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Congenital diarrhoeas and enteropathies (CODE) are a heterogeneous group of disorders. Many affected infants present with catastrophic dehydration in the first few days of life, although the clinical phenotype is variable. Advances in the understanding of underlying pathomechanisms and genetic testing, as well as improved management, in particular intravenous nutrition support, have allowed affected patients to survive well beyond childhood. Awareness and understanding of these rare diseases are hence needed, both amongst paediatricians and adult physicians. In this review, we discuss the different groups of disorders based on a review of the current literature and provide a diagnostic and therapeutic approach. Many of the subtypes of CODE result in the need for prolonged or indefinite parenteral nutrition. Further research is needed to identify new CODE to improve the recognition and management of these children, which can assist in developing new targeted therapies and potentially a long-term cure.

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. SBS is characterised by abnormal microbiome patterns, which might serve as predictive indicators for IR. We aim to characterise the microbiome profiles of children with SBS during IR, concurrently exploring how parental perspectives of QoL relate to IR., Methods and Analysis: This study will enrol a minimum of 20 paediatric patients with SBS (0-18 years). Clinical data and biological samples will be collected over a 2-year study period. We will apply 16S rRNA gene sequencing to analyse the microbiome from faecal and gut tissue samples, with additional shotgun metagenomic sequencing specifically on samples obtained around the time of IR. Gas chromatography with flame ionisation detection will profile faecal short-chain fatty acids. Plasma citrulline and urinary intestinal fatty acid binding proteins will be measured annually. We will explore microbiome-clinical covariate interactions. Furthermore, we plan to assess parental perspectives on QoL during PN and post-IR by inviting parents to complete the Paediatric Quality of Life questionnaire at recruitment and after the completion of IR., Ethics and Dissemination: Ethical approval was obtained from the East Midlands-Nottingham 2 Research Ethics Committee (22/EM/0233; 28 November 2022). Recruitment began in February 2023. Outcomes of the study will be published in peer-reviewed scientific journals and presented at scientific meetings. A lay summary of the results will be made available to participants and the public., Trial Registration Number: ISRCTN90620576., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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. The aim of this study was to describe causes and outcomes of CVC repairs in home PN dependent children., Methods: All patients (ages 0-17 years) with CVC dependency enrolled in the IF rehabilitation program of a pediatric referral center were identified, and those who underwent a CVC repair between January 2019 and November 2020 included. Data on associated cause for breakage and incidence of central line-associated bloodstream infections (CLABSIs) post repair were documented retrospectively. Descriptive statistics including medians, percentages, and frequencies were used., Results: Forty children, 15 males (37%) and 25 females (63%), were identified. Fifteen of 40 (37.5%) patients underwent a total of 29 CVC repairs (0.1 repairs per 1000 catheter days); 8 of 15 (53%; 33% females) were <5 years of age. The most common reason for repair was CVC fracture by biting (41%) followed by catheter occlusion with intraluminal PN deposition (13.2%). Repair was successful in 100% cases. Only 1 patient had a confirmed CLABSI post repair (1/29 repairs; 3%) who presented 3 days late after the initial catheter breakage. Catheter salvage was successful with antibiotics., Conclusion: CVC repair in our cohort was very successful and infection rate after repair minimal. CVC repair rather than removal is recommended to preserve central venous access., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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. Target gene mutations commence in utero, resulting in a profusion of clonal expansions, with only a few haematopoietic stem cell lineages (mean 8, range 1-24) contributing ~50% of haematopoietic colonies across 8 individuals (range 4-100% clonality) by young adulthood. Rapid clonal expansion during disease transformation is associated with biallelic TP53 mutations and increased mutation burden. Our study highlights how convergent somatic mutation of the p53-dependent nucleolar surveillance pathway offsets the deleterious effects of germline ribosomopathy but increases opportunity for TP53-mutated cancer evolution., (© 2023. Springer Nature Limited.)

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. Therefore, the aim of this study is to create a core outcome set (COS) to be used in studies on pediatric chronic intestinal failure., Methods: Candidate outcomes were selected from a recent systematic review. A three-round Delphi study among key stakeholders and a consensus meeting with an expert panel were undertaken to achieve consensus on the COS., Results: Seventy-two stakeholders (79%) completed all three rounds of the Delphi process. Ninety-eight outcomes were assessed, and five new outcomes were added after the first round. Ten outcomes were included in the final COS: weaning from parenteral nutrition, growth, mortality, central line-related infection, central line longevity, sepsis not related to central line infection, central line-related thrombosis, intestinal failure-associated liver disease, (serious) adverse events, and health-related quality of life., Conclusion: This pediatric chronic intestinal failure COS consists of 10 outcomes important for all key stakeholders. Usage of this set in future research should minimize outcome heterogeneity and enhance the value of evidence synthesis. This will lead to better management in this field of rare gastrointestinal conditions., (© 2022 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

