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Abstract Background Constipation during pregnancy is a common problem. Nowadays only few effective interventions are published preventing or treating constipation during pregnancy. However, their use is limited due to side-effects. This uncontrolled intervention study was performed to determine if a mixture of probiotics in the treatment of constipation during pregnancy is effective. Methods Women aged ≥ 18 years with functional constipation were included at the Obstetrical outpatient clinic and midwife practices. Patients received during four weeks a daily dose of Ecologic®Relief (Bifidobacterium bifidum W23, Bifidobacterium lactis W52, Bifidobacterium longum W108, Lactobacillus casei W79, Lactobacillus plantarum W62 and Lactobacillus rhamnosus W71 (total 4*109 CFU)). For all analyses, the non-parametric paired Wilcoxon test was used. Primary outcome measure was change in defecation frequency. Secondary outcome measures were stool consistency, sensation of incomplete evacuation, sensation of anorectal obstruction, manual manoeuvres to facilitate defecation, abdominal pain, adverse effects, presence of reflux episodes and intake of Bisacodyl. Results 20 women were included. Defecation frequency significantly increased from 3.1 at baseline to 6.7 in week four (p Conclusions Ecologic®Relief is effective in the treatment of constipation during pregnancy. A randomised placebo controlled trial is required to confirm these data.

Abstract Background Constipation is a frustrating symptom affecting 3% of children worldwide. Randomised controlled trials show that both polyethylene glycol and lactulose are effective in increasing defecation frequency in children with constipation. However, in 30–50%, these children reported abdominal pain, bloating, flatulence, diarrhoea, nausea and bad taste of the medication. Two recent studies have shown that the fermented dairy product containing Bifidobacterium lactis strain DN-173 010 is effective in increasing stool frequency in constipation-predominant irritable bowel syndrome patients with a defecation frequency < 3/week and in constipated women with a defecation frequency < 3/week. Goal of this study is to determine whether this fermented dairy product is effective in the treatment of constipated children with a defecation frequency < 3/week. Methods/design It is a two nation (The Netherlands and Poland) double-blind, placebo-controlled randomised multicentre trial in which 160 constipated children (age 3–16 years) with a defecation frequency Bifidobacterium lactis DN-173 010 or a control product, twice a day, for 3 weeks. During the study all children are instructed to try to defecate on the toilet for 5–10 minutes after each meal (3 times a day) and daily complete a standardized bowel diary. Primary endpoint is stool frequency. Secondary endpoints are stool consistency, faecal incontinence frequency, pain during defecation, digestive symptoms (abdominal pain, flatulence), adverse effects (nausea, diarrhoea, bad taste) and intake of rescue medication (Bisacodyl). Rate of success and rate of responders are also evaluated, with success defined as ≥ 3 bowel movements per week and ≤1 faecal incontinence episode over the last 2 weeks of product consumption and responder defined as a subject reporting a stool frequency ≥ 3 on the last week of product consumption. To demonstrate that the success percentage in the intervention group will be 35% and the success percentage in the control group (acidified milk without ferments, toilet training, bowel diary) will be 15%, with alpha 0.05 and power 80%, a total sample size of 160 patients was calculated. Conclusion This study is aimed to show that the fermented dairy product containing Bifidobacterium lactis strain DN-173 010 is effective in increasing stool frequency after 3 weeks of product consumption in children with functional constipation and a defecation frequency < 3/week.

Aim: This study aimed to develop a universally applicable core set of quality indicators for Hirschsprung's disease care through a consensus-driven process, to standardise and improve care quality across Europe., Methods: A modified Delphi method was used to achieve consensus among healthcare professionals (HPs) and patient representatives (PRs) across Europe. Participants completed three rounds of anonymous surveys, rating quality indicators for Hirschsprung's disease care. A systematic literature review informed the initial item list. Results were analysed using predefined criteria, and a final consensus meeting established the core set of indicators., Results: An international panel of 8 PRs and 96 multidisciplinary health care professionals representing 59 European hospitals completed all questionnaires, eventually including 12 baseline characteristics and 39 indicators. Six of the top 10 indicators were commonly prioritised by both groups. The remaining items were refined through debate and finalised during a consensus meeting., Conclusion: This study established a core set of 12 baseline characteristics and 14 quality indicators for evaluating Hirschsprung's disease care. These indicators will support benchmarking and continuous quality improvement within the European Paediatric Surgical Audit framework, ultimately enhancing outcomes and care for children with Hirschsprung's disease., (© 2025 The Author(s). Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

