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Background and aimsTo investigate the incidence and the severity of COVID-19 infection in patients enrolled in the database for home parenteral nutrition (HPN) for chronic intestinal failure (CIF) of the European Society for Clinical Nutrition and Metabolism (ESPEN).MethodsPeriod of observation: March 1st, 2020 March 1st, 2021. Inclusion criteria: patients included in the database since 2015 and still receiving HPN on March 1st, 2020 as well as new patients included in the database during the period of observation. Data related to the previous 12 months and recorded on March 1st 2021: 1) occurrence of COVID-19 infection since the beginning of the pandemic (yes, no, unknown); 2) infection severity (asymptomatic; mild, no-hospitalization; moderate, hospitalization no-ICU; severe, hospitalization in ICU); 3) vaccinated against COVID-19 (yes, no, unknown); 4) patient outcome on March 1st 2021: still on HPN, weaned off HPN, deceased, lost to follow up.ResultsSixty-eight centres from 23 countries included 4680 patients. Data on COVID-19 were available for 55.1% of patients. The cumulative incidence of infection was 9.6% in the total group and ranged from 0% to 21.9% in the cohorts of individual countries. Infection severity was reported as: asymptomatic 26.7%, mild 32.0%, moderate 36.0%, severe 5.3%. Vaccination status was unknown in 62.0% of patients, non-vaccinated 25.2%, vaccinated 12.8%. Patient outcome was reported as: still on HPN 78.6%, weaned off HPN 10.6%, deceased 9.7%, lost to follow up 1.1%. A higher incidence of infection (p = 0.04), greater severity of infection (p < 0.001) and a lower vaccination percentage (p = 0.01) were observed in deceased patients. In COVID-19 infected patients, deaths due to infection accounted for 42.8% of total deaths.ConclusionsIn patients on HPN for CIF, the incidence of COVID-19 infection differed greatly among countries. Although the majority of cases were reported to be asymptomatic or have mild symptoms only, COVID-19 was reported to be fatal in a significant proportion of infected patients. Lack of vaccination was associated with a higher risk of death. Background and aimsTo investigate the incidence and the severity of COVID-19 infection in patients enrolled in the database for home parenteral nutrition (HPN) for chronic intestinal failure (CIF) of the European Society for Clinical Nutrition and Metabolism (ESPEN).MethodsPeriod of observation: March 1st, 2020 March 1st, 2021. Inclusion criteria: patients included in the database since 2015 and still receiving HPN on March 1st, 2020 as well as new patients included in the database during the period of observation. Data related to the previous 12 months and recorded on March 1st 2021: 1) occurrence of COVID-19 infection since the beginning of the pandemic (yes, no, unknown); 2) infection severity (asymptomatic; mild, no-hospitalization; moderate, hospitalization no-ICU; severe, hospitalization in ICU); 3) vaccinated against COVID-19 (yes, no, unknown); 4) patient outcome on March 1st 2021: still on HPN, weaned off HPN, deceased, lost to follow up.ResultsSixty-eight centres from 23 countries included 4680 patients. Data on COVID-19 were available for 55.1% of patients. The cumulative incidence of infection was 9.6% in the total group and ranged from 0% to 21.9% in the cohorts of individual countries. Infection severity was reported as: asymptomatic 26.7%, mild 32.0%, moderate 36.0%, severe 5.3%. Vaccination status was unknown in 62.0% of patients, non-vaccinated 25.2%, vaccinated 12.8%. Patient outcome was reported as: still on HPN 78.6%, weaned off HPN 10.6%, deceased 9.7%, lost to follow up 1.1%. A higher incidence of infection (p = 0.04), greater severity of infection (p < 0.001) and a lower vaccination percentage (p = 0.01) were observed in deceased patients. In COVID-19 infected patients, deaths due to infection accounted for 42.8% of total deaths.ConclusionsIn patients on HPN for CIF, the incidence of COVID-19 infection differed greatly among countries. Although the majority of cases were reported to be asymptomatic or have mild symptoms only, COVID-19 was reported to be fatal in a significant proportion of infected patients. Lack of vaccination was associated with a higher risk of death.

