case of chronic stimuli, body metabolism changes. LC PUFA again, prove to be a potent tool to reverse or enhance these changes. In this chapter we want to focus on the effects of n-3 PUFA with regard to brain development and obesity.

la nutrizione pediatrica intesa secondo le sue diverse applicazioni con aspetti di base utili per comprendere come trattare un essere umano in evoluzione in modo corretto, a cio' segue l'applicazione della nutrizione in patologia. Da oltre un secolo si parla sempre più intensamente di nutrizione pediatrica anche se, sotto aspetti diversi, a seguito della modificazione dei target da affrontare. Questo processo in espansione vede antichi problemi da affrontare come la malnutrizione da carenza di cibo e da patologia, ma, all’opposto, nuove emergenze date dall’obesità e dalla diffusione di alimenti sempre meno genuini, di cui si conoscono i vari costituenti nutritivi quasi nei minimi particolari e non i “contaminanti” volontariamente od involontariamente utilizzati. Per far fronte a tali challenge quindi, non si può non conoscere la fisiologia del bambino che rappresenta un essere in continua evoluzione e la cui nutrizione funge da mantenimento, ma anche da fattore di crescita.

Phytosterols have been claimed as a risk factor of Parenteral Nutrition-Associated Cholestasis (PNAC). This study aims to determine phytosterol levels in plasma and liver cells and relates them to the occurrence of cholestasis in rats receiving parenteral, enteral or standard oral diet. Plasma and liver levels of phytosterols, cholesterol and other sterol were determined in 30 weaning rats divided in 6 groups receiving parenteral nutrition (PN) with soybean oil (PNSB), PN with soybean oil plus glutamine (PNSBG), PN with olive oil (PNO), PN with structured triglycerides, soy enteral formula and chow, respectively for 5 days. Phytosterols were also determined in hepatic tissues by gas-chromatography-flame ionization detector and correlated to with liver histology findings. Among phytosterols, sitosterol had the highest plasma level in all the groups. Its highest concentrations were found in the PNBS and PNO groups. Plasma stigmaterol levels were significantly higher in the PNSB and PNSBG groups. Plasma cholestanol and campesterol levels were similar across all groups. Liver campesterol, sitosterol and stigmasterol levels were highest in the PNSB and PNSBG groups. When comparing plasma and liver tissue levels, campesterol and sitosterol were more present in the liver than in the plasma, while the opposite was true for stigmasterol. None of the liver specimens showed PNAC findings. In conclusion, phytosterols seem to accumulate more in the liver than in the plasma, particularly in rats receiving Parenteral Nutrition with soybean oil (PNSB) and Parenteral Nutrition with soybean oil plus glutamine (PNSBG). No cases of PNAC appear to occur.

A case with short bowel syndrome on prolonged home-parenteral nutrition developed cholestasis, which was promptly treated with multiple therapies.

The chapter describes the principles of enteral nutrition and its applications in pediatrics.

Principi generali sull'utilizzo della nutrizione artificiale in ambito domiciliare di vitale importanza per il bambino con l'aumento delle patologie croniche

association between a rare benign tumor of the pancreas and celiac disease in a young girl.

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.

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 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.

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.

Background & Aims Interventions aimed at lifestyle changes are pivotal for the treatment of non-alcoholic fatty liver disease (NAFLD), and web-based programs might help remove barriers in both patients and therapists. Methods In the period 2010–15, 716 consecutive NAFLD cases (mean age, 52; type 2 diabetes, 33%) were treated in our Department with structured programs. The usual protocol included motivational interviewing and a group-based intervention (GBI), chaired by physicians, dietitians and psychologists (five weekly meetings, n = 438). Individuals who could not attend GBI entered a web-based intervention (WBI, n = 278) derived from GBI, with interactive games, learning tests, motivational tests, and mail contacts with the center. The primary outcome was weight loss ≥10%; secondary outcomes were alanine aminotransferase within normal limits, changes in lifestyle, weight, alanine aminotransferase, and surrogate markers of steatosis and fibrosis. Results GBI and WBI cohorts had similar body mass index (mean, 33 kg/m2), with more males (67% vs. 45%), younger age, higher education, and more physical activity in the WBI group. The two-year attrition rate was higher in the WBI group. Healthy lifestyle changes were observed in both groups and body mass index decreased by almost two points; the 10% weight target was reached in 20% of WBI cases vs. 15% in GBI (not significant). In logistic regression analysis, after adjustment for confounders and attrition rates, WBI was not associated with a reduction of patients reaching short- and long-term 10% weight targets. Liver enzymes decreased in both groups, and normalized more frequently in WBI. Fatty liver index was reduced, whereas fibrosis remained stable (NAFLD fibrosis score) or similarly decreased (Fib-4). Conclusion WBI is not less effective than common lifestyle programs, as measured by significant clinical outcomes associated with improved histological outcomes in NAFLD. eHealth programs may effectively contribute to NAFLD control. Lay summary In patients with non-alcoholic fatty liver disease, participation in structured lifestyle programs may be jeopardized by job and time constraints. A web-based intervention may be better suited for young, busy patients, and for those living far from liver units. The study shows that, following a structured motivational approach, a web-based, interactive intervention coupled with six-month face-to-face meetings is not inferior to a standard group-based intervention with respect to weight loss, adherence to healthy diet and habitual physical activity, normalization of liver enzymes, and stable surrogate markers of fibrosis.

Oral chronic graft versus host disease (cGVHD) is often refractory to systemic therapies. Additional topical treatment is commonly required. The potency of the agent, the vehicle and formulation in which it is delivered are all critical factors in determining the effectiveness of topical therapies. High potency of budesonide, combined with its very low bioavailability when absorbed through mucosal surfaces, increased the potential role in topical application for oral cGVHD. Viscous formulation increases mucosal contact time resulting in a greater decrease in mucosal inflammation. This short communication suggests that oral viscous budesonide should be considered as a potential new therapy in the management of oral cGVHD.

Home Artificial Nutrition (HAN) is a safe and efficacious technique that insures children&rsquo, s reintegration into the family, society and school. Epidemiological data on paediatric HAN in Italy are not available. Aim: to detect the prevalence and incidence of Home Parenteral Nutrition (HPN) and Home Enteral Nutrition (HEN), either via tube or mouth, in Italy in 2016. Materials and methods: a specific form was sent to all registered SIGENP members and investigators of local HAN centres, inviting them to provide the requested centre&rsquo, s data and demographics, underlying diseases and HAN characteristics of the patients. Results: we recorded 3403 Italian patients on HAN aged 0 to 19 years from 22 centres: 2277 HEN, 950 Oral Nutritional Supplements (ONS) and 179 HPN programs. The prevalence of HEN (205 pts/million inhabitants) and HPN (16 pts/million inhabitants) has dramatically increased in Italy in the last 9 years. Neurodisabling conditions were the first indication for HEN by tube or mouth while HPN is mainly requested in digestive disorders. Conclusions: HAN is a widespread and rapidly growing treatment in Italy, as well as in other European countries. Awareness of its extent and characteristics helps improving HAN service and patients&rsquo, quality of life.

Summary No data exist for vitamin A group and vitamin D2/D3 content in branded intravenous lipid emulsions (ILEs). Our goal is to evaluate and quantify their concentrations in different ILEs to assess whether they are clinically relevant. Analyses were carried out in triplicates on six ILEs: 1) 30% soybean oil-based, 2) 20% olive-soybean oil based, 3) 10 + 10% soybean - MCT coconut oil based, 4) 20% soybean-olive-MCT-fish oil based, 5) 20% soybean-MCT-fish oil based and 6) 10% pure fish oil based, respectively. Retinol group (vitamin A) and ergo-chole-calciferol (vitamin D2/D3) were analyzed and quantified by a quali-quantitative Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS) method after potassium hydroxide alkaline hydrolysis, hexane extraction, reverse phase-liquid chromatography and specific multiple-reaction-monitoring (MRM) detection. On average, measured retinol content was in the range of 200–1000 μg/L in ILEs (1,2, and 3), whereas it was higher (1000–2000 μg/L) in the ILEs containing fish-oil. Vitamin D content was in the range of 1–10 μg/L in the fish-oil based ILEs, but undetectable in those ILEs containing purely vegetable oils. This study shows that vitamin A and D contents are variably present in ILEs based on their different lipid sources. Both contents should be explicitly mentioned in the products.

Objectives: A gluten-free diet (GFD) may carry high energy and fat load. We verified lipid profile and dietary indicators cross-sectionally and prospectively in patients with celiac disease (CD). Methods: In any consecutive child receiving a GFD (group 1) or newly diagnosed as having CD (group 2), total cholesterol (TC), triglycerides (TG), high-density lipoprotein (HDL) cholesterol, blood pressure (BP), anthropometric data, physical activity, and a 24-hour food diary were collected during follow-up visits (yearly in group 1 and during the first year of GFD in group 2). Results: In group 1 (132 girls, 73 boys, 10.7±4.2 years), TC (P=0.006), TG (P=0.014), and HDL (P=0.019) were significantly higher in girls than in boys. Compared with the general pediatric population, group 1 girls had higher TC, TG, HDL, and low-density lipoprotein; group 1 boys had lower TC, TG, and low-density lipoprotein and higher HDL. TC was significantly and positively affected by age, sex, and time receiving GFD, whereas HDL was significantly and positively affected by body mass index, diastolic BP, and sex; TG was negatively affected by diastolic BP. Compared with recommendations, group 1 children introduced less calories, iron, and calcium; one-third more sodium; similar amounts of fiber; and twice as many proteins. In group 2 (20 girls, 10 boys, 8.6±3.55 years), TC did not change over time and TG diminished, whereas HDL, blood glucose, and body mass index increased; saturated fats and caloric intake were below recommendations, whereas proteins were excessively introduced. Fibers were optimal. HDL was inversely correlated to calories and saturated fat (R2=80, P=0.011). Conclusions: Lipid profiles of children with CD differ across sexes and from reference population. GFD, being unexpectedly appropriate in fibers and fat proportion, may be a contributor.