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., Aim: To conduct an online cross-sectional study among medical professionals and researchers across a range of countries, to propose a consensus definition, terminology and duration of medically unresponsive constipation., Method: An expert-designed questionnaire was disseminated via Google Forms in a two-stage study over 2 months targeting paediatric gastroenterology professionals globally and Latin American clinicians with a translated version. The questionnaire had seven critical questions containing details needed to define medically unresponsive constipation. The study protocol was approved by the ethics review panel., Results: The survey involved 1079 participants: 87 from various countries in the first phase and 992 from Latin America in the second. There were 619 (57.3%) general paediatricians and 462 (43 %) paediatric gastroenterologists. The preferred term to indicate poorly responding constipation was 'therapy-resistant constipation' (47.8%), followed by 'refractory constipation' (43.6%). The majority of respondents (92.9%) agreed on considering a time frame for defining refractory constipation, with 37.7% suggesting 2-3 months. 467 (43.2%) recommended including failure despite maximum laxative therapy with two agents should be considered as previous therapy failure. Compliance with therapy was deemed essential for successful treatment by 91.1%, assessed through detailed history-taking (47.4%) or medical/pharmacy records (29.4%)., Conclusion: Based on the professional views collected in this study, we propose the term 'therapy-resistant constipation' and it can be defined as constipation that is not responding to a maximum dose of at least two laxatives of different classes for a minimum of 3 months with good compliance in a secondary or tertiary care facility., 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 Group.)

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. Our findings indicate that FAP patients exhibited higher Social Responsiveness Scale (SRS) scores, suggesting a greater prevalence of autistic traits when compared to unaffected siblings (mean 53.8 vs. 47.4, Wilcoxon p = 0.018). The distribution of SRS scores in cases suggested a bimodal pattern, potentially linked to the location of the APC variant, with scores increasing from the 5' to 3' end of the gene (Pearson's r = 0.33, p = 0.022). While we observed a trend toward lower educational attainment in cases, this difference was not statistically significant. This study is the first to explore the connection between APC variant location and neurodevelopmental traits in FAP, expanding our understanding of the genotype-phenotype correlation. Our results emphasize the importance of clinical assessment for autistic traits in FAP patients, shedding light on the potential role of APC gene variants in these behavioral and cognitive challenges., (© 2024 The Author(s). American Journal of Medical Genetics Part B: Neuropsychiatric Genetics published by Wiley Periodicals LLC.)

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., Methods: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements., Results: The consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus., Conclusions: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center., Type of Study: Clinical consensus statement., Level of Evidence: 3a., Competing Interests: Declaration of competing interest All contributing authors have submitted a declaration of interest form. No competing interests were noticed, all declarations are available on request., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

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. Several pediatric scientific organizations, expert panels, and authorities have recently recommended that standardized PN should generally be used over individualized PN in the majority of pediatric patients including very low birth weight premature infants. In addition, PN admixtures produced and validated by a suitably qualified institution are recommended over locally produced PN. Licensed multi chamber bags are standardized PN bags that comply with Good Manufacturing Practice and high-quality standards for the finished product in the frame of their full manufacturing license. The purpose of this article is to review the practical aspects of PN and the evidence for using such multi-chamber bags in pediatric patients. It highlights the safety characteristics and the limitations of the different PN practices and provides some guidance for ensuring safe and efficient therapy in pediatric patients., Competing Interests: Conflict of Interest VC, MD, NH, JR, MSDP, ST, FV, DW, and CvdA have received honoraria from Baxter Healthcare Corporation for symposium participation or scientific education activity. HA has received honoraria from Baxter Healthcare Corporation and Fresenius Kabi for symposium participation or scientific education activity. MP has received honoraria from Baxter Healthcare Corporation, Fresenius Kabi, and B. Braun for symposium participation or education scientific activity. MD, VC and MT have received investigator-initiated research grants from the Baxter Healthcare Corporation (money paid to their institutions). TS is an employee of Baxter Healthcare Corporation. PL and LO have no conflicts of interest to declare., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

