Approach to Endoscopic Balloon Dilatation in Pediatric Stricturing Crohn Disease: A Position Paper of the Endoscopy Special Interest Group of ESPGHAN.

Crohn disease (CD) is often complicated by bowel strictures that can lead to obstructive symptoms, resistant inflammation, and penetrating complications. Endoscopic balloon dilatation of CD strictures has emerged as a safe and effective technique for relieving these strictures, which may obviate the need for surgical intervention in the short and medium term. This technique appears to be underutilized in pediatric CD. This position paper of the Endoscopy Special Interest Group of European Society for Pediatric Gastroenterology, Hepatology and Nutrition describes the potential applications, appropriate evaluation, practical technique, and management of complications of this important procedure. The aim being to better integrate this therapeutic strategy in pediatric CD management.

Identification of Iatrogenic Perforation in Pediatric Gastrointestinal Endoscopy.

OBJECTIVES: Iatrogenic viscus perforation in pediatric gastrointestinal endoscopy (GIE) is a very rare, yet potentially life-threatening event. There are no evidence-based recommendations relating to immediate post-procedure follow-up to identify perforations and allow for timely management. This study aims to characterize the presentation of children with post-GIE perforation to better rationalize post-procedure recommendations. METHODS: Retrospective study based on unrestricted pooled data from centers throughout Europe, North America, and the Middle East affiliated with the Endoscopy Special Interest Groups of European Society for Paediatric Gastroenterology Hepatology and Nutrition and North American Society for Pediatric Gastroenterology Hepatology and Nutrition. Procedural and patient data relating to clinical presentation of the perforation were recorded on standardized REDCap case-report forms. RESULTS: Fifty-nine cases of viscus perforation were recorded [median age 6 years (interquartile range 3-13)]; 29 of 59 (49%) occurred following esophagogastroduodenoscopy, 26 of 59 (44%) following ileocolonoscopy, with 2 of 59 (3%) cases each following balloon enteroscopy and endoscopic retrograde cholangiopancreatography; