Towards a definition of refractory/therapy-resistant/intractable constipation in children: a cross-sectional, questionnaire-based, online survey.

Background: The Rome criteria define childhood functional constipation but do not address refractory constipation. Attempts to define refractory constipation lack consensus. The interchangeable use of 'refractory' and 'intractable' or 'therapy-resistant' constipation and lack of understanding of the therapeutic ceilings before this diagnosis complicates the definition.

Incidence, diagnostics, therapeutic management and outcomes of paediatric intestinal pseudo-obstruction in the Netherlands: A 20-year retrospective cohort study.

Objectives: To describe incidence, clinical course, diagnostic and therapeutic management and long-term follow-up of paediatric intestinal pseudo-obstruction (PIPO) in the Netherlands between 2000 and 2020.

Methods: Multicenter, national, retrospective, observational study including patients aged <18 years diagnosed with PIPO and treated between 2000 and 2020 in Dutch academic medical centres. Outcomes included demographics, incidence, symptoms, diagnostic- and treatment methods used during follow-up, number of hospital admissions and mortality.

Playing sport as a central-line carrier: a survey to collect the European pediatric intestinal failure centers' view.

Background: The administration of home parenteral nutrition improves quality of life for patients with intestinal failure, thus fostering their will to actively participate to social activities. Nevertheless, sports participation can be risky for patients with a central venous catheter (CVC). Despite literature thoroughly proving the positive impact of sports on motor-psychosocial development, no consistent evidence assessing its role on central-line complications is available.

Antegrade Continence Enemas for Pediatric Functional Constipation: A Systematic Review.

Background: Despite optimal conservative and medical treatment, some children with functional constipation (FC) continue to experience symptoms. Antegrade continence enema (ACE) surgery has been suggested as the primary surgical option after less invasive pharmacological and non-pharmacological interventions have not been effective. The purpose of this systematic review was to assess the outcomes of ACE for children with FC.

Autistic traits in youth with familial adenomatous polyposis: A Dutch-Canadian case-control study.

This study investigated the neurodevelopmental impact of pathogenic adenomatous polyposis coli (APC) gene variants in patients with familial adenomatous polyposis (FAP), a cancer predisposition syndrome. We hypothesized that certain pathogenic APC variants result in behavioral-cognitive challenges. We compared 66 FAP patients (cases) and 34 unaffected siblings (controls) to explore associations between APC variants and behavioral and cognitive challenges.

Transition from pediatric to adult care in patients with chronic intestinal failure on home parenteral nutrition: How to do it right?

Background: Life expectancy of children with chronic intestinal failure (CIF) on home parenteral nutrition has greatly improved. Children are now able to grow into adulthood which requires transfer from pediatric to adult health care. A guideline for structured transition is lacking and the demand for a more standardized care for this patient group is necessary.

How do we define therapy-resistant constipation in children aged 4-18 years old? A systematic review with meta-narrative synthesis.

Background: Therapy-resistant constipation often is a frustrating clinical entity recognised by the persistence of infrequent and painful bowel movements faecal incontinence and abdominal pain despite intensive treatment. It is important to clearly define therapy-resistant constipation before children are subjected to invasive diagnostic and therapeutic procedures.

Aim: To conduct a systematic review determining how paediatric interventional studies define therapy-resistant constipation.

Safe and efficient practice of parenteral nutrition in neonates and children aged 0-18 years - The role of licensed multi-chamber bags.

Parenteral nutrition (PN) is recognized as a complex high-risk therapy. Its practice is highly variable and frequently suboptimal in pediatric patients. Optimizing care requires evidence, consensus-based guidelines, audits of practice, and standardized strategies.

Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis.

Background: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis.

Catheter salvage or removal in catheter-related bloodstream infections with Staphylococcus aureus in children with chronic intestinal failure receiving home parenteral nutrition and the use of prophylactic taurolidine catheter lock solution: A descriptive cohort study.

Background: Children with chronic IF require long-term home parenteral nutrition (HPN), administered through a central venous catheter. Catheter-related bloodstream infection (CRBSI) with Staphylococcus aureus is known to be a serious infection with a high mortality rate and risk of complications. A standardized protocol on the management of S aureus CRBSIs in children receiving HPN is lacking.

Systematic review on management of high-output enterostomy in children: An urgent call for evidence.

Objectives/background: High-output stoma is one of the most common major morbidities in young children with an enterostomy that could lead to intestinal failure. Management of high-output enterostomy in children is mostly based on personal experience. This systematic review aims to clarify the evidence-based therapeutic approach of high-output enterostomy in children.

ESPGHAN and NASPGHAN 2023 protocol for paediatric FAPD treatment guidelines (standard operating procedure).

Introduction: To date, no international guidelines have been published for the treatment of paediatric functional abdominal pain disorders (FAPDs), subcategorised into functional abdominal pain-not otherwise specified (FAP-NOS), irritable bowel syndrome (IBS), functional dyspepsia and abdominal migraine (AM). We aim for a treatment guideline, focusing on FAP-NOS, IBS and AM, that appreciates the extensive array of available therapies in this field. We present the prospective operating procedure and technical summary protocol in this manuscript.

