[Extra-intestinal manifestations associated with Crohn's disease].

Extra-intestinal manifestations (EIM) associated with Crohn's disease (CD) are frequently observed and can manifest as either the initial symptom or develop later in the disease course following gastrointestinal symptoms. The most common EIM affect the joints, skin, or eyes, with some correlating with active CD activity while others may occur during periods of inactivity. EIM can affect both pediatric and adult CD patients, potentially reducing their quality of life if not promptly identified and treated.

Randomised clinical trial: First-line infliximab biosimilar is cost-effective compared to conventional treatment in paediatric Crohn's disease.

Background: Data on cost-effectiveness of first-line infliximab in paediatric patients with Crohn's disease are limited. Since biologics are increasingly prescribed and accompanied by high costs, this knowledge gap needs to be addressed.

Aim: To investigate the cost-effectiveness of first-line infliximab compared to conventional treatment in children with moderate-to-severe Crohn's disease.

Gastric emptying and myoelectrical activity testing in children with esophageal atresia: A pilot study.

Objectives: Abnormalities of gastric function in children with esophageal atresia (EA) could potentially contribute to gastrointestinal symptoms and reduced quality of life (QOL). Therefore, we aimed to determine the feasibility and clinical usefulness of gastric function testing in children with EA.

Methods: The validated PedsQL Gastrointestinal Symptoms Questionnaire (PedsQL-GI) was completed to assess gastrointestinal symptoms and symptom-related QOL.

Unintentional Suture Fistula Between the Proximal and Distal Esophagus in a Preterm Neonate with Type C Esophageal Atresia.

We present the case of a patient with gross type C esophageal atresia in a preterm neonate (gestational age of 31 weeks + 1 day) with a birth weight of 1,470 g. The fistula was released via a thoracotomy, but no primary anastomosis could be made, due to an unexpected long gap. The distal esophagus was closed and approximated to the blind pouch using traction sutures until an adjacent position was reached.

Age-dependent normal values for the 'Infant Gastroesophageal Reflux Questionnaire Revised'.

The Infant Gastroesophageal Reflux Questionnaire Revised (I-GERQ-R) is used to score and evaluate gastroesophageal reflux (GER) related symptoms such as regurgitation, irritability and crying. A cut-off of ≥ 16 has been suggested to discriminate between GER and GER-disease. However, age-specific normal values were not assessed.

Protocol for an international multicenter randomized controlled trial assessing treatment success and safety of peroral endoscopic myotomy vs endoscopic balloon dilation for the treatment of achalasia in children.

Introduction: Achalasia is a rare neurodegenerative esophageal motility disorder characterized by incomplete lower esophageal sphincter (LES) relaxation, increased LES tone and absence of esophageal peristalsis. Achalasia requires invasive treatment in all patients. Conventional treatment options include endoscopic balloon dilation (EBD) and laparoscopic Heller's myotomy (LHM).

Development and validation of a condition-specific quality of life instrument for adults with esophageal atresia: the SQEA questionnaire.

The importance of multidisciplinary long-term follow-up for adults born with esophageal atresia (EA) is increasingly recognized. Hence, a valid, condition-specific instrument to measure health-related quality of life (HRQoL) becomes imperative. This study aimed to develop and validate such an instrument for adults with EA.

Fatal gastrointestinal complications in Pitt-Hopkins syndrome.

Pitt-Hopkins syndrome (PTHS) is a rare neurodevelopmental disorder caused by mutations of the transcription factor 4 (Tcf4) gene. Individuals with PTHS often suffer from severe abdominal bloating and constipation. In this short communication, we discuss two individuals with PTHS who died unexpectedly due to gastrointestinal complications.

Psychometric Performance of a Condition-Specific Quality-of-Life Instrument for Dutch Children Born with Esophageal Atresia.

A condition-specific instrument (EA-QOL©) to assess quality of life of children born with esophageal atresia (EA) was developed in Sweden and Germany. Before implementing this in the Netherlands, we evaluated its psychometric performance in Dutch children. After Swedish−Dutch translation, cognitive debriefing was conducted with a subset of EA patients and their parents.

Management of Gastroesophageal Reflux Disease in Esophageal Atresia Patients: A Cross-Sectional Survey amongst International Clinicians.

Objectives: After surgical repair, up to 70% of esophageal atresia (EA) patients suffer from gastroesophageal reflux disease (GERD). The ESPGHAN/NASPGHAN guidelines on management of gastrointestinal complications in EA patients were published in 2016. Yet, the implementation of recommendations on GERD management remains poor.

Evaluation of exclusive enteral nutrition and corticosteroid induction treatment in new-onset moderate-to-severe luminal paediatric Crohn's disease.

To induce remission in luminal paediatric Crohn's disease (CD), the ESPGHAN/ECCO guideline recommends treatment with exclusive enteral nutrition (EEN) or oral corticosteroids. In newly diagnosed moderate-to-severe paediatric CD patients, we determined the proportion of patients in which EEN or corticosteroids induced remission and maintained remission on azathioprine monotherapy. We included patients from the "TISKids" study assigned to the conventional treatment arm.