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. A list of topics was developed, and individual leads were assigned to topics along with other members, who reviewed the current literature. A single face-to-face meeting was held in February 2020. Provisional conclusions and recommendations were developed between 2020 and 2021, and these were voted on electronically by all members of the working group between 2021 and 2022. Where >90% consensus was not achieved, online discussion meetings were held, along with further voting until agreement was reached., Results: In general, there is a lack of strong evidence for most nutrients and topics. The summary paper is supported by additional supplementary digital content that provide a fuller explanation of the literature and relevant physiology: introduction and overview; human milk reference data; intakes of water, protein, energy, lipid, carbohydrate, electrolytes, minerals, trace elements, water soluble vitamins, and fat soluble vitamins; feeding mode including mineral enteral feeding, feed advancement, management of gastric residuals, gastric tube placement and bolus or continuous feeding; growth; breastmilk buccal colostrum, donor human milk, and risks of cytomegalovirus infection; hydrolyzed protein and osmolality; supplemental bionutrients; and use of breastmilk fortifier., Conclusions: We provide updated ESPGHAN CoN consensus-based conclusions and recommendations on nutrient intakes and nutritional management for preterm infants., Competing Interests: N.D.E. reports receipt of research grant supports from National Institutes for Health Research (UK), Prolacta Bioscience (US), Danone Early Life Nutrition, Medical Research Council, and Action Medical Research; payment/honorarium for lectures from Nestle Nutrition Institute, Astarte Medical. S.J.M. reports receipt of research support from DSM Nutritional Products and payment/honorarium for lectures from Baxter. A.L. reports receipt of lecture fees and/or nonfinancial support from Baxter, Fresenius, Nestle, and Mead Johnson Nutrition. C.H.P.v.d.A. reports receipt of speakers and consultancy honoraria from Nestlé Nutrition Institute, Nutricia Early Life Nutrition, and Baxter. V.C. reports speaker fees from Baxter and Chiesi Pharma. C.F. reports receipt of lecture fees from Nestle Nutrition Institute, Medela, Prolacta, and Neobiomics and consultancy fees for Medela, Baxter, Hipp, and Nutricia. K.G. reports receipt of research grants, speakers and consultancy fees and hospitality from Nestle Health Sciences, Nutricia-Danone, Baxter, Mylan, Dr. Falk Pharma, Abbott, Servier, and Janssen. J.B.v.G. reports a research grant from Nutrinia and FP7/Danone. Founder & Director of Dutch National Human Milk Bank and council member of National Health Council. N.H. reports consulting fees from Medis, MAM, Baxter, and Nestle and speaker fees from Nestle, Baxter, Danone, and Hipp outside the submitted work. M.J.J. reports research grants from NeoKare UK Ltd. S.M. reports research support from Nestle Health Science. L.N. reports consultancy advice for Takaeda and Nestle. J.B. reports personal fees and nonfinancial support from AbbVie, Nutricia, and Biocodex, personal fees from MSD, Nestlé, and Ferring. F.I. reports receipt of payment/honorarium for lectures from Biogaia, Nestle, Danone, and Abbot, and consultancy fees from Biogaia. E.V. reports grant/research support from Nutricia Italia Spa, Nestle Health Science – Vitaflo Italy, FoodAR srl Italy, PIAM Pharma, and Integrative Care. M.D. reports a research grant from Prolacta and speaker fees from Semper, Baxter, Mead Johnson, and Nestlé. The remaining authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, 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. Final consensus was obtained by multiple e-mail exchange and virtual meetings of the CAHP and CON., Results: Reported benefits of blended diets include reduced GERD and infections, improved defecation, level of alertness and attention span, skin conditions, and appearance of hair and nails. Families report a sense of greater normality. Small case series, cross-sectional surveys, questionnaire-based small case studies, reports of personal experience, and single-center pilot studies are available in the medical literature. A total of 20 recommendations for practice were made based on the results and consensus process., Conclusions: There is little evidence published to formally inform about the potential health benefits or risks of this practice and how to use it in the best way. This leaves health professionals caring for such patients in a relative vacuum regarding what to consider when providing a duty of care to patients and carers who wish to pursue this method of feeding. This article provides guidelines for safe and appropriate use of a BD, but more research is needed., Competing Interests: The authors report no conflicts of interest. This is a joint position paper of the ESPGHAN Committees of Allied Health Professionals and Nutrition., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Methods: A literature search was performed from 1992 to 2022 using Pubmed, MEDLINE and Cochrane Database of Systematic Reviews, and recent guidelines were reviewed. In the absence of evidence, recommendations reflect the authors' expert opinion., Results: Consensus on the best possible way of feeding children with IF-SBS is lacking and practice varies widely between centres. Feeding should commence as soon as possible following surgery. Oral feeding is the preferred route and breast milk (BM) the first milk of choice in infants. Donor BM, standard preterm or term formula are alternatives in the absence of maternal BM. Extensively hydrolysed or amino acid-based feeds are used when these are not tolerated. Solids should be introduced as soon as clinically appropriate. Children are encouraged to eat by mouth and experience different tastes and textures to avoid oral aversion. Aggressive weaning of PN and tube (over-) feeding are now discouraged., Conclusions: To date, uniform agreement on the optimal type of feed, timing of food introduction and feeding regime used is lacking and great difference in practice remains. There is need for more research to establish common treatment protocols.

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). Full blood count, and whenever available, ferritin and C-reactive protein were collected at least 3 times for each patient. Liver function tests were checked at the beginning and end of the study and number of blood transfusions/iron infusions recorded., Results: Forty-one HPN patients (61% girls; 51% motility disorder, 29% enteropathy, 20% short bowel syndrome) were identified. Eighty-three percent of children were anaemic at the beginning of the study with the number decreasing to 73% by the end. Iron deficiency anaemia was most commonly seen and treated with blood transfusion in 46% of cases and iron infusions in 29%. There was no statistical difference in the level of haemoglobin improvement between the 2 treatments (50% vs 33%, P  = 0.657) nor a significant difference in haemoglobin (g/L) level achieved. The results for both were, however, more favourable in the infusion group. Patients receiving transfusions had a significantly higher incidence of abnormal liver function compared with those who hadinfusions (93% vs 37%, P = 0.009)., Conclusions: Iron deficiency anaemia is common in children receiving HPN. A large proportion of patients still receive blood transfusions as first-line therapy but intravenous iron can be a suitable alternative. Treatment guidelines are required., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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., Patients: Children with severe NI referred to our IF rehabilitation programme from 2009 to 2019., Main Outcome Measures: Demographic and social data, diagnosis, clinical condition, use of home parenteral nutrition (HPN), complications, ethics review outcome and advance care plans., Results: Six patients with severe NI were referred to our IF rehabilitation service. Consent for publication was obtained from five families. After thorough medical review and clinical ethics committee assessment, three children started HPN, one had intravenous fluids in addition to enteral feed as tolerated and one intravenous fluids only. The HPN children survived 3-7.08 years (median 4.42 years) on treatment. Objective gastrointestinal signs, for example, bleeding improved without excessive HPN-related complications. Symptomatic improvement was less clear. Analgesia was reduced in three of the five children. All cases had detailed symptom management and advance care plans regularly updated., Conclusions: HPN can play a role in relieving gastrointestinal signs/symptoms in children with severe NI and IF. HPN can be conceptualised as part of good palliative care if judged to be in the child's best interests. However, given its risks and that HPN has the potential to become inappropriately life-sustaining, a thorough ethics review and evaluation should be performed before it is initiated, withheld or withdrawn in children with severe NI., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Congenital chloride diarrhoea [CLD] is a rare autosomal recessive disease caused by mutations in the solute family carrier 26 member 3 [SLC26A3] gene. Patients suffer from life-long watery diarrhoea and chloride loss. Inflammatory bowel disease [IBD] has been reported in individual patients with CLD and in scl26a3-deficient mice., Methods: We performed an international multicentre analysis to build a CLD cohort and to identify cases with IBD. We assessed clinical and genetic characteristics of subjects and studied the cumulative incidence of CLD-associated IBD., Results: In a cohort of 72 patients with CLD caused by 17 different SLC26A3 mutations, we identified 12 patients [17%] diagnosed with IBD. Nine patients had Crohn's disease, two ulcerative colitis and one IBD-unclassified [IBD-U]. The prevalence of IBD in our cohort of CLD was higher than the highest prevalence of IBD in Europe [p < 0.0001]. The age of onset was variable [13.5 years, interquartile range: 8.5-23.5 years]. Patients with CLD and IBD had lower z-score for height than those without IBD. Four of 12 patients had required surgery [ileostomy formation n = 2, ileocaecal resection due to ileocaecal valve stenosis n = 1 and colectomy due to stage II transverse colon cancer n = 1]. At last follow-up, 5/12 were on biologics [adalimumab, infliximab or vedolizumab], 5/12 on immunosuppressants [azathioprine or mercaptopurine], one on 5-ASA and one off-treatment., Conclusions: A substantial proportion of patients with CLD develop IBD. This suggests the potential involvement of SL26A3-mediated anion transport in IBD pathogenesis. Patients with CLD-associated IBD may require surgery for treatment failure or colon cancer., (© The Author(s) 2021. Published by Oxford University Press on behalf of European Crohn’s and Colitis Organisation. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Abstract: 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. A complex interplay exists between diet, microbiome, and epigenetic factors. The effect of single nutrients on immune function may hence be difficult to study. Well-designed intervention studies, investigating the effects of whole dietary pattern on the immune system, are needed., Competing Interests: The authors certify that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript. In addition, the authors report no involvement in the research by the sponsor that could have influenced the outcome of this work., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and 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. The Medline database and the Cochrane Library were used in the search for relevant publications. The quality of evidence was reviewed and discussed before voting on recommendations, and a consensus of 90% or more was required for the final approval. Important research gaps were also identified., Results: This position paper provides clinical recommendations on nutritional support during different phases of critical illness in preterm and term neonates based on available literature and expert opinion., Conclusion: Basic research along with adequately powered trials are urgently needed to resolve key uncertainties on metabolism and nutrient requirements in this heterogeneous patient population., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Abstract: 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. We present data from systematic reviews and meta- analysis, randomised controlled trials (RCTs) and large observational studies, published from 2011 onwards that consider the possible role of the following factors in obesity development: breast-feeding; macronutrient composition and method of complementary feeding; parenting style; dietary patterns; sugar-sweetened beverage consumption; eating behaviour (eg, skipping breakfast, family dinners. etc); meal frequency and composition (fast foods, snacking), portion size; dietary modulators of gut microbiota (including pre-, pro-, and synbiotics); physical activity and sedentary behaviour. We used the Medline database and the Cochrane Library to search for relevant publications. Important research gaps were also identified. This position paper provides recommendations on dietary factors, food habits, and lifestyle to prevent childhood obesity development, based on the available literature and expert opinion. Clinical research and high-quality trials are urgently needed to resolve numerous areas of uncertainty., Competing Interests: The authors report no conflicts of interest relevant to this article but many of the authors have received research funding or honorarium for lectures from pharmaceutical or infant food/milk companies. E.V. reports grant/research support from Nutricia Italia Spa, Nestle Health Science—Vitaflo Italy, FoodAR srl Italy, PIAM Pharma, and Integrative Care. J.B. reports personal fees and non-financial support from AbbVie, Nutricia, Biocodex and personal fees from MSD, Nestlé, Ferring, Walmark. N.E. reports receipt of grants/research supports from National Institutes for Health Research (UK), Prolacta Bioscience (US), and Danone Early life Nutrition. He also served as member of Advisory board for Danone Early life Nutrition and received payment/honorarium for lectures from Danone Early life Nutrition, Nestle Nutrition Institute, Baxter, and Fresenius Kabi. K.G. reports receipt of research grants, speakers and consultancy fees, and hospitality from Nestle Health Sciences, Nutricia-Danone, Baxter, Mylan, DrFalk, and Abbott. F.I. reports receipt of payment/honorarium for lectures from Biogaia, Nestle, Danone, Abbot, and consultancy fees from Biogaia. A.L. reports receipt of lecture fees and/or non-financial support from Baxter, Fresenius, Nestle, and Nead Johnson Nutrition. S.J.M. reports receipt of research support from DSM Nutritional Products and payment/honorarium for lectures from Baxter. M.D. reports a research grant from Baxter and speaker fees from Semper, Baxter, Nutricia, and Abbvie. The remaining authors (J.K., Bd.K., and L.N.) report no conflict of interests., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and 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. Clinical signs of VTE deficiencies usually present only when body stores are substantially depleted and are often difficult to detect or differentiate from other nonnutrient-related causes. In isolation, dietary assessment of micronutrients can be inaccurate and imprecise, in disease and in individual patient assessment but may be useful to complement findings from other VTE assessment methods. Use of biomarkers is the most common approach to assess VTE status in routine practice but in the presence of systemic inflammatory response and in the absence of appropriate paediatric reference intervals, interpretation of biomarker results might be challenging and potentially mislead clinical practice. The use of a multimodal approach, including clinical examination, dietary assessment, and laboratory biomarkers is proposed as the optimal way to ascertain the VTE status of individual patients. In the presence of acute inflammatory conditions, VTE measurements in plasma should be replaced by biomarkers not affected by systemic inflammatory response or delayed until inflammatory state is resolved.