Children with Intestinal Failure are at Risk for Psychopathology and Trauma.

Objectives: The objective of this study is to assess the psychopathology and medical traumatic stress in children with intestinal failure (IF) and identify associated risk factors.

Methods: Two-center study, performed from September 2019 until April 2022 (partly during COVID-19 pandemic), including children (1.5-17 years) with IF, dependent on parenteral nutrition (PN) or weaned off PN, treated by a multidisciplinary IF-team.

What are Normal Defecation Patterns in Healthy Children up to Four Years of Age? A Systematic Review and Meta-Analysis.

Objective: To summarize available data on defecation frequency and stool consistency of healthy children up to age 4 in order to estimate normal references values.

Study Design: Systematic review including cross-sectional, observational, and interventional studies published in English, that reported on defecation frequency and/or stool consistency in healthy children 0-4 years old.

Results: Seventy-five studies were included with 16 393 children and 40 033 measurements of defecation frequency and/or stool consistency.

Pediatric Intestinal Pseudo-Obstruction: An International Survey on Diagnostic and Management Strategies in the European Reference Network for Rare Inherited and Congenital Anomalies Intestinal Failure Teams.

Objectives: Pediatric intestinal pseudo-obstruction (PIPO) management is based on nutritional, medical, and surgical care while available evidence is scarce. The aim of this study was to outline the current diagnostic and management strategies in intestinal failure (IF) teams of the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) and to compare these practices to the latest PIPO international guidelines.

Methods: An online survey on institutional diagnostic and management strategies of PIPO was conducted among the ERNICA IF teams.

Impact of Presence, Level, and Closure of a Stoma on Growth in Young Children: A Retrospective Cohort Study.

Introduction:  A stoma will cause nutrients loss which could result in impaired growth. Impaired growth can negatively impact long-term development. This study aims to evaluate: (1) the effect of stomas on growth comparing small bowel stoma versus colostomy and (2) if early closure (within 6 weeks), proximal small bowel stoma (within 50 cm of Treitz), major small bowel resection (≥ 30 cm), or adequate sodium supplementation (urinary level ≤ 30 mmol/L) influences growth.

An Update of Pharmacological Management in Children with Functional Constipation.

Functional constipation is a common problem in childhood worldwide and has a great impact on social, physical, and emotional functioning of affected children and their caregivers. It is a clinical diagnosis based on the Rome IV criteria. Non-pharmacological treatment involves education, demystification, lifestyle advice, and toilet training.

Development of a core outcome set for pediatric chronic intestinal failure.

Background: In research on pediatric chronic intestinal failure, heterogeneity in reported definitions and outcomes exists. This leads to a risk of reporting bias and impossibility of evidence synthesis. Also, reported outcomes should be relevant to both healthcare providers and patients and their parents.

Ghrelin Levels in Children With Intestinal Failure Receiving Long-Term Parenteral Nutrition.

Background: Children with intestinal failure (IF) require parenteral nutrition (PN). Transition to oral and enteral nutrition (EN) can be difficult also due to abnormal gastrointestinal motility. The gut hormone ghrelin is increased in states of negative energy balance, functioning to preserve euglycemia, and also has appetite stimulating and prokinetic properties.

A therapeutic guide on pediatric irritable bowel syndrome and functional abdominal pain-not otherwise specified.

Unlabelled: Disorders of the gut-brain interaction negatively impact quality of life and carry a substantial socioeconomic burden. Irritable bowel syndrome (IBS) and functional abdominal pain-not otherwise specified (FAP-NOS) are common functional abdominal pain disorders in childhood. The pathophysiology is not fully understood, and high-quality intervention trials and international guidelines are missing.

Psychosocial Interventions for the Treatment of Functional Abdominal Pain Disorders in Children: A Systematic Review and Meta-analysis.

Importance: Functional abdominal pain disorders (FAPDs) can severely affect the life of children and their families, with symptoms carrying into adulthood. Management of FADP symptoms is also a financial and time burden to clinicians and health care systems.

Objective: To systematically review various randomized clinical trials (RCTs) on the outcomes of cognitive behavioral therapy (CBT), educational support, yoga, hypnotherapy, gut-directed hypnotherapy, guided imagery, and relaxation in the management of FAPDs.

Dietary interventions for functional abdominal pain disorders in children: a systematic review and meta-analysis.

Background: Functional abdominal pain disorders (FAPDs) are common among children and are associated with decreased quality of life and school attendance. Several dietary interventions have been suggested to improve symptoms of FAPDs. This systematic review assessed the efficacy and safety of dietary interventions for pediatric FAPDs.

Development of a Core Outcome Set for Children Aged 1-18 Years with Gastroesophageal Reflux Disease.

Objective: To develop a core outcome set for clinical studies assessing gastroesophageal reflux disease (GERD) in children.

Study Design: This core outcome set was developed using a 2-round Delphi technique and adhering to the Outcome Measures in Rheumatology Initiative (OMERACT 2.0) recommendations.