Gut microbiota and intestinal rehabilitation: a prospective childhood cohort longitudinal study of short bowel syndrome (the MIRACLS study): study protocol.

Introduction: Short bowel syndrome (SBS) is the predominant cause of paediatric intestinal failure. Although life-saving, parenteral nutrition (PN) is linked to complications and may impact quality of life (QoL). Most children will experience intestinal rehabilitation (IR), but the mechanisms underpinning this remain to be understood.

Causes and Outcome of Central Venous Catheter Repair in Children with Intestinal Failure on Home Parenteral Nutrition.

Objectives: Children requiring a central venous catheter (CVC) for long-term parenteral nutrition (PN) are at risk of CVC breakage. Modern intestinal failure (IF) management aims to preserve vascular access sites. CVC repair rather than removal is hence attempted for broken catheters.

Convergent somatic evolution commences in utero in a germline ribosomopathy.

Clonal tracking of cells using somatic mutations permits exploration of clonal dynamics in human disease. Here, we perform whole genome sequencing of 323 haematopoietic colonies from 10 individuals with the inherited ribosomopathy Shwachman-Diamond syndrome to reconstruct haematopoietic phylogenies. In ~30% of colonies, we identify mutually exclusive mutations in TP53, EIF6, RPL5, RPL22, PRPF8, plus chromosome 7 and 15 aberrations that increase SBDS and EFL1 gene dosage, respectively.

Enteral Nutrition in Preterm Infants (2022): A Position Paper From the ESPGHAN Committee on Nutrition and Invited Experts.

Objectives: To review the current literature and develop consensus conclusions and recommendations on nutrient intakes and nutritional practice in preterm infants with birthweight <1800 g.

Methods: The European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Committee of Nutrition (CoN) led a process that included CoN members and invited experts. Invited experts with specific expertise were chosen to represent as broad a geographical spread as possible.

Nutritional Management of Intestinal Failure due to Short Bowel Syndrome in Children.

Background: The most common cause of intestinal failure (IF) in childhood remains short bowel syndrome (SBS), where bowel mass is significantly reduced due to a congenital atresia or resection and parenteral nutrition (PN) needed. Home PN has improved outcome and quality of life, but the long-term therapeutic goal is to achieve enteral autonomy whilst avoiding long term complications. This paper is aimed at discussing nutritional strategies available to clinicians caring for these patients.

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.

The Use of Blended Diets in Children With Enteral Feeding Tubes: A Joint Position Paper of the ESPGHAN Committees of Allied Health Professionals and Nutrition.

Objectives: This joint position paper of the Committees of Allied Health Professionals (CAHP) and Nutrition (CON) of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) provides a comprehensive guide for health professionals to manage blended diets in children via gastrostomy tubes.

Methods: A systematic literature search was performed from 1992 to 2021 using Pubmed, MEDLINE, and Cochrane Database of Systematic Reviews and recent guidelines reviewed. In the absence of evidence, recommendations reflect the authors' expert opinion.

An ESPGHAN Position Paper on the Use of Low-FODMAP Diet in Pediatric Gastroenterology.

Excluding oligo-, di-, monosaccharides and polyols (FODMAPs) from the diet is increasingly being used to treat children with gastrointestinal complaints. The aim of this position paper is to review the available evidence on the safety and efficacy of its use in children and provide expert guidance regarding practical aspects in case its use is considered . Members of the Gastroenterology Committee, the Nutrition Committee and the Allied Health Professionals Committee of the European Society for Pediatric Gastroenterology Hepatology and Nutrition contributed to this position paper.

Anaemia in Children Receiving Home Parenteral Nutrition: A Common Problem?

Objectives: Children receiving home parenteral nutrition (HPN) are at risk of iron deficiency anaemia. Our aim was to determine the incidence of iron deficiency anaemia in paediatric HPN and efficacy of each therapeutic approach.

Methods: Retrospective data collection from children receiving HPN at a tertiary referral centre over a 12-month period (2015).

Use of home parenteral nutrition in severely neurologically impaired children.

Objective: To review the outcome of children with severe neurological impairment (NI) and intestinal failure (IF) referred to our specialist multidisciplinary IF rehabilitation service and to discuss implications.

Design: Case report series, descriptive analysis.

Setting: IF rehabilitation programme at a tertiary children's hospital in the UK.

Immunomodulation in Children: The Role of the Diet.

Immune maturation and response to inflammation depend on good nutritional status. Protein and amino acid deficiencies can compromise innate and adaptive immune functions, particularly following injury or during illness. Dietary omega-3 long-chain fatty acids, prebiotics and micronutrients are beneficial to the immune system.

