The French Experience with a Population-Based Esophageal Atresia Registry (RENATO).

This paper presented a national register for esophageal atresia (EA) started in January 2008. We report our experience about the conception of this database and its coordination. Data management and data quality are also detailed.

Porous Ceramic Sternal Prosthesis Implantation in a 13-Year-Old Patient Presenting with Metastatic Ewing's Sarcoma.

Ewing's sarcoma is the second most frequent primary malignant bone tumor in adolescents and young adults. Locations on the thoracic wall represent up to 20% of primary and secondary locations. We present the case of a 13-year-old patient treated with the use of a radiolucency porous bioceramic prosthesis as a sternal replacement for a wide tumor resection in an oncologic context.

Patient-matched analysis identifies deregulated networks in prostate cancer to guide personalized therapeutic intervention.

Prostate cancer (PrCa) is the second most common malignancy in men. More than 50% of advanced prostate cancers display the TMPRSS2-ERG fusion. Despite extensive cancer genome/transcriptome data, little is known about the impact of mutations and altered transcription on regulatory networks in the PrCa of individual patients.

Clinical Remission and Psychological Management are Major Issues for the Quality of Life in Pediatric Crohn Disease.

Objectives: Crohn disease (CD) can affect patient's quality of life (QOL) with physical, social, and psychological impacts. This study aimed to investigate the QOL of children with CD and its relationship with patient and disease characteristics.

Methods: Children ages from 10 to 17 years with diagnosed CD for more than 6 months were eligible to this cross-sectional study conducted in 35 French pediatric centers.

Growth, stool consistency and bone mineral content in healthy term infants fed sn-2-palmitate-enriched starter infant formula: A randomized, double-blind, multicentre clinical trial.

Background: Palmitate in breast milk is predominantly located in the triacylglycerol sn-2 position, while infant formulae contain palmitate predominantly in the sn-1 and sn-3 positions. During digestion, palmitate in the sn-1 and sn-3 positions is hydrolyzed to free palmitic acid that can subsequently complex with calcium to form insoluble soaps; this may partially explain why formula-fed infants have harder stools than breast-fed infants.

Methods: This large (n = 488) randomized, double-blind, multicentre trial investigated whether increasing the sn-2 palmitate content of infant formula improves stool consistency and bone mineral content (measured by dual-energy x-ray absorptiometry), without affecting growth or health.

Diagnostic Yield of Next-generation Sequencing in Very Early-onset Inflammatory Bowel Diseases: A Multicentre Study.

Background And Aims: An expanding number of monogenic defects have been identified as causative of severe forms of very early-onset inflammatory bowel diseases [VEO-IBD]. The present study aimed at defining how next-generation sequencing [NGS] methods can be used to improve identification of known molecular diagnosis and to adapt treatment.

Methods: A total of 207 children were recruited in 45 paediatric centres through an international collaborative network [ESPGHAN GENIUS working group] with a clinical presentation of severe VEO-IBD [n = 185] or an anamnesis suggestive of a monogenic disorder [n = 22].

National Esophageal Atresia Register.

National Esophageal Atresia was created in 2008 by the National Reference Center for Esophageal Congenital Abnormalities created in 2006. Primary goal was estimation of live birth prevalence in France. A national network of surgeons and pediatricians was initiated and entire teams dealing with esophageal atresia accepted to participate in an exhaustive national register.

The relationship between school lunch attendance and the food intakes of French schoolchildren aged 3-17 years.

Objective: Recently, school meal composition regulations have been implemented in France in order to improve the nutritional status of children. The present study investigated the link between school lunch attendance and the food intakes of schoolchildren aged 3-17 years.

Design: Second French cross-sectional dietary survey (2006-2007).

Iron requirements of infants and toddlers.

Iron deficiency (ID) is the most common micronutrient deficiency worldwide and young children are a special risk group because their rapid growth leads to high iron requirements. Risk factors associated with a higher prevalence of ID anemia (IDA) include low birth weight, high cow's-milk intake, low intake of iron-rich complementary foods, low socioeconomic status, and immigrant status. The aim of this position paper was to review the field and provide recommendations regarding iron requirements in infants and toddlers, including those of moderately or marginally low birth weight.

Donor human milk for preterm infants: current evidence and research directions.

The Committee on Nutrition of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition aims to document the existing evidence of the benefits and common concerns deriving from the use of donor human milk (DHM) in preterm infants. The comment also outlines gaps in knowledge and gives recommendations for practice and suggestions for future research directions. Protection against necrotizing enterocolitis is the major clinical benefit deriving from the use of DHM when compared with formula.

