Symptoms recurred frequently and required repetition of the interventions.In this retrospective multicentre study, endoscopic management of gastroparesis by balloon dilatation or botulinum toxin was safe in children and seemed to be partially efficient within the first months. Symptoms recurred frequently and required repetition of the interventions. Cystic fibrosis related liver disease (CFLD) with consecutive cirrhosis is the third most common cause of death in CF patients (3). The aim of this study is to identify the potential long-term benefits of liver transplantation (LTx) in a match-control comparison. Retrospective single-center data analysis of all pediatric LTx for CFLD between 1998 and 2014. A control group was selected from the local CF patient registry. Data were collected from case report forms and included clinical and laboratory data, lung function tests, the indication for LTx and details of surgical procedures. At our institution 23 patients with severe CFLD median age 13.8 years (range 8.7-17.4; 16 boys) underwent LTx between 1998 and 2014. In all patients, normalization of hepatic CF manifestations were achieved after LTx. But obviously there was no significant positive influence on nutritional status. Signs of post-transplant liver steatosis were documented by ultrasound in 17 patients. check details Liver biopsies after LTx were performed interm perspectives of affected patients. To describe a cohort of Wilson's disease (WD) paediatric cases, and to point out the diagnostic particularities of this age group and the long-term outcome. Clinical data of 182 paediatric patients included in the French WD national registry from 01/03/1995 to 01/06/2019 were gathered. Diagnosis of WD was made at a mean age of 10.7 ± 4.2 years (range 1-18 years). At diagnosis, 154 patients (84.6%) had hepatic manifestations, 19 (10.4%) had neurological manifestations, and 9 patients (4.9%) were asymptomatic. The p.His1069Gln mutation was the most frequently encountered (14% of patients).Neurological patients were diagnosed at least one year after they presented their first symptoms. At diagnosis, the median urinary copper excretion (UCE) was 4.2 μmol/24 hours (0.2-253). The first-line treatment was D-penicillamine (DP) for 131 (72%) patients, zinc salts for 24 (13%) patients, and Trientine for 17 (9%) patients. Liver transplantation was performed in 39 (21.4%) patients, for hepatic indications in 33/39 nd proper treatment ensure excellent outcomes, subject to good long-term treatment compliance. LT is a valid option for end-stage liver disease not responding to treatment and can be discussed for selected cases of neurological deterioration. Pediatric functional gastrointestinal disorders including irritable bowel syndrome with constipation and functional constipation are common conditions in childhood, but no drugs are FDA approved for chronic use in pediatric patients with these disorders. Despite efforts to better standardize the diagnosis of these conditions in children (including recent modifications to the Rome criteria), conducting pediatric clinical trials to support drug approval remains a challenge. In March 2018, the U.S. Food and Drug Administration, in collaboration with the NASPGHAN, AGA, and ACG, convened a public workshop to discuss the challenges and opportunities in conducting pediatric clinical trials in functional GI conditions. The workshop assembled gastroenterologists, psychologists, patients, patient advocates, regulators, and industry representatives to discuss trial design and conduct including alternative designs, eligibility criteria, instruments for patient- and observer-reported outcomes, and optimal primary endpoits, patients, patient advocates, regulators, and industry representatives to discuss trial design and conduct including alternative designs, eligibility criteria, instruments for patient- and observer-reported outcomes, and optimal primary endpoints to support regulatory approval. This report summarizes the workshop, key challenges and knowledge gaps identified, and outlines areas where further research efforts are needed to overcome barriers to developing drugs to treat these conditions. To evaluate the safety and use of the 3D-Transit system (Motilis SA, Lausanne, Switzerland) and to describe regional gastrointestinal transit times, segmental colonic transit times and colonic movement patterns in healthy children. 21 healthy children (11 girls, median age 10.5 years, range 7-15 years) were included. For evaluation of gastrointestinal transit times and colonic movement patterns, we used the minimally-invasive electromagnetic 3D-Transit system. A small electromagnetic capsule (21.5 x 8.3 mm) was ingested and tracked through the gastrointestinal tract by a body-worn detector. Regional gastrointestinal transit times were assessed as time between capsule passage of anatomical landmarks. Colonic movement patterns were described and classified based on capsule movement velocity, direction, and distance. One child could not swallow the capsule and 20 children completed the study without any discomfort or side-effects. Median whole gut transit time was 33.6 (range 10.7-80.5) hours, median gastric emptying time was 1.9 (range 0.1-22.1) hours, median small intestinal transit time was 4.9 (range 1.1-15.1) hours, and median colonic transit time was 26.4 (range 6.8-74.5) hours. Median ascending colon/cecum transit time was 9.7 (range 0.3-48.1) hours, median transverse colon transit time was 5.6 (range 0.0-11.6) hours, median descending colon transit time was 2.6 (range 0.01-22.3) hours, and median sigmoid colon/rectum transit time was 7.5 (range 0.1-31.6) hours. Colonic movement patterns among children corresponded to those previously described in healthy adults. The 3D-Transit system is a well-tolerated and minimally-invasive method for assessment of gastrointestinal motility in children.The 3D-Transit system is a well-tolerated and minimally-invasive method for assessment of gastrointestinal motility in children. Evaluate accuracy of skinfold thicknesses and body mass index (BMI) for the prediction of fat mass percentage (FM%) in pediatric inflammatory bowel disease (IBD) and to develop population specific formulae based on anthropometry for estimation of FM%. IBD children (n = 30) and healthy controls (HCs, n = 144) underwent anthropometric evaluation and Dual-energy X-ray absorptiometry (DEXA) scan, as the clinical reference for measurement of body composition. Body FM% estimated with skinfolds thickness was compared with FM% measured with DEXA. By means of four prediction models, population specific formulae for estimation of FM% were developed. No significant difference in terms of FM% measured by DEXA was found between IBD population and HCs (FM% 29.6% vs 32.2%, p = 0.108). Triceps skinfold thickness (TSF, Model 2) was better than BMI (Model 1) at predicting FM% (82% vs. 68% of variance). The sum of two skinfolds (biceps + triceps; SF2, Model 3) showed an improvement in the prediction of FM% as compared to TSF, Model 2 (86% vs.