Variants in the ACTG2 gene, encoding a protein crucial for correct enteric muscle contraction, have been found in patients affected with chronic intestinal pseudo-obstruction, either congenital or late-onset visceral myopathy, and megacystis-microcolon-intestinal hypoperistalsis syndrome. Here we report about ten pediatric and one adult patients, from nine families, carrying ACTG2 variants: four show novel still unpublished missense variants, including one that is apparently transmitted according to a recessive mode of inheritance. Four of the remaining five probands carry variants affecting arginine residues, that have already been associated with a severe phenotype. A de novo occurrence of the variants could be confirmed in six of these families. Since a genotype-phenotype correlation is affected by extrinsic factors, such as, diagnosis delay, quality of clinical management, and intra-familial variability, we have undertaken 3D molecular modeling to get further insights into the effects of the variants here described. The present findings and further ACTG2 testing of patients presenting with intestinal pseudo-obstruction, will improve our understanding of visceral myopathies, including implications in the prognosis and genetic counseling of this set of severe disorders.

Background Malnutrition is common among hospitalized children with chronic diseases and increases hospital care needs. The aim of this study is to estimate the clinical consequences of nutrition therapy (NT) after discharge. Methods A retrospective analysis of all pediatric inpatients with diagnosis of malnutrition hospitalized at our center from January 2017 to February 2018 was conducted. Malnutrition was assessed according to body mass index (BMI) z-score, routinely recorded in patient's files. The treatment group consists of all patients referred to nutrition assessment and treated by the clinical nutrition team; all the other patients not receiving NT are selected as the control group. The effect of NT on rehospitalization rates, length of stay (LOS), and emergency room (ER) visits was estimated for the total cohort and in a propensity score (PS) matched sample. Results 277 malnourished pediatric inpatients were enrolled and analyzed. NT was prescribed in 111 patients (40%). Rehospitalization rate was lower in the treated group (rate ratio [RR] = 0.797; 95% CI, 0.630-1.009); particularly, nonelective hospital admissions are considerably lower (RR = 0.556; 95% CI, 0.325-0.952). The strength of this association increased in the PS-matched sample. There is no clear evidence of NT's effect on ER visits (RR = 0.892; 95% CI, 0.580-1.373) or LOS per episode (Δ = 1.46 days; 95% CI, -3.39 to 6.31). Conclusions Detecting and treating malnutrition seems to promptly improve the patients' clinical course after discharge, reducing the number of subsequent hospitalizations, particularly nonelective ones, probably caused by unresolved, ongoing malnutrition.

Simple Summary Nutritional management is an underestimated issue in treating pediatric cancer, since a systematic approach is currently lacking. In this consensus statement, a cohort of 12 experts selected from four different tertiary pediatric oncology centers formulated 21 clinical questions regarding the identification and treatment of nutritional issues in children with cancer. These questions were discussed, and practical recommendations were provided. With this paper, we aimed to give consensus-based guidance for addressing the nutritional needs of children with cancer, filling a gap in the field. Malnutrition, intended as both overnutrition and undernutrition, is a common problem in children with cancer, impacting quality of life as well as survival. In addition, nutritional imbalances during childhood can significantly affect proper growth. Nevertheless, there is currently a lack of a systematic approach to this issue in the pediatric oncology population. To fill this gap, we aimed to provide practice recommendations for the uniform management of nutritional needs in children with cancer. Twenty-one clinical questions addressing evaluation and treatment of nutritional problems in children with cancer were formulated by selected members from four Italian Association of Pediatric Hematology and Oncology (AIEOP) centers and from the Survivorship Care and Nutritional Support Working Group of Alliance Against Cancer. A literature search in PubMed was performed; during two consensus meetings, all recommendations were discussed and finalized using the nominal group technique. Members representing every institution voted on each recommendation. Finally, recommendations were approved by all authors.