Colonic Function Investigations in Children: Review by the ESPGHAN Motility Working Group.

Disorders of colonic motility, most often presenting as constipation, comprise one of the commonest causes of outpatient visits in pediatric gastroenterology. This review, discussed and created by the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Motility Working Group, is a practical guide, which highlights the recent advances in pediatric colonic motility testing including indications, technical principles of the tests, patient preparation, performance and basis of the results' analysis of the tests. classical methods, such as colonic transit time (cTT) with radiopaque markers and colonic scintigraphy, as well as manometry and novel techniques, such as wireless motility capsule and electromagnetic capsule tracking systems are discussed.

Fundoplication in children with esophageal atresia: preoperative workup and outcome.

Up to 45% of esophageal atresia (EA) patients undergo fundoplication during childhood. Their esophageal dysmotility may predispose to worse fundoplication outcomes compared with patients without EA. We therefore compared fundoplication outcomes and symptoms pre- and post-fundoplication in EA patients with matched patients without EA.

Adverse Events of Thiopurine Therapy in Pediatric Inflammatory Bowel Disease and Correlations with Metabolites: A Cohort Study.

Background: In the recent era of growing availability of biological agents, the role of thiopurines needs to be reassessed with the focus on toxicity.

Aims: We assessed the incidence and predictive factors of thiopurine-induced adverse events (AE) resulting in therapy cessation in pediatric inflammatory bowel disease (IBD), related to thiopurine metabolites and biochemical abnormalities, and determined overall drug survival.

Methods: We performed a retrospective, single-center study of children diagnosed with IBD between 2000 and 2019 and treated with thiopurine therapy.

First-line treatment with infliximab versus conventional treatment in children with newly diagnosed moderate-to-severe Crohn's disease: an open-label multicentre randomised controlled trial.

Objective: In newly diagnosed paediatric patients with moderate-to-severe Crohn's disease (CD), infliximab (IFX) is initiated once exclusive enteral nutrition (EEN), corticosteroid and immunomodulator therapies have failed. We aimed to investigate whether starting first-line IFX (FL-IFX) is more effective to achieve and maintain remission than conventional treatment.

Design: In this multicentre open-label randomised controlled trial, untreated patients with a new diagnosis of CD (3-17 years old, weighted Paediatric CD Activity Index score (wPCDAI) >40) were assigned to groups that received five infusions of 5 mg/kg IFX at weeks 0, 2, 6, 14 and 22 (FL-IFX), or EEN or oral prednisolone (1 mg/kg, maximum 40 mg) (conventional).

Clinical Experience With Performing Esophageal Function Testing in Children.

Objectives: Pediatric high-resolution manometry (HRM) and 24-hour pH-impedance with/without ambulatory manometry (pH-MII+/-mano) tests are generally performed using adult-derived protocols. We aimed to assess the feasibility of these protocols in children, the occurrence of patient-related imperfections and their influence on test interpretability.

Methods: Esophageal function tests performed between 2015 and 2018 were retrospectively analyzed.

Evaluation of Gastroesophageal Reflux in Children Born With Esophageal Atresia Using pH and Impedance Monitoring.

Objectives: The aim of the study was to evaluate acid and nonacid gastroesophageal reflux in infants and school-aged children with esophageal atresia (EA) using pH-impedance (pH-MII) monitoring.

Methods: Between 2012 and 2017, all 24-hour pH-MII studies performed in infants (≤18 months) and 8-year olds with EA were included. Antiacid therapy was discontinued before study.

Oesophageal atresia.

Oesophageal atresia (EA) is a congenital abnormality of the oesophagus that is caused by incomplete embryonic compartmentalization of the foregut. EA commonly occurs with a tracheo-oesophageal fistula (TEF). Associated birth defects or anomalies, such as VACTERL association, trisomy 18 or 21 and CHARGE syndrome, occur in the majority of patients born with EA.

Clinical Management of Pediatric Achalasia: A Survey of Current Practice.

Objectives: Pediatric achalasia is a rare neurodegenerative disorder of the esophagus that requires treatment. Different diagnostic and treatment modalities are available, but there are no data that show how children can best be diagnosed and treated. We aimed to identify current practices regarding the diagnostic and therapeutic approach toward children with achalasia.

Measurement of Salivary Pepsin to Detect Gastroesophageal Reflux Disease Is Not Ready for Clinical Application.

A noninvasive test for gastroesophageal reflux disease (GERD) is desirable for adults and children. Salivary pepsin measurement has been proposed as such a test. A previous study from our group demonstrated that a maximal salivary pepsin cutoff of >210 ng/mL using the PepTest device (RD Biomed, Hull, United Kingdom) had excellent specificity of 96% but modest sensitivity of 44% to diagnose GERD, leading to optimism about its potential use.