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Level of evidence and grade of recommendations according to the National Guidelines System [4]. (DOCX 15Â kb)

Supplemental Material, jspr-17-253-File006 for Compassion in a heartbeat: Physiology during couples’ loss discussions by Geoffrey W. Corner, Darby E. Saxbe, Theodora Chaspari, Hannah F. Rasmussen, Laura Perrone, Corey Pettit, Madeleine Friendly, Adela C. Timmons, and Gayla Margolin in Journal of Social and Personal Relationships

Background The Glu167Lys (E167K) transmembrane 6 superfamily member 2 (TM6SF2) variant has been associated with liver steatosis, high alanine transaminase (ALT) levels and reduced plasma levels of liver-derived triglyceride-rich lipoproteins. Objectives The objectives of this study were to investigate in a group of obese children the association among the 167K allele of TM6SF2 gene and ALT, cholesterol and triglycerides levels, and hepatic steatosis, and to evaluate the potential interaction between this variant and the I148M patatin like phospholipase 3 gene (PNPLA3) polymorphism on liver enzymes. Methods We genotyped 1010 obese children for TM6SF2 E167K and PNPLA3 I148M polymorphisms. Anthropometrical and biochemical data were collected. Ultrasound imaging of the liver was performed. Results The 167K allele showed an association with steatosis (P < 0.0001), higher ALT levels (P < 0.001) and lower total cholesterol (P < 0.00001), low-density lipoprotein cholesterol (P < 0.0001), triglycerides (P = 0.02) and non-high-density lipoprotein cholesterol levels (P < 0.000001). The subjects homozygous for the PNPLA3 148M allele carrying the rare variant of TM6SF2 showed an odds ratio of 12.2 (confidence interval 3.8-39.6, P = 0.000001) to present hypertransaminasaemia compared with the remaining patients. Conclusion Although the TMS6SF2 E167K variant predisposes the obese children to non-alcoholic fatty liver disease, there is an association between this variant and lower levels of cardiovascular risk factors. Overall, the data suggest differential effects of TMS6SF2 E167K variant on liver and heart health.

The association of autoimmune type 1 diabetes mellitus and celiac disease in children and adolescents has been known for a long time. This critical review of the literature should encourage readers to reassess many of the recent concepts on celiac disease screening accepted for the general population but that must be carefully evaluated before they are passively applied in high-risk pediatric populations predisposed to autoimmune diseases. The high frequency of potential celiac disease in diabetic patients, for example, implies the need for duodenal biopsy to establish a precise diagnosis. The diagnosis of celiac disease in a child or a teenager with diabetes mellitus may have negative effects on compliance with therapy and metabolic control. Then too, the introduction of glutenfree foods, for example, giving a high intake of rapidly absorbed carbohydrates, may make it necessary to increase the insulin dose.

Background: Bisphenol A (BPA) exposure has been associated with increased incidence of diabetes and obesity in adults. Objectives: To evaluate whether an association between BPA urinary levels and insulin resistance as well as adiponectin and resistin production and serum concentrations may occur in obese children. Methods: Clinical and biochemical features of 141 obese children were collected. Serum resistin and adiponectin were evaluated. Insulin resistance and urinary BPA levels were assessed. Moreover, the effect of BPA on adiponectin and resistin gene expression in adipocytes from eight normal weight prepubertal children was investigated by quantitative real-time RT-PCR (qPCR). Results: Direct association between BPA and homeostasis model assessment (r=0.23; p: 0.0069) and a strong inverse association between BPA and adiponectin have been found (r=-0.48; p

Table of contents A1 Preterm and low birth weight nutrition in the first month life: implications for the outcome Massimo Agosti A2 Behind human milk and breastfeeding: not only food, not only growth Carlo Agostoni A3 To prevent obesity: importance and issues of cultural adaptation from weaning to 3 years of age Serge Chalons A4 Diet before and during pregnancy and child health: lessons from animal models Pascale Chavatte-Palmer A5 Infant nutrition: an opportunity to optimize future health José Manuel Moreno Villares A6 Complementary feeding strategies to facilitate acceptance of fruits and vegetables Sophie Nicklaus A7 Diet of young children in the Mediterranean region Luís Pereira-da-Silva A8 Proposal of 10 good practices to help prevent obesity in the first 1,000 days Angelo Pietrobelli, the MeNu Group A9 Macronutrient intakes in early life and subsequent risk of obesity Marie Françoise Rolland-Cachera A10 The burden of childhood obesity in Italy and the results of Nutrintake study Gian Vincenzo Zuccotti A11 Growth body composition and growth hormone therapy: linear growth Marco Cappa A12 Early nutrition pattern and late metabolic consequences Manuela Caruso-Nicoletti A13 Nutrition and Insulin-like Growth Factor (IGF) System Elena Inzaghi, Stefano Cianfarani A14 Nutrition of preterm infants Mario De Curtis A15 Early nutrition patterns and later metabolic outcomes- I part: Genetic and metabolic mechanisms Laura Guazzarotti A16 Diagnosis of metabolic disease by imaging techniques Lorenzo Iughetti A17 Nutrition, growth and cardiovascular diseases Francesco Chiarelli, Laura Comegna, Simone Franchini A18 Body fat mass and gender Laura Perrone, Giuseppina Rosaria Umano A19 Lifestyle interventions for an appropriate birth weight Elisabetta Petrella, Raffaele Bruno, Valentina Bertarini, Giulia Pedrielli, Isabella Neri, Fabio Facchinetti A20 Nutrition, growth and body composition Flavia Prodam A21 Nation-specific reference growth charts in the daily practice Alessandro Sartorio, John M. H. Buckler, Nicoletta Marazzi A22 Growth patterns in inflammatory bowel diseases (IBD) and in cystic fibrosis (CF) Maria E. Street, Viviana D. Patianna, Paola Accorsi, Sara Lo Scocco, Sergio Amarri A23 Newborn in the digital era and their body feeling: physical exercise to counteract hyperphagia Alberto Pellai A24 Nutrition, young athletes and effects of exercise. Practical suggestions Giampiero Merati A25 Physical exercise as a way to prevent criminality in minors and teenagers Isabella Merzagora A26 The measurement of daily energy expenditure in children. Evaluation of a new wrist portable device vs breath-by-breath metabolimeter Susanna Rampichini, Arsenio Veicsteinas A27 Probiotic and inflammasomes Mario Clerici A28 Probiotics and newborns Paolo Manzoni, Elena Tavella, Elena Boano, Daniele Farina A29 Relationship between gut microbiota and obesity Fabio Pace