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., Method: We searched CENTRAL, MEDLINE, Embase, WHO ICTR and ClinicalTrials.gov. Studies that included patients with therapy-resistant constipation were identified. Data were extracted on criteria used for defining therapy-resistant constipation and reported using a meta-narrative approach highlighting areas of convergence and divergence in the findings., Results: A total of 1553 abstracts were screened in duplicate, and 47 studies were included in the review. There were at least seven definitions used in the paediatric literature to define medically resistant constipation. The term intractable was used in 24 articles and 21 used the term refractory to describe therapy-resistant constipation. Out of them, only 14 articles have attempted to provide an explicit definition including a predefined time and prior therapy. There were 10 studies without a clear definition for therapy-resistant constipation. The duration before being diagnosed as therapy-resistant constipation varied from 1 months to 2 years among studies. Seven studies employed the Rome criteria (Rome III or Rome IV) to characterising constipation while five adopted the Rome III and European and North American paediatric societies definition of paediatric gastroenterology, hepatology and nutrition guideline of management of constipation in children., Conclusion: The current literature has no explicit definition for therapy-resistant constipation in children. There is a need for a detailed consensus definition to ensure consistency of future research and to avoid unnecessary and maybe even harmful, invasive diagnostic and therapeutic interventions., Competing Interests: Competing interests: VS, MG, SR, WH, ABR, AS, MT, CDL and MS: Abbvie, Ironwood and IQVIA consultant, MAB: Consultant for HIPP, Danone, FrieslandCampina, United Pharmaceuticals, Norgine, Coloplast, Wellspect, Mallinckrodt, Allergan., (© 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.)

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., Methods:  Young children (≤ 3 years) treated with stomas between 1998 and 2018 were retrospectively identified. Growth was measured with weight-for-age Z-scores. Malnourishment was defined using the World Health Organization's definition. Comparison between changes in Z-scores at creation, closure, and a year following closure was done by Friedman's test with post hoc Wilcoxon's signed rank test or Wilcoxon's rank-sum test when necessary., Results:  In the presence of a stoma in 172 children, 61% showed growth decline. Severe malnourishment was seen at the time of stoma closure in 51% of the patients treated by small bowel stoma and 16% of those treated by colostomy. Within a year following stoma closure, 67% showed a positive growth trend. Having a proximal small bowel stoma and undergoing major small bowel resection led to significantly lower Z-scores at closure. Adequate sodium supplementation and early closure did not lead to significant changes in Z-scores., Conclusion:  Stomas have a negative impact on growth in the majority of children. This impact might be decreased by preventing small bowel stomas when possible, specifically proximal stomas, and limiting small bowel resection. Since stoma closure is essential in reversing the negative effect on growth, we opt that early closure might result in an early shift to catch-up growth., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

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., Methods: Grading of Recommendations, Assessment, Development and Evaluation (GRADE) will be followed in the development of the guideline, following the approach as laid out in the GRADE handbook, supported by the WHO. The Guideline Development Group (GDG) is formed by paediatric gastroenterologists from both the European Society for Pediatric Gastroenterology, Hepatology and Nutrition, as well as the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Also, one clinical psychologist with expertise in FAPDs is a voting member in the GDG. A final consensus list of treatment options is translated into 'patient, intervention, comparison, outcome' format options. Prospective agreement on the magnitude of health benefits or harms categories was reached through a Delphi process among the GDG to support grading of the literature.There will be a detailed technical evidence review with randomised controlled trial data that will be judged for risk of bias with the Cochrane tool. Recommendations are preferably based on GRADE but could also be best practice statements following the available evidence. A full Delphi process will be used to make recommendations using online response systems. This set of procedures has been approved by all members of the GDG., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

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., Results: In total, 11 of 21 ERNICA IF centers from 8 countries participated. On average, 64% of teams had ≥6 and 36% had 1-5 PIPO patients under active follow-up. In total, 80 of 102 PIPO patients were parenteral nutrition (PN) dependent while each IF team had median 4 (range 0-19) PN dependent PIPO patients under follow-up. On average, each center received 1-2 new PIPO patients per year. Diagnostics mostly followed current guidelines while medical and surgical management strategies were diverse., Conclusions: Numbers of PIPO patients are low and management strategies are diverse among ERNICA IF teams. To improve PIPO patient care, regional reference centers with specialized multidisciplinary IF teams and continuous collaboration across centers are needed., 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.)

Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1−4 and 14−18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores < −2. One-third had %IVSE/REE > 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children.