Protein‐losing enteropathy and chyle leakage may lead to severe malnutrition in heart transplantation for failing Fontan. Nutritional management may be challenging from defining nutrient needs to diagnosis of malnutrition enteropathy, and expertise is necessary. Body composition and hematological nutritional indices may help define malnutrition severity and guide nutritional strategy.

Background Malnutrition constitutes one of the major public health challenges throughout the developing world. Urban poverty and malnutrition have been on the rise, with an increased rate of morbidity. We herein explore the relationship between infections and nutritional status and the related association with hygienic conditions as risk of infection in children residing in the slums of Nairobi. Methods Case-control study based on a secondary analysis of quantitative data collected from a cluster randomized trial carried out in two slums of Nairobi. The following information about resident children were selected: babies’ anthropometric measurements, related life conditions, data on infant-feeding practices, food security, hygiene, immunization coverage and morbidity were collected and updated with structured questionnaires until 12 months of life. Prevalence of malnutrition was calculated, then both bivariate and multivariate analysis were used to explore the relationship between malnutrition and its determinants. Results The study involved a total of 1119 babies registered at birth (51.28% male and 48.03% female infants). Overall the prevalence of malnutrition was high, with 26.3% of the children being stunted, 6.3% wasted and 13.16% underweight. Prevalence of wasting was higher in the first months of life, while in older children more case of stunting and underweight were captured. Wasted infants were significantly associated with common childhood illnesses: with cough and rapid breathing as well as with diarrhea (p-value

Hypothalamic obesity (HO) is delineated by an inexorable weight gain in subjects with hypothalamic disorder (congenital or acquired). The aim of the present study was to evaluate the effect of a multidisciplinary approach on weight trend and metabolic outcome in children and adolescents with hypothalamic disease who were overweight or obese. Thirteen patients (aged 8.1–16.1 years) received a personalized diet, accelerometer-based activity monitoring, and psychological assessment. Height, weight, body mass index (BMI), and serum metabolic parameters were assessed at baseline (T0) and after six months (T1). Metformin was introduced at T1 in four subjects who were then re-evaluated after six months (T2). At T1, weight gain was significantly reduced compared with T0 (0.29 ± 0.79 kg/month vs. 0.84 ± 0.55 kg/month, p = 0.03), and weight standard deviation score (SDS) and BMI SDS did not change significantly, as serum metabolic parameters. The four subjects treated with metformin showed a reduction of weight SDS and BMI SDS at T2. In conclusion, patients treated with our multidisciplinary approach showed, after 6 months, favorable results characterized by decreased weight gain and stabilization of weight SDS and BMI SDS in a condition usually characterized by inexorable weight gain. However, further analysis, larger cohorts, and longer follow-up are needed to confirm these preliminary data.

Failure to thrive (FTT) is an abnormal growth pattern determined by inadequate nutrition. It is a common problem in children, representing 5% to 10% of patients seen in an outpatient setting. Many definitions have been proposed based on anthropometric deterioration; however, they show poor concordance. No single definition is sufficiently sensitive in identifying faltering growth whilst a combination of multiple criteria seems more accurate. In light of the recent redefinition of pediatric malnutrition as a disequilibrium between requirements and intakes of energy, and macro- and micronutrients, a wider conception of FTT as an unsatisfactory nutritional status related to poor growth and health is useful. Although the most severe problems of micronutrient malnutrition are found in developing countries, people of all regions of the world can be affected by micronutrient deficiencies (MNDs), a form of undernutrition with relevant effects on growth and health. Changes in diets and lifestyle, elimination diets, food insecurity, and food intake disorders create the conditions at risk of faltering growth and MNDs. This new insight integrates the “classical” anthropometric criteria for definition and treatment, in the aim of warranting both a regular increase in size and an overall adequate development and health status.

Pediatric Short Bowel Syndrome (SBS) can require prolonged parenteral nutrition (PN). Over the years, SBS management has been implemented by autologous gastrointestinal reconstructive surgery (AGIR). The primary objective of the present review was to assess the effect of AGIR on weaning off PN. We also evaluated how AGIR impacts survival, the need for transplantation (Tx) and the development of liver disease (LD). We conducted a systematic literature search to identify studies published from January 1999 to the present and 947 patients were identified. PN alone was weakly associated with higher probability of weaning from PN (OR = 1.1, p = 0.03) and of surviving (OR = 1.05, p = 0.01). Adjusting for age, the probability of weaning off PN but of not surviving remained significantly associated with PN alone (OR = 1.08, p = 0.03). Finally, adjusting for age and primary diagnosis (gastroschisis), any association was lost. The prevalence of TX and LD did not differ by groups. In conclusion, in view of the low benefit in terms of intestinal adaptation and of the not negligible rate of complications (20%), a careful selection of candidates for AGIR should be required. Bowel dilation associated with failure of advancing EN and poor growth, should be criteria to refer for AGIR.

In 2019 the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and the Italian Society of Artificial Nutrition and Metabolism (SINPE) created a joint panel of experts with the aim of preparing an official statement on transition in Chronic Intestinal Failure (CIF). The transition from pediatric to adult care has a key role in managing all chronic diseases and in optimizing the compliance to care. Thus SIGENP and SINPE, in light of the growing number of patients with IF who need long-term Parenteral Nutrition (PN) and multidisciplinary rehabilitation programs throughout adulthood, shared a common protocol to provide an accurate and timely process of transition from pediatric to adult centers for CIF. The main objectives of the transition process for CIF can be summarized as the so-called "acronym of the 5 M": 1)Motivate independent choices which are characteristics of the adult world; 2)Move towards adult goals (e.g. self-management of his pathology and sexual issues); 3)Maintain the habitual mode of care; 4) Minimize the difficulties involved in the transition process and 5)Modulate the length of the transition so as to fully share with the adult's team the children's peculiarities.

Background: Mixed lipid emulsions (LE) containing fish oil present several advantages compared to the sole soybean oil LE, but little is known about the safety of essential fatty acids (EFA) profile in paediatric patients on long-term Parenteral Nutrition (PN). Aim of the study: to assess glycerophosfolipid polyunsaturated fatty acids (PUFA) levels on plasma and red blood cell (RBC) membrane of children on long term PN with composite LE containing fish oil (SMOF), and to compare it with a group receiving olive oil LE (Clinoleic&reg, ) and to the reference range for age, previously determined on a group of healthy children. Results: A total of 38 patients were enrolled, median age 5.56 (0.9&ndash, 21.86) years, 15 receiving Clinoleic&reg, 23 receiving SMOF. Patients on SMOF showed significantly higher levels of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA), lower levels of arachidonic acid (ARA) and Mead acid (MEAD)/ARA ratio in plasma and RBC compared with patients on Clinoleic&reg, and with healthy children. Triene:tetraene (T:T) ratio of both groups of patients did not differ from that of healthy children-median plasma (MEAD/ARA: 0.01, interquartile rage (IQR) 0.01, p = 0.61 and 0.02, IQR 0.02, p = 0.6 in SMOF and Clinoleic&reg, patients, respectively), and was considerably lower than Holman index (&gt, 0.21). SMOF patients showed no statistically significant differences in growth parameters compared with Clinoleic&reg, patients. Patients of both groups showed stiffness class F0-F1 of liver stiffness measure (LSM) 5.6 (IQR 0.85) in SMOF patients and 5.3 (IQR 0.90) in Clinoleic&reg, patients, p = 0.58), indicating absence of liver fibrosis. Conclusions: Fatty acids, measured as concentrations (mg/L), revealed specific PUFA profile of PN patients and could be an accurate method to evaluate nutritional status and eventually to detect essential fatty acid deficiency (EFAD). SMOF patients showed significantly higher EPA, DHA and lower ARA concentrations compared to Clinoleic&reg, patients. Both LEs showed similar hepatic evolution and growth.