Cow's milk and goat's milk.

Cow's milk is increasingly suggested to play a role in the development of chronic degenerative, non-communicable disorders whereas goat's milk is advocated as having several health benefits. Cow's milk is a rich and cheap source of protein and calcium, and a valuable food for bone health. Despite their high content in saturated fats, consumption of full-fat dairy products does not seem to cause significant changes in cardiovascular disease risk variables.

World Health Organization 2006 child growth standards and 2007 growth reference charts: A discussion paper by the committee on Nutrition of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition.

Growth charts are essential for evaluating children's health including their nutrition; however, the evaluation of child growth trajectories and consequently the decision to intervene are highly dependent on the growth charts used. The aim of this discussion paper of the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition Committee on Nutrition is to provide information on the background and rationale of the World Health Organization (WHO) 2006 child growth standards and WHO 2007 growth reference charts, describe their development, outline their main innovative aspects, discuss potential limitations, and make recommendations. WHO 2006 child growth standards (0-5 years) are based on prospectively collected data describing the growth of healthy infants who were breast-fed according to WHO recommendations, showing a pattern of linear growth, which is remarkably consistent between different countries and ethnic groups.

Safety aspects in preparation and handling of infant food.

Powdered infant formula (PIF) can become contaminated during production with harmful bacteria such as Cronobacter spp. (formerly Enterobacter sakazakii). Inadequate conditions of preparation and handling of PIF can therefore exacerbate the risk of severe infection, especially in preterm infants.

Anastomotic stricture after surgical repair of esophageal atresia: frequency, risk factors, and efficacy of esophageal bougie dilatations.

Aims: The aim of this study was to evaluate the frequency and risk factors of postoperative anastomotic stricture, and the efficacy and complications of esophageal bougie dilatations for symptomatic anastomotic stricture in a population of children with esophageal atresia.

Patients And Methods: The medical records of 62 children operated on for esophageal atresia type III (Ladd and Gross) over a 5-year period were retrospectively reviewed.

Results: Anastomotic stricture developed in 23 (37%) of patients.

Dumping syndrome after esophageal atresia repair without antireflux surgery.

In childhood, the surgical treatment of gastroesophageal reflux is the main cause of dumping syndrome. We report the cases of 2 children with esophageal atresia who presented with dumping syndrome without any precipitating known factors, such as gastroesophageal reflux surgery or associated microgastria. Our data suggest (1) that dumping syndrome can occur after primary anastomosis of esophageal atresia without antireflux surgery and (2) that dumping syndrome should be considered in every child treated surgically for esophageal atresia presenting with digestive symptoms, malaise, failure to thrive, or refusal to eat.

Longevity of balloon-stabilized skin-level gastrostomy device.

Objective: The gastrostomy button is increasingly used in patients requiring long-term enteral feeding. No data are available addressing the longevity of balloon-type gastrostomy buttons. The aim of this study was to evaluate the longevity of balloon-type gastrostomy buttons in children and the cause of the button removal.

Incidence and risk factors of oral antibiotic-associated diarrhea in an outpatient pediatric population.

Background: Little information is available on the epidemiologic characteristics of antibiotic-associated diarrhea (AAD) in children. The authors' aim was to evaluate the incidence of AAD in an outpatient pediatric population and to identify risk factors.

Methods: Children aged 1 month to 15.

Auto-immune pancytopenia in a child with DiGeorge syndrome.

Unlabelled: We report on the development of auto-immune pancytopenia in a child with DiGeorge syndrome carrying the 22q11 microdeletion. She had congenital heart disease, dysmorphic facies, thymic hypoplasia, immunodeficiency, velopharyngeal insufficiency, scoliosis, and a hearing deficit. She had a low T-cell count with a normal CD4/CD8 ratio, IgA deficiency and a normal lymphoblastic response to mitogens.

Neonatal severe intractable diarrhoea as the presenting manifestation of an unclassified congenital disorder of glycosylation (CDG-x).

A case of severe and protracted diarrhoea is reported, which started in the neonatal period and progressively associated with neurological impairment, dysmorphy, hepatosplenomegaly, and hepatic insufficiency, from which the patient died at 2 years of age. Isoelectric focusing of serum transferrin showed a congenital disorder of glycosylation type I pattern but the basic defect could not be identified. This observation shows that congenital disorder of glycosylation is a cause of intractable diarrhoea in neonates.