Objectives: Short bowel syndrome (SBS) is a complex and rare condition (incidence 1200/100,000 live births) that requires a multidisciplinary team approach to management. In January 2019, the first European Reference Network on Rare and Inherited Congenital Anomalies (ERNICA) Intestinal Failure (IF) workshop was held. Several questions about the strategies used in managing IF associated with SBS were devised. The aim of our study was to collect data on the enteral feeding strategies adopted by the ERNICA centres., Methods: A questionnaire (36 questions) about strategies used to introduce enteral nutrition post-operatively and start complementary food/solids in infants with SBS associated IF was developed and sent to 24 centres in 15 countries that participated in the ERNICA-IF workshop. The answers were collated and compared with the literature., Results: There was a 100% response rate. In infants, enteral nutrition was introduced as soon as possible, ideally within 24-48 hours post-small intestinal surgical resection. In 10 of 24 centres, bolus feeding was used, in nine continuous, and in five a combination of both. Twenty-three centres used mothers' own milk as the first choice of feed with extensively hydrolysed feed, amino acid-based feed, donor human milk or standard preterm/term formula as the second choice. Although 22 centres introduced complementary/solid food by 6 months of age, food choice varied greatly between centres and appeared to be culturally based., Conclusions: There is diversity in post-surgical enteral feeding strategies among centres in Europe. Further multi-centre studies could help to increase evidence-based medicine and management on this topic., Competing Interests: Conflicts of Interest: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. The other authors declare that there are no conflicts of interest, and has nothing to disclose., (Copyright © 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Myosin Vb (MYO5B) is a motor protein that facilitates protein trafficking and recycling in polarized cells by RAB11- and RAB8-dependent mechanisms. Biallelic MYO5B mutations are identified in the majority of patients with microvillus inclusion disease (MVID). MVID is an intractable diarrhea of infantile onset with characteristic histopathologic findings that requires life-long parenteral nutrition or intestinal transplantation. A large number of such patients eventually develop cholestatic liver disease. Bi-allelic MYO5B mutations are also identified in a subset of patients with predominant early-onset cholestatic liver disease. We present here the compilation of 114 patients with disease-causing MYO5B genotypes, including 44 novel patients as well as 35 novel MYO5B mutations, and an analysis of MYO5B mutations with regard to functional consequences. Our data support the concept that (1) a complete lack of MYO5B protein or early MYO5B truncation causes predominant intestinal disease (MYO5B-MVID), (2) the expression of full-length mutant MYO5B proteins with residual function causes predominant cholestatic liver disease (MYO5B-PFIC), and (3) the expression of mutant MYO5B proteins without residual function causes both intestinal and hepatic disease (MYO5B-MIXED). Genotype-phenotype data are deposited in the existing open MYO5B database in order to improve disease diagnosis, prognosis, and genetic counseling.

Background & Aims: Chronic intestinal failure (IF) in children is a rare and heterogeneous disease requiring treatment with parenteral nutrition. A uniform definition for chronic IF and standardized outcome measures to compare therapeutic trials in these children are lacking. Therefore, the aim of this study is to systematically assess how definitions and outcome measures are defined in therapeutic trials of children with chronic IF., Methods: MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception till August 2018. No language restriction was used., Results: A total of 1766 articles was found of which 70 studies fulfilled our inclusion criteria. 54 studies (76%) did not report any definition of IF. Of the 16 studies (23%) which reported a definition of IF, 7 different definitions were found. The two most frequently used definitions were: (1) the inability to absorb adequate nutrients to maintain body weight or normal growth and development (n = 5), and (2) the dependence upon parenteral nutrition to maintain minimal energy requirements for growth and development (n = 5). A total of 117 different outcomes were reported. The three most reported outcome measures were: mortality (n = 27), liver enzymes (alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, and gamma-glutamyl trans peptidase) (n = 27), and growth (n = 23). Quality of reporting was considered fair to poor in most studies., Conclusion: There is a lack of reported definitions in studies concerning pediatric IF. Heterogeneity exists in outcome reporting in research concerning pediatric chronic IF. Therefore, we recommend the development of a core outcome set., (Copyright © 2019 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Background: Children with intestinal failure (IF) are at risk of growth failure, but little information about body composition is available. Our aim was to assess body composition using air displacement plethysmography (ADP) and relate it to clinical and growth parameters., Methods: In this prospective descriptive observational 2-center cohort study, children aged 2-18 years receiving home parenteral nutrition (PN) for ≥6 months underwent ADP measurement. Fat mass index (FMI) and fat-free mass index (FFMI) standard deviation scores (SDSs) were calculated to normalize for small body size., Results: Twenty-one out of 22 children, median age 7.4 years, underwent successful ADP measurement after a median PN duration of 5.5 years. They were significantly lighter (median weight for age SDS -0.71, P = 0.004) and shorter (median height for age SDS -1.55, P < 0.001) than the normal population mean; 52% were growing below target height range. They had low FFMI (median SDS -1.53, P < 0.001) and high FMI (median SDS 0.80, P = 0.002). Weight for height and body mass index (BMI) were significantly associated with FFMI and BMI with FMI, but children with the same weight and height showed different body composition. In 13 patients with 1-year follow-up, growth and body composition did not change significantly., Conclusion: Children with IF receiving long-term PN show lower FFM and higher FM than healthy children. Additionally, children with similar routine growth parameters showed different body composition. Further studies should evaluate the effect of a patient-tailored approach including physical activity and nutrition advice based on body composition., (© 2019 American Society for Parenteral and Enteral Nutrition.)