Purpose: To estimate the impact of increasing pre-pregnancy Body Mass Index (BMI) on the risk of adverse maternal and perinatal outcomes, in patients who delivered in an Italian tertiary care Obstetric department. Methods: Data, related to women who delivered at Sant’Anna Hospital, Turin, between 2011 and 2015, were collected retrospectively from the hospital database. According to BMI, women were considered as normal weight, overweight, and class 1, 2 and 3 obese (WHO criteria). Logistic regression analysis studied the impact of BMI on maternal and neonatal outcomes, adjusting results for maternal age and parity. Adjusted absolute risks of each outcome were reported according to incremental values in pre-pregnancy BMI. Results: A total of 27,807 women were included. 75.8% of pregnancies occurred among normal-weight women, whereas 16.7% were overweight, and 7.5% obese women (5.4% class 1, 1.7% class 2 and 0.4% class 3). A 10% decrease in pre-pregnancy BMI was associated with a reduction of at least 15% of Gestational diabetes mellitus (GDM), preeclampsia, maternal admission to intensive care unit (ICU), macrosomia, APGAR 5′ < 6 and neonatal admission to ICU. GDM and preeclampsia resulted in the highest reduction being almost 30%. Larger differences in BMI (20–25%) corresponded to at least a 10% in reduction of risk of preterm and very preterm delivery and emergency cesarean section. Differences in maternal pre-pregnancy BMI had no impact on the frequency of shoulder dystocia and stillbirth. Conclusions: This study offers a quantitative estimation of negative impact of pre-pregnancy obesity on the most common pregnancy and perinatal complications.

BACKGROUND Iodine is an essential micronutrient for intellectual development in children. Information on iodine intakes based on 24-h urinary iodine excretion (UIE) is scant, because iodine status is only assessed by the measurement of urinary iodine concentration (UIC) in spot urine samples. OBJECTIVES The aim of our study was to evaluate the iodine intake of school-age children and adolescents, using UIE measurement in 24-h urine collections. METHODS The study population included 1270 healthy subjects (677 boys, 593 girls) aged 6-18 y (mean age ± SD: 10.3 ± 2.9) from 10 Italian regions. Daily iodine intake was estimated as UIE/0.92, based on the notion that $\sim$92% of the dietary iodine intake is absorbed. The adequacy of intakes was assessed according to the Dietary Reference Values for iodine of the European Food Safety Authority (EFSA). Body mass index (BMI) and UIC were also measured for each subject. RESULTS Based on the scientific opinion of EFSA, 600 of 1270 subjects (47.2%) had a lower than adequate iodine intake, with a higher prevalence among girls (54.6%) compared with boys (40.2%) (P

Plasma and erythrocyte PUFAs are useful in clinical practice to optimize the parenteral nutrition therapy. The aim of this study was the definition of pediatric PUFAs reference ranges to follow-up patients with IICB. We validated a method to quantify, in plasma and erythrocyte membranes, glycerophospholipids PUFAs by gas chromatography coupled with the highly specific Mass spectrometer. The analyzed PUFAs are only those metabolized by liver: no interferences by oral nor parenteral nutrition are expected. Metabolically significative PUFAs were selected for analysis: ARA (ω6), EPA and DHA (ω3) were quantified because they are precursors of important mediators of inflammation, while Mead Acid determination is useful to calculate the ratio ARA (ω6)/ Mead (ω9), a known marker of essential fatty acid deficiency. We quantified these PUFAs in healthy pediatric subjects to build reference ranges. A pilot study was performed to compare PUFAs of two groups of pediatric patients with IICB (group A fed with Clinoleic® and group B with SMOF®) with reference ranges. Pediatric reference ranges of PUFAs were proposed. A good correlation between plasma and erythrocyte results was shown for the ratio ARA/(EPA+DHA), (R2=0.941). PUFAs assessment is highly recommended in plasma rather than in erythrocytes because plasma is less prone to peroxidation artifacts. Our fast method can offer an accurate, specific and easy-to-handle PUFAs analysis and a useful marker of w6/w3 balance in tissues for routine clinical practice. This work could be a step towards standardization of routine determination of PUFAs.

The purpose of the present study is to compare the effects of four different breads (one commercial par-baked wheat bread, three sourdough breads prepared with commercial wheat flour, organic wheat flour, organic einkorn flour) in 16 healthy subjects. The primary outcome of this randomized cross-over trial was evaluating intra-individual changes in glycemic areas-under-the-curve (AUCs) after 50g carbohydrate portions of each bread; secondary outcomes were changes in insulin, fatty free acids (FFA), triglyceride, acylated ghrelin and satiety AUCs. Blood samples and satiety ratings were collected every 30-min for 2-h after the consumption of each bread. The einkorn flour showed the lowest amylase activity, the commercial flour the highest; commercial bread had the highest carbohydrate content and the lowest dietary fiber content. Glucose AUCs were significantly lower after the consumption of sourdough breads made with organic (12,754±1433mg/dL×h) and einkorn flour (12,216±1210mg/dL×h), with respect to the commercial bread (13,849±2193mg/dL×h). Insulin AUCs decreased after the consumption of all sourdough breads when compared to commercial bread. FFA and triglyceride AUCs did not differ by kind of breads. Median ghrelin AUC was significantly lower and satiety higher after the einkorn bread (3710pg/mL×h; 3225±2414, respectively) than after commercial bread consumption (4140pg/mL×h; 1706±1766, respectively), but not with other sourdough breads. In conclusion, the use of sourdough may improve the nutritional features of breads; einkorn bread induced the least disturbance in carbohydrate homeostasis and the greater satiety. If confirmed by further research, these results might have implications in the approach towards chronic dysmetabolic diseases.

none 34 no Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy. Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family’s place of residence and underlying diagnosis determining IF. Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20–18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53–2.20). Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries. open Diamanti, Antonella; Capriati, Teresa; Gandullia, Paolo; Dileo, Grazia; Lezo, Antonella; Lacitignola, Laura; Spagnuolo, Mariaimmacolata; Gatti, Simona; D’antiga, Lorenzo; Verlato, Giovanna; Roggero, Paola; Amarri, Sergio; Baldassarre, Mariaelisabetta; Cirillo, Francesco; Elia, Domenica; Boldrini, Renata; Campanozzi, Angelo; Catassi, Carlo; Aloi, Marina; Romano, Claudio; Candusso, Manila; Cecchi, Nicola; Bellini, Tommaso; Tyndall, Elaine; Fusaro, Fabio; Caldaro, Tamara; Alberti, Daniele; Gamba, Piergiorgio; Lima, Mario; Bagolan, Pietro; De Goyet, Jean De Ville; Dall’oglio, Luigi; Spada, Marco; Grandi, Francesca Diamanti, Antonella; Capriati, Teresa; Gandullia, Paolo; Dileo, Grazia; Lezo, Antonella; Lacitignola, Laura; Spagnuolo, Mariaimmacolata; Gatti, Simona; Dâ antiga, Lorenzo; Verlato, Giovanna; Roggero, Paola; Amarri, Sergio; Baldassarre, Mariaelisabetta; Cirillo, Francesco; Elia, Domenica; Boldrini, Renata; Campanozzi, Angelo; Catassi, Carlo; Aloi, Marina; Romano, Claudio; Candusso, Manila; Cecchi, Nicola; Bellini, Tommaso; Tyndall, Elaine; Fusaro, Fabio; Caldaro, Tamara; Alberti, Daniele; Gamba, Piergiorgio; Lima, Mario; Bagolan, Pietro; De Goyet, Jean De Ville; Dallâ oglio, Luigi; Spada, Marco; Grandi, Francesca