BACKGROUND: The major burden of acute gastroenteritis (AGE) in childhood is related to its high frequency and the large number of hospitalizations, medical consultations, tests and drug prescriptions. The adherence to evidence-based recommendations for AGE management in European countries is unknown. The purpose of the study was to compare hospital medical interventions for children admitted for AGE with recommendations reported in the European Societies of Pediatric Gastroenterology, Hepatology and Nutrition and Pediatric Infectious Diseases guidelines. METHODS: A multicenter prospective study was conducted in 31 Italian hospitals. Data on children were collected through an online clinical reporting form and compared with European Societies of Pediatric Gastroenterology, Hepatology and Nutrition and Pediatric Infectious Diseases guidelines for AGE. The main outcomes were the inappropriate hospital admissions and the percentage of compliance to the guidelines (full >90%, partial >80% compliance) based on the number and type of violations to evidence-based recommendations. RESULTS: Six-hundred and twelve children (53.6% male, mean age 22.8 ± 15.4 months) hospitalized for AGE were enrolled. Many hospital admissions (346/602, 57.5%) were inappropriate. Once admitted, 20.6% (126/612) of children were managed in full compliance with the guidelines and 44.7% (274/612) were managed in partial compliance. The most common violations were requests for microbiologic tests (404; 35.8%), diet changes (310; 27.6%) and the prescription of non-recommended probiotics (161; 14.2%), antibiotics (103; 9.2%) and antidiarrheal drugs (7; 0.6%). CONCLUSIONS: Inappropriate hospital admissions and medical interventions are still common in the management of children with AGE in Italy. Implementation of guidelines recommendations is needed to improve quality of care

Background: Homozygous mutations in acid-labile subunit (IGFALS) gene result in short stature, very low circulating levels of acid-labile subunit (ALS), insulin growth factor 1 (IGF1) and insulin growth factor binding protein 3 (IGFBP3) and a poor response to growth hormone (GH). The impact of IGFALS mutations heterozygosity on growth is unknown. Patient and Methods: We describe a 10-year-old girl with severe short stature (height -3.2 SDS), heterozygous for a new IGFALS mutation. Results: The girl showed low circulating IGF1, IGFBP3 and ALS levels and normal GH secretion. We found a novel heterozygous frameshift IGFALS mutation (c.1283delA, p.Gln428Argfs*14). Size-exclusion chromatography showed a reduction of the IGF1, IGFBP3 and ALS 150-kDa ternary complex (by about 55%) compared to a control. An IGF-1 generation test, with two different GH dosages, showed a good response in term of increase in IGF1 and in formation of the ternary complex at size-exclusion chromatography. Clinical response after 6 months of therapy with GH was satisfactory (height velocity increased from 3 to 8 cm/year). Conclusion: We suggest that (1) heterozygous IGFALS mutations can be responsible for a subset of patients with severe short stature (below -2.5 SDS), low IGF1 (below -2 SDS) and normal GH secretion, and (2) the identification by IGFALS molecular screening of this subset of patients could help in the administration of the appropriate therapy. Background: Homozygous mutations in acid-labile subunit (IGFALS) gene result in short stature, very low circulating levels of acid-labile subunit (ALS), insulin growth factor 1 (IGF1) and insulin growth factor binding protein 3 (IGFBP3) and a poor response to growth hormone (GH). The impact of IGFALS mutations heterozygosity on growth is unknown. Patient and Methods: We describe a 10-year-old girl with severe short stature (height -3.2 SDS), heterozygous for a new IGFALS mutation. Results: The girl showed low circulating IGF1, IGFBP3 and ALS levels and normal GH secretion. We found a novel heterozygous frameshift IGFALS mutation (c.1283delA, p.Gln428Argfs*14). Size-exclusion chromatography showed a reduction of the IGF1, IGFBP3 and ALS 150-kDa ternary complex (by about 55%) compared to a control. An IGF-1 generation test, with two different GH dosages, showed a good response in term of increase in IGF1 and in formation of the ternary complex at size-exclusion chromatography. Clinical response after 6 months of therapy with GH was satisfactory (height velocity increased from 3 to 8 cm/year). Conclusion: We suggest that (1) heterozygous IGFALS mutations can be responsible for a subset of patients with severe short stature (below -2.5 SDS), low IGF1 (below -2 SDS) and normal GH secretion, and (2) the identification by IGFALS molecular screening of this subset of patients could help in the administration of the appropriate therapy. © 2013 S. Karger AG, Basel.