Pediatric Patients with Eosinophilic Esophagitis and Their Parents Identify Symptoms as the Most Important Treatment Outcome.

Introduction: Given the lack of data, we aimed to explore which therapeutic endpoints pediatric patients with eosinophilic esophagitis (EoE) and their parents consider to be relevant.

Methods: We created an educational brochure on EoE and a questionnaire, both of which were content-validated by pediatric patients and parents. Validated documents were sent to 112 patients and parents.

[Digestive and nutritional problems of children with cerebral palsy].

Digestive and nutritional problems of children with cerebral palsy put them at risk of malnutrition. Identification of these problems through measurements of weight, height, and body composition is essential. Feeding difficulties may be caused by a combination of oral and digestive problems, such as swallowing difficulties, gastroesophageal reflux, and constipation.

Vitamin D Insufficiency Prior to Paediatric Liver Transplantation Is Associated with Early T-Cell Mediated Rejection.

: T-cell mediated rejection (TCMR) can compromise long-term liver allograft survival. The immunomodulatory properties of vitamin D are increasingly recognized. We investigated whether perturbations in vitamin D metabolism prior to LT may predispose to TCMR in a representative cohort of paediatric LT recipients.

E-Learning Training to Improve Pediatric Parenteral Nutrition Practice: A Pilot Study in Two University Hospitals.

Background: Education and training may improve the prescription of pediatric parenteral nutrition. The aim was to evaluate the impact of an e-learning method on parenteral nutrition prescription skills among pediatric residents in 2 pediatric hospitals.

Methods: A randomized double-blind control study was conducted over a 9-month period among pediatric residents in HOSP1, Geneva, Switzerland, where physicians prescribe parenteral nutrition directly, and in HOSP2, Montreal, Canada, where physicians prescribe only occasionally because clinical pharmacists are devoted to this activity.

Preformed and de novo DSA are associated with T-cell-mediated rejection in pediatric liver transplant recipients requiring clinically indicated liver biopsy.

Despite growing interest about the impact of donor-specific HLA antibodies (DSA) in LT limited data are available for pediatric recipients. Our aim was to perform a retrospective single-center chart review of children (0-16 years) having undergone LT between January 1, 2005 and December 31, 2017, to characterize DSA, to identify factors associated with the development of de novo DSA, and to analyze potential associations with the diagnosis of TCMR. Information on patient- and donor-characteristics and LB reports were analyzed retrospectively.

Should Proton Pump Inhibitors be Systematically Prescribed in Patients With Esophageal Atresia After Surgical Repair?

Objective: To evaluate outcomes of patients with esophageal atresia (EA) on systematic treatment with proton pump inhibitors (PPI) since the neonatal period and to determine factors associated with successful discontinuation of PPI.

Study Design: Longitudinal cohort study with prospective data collection of 73 EA patients, over 11 years systematically treated with PPI. Outcome and predictive factors for discontinuation of PPI treatment were evaluated at study end in February 2017.

[Vegan diet in children : what potential deficits to monitor ?].

The vegan diet, although beneficial for adults in the prevention of metabolic diseases, raises questions about its benefits for a growing child : adequate caloric and protein intake, quality of essential amino acids, presence of essential fatty acids, inhibition of absorption of trace elements (including iodine, iron and zinc) and supply of various vitamins. Whereas vegan food is vitamin B12 deprived, the biggest challenge remains the vitamin B12 substitution in pregnant women, breastfeeding mothers and children at any age. Specific management by pediatricians using dietetic support and blood analyzes are required for children under vegan diet with respect of moral and ethical values related to this lifestyle choice.

Combined Pancreatic Islet-Lung-Liver Transplantation in a Pediatric Patient with Cystic Fibrosis-Related Diabetes.

Background: Cystic fibrosis-related diabetes (CFRD) is the most frequent extrapulmonary complication of cystic fibrosis (CF).

Methods: We report the first combined pancreatic islet-lung-liver transplantation in a 14-year-old adolescent. CFTR was analyzed by Sanger sequencing.

A new mutation in the C-terminal end of TTC37 leading to a mild form of syndromic diarrhea/tricho-hepato-enteric syndrome in seven patients from two families.

Syndromic diarrhea/tricho-hepato-enteric syndrome (SD/THE) is a rare congenital enteropathy with seven main clinical features: intractable diarrhea of infancy, hair abnormalities, intrauterine growth restriction (IUGR), facial dysmorphism, immune dysfunction, and liver and skin abnormalities. SD/THE is caused by mutations in TTC37 or SKIV2L, two genes encoding components of the human SKI complex. To date, approximately 50 SD/THE patients have been described with a wide spectrum of mutations, and only one recurrent mutation has been identified in independent families.

Age at disease onset of inflammatory bowel disease is associated with later extraintestinal manifestations and complications.

Introduction: A small but increasing number of patients with inflammatory bowel disease are diagnosed during childhood or adolescence, and disease distribution and severity at onset vary according to the age at diagnosis. Clinical factors present at the time of diagnosis can be predictive of the disease course.

Aim: The aim of this study was to characterize disease behavior and the cumulative complications and extraintestinal manifestations 10 years after the diagnosis and to assess their association with age at diagnosis.

Food protein-induced enterocolitis syndrome - a review of the literature with focus on clinical management.

Food protein-induced enterocolitis syndrome (FPIES) is a potentially severe presentation of non-IgE-mediated gastrointestinal food allergy (non-IgE-GI-FA) with heterogeneous clinical manifestations. Acute FPIES is typically characterized by profuse vomiting and lethargy, occurring classically 1-4 hours after ingestion of the offending food. When continuously exposed to the incriminated food, a chronic form has been described with persistent vomiting, diarrhea, and/or failure to thrive.

Extraintestinal Manifestations of Pediatric Inflammatory Bowel Disease: Prevalence, Presentation, and Anti-TNF Treatment.

Background: There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD).

Methods: Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively.

Outcome of home parenteral nutrition in 251 children over a 14-y period: report of a single center.

Background: Parenteral nutrition (PN) is the main treatment for intestinal failure.

Objective: We aimed to review the indications for home parenteral nutrition (HPN) in children and describe the outcome over a 14-y period from a single center.

Design: We conducted a retrospective study that included all children who were referred to our institution and discharged while receiving HPN between 1 January 2000 and 31 December 2013.

Weaning Off Prognosis Factors of Home Parenteral Nutrition for Children With Primary Digestive Disease.

Objectives: The aim of the present study was to describe the indications for home parenteral nutrition (HPN) in children with primary digestive diseases and to identify factors associated with weaning off.

Methods: All the children initially discharged on HPN between January 1, 2000, and December 31, 2009, for chronic intestinal failure (IF) were included. The associations between clinical factors and weaning off of HPN were assessed using a multivariable Cox regression model.

Intestinal absorption rate in children after small intestinal transplantation.

Background: Small bowel transplantation has now become a recognized treatment of irreversible, permanent, and subtotal intestinal failure.

Objective: The aim of this study was to assess intestinal absorption at the time of weaning from parenteral nutrition in a series of children after intestinal transplantation.

Design: Twenty-four children (age range: 14-115 mo) received intestinal transplantation, together with the liver in 6 children and the colon in 16 children.