Introduction the prevalence of malnutrition in children and its impact on clinical outcomes is underrecognized by clinicians in Italy as well as worldwide. A novel definition of pediatric malnutrition has been recently proposed by a working group of the Academy of Nutrition and Dietetics and American Society for Parenteral and Enteral Nutrition (A.S.P.E.N.), based on the correlation between illness and the use of zscores of anthropometric measurements. Aim to investigate the prevalence of malnutrition and related nutritional support among hospitalized children in Italy, in a nationwide survey performed in a single day (16/4/2015). Methods an open access website (http://nday.biomedia.net) was used to collected data from 73 hospitals and 101 wards in 14 Italian regions (1994 patients). Anonymous information was collected on hospitals' characteristics, patient's anthropometry, admission diagnosis, presence of chronic diseases and use of nutritional support: oral nutritional supplements (ONS), enteral nutrition (EN) or parenteral nutrition (PN). Z-scores of anthropometric measurements, calculated with Epi Info 7.1.5, defined nutritional status: wasting was identified by BMI or Weight-for-Length z-score (

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.

Chylomicron retention disease is a recessive inherited disorder characterized by fat malabsorption and steatorrhea and is associated with failure to thrive in infancy. We describe a kindred carrying a mutation of Sara2 gene causing a chylomicron retention phenotype. The proband was a 5-month-old baby, born of consanguineous, apparently healthy parents from Morocco, with failure to thrive. There was a large quantity of fats in feces and malabsorption of fat-soluble vitamins. Intestinal biopsies showed a diffused enterocyte vacuolization with large cytosolic lipid droplets. Chylomicron retention disease or Anderson disease was hypothesized, and the Sara2 gene was analyzed by direct sequencing. Analysis of the Sara2 gene in the proband identified a 2-nucleotide homozygous deletion in exon 3 leading to a premature stop codon (c.75-76 del TG-L28fsX34). The father was heterozygous for the same mutation, whereas the proband's mother was homozygous, suggesting a variable phenotypic expression of the molecular defect. More studies are needed to understand the reasons of the phenotypic variability of the same molecular defect in the same family.

Feeding gastrostomy is used worldwide for adults and children with feeding impairment to obtain long-term enteral nutrition. Percutaneous endoscopic gastrostomy insertion is considered the gold standard, but after the first months requires gastrostomy tube replacement with a low-profile button. The replacement is known as an easy procedure, but several minor and major complications may occur during and after the manoeuvre. We describe intraperitoneal bumper migration in a 3-year-old boy, a rare complication following gastrostomy tube replacement, and we discuss the recent literature regarding similar cases.

Background/Objectives: The practice of home enteral nutrition (HEN) represents a relevant aspect of the clinical management of both malnourished children and well-nourished children unable to be fed using an oral diet. The aim of this study was to estimate in an Italian paediatric population over a 14-year period (1996-2009), the clinical relevance and results over time of HEN activity. Subjects/methods: HEN-computerized database and medical/dietetic charts were evaluated for patients aged at start of HEN 1 month. Results: During the study period, we recorded 757 HEN programs. HEN began at a median age of 2 years for a median duration of 8.1 months. The complication rate was 14.8%. In the second period of the survey (2003-2009), the main changes concerned the underlying diseases requiring HEN, choice of formula feeding and access route. In 2009, the estimated overall prevalence of HEN was 3.47 and the incidence 2.45 per 100 000 inhabitants from 0 to 18 years of age. Conclusions: The epidemiological data of this study demonstrate that HEN concerns a growing number of Italian children and families. Some aspects of HEN clinical management should be modified to reach the recommended standards. © 2013 Macmillan Publishers Limited.