Background: Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare, severe, sporadically occurring disorder characterized by multiple venous malformations., Aims: To present and analyze a case series of pediatric patients with BRBNS and to describe diagnostic approaches and management options applied., Patients and Methods: Multicenter, retrospective study, evaluating the diagnosis and management of children with BRBNS., Results: Eighteen patients diagnosed with BRBNS were included. Cutaneous venous malformations were observed in 78% and gastrointestinal venous malformations in 89%. Lesions were also found in other organs including muscles, joints, central nervous system, eyes, parotid gland, spine, kidneys and lungs. Gastrointestinal lesions were more common in the small intestine than in stomach or colon. The management varied significantly among centers. Endoscopic therapy and surgical therapy alone failed to prevent recurrence of lesions. In younger children and in patients with musculoskeletal or other organ involvement, sirolimus was used with 100% success rate in our series (5 patients treated) although poor compliance with subtherapeutic sirolimus trough levels led to recurrence in a minority., Conclusions: Considering the multi-organ involvement in BRBNS, diagnosis and management requires a multidisciplinary approach. The treatment includes conservative, medical, endoscopic and surgical options. Prospective multicenter studies are needed to identify the optimal management of this rare condition., (Copyright © 2019 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Objectives: Gastroesophageal reflux disease (GERD) is defined as gastroesophageal reflux causing troublesome symptoms or complications. In this study we reviewed the literature regarding the prevalence of GERD symptoms in infants and children., Methods: Databases of PubMed, EMBASE, and Cochrane were systematically searched from inception to June 26, 2018. English-written studies based on birth cohort, school-based, or general population samples of ≥50 children aged 0 to 21 years were included. Convenience samples were excluded., Results: In total, 3581 unique studies were found, of which 25 studies (11 in infants and 14 in children) were included with data on the prevalence of GERD symptoms comprising a total population of 487,969 children. In infants (0-18 months), GERD symptoms are present in more than a quarter of infants on a daily basis and show a steady decline in frequency with almost complete disappearance of symptoms at the age of 12 months. In children older than 18 months, GERD symptoms show large variation in prevalence between studies (range 0%-38% of study population) and overall, are present in >10% and in 25% on respectively a weekly and monthly basis. Of the risk factors assessed, higher body mass index and the use of alcohol and tobacco were associated with higher GERD symptom prevalence., Conclusions: This systematic review demonstrates that the reported prevalence of GERD symptoms varies considerably, depending on method of data collection and criteria used to define symptoms. Nevertheless, the high reported prevalence rates support better investment of resources and educational campaigns focused on prevention.