Role of Dietary Factors, Food Habits, and Lifestyle in Childhood Obesity Development: A Position Paper From the European Society for Paediatric Gastroenterology, Hepatology and Nutrition Committee on Nutrition.

Childhood obesity has high societal and economic impact but current treatment approaches are sub-optimal. In the last decade, important studies have been conducted aiming to identify strategies to prevent obesity during critical periods of life. Updated recommendations for childhood obesity prevention are needed.

Nutritional Management of the Critically Ill Neonate: A Position Paper of the ESPGHAN Committee on Nutrition.

Objectives: The nutritional management of critically ill term neonates and preterm infants varies widely, and controversies exist in regard to when to initiate nutrition, mode of feeding, energy requirements, and composition of enteral and parenteral feeds. Recommendations for nutritional support in critical illness are needed.

Methods: The ESPGHAN Committee on Nutrition (ESPGHAN-CoN) conducted a systematic literature search on nutritional support in critically ill neonates, including studies on basic metabolism.

Mucosal Abnormalities in Children With Congenital Chloride Diarrhea-An Underestimated Phenotypic Feature?

Congenital chloride diarrhea (CCD) is a rare, autosomal recessive disorder caused by mutations in the gene encoding a transmembrane chloride/bicarbonate ion exchanger mainly expressed in the apical brush border of the ileal and colonic epithelium. Lifelong, secretory, chloride-rich diarrhea and hypochloremic, hypokalemic metabolic alkalosis are characteristic. Histological evidence of bowel inflammation is not typically described in CCD and has only been reported in a few patients.

Assessment and Interpretation of Vitamin and Trace Element Status in Sick Children: A Position Paper From the European Society for Paediatric Gastroenterology Hepatology, and Nutrition Committee on Nutrition.

Assessment of vitamin and trace element status (VTE) is important in the clinical management of the sick child. In this position paper, we present the various assessment methods available to the clinical practitioner, and critically discuss pitfalls with interpretation of their results. There are 4 main approaches to assess the VTE body status of an individual patient including clinical examination, dietary assessment, and measurement of direct and indirect biomarkers of VTE in biological samples.

Colonic Basidiobolomycosis-An Unusual Presentation of Eosinophilic Intestinal Inflammation.

Basidiobolomycosis is a rare fungal disease caused by . Involvement of the gastrointestinal tract is unusual and poses both a diagnostic and therapeutic challenge, as clinical signs are non-specific and predisposing risk factors are lacking. It can mimick inflammatory bowel disease, primary immunodeficiency, or a malignancy and should be considered in patients who do not respond to standard therapy.

Renal Function in Children on Long Term Home Parenteral Nutrition.

To assess renal function in pediatric intestinal failure (IF) patients on long term home parenteral nutrition (HPN). Children who received HPN for a minimum of 3 years between 2007 and 2017 were identified from the IF clinic of a large tertiary referral center. Estimated glomerular filtration rate (eGFR) was calculated using the Schwartz formula at discharge on HPN, after 6 months, 1, 2, and 3 years.

Central Line in Long-term Parenteral Nutrition in Children: A European Survey.

Background And Aims: The guidelines for the insertion and maintenance of the central venous catheter (CVC) in children on long-term parenteral nutrition (PN) were published 12 years ago and studies evaluating the outcomes are limited. Therefore, the aim of the present study was to perform a survey about criteria for CVC insertion and maintenance in intestinal failure/rehabilitation centers treating children on home PN.

Methods: An online cross-sectional survey based on previous European Society of Paediatric Gastroenterology, Hepatology and Nutrition PN guidelines was distributed electronically to the members of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition networking group, Network for Intestinal Failure and Transplantation in Europe and tertiary pediatric gastroenterology centers in Europe.

Indications and complications of inpatient parenteral nutrition prescribed to children in a large tertiary referral hospital.

Background: Parenteral Nutrition (PN) is prescribed to children with intestinal failure. Although life saving, complications are common. Recommendations for indications and constituents of PN are made in the 2005 guidelines by the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN).

A survey of oral and dental health in children on home parenteral nutrition.

Aim: To determine oral and dental problems in children who are receiving long term intravenous nutrition at home.

Design: Children who had been at home on parenteral nutrition (PN) for a period of at least 3 months were recruited from the intestinal failure clinic database of a large tertiary nutrition centre. The parents were contacted by email, face to face or telephone and asked to fill in a questionnaire.

Travelling With Children on Home Parenteral Nutrition.

Background: Home parenteral nutrition (HPN) is an established therapy in children with intestinal failure. Parenteral nutrition (PN) management allows most paediatric patients to participate in age-appropriate activities; however, HPN may lead to significant restrictions, particularly going on holiday. We aimed to identify sociodemographic and illness-specific variables that influence if and how families with children on HPN travel.