Ectopic adrenocorticotrophic hormone (ACTH) secretion is a rare cause of Cushing syndrome in paediatric age, due to tumours arising from different tissues. To date, only 11 reports of ACTH-secreting pancreatic tumours in children and adolescents exist in the literature. We present a paediatric case of Cushing syndrome caused by ectopic ACTH secretion. This was caused by a large acinar cell carcinoma that developed in the pancreas of a 3-year-old girl.

Intestinal function in children with very short bowel syndrome and related intestinal failure may improve after isolated liver transplantation. An infant with an ultrashort gut, ileo-cecal valve, and whole colon received total parenteral nutrition from the first days of life. Enteral feeding failed because of the progressive dilatation of the jejunal portion and motility disorders. He developed early severe cholestatic liver disease (aspartate transferase 186, alanine transferase 103 U/L, serum bilirubin 8.4 mg/dL) and subsequent liver failure. At 8 months of age, he benefited from isolated liver transplantation (left segment graft from living donor). His early posttransplant evolution was characterized by recovery of oral alimentation, improvement of digestive and absorption functions, but he did not achieve TPN-independence. At 20 months, 50% to 60% of his energy needs were covered by parenteral nutrition and he has satisfactory growth indices (3rd percentile for weight and height), reduced stool volume, and frequency. Isolated liver transplantation allowed, in this particular case, time for further intestinal adaptation thereby avoiding the need for intestinal transplantation early in life.

s / Digestive and Liver Disease 46 (2014) e85–e127 e95 4.5±0.6pg/ml, mean FT4 1.2± .4 ng/dl. TSH levels were above cutoff laboratory range in 10% of our population and the 2 patients (0.2%) of the whole population presented TSH≥10 UI/ml. We found a significant positive correlation between TSH levels and BMI z-score (rho=0.121, p=0.001), even after adjustment for age and sex (r=0.117, p=0.001); a positive correlation was also observed between FT3 and BMI z-score (rho=0.161, p

Since the year 2000, infants born in the Piedmont region of Italy have been screened for cystic fibrosis (CF). The investigators estimated the time to initial infection by Pseudomonas aeruginosa in 71 children who were diagnosed as having CF in the years 1997-2004, which includes the years before and after newborn screening was introduced. Infants were followed up for at least 12 months. Forty-four of the 71 infants in the study (62%) were diagnosed by newborn screening, while the remaining 38% were diagnosed clinically before screening came into use, by sweat chloride testing after symptoms of CF had been observed. Introduction of newborn screening significantly lowered the median age at diagnosis of CF from 323 to 42 days. Pancreatic insufficiency was found in 35 children (50% of the newborn screen group, 48% of the clinically diagnosed group) and meconium ileus was the presenting feature in 12 (16% and 18%, respectively). P aeruginosa was diagnosed in 20 (45%) screened and 14 (52%) clinically diagnosed infants. Children diagnosed by newborn screening had a significantly shorter median time to P aeruginosa infection compared with the clinically diagnosed subjects (183 versus 448 days). Median ages at the time of initial infection were 201 and 511 days, respectively. Overall annual rates of initial infection by P aeruginosa did not differ significantly between the screened and nonscreened children. Neither univariate nor multivariate regression analyses showed a significant association between the performance of newborn screening and respiratory tract infection by P aeruginosa. Semiparametric analyses, however, showed a significant correlation between the need for pancreatic enzyme supplementation and an increased hazard ratio for P aeruginosa infection. Both screened and nonscreened children have been followed for 71 to 97 person-years; to reach a statistical power of 90%, the overall follow-up would have to be 1400 person-years. These findings suggest that health care authorities considering the institution of newborn screening for CF should do so only in settings where infection control policies are in place.