Background & Aims: We aimed to assess the current organisation and clinical practice of teams treating children with intestinal failure (IF) across Europe and compare the results with the current guideline., Methods: A two-part online survey was sent to all the major European specialist IF services. The first part concerned general information about the team and patients monitored. The second part concerned important care topics such as vascular access and monitoring of complications. No patient identifiers were collected., Results: Seventy-three respondents completed the first part, representing 61 teams in 20 countries. The median number of children on parenteral nutrition (PN) at home per team was 15 (range 1-125). Teams consisted of the following members: paediatric gastroenterologist (present in 100% of the teams), dietitian (95%), specialist nurse (92%), paediatric surgeon (89%), pharmacist (82%), psychologist (66%), social worker (62%), speech therapist (48%), physiotherapist (38%), general paediatrician (33%). The second part was completed by 67/73 respondents (59/61 teams). Vascular access care was comparable with the guideline. Somatostatin analogues were prescribed by 14% of the IF teams and probiotics by 44% of the teams. Prophylactic anticoagulation was used by 46% of the teams. In 81% of the teams a multicomponent lipid emulsion containing fish oil was routinely used. Bone densitometry was regularly performed in 75% of teams, but never performed in 19%., Conclusions: In conclusion, there is a wide diversity of composition of IF teams and their number of patients treated. Overall, there is good compliance to the current guideline. Clinical practice that varied most was the standard use of medication such as probiotics and somatostatin analogues, and standard monitoring of long-term complications. Experience regarding specific treatment options should be shared. Moreover, international agreement on standards of care with focus on implementation of the guideline is needed to optimise care and improve outcomes of children with IF., (Copyright © 2017 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Objectives: This study aimed to provide an overview of the characteristics of thickened formulas to aid health care providers manage infants with regurgitations., Methods: The indications, properties, and efficacy of different thickening agents and thickened formulas on regurgitation and gastroesophageal reflux in infants were reviewed. PubMed and the Cochrane database were searched up to December 2016., Results: Based on the literature review, thickened formulas reduce regurgitation, may improve reflux-associated symptoms, and increase weight gain. However, clinical efficacy is related to the characteristics of the formula and of the infant. Commercial thickened formulas are preferred over the supplementation of standard formulas with thickener because of the better viscosity, digestibility, and nutritional balance. Rice and corn starch, carob bean gum, and soy bean polysaccharides are available as thickening agents. Hydrolyzed formulas have recently shown promising additional benefit., Conclusions: Thickened formulas reduce the frequency and severity of regurgitation and are indicated in formula-fed infants with persisting symptoms despite reassurance and appropriate feeding volume intake., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To evaluate the (cost-)effectiveness of online consultations in follow-up of patients with celiac disease (CD)., Study Design: Multicenter randomized, controlled trial involving 304 patients aged ≤25 years with CD for ≥1 year, randomized to an online (n = 156) or outpatient consultation (n = 148). An online consultation included questionnaires for symptom and growth measurement. Antitransglutaminase-type-2 antibodies were determined using a point-of-care (POC) test. Controls had a traditional consultation with antitransglutaminase-type-2 antibodies testing in laboratories. Both groups completed questionnaires concerning CD-specific health-related quality of life (HRQOL), gluten-free diet adherence, and patient satisfaction. Six months later, participants repeated HRQOL and patient satisfaction questionnaires and the POC test. The primary outcome was anti-transglutaminase-type-2 antibodies after 6 months, and the secondary outcomes were health problems, dietary adherence, HRQOL, patient satisfaction, and costs., Results: The performance of the POC test was inferior to laboratory testing (2/156 positive POC tests vs 13/148 positive laboratory tests; P = .003). Health problems were detected significantly more frequently using online consultation. The detection of growth problems and dietary transgressions was similar. HRQOL (from 1 [good] to 5 [poor]) improved after online consultation (from 3.25 to 3.16 [P = .013] vs controls from 3.10 to 3.23; P = .810). Patient satisfaction (from 1 [low] to 10 [high]) was 7.6 (online) vs 8.0 (controls; P = .001); 58% wished to continue online consultations. Mean costs per participant during the studied period were €202 less for the online group (P < .001)., Conclusions: The primary outcome could not be tested because the POC test was unreliable. Nevertheless, our results indicate that online consultations for children and young adults with CD are cost saving, increase CD-specific HRQOL, and are satisfactory for the majority., Trial Registration: Trialregister.nl: NTR3688., (Copyright © 2017 Elsevier Inc. All rights reserved.)

This document serves as an update of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) 2009 clinical guidelines for the diagnosis and management of gastroesophageal reflux disease (GERD) in infants and children and is intended to be applied in daily practice and as a basis for clinical trials. Eight clinical questions addressing diagnostic, therapeutic and prognostic topics were formulated. A systematic literature search was performed from October 1, 2008 (if the question was addressed by 2009 guidelines) or from inception to June 1, 2015 using Embase, MEDLINE, the Cochrane Database of Systematic Reviews and the Cochrane Central Register of Controlled Clinical Trials. The approach of the Grading of Recommendations Assessment, Development and Evaluation (GRADE) was applied to define and prioritize outcomes. For therapeutic questions, the quality of evidence was also assessed using GRADE. Grading the quality of evidence for other questions was performed according to the Quality Assessment of Studies of Diagnostic Accuracy (QUADAS) and Quality in Prognostic Studies (QUIPS) tools. During a 3-day consensus meeting, all recommendations were discussed and finalized. In cases where no randomized controlled trials (RCT; therapeutic questions) or diagnostic accuracy studies were available to support the recommendations, expert opinion was used. The group members voted on each recommendation, using the nominal voting technique. With this approach, recommendations regarding evaluation and management of infants and children with GERD to standardize and improve quality of care were formulated. Additionally, 2 algorithms were developed, 1 for infants <12 months of age and the other for older infants and children.