Objectives: To assess renal function in pediatric intestinal failure (IF) patients on long term home parenteral nutrition (HPN). Methods: 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. Results: Twenty five patients (40% male) fulfilled the inclusion criteria. The indications for HPN were due to an underlying motility disorder in 56% (14/25), enteropathy in 24% (6/25), and short bowel syndrome in 20% (5/25). At the start of HPN 80% (20/25) had a normal eGFR. Four children (17%) had an abnormal eGFR. In the group of patients with normal eGFR at the start of HPN 30% (6/20) had at least one episode of decreased eGFR in the following 3 years, however there was no significant decline in eGFR at the end of the 3 year study period. Overall there was no statistically significant deterioration of eGFR in the study population ( p = 0.7898). Conclusion: In our cohort of children on long term HPN no significant decline of eGFR could be demonstrated within 3 years of starting PN.

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., Results: Overall, 55 responses from 49 centers in 18 European countries and Israel were collected. The majority of respondents were from the United Kingdom (10, 19%), followed by Germany (7, 13%) and France (6, 11%). Eleven centers (21%) cared for >30 patients, 8 (15%) centers between 20 and 30 patients, 18 (34%) centers between 10 and 20 patients, and 16 (30%) <10 patients on home PN. There was a high variability in the majority of answers to the cross-sectional survey., Conclusions: CVC insertion and maintenance in children on home PN varies largely amongst centers in Europe. These differences could be at least partially explained by the lack of updated guidelines and limited evidence. There is an urgent need for collaborative research to make recommendations about the best possible practice.

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. Information about the PN, enteral nutrition, type of feeding in infancy, weaning, dental and oral problems was collected. Results A total of 35 patients were identified, of which 28 participated in the study. The age of the children ranged from 1-18 years with a median age of 5.5 years. The average duration of PN administration was 4.3 years. Just over half were also orally fed and three quarters had a history of breast and/or bottle feeding in infancy. Around 60% of children reported oral problems including teeth staining (29%), gum infections (11%), teeth decay and delayed dentition (16%). 68% regularly visited the dentist at 2-12 monthly intervals., Conclusion: In comparison to the general UK paediatric population, oral and dental problems occurred less commonly in our study group of children on HPN, unlike adult patients on intravenous nutrition where poor oral health was much more prominent compared to the general public. However the overall prevalence was similar in the adult and paediatric age group receiving long-term HPN highlighting the need for specific health advice in this patient group.

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., Methods: A standardised questionnaire was sent to all 40 children on HPN within a large tertiary intestinal failure centre in the United Kingdom. Depending on whether the family had/had not been on holiday since their child had started HPN, questions were asked to understand the reasons for not travelling or to gather information about individual travel experiences., Results: A total of 30 children were enrolled, 20 of 30 went at least once on holiday, and 5 of 30 travelled more than once per year, 70% travelled outside Britain. Going on vacation was more common, the longer the child had been on HPN (P = 0.022); hours spent on PN tolerance of enteral feeds or the child's age did not influence travel behaviour; 80% of parents who went on vacation had a good/worthy experience, 95% would travel again. The biggest reported obstacle was the transportation of PN bags. Ten families sacrificed a holiday over fear that it may be difficult to arrange or because of the child's unstable medical condition., Conclusions: A significant proportion of families chose to go on holiday away from home despite their child being on HPN. The experience is considered good by most.

Objective: In Shwachman-Diamond syndrome (SDS), pancreatic insufficiency can lead to malabsorption of fat-soluble vitamins and trace elements. The aim of this study was to assess the serum concentrations of vitamins A and E, zinc, copper, and selenium and their deficiencies., Methods: This retrospective review was performed in 21 children (12 were male; median age, 7.8 years) with genetically confirmed SDS at a tertiary pediatric hospital. Pancreatic enzyme replacement therapy (PERT) and vitamin or trace elements supplements were documented., Results: Twenty patients (95%) had pancreatic insufficiency receiving PERT, 10 (47%) had a combined vitamin and trace element deficiency, 6 (29%) had an isolated vitamin deficiency, and 4 (19%) had an isolated trace element deficiency. Vitamins A and E deficiency occurred in 16 (76%) and 4 (19%) of 21, respectively. Low serum selenium was found in 10 (47%), zinc deficiency in 7 (33%), and copper deficiency in 5 (24%). Eleven patients (52%) were on multivitamin supplementation, and 2 (10%) on zinc and selenium supplements. No statistical differences were found between repeated measurements for all micronutrients., Conclusions: More than 50% of the children had vitamin A and selenium deficiencies despite adequate supplementation of PERT and supplements. Micronutrients should be routinely measured in SDS patients to prevent significant complications.