Functional constipation and functional non-retentive faecal incontinence are common problems in childhood; these functional defaecation disorders are diagnosed according to the Rome IV criteria. Here we describe the role of an abdominal X-ray in diagnosing functional defaecation disorders, and address the limited additional value of a plain abdominal X-ray. In limited cases it may be helpful to determine colonic transit time using an abdominal X-ray to differentiate between functional constipation and functional non-retentive faecal incontinence. The diagnostic value of abdominal X-rays in children with functional abdominal pain will be discussed. The aims of this article are to explore ways of reducing unnecessary use of abdominal X-rays and reducing unnecessary radiation exposure in children with functional defaecation disorders and functional abdominal pain.

- The guideline entitled 'Functional abdominal pain in children' has been developed by a multidisciplinary working group on behalf of the Dutch Pediatric Association in 2015. - History and physical examination are essential in diagnosing pediatric functional abdominal pain. Additional diagnostic testing is of limited value when alarm symptoms are absent. - Reassurance and education of both the child and parent(s), are the cornerstones in the treatment of pediatric functional abdominal pain. - In case of persisting symptoms, hypnotherapy or cognitive behavioral therapy can be started. There is no evidence for any pharmacological treatment.

Background & Aims: Exact data on Dutch patients with chronic intestinal failure (CIF) and after intestinal transplantation (ITx) have been lacking. To improve standard care of these patients, a nationwide collaboration has been established. Objectives of this study were obtaining an up-to-date prevalence of CIF and characterizing these patients using the specially developed multicenter web-based Dutch Registry of Intestinal Failure and Intestinal Transplantation (DRIFT)., Methods: Cross-sectional study. CIF was defined as type 3 intestinal failure in which >75% of nutritional requirements were given as home parenteral nutrition (HPN) for ≥ 4 weeks in children and >50% for ≥3 months in adults. All patients with CIF receiving HPN care by the three Dutch specialized centers on January 1, 2013 and all ITx patients were registered in DRIFT (https://drift.darmfalen.nl)., Results: In total, 195 patients with CIF (158 adults, 37 children) were identified, of whom 184 were registered in DRIFT. The Dutch point prevalence of CIF was 11.62 per million (12.24 for adults, 9.56 for children) on January 1, 2013. Fifty-seven patients (31%) had one or more indications for ITx, while 12 patients actually underwent ITx since its Dutch introduction. Four patients required transplantectomy of their intestinal graft and 3 intestinal transplant patients died., Conclusion: The multicenter registry DRIFT revealed an up-to-date prevalence of CIF and provided nationwide insight into the patients with CIF during HPN and after ITx in the Netherlands. DRIFT will facilitate the multicenter monitoring of individual patients, thereby supporting multidisciplinary care and decision-making., (Copyright © 2015 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Importance: Histamine-2 receptor antagonists (H2RAs) are frequently used in the treatment of gastroesophageal reflux disease (GERD) in children; however, their efficacy and safety is questionable., Objective: To systematically review the literature to assess the efficacy and safety of H2RAs in pediatric GERD., Evidence Review: PubMed, EMBASE, and the Cochrane database were searched for randomized clinical trials investigating the efficacy and safety of H2RAs in pediatric GERD. Two reviewers independently extracted data from the included articles. The quality of the evidence was assessed using the Grades of Recommendations, Assessment, Development, and Evaluation approach. When possible, infants and children were analyzed separately., Findings: Eight studies with a total of 276 children (0-15 years of age) were included. Compared with the placebo, H2RAs were more effective in the reduction of symptoms in terms of histologic healing and increasing gastric pH and had a larger overall treatment effect. In infants, H2RAs were only more effective in terms of histologic healing. Comparing H2RAs with antacids, H2RAs were more effective in symptom reduction in only 1 study. H2RAs compared with proton pump inhibitors were not significantly different in any of the outcome measures. For safety analysis, data were not reported in a quantitative manner and for all outcomes, the quality of evidence was very low., Conclusions and Relevance: Evidence to support the efficacy and safety of H2RAs in infants and children is limited and of poor quality. Well-designed placebo-controlled trials are needed before thorough conclusions can be drawn.

We present four women with seven ongoing pregnancies. Five pregnancies were complicated by intrahepatic cholestasis of pregnancy (ICP) and severe haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome with uncommon maternal morbidity. The combination of ICP and HELLP syndrome has not previously been reported. Awareness is warranted to accurately identify this combination of pregnancy-specific diseases with severe maternal morbidity.

In 2102, a multidiscplinary guideline was developed on behalf of the Dutch Association of Pediatrics entitled 'Gastroesophageal reflux disease in children from 0-18 years'. The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) guideline from 2009 served for guidance. History and physical examination are usually sufficient for diagnosing gastroesophageal reflux disease. It is important to make a distinction between gastroesophageal reflux, a physiological phenomenon in children, and gastroesophageal reflux disease. In practice, medications are provided too often, especially in infants. Only children suffering from gastroesophageal reflux disease should be treated according to the therapeutical action plan.

Background: Home parenteral nutrition (PN) has improved the survival of children with intestinal failure. Important complications include catheter-related thrombosis, occlusion, and infection. This study evaluated the efficacy and safety of prophylactic anticoagulation in the prevention of these complications., Methods: Medical records were retrospectively reviewed of all children (0-18 years) with PN between January 1994 and March 2007 in 1 tertiary center. After introduction of prophylactic low molecular weight heparin or vitamin K antagonists in March 2007, all patients were prospectively followed until March 2010., Results: In sum, 14 patients did not receive prophylaxis; 13 switched from no prophylaxis to prophylaxis in March 2007; and 5 directly received prophylaxis. Median age of PN onset was 4 months (range, 0.1-202) in the nonprophylaxis group (n = 27) and 25 (range, 2-167) in the prophylaxis group (n = 18); 16 children received low molecular weight heparin and 2, vitamin K antagonists. Catheter-related thrombosis developed in 9 patients with no prophylaxis (33%) and 1 with prophylaxis (6%) (P = .034). Cumulative 5-year thrombosis-free survival was 48% and 93% in the nonprophylaxis and prophylaxis groups, respectively (P = .047). Per 1,000 PN days, the nonprophylaxis and prophylaxis groups had 2.6 and 0.1 occlusions (P = .04) and 4.6 and 2.1 infections (P = .06), respectively. Cumulative infection-free survival after 3 years was 19% and 46% in the nonprophylaxis and prophylaxis groups, respectively (P = .03). Bleeding complications did not occur., Conclusion: Thromboprophylaxis significantly decreased catheter-related thrombosis and occlusion in children with PN without complications.

Endoscopic investigation of small bowel pathology in children has historically been difficult due to location, length and tortuosity of the small bowel. Recently, video capsule endoscopy and balloon-assisted enteroscopy techniques have evolved as new diagnostic tools and are increasingly used in the paediatric population. In this review the current literature is appraised to define the clinical indications and practical aspects of capsule endoscopy and balloon-assisted enteroscopy in children., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Probiotics are defined as 'live micro-organisms that confer a health benefit on the host if administered in adequate amounts'. There are only limited data in the literature regarding the efficacy and safety of lactobacilli for various gastrointestinal indications in children. Lactobacillus rhamnosus GG (LGG) has no added value in the treatment of children with functional constipation. Lactobacilli do seem to be effective in rotavirus diarrhoea. They seem safe and shorten the duration of the diarrhoea by about 1 day. For the prevention of infectious diarrhoea, lactobacilli are only moderately effective and the clinical relevance is unclear. There is 1 controlled study in which LGG had a preventive effect on the development of atopic eczema (but not of allergy to cow's milk) in newborn infants.

Video-capsule endoscopy was used in 4 children with unexplained symptoms of the small intestine. Each patient swallowed a capsule of 11 by 27 mm, which contained a camera that takes 2 images per second (in children aged less than 8 years, the capsule was placed in the duodenum under sedation). In a 3-year-old girl with rectal bleeding following partial resection of the colon and small intestine, ulcers were seen proximal to the ileorectal anastomosis. In a 14-year-old boy with Crohn's disease and an abnormal growth curve, multiple stenoses of the small intestine were seen. In an 8-year-old boy with rectal bleeding, a solitary polypoid mass was seen that, upon operation, appeared to be the result of a partially invaginated Meckel's diverticulum. In a 17-year-old boy with weight loss, rectal bleeding and colitis, abnormalities were seen that were consistent with Crohn's disease. Patients were treated based on the endoscopic results and subsequently recovered. Video-capsule endoscopy is non-invasive and painless and provides better images of the small intestine than a standard endoscopic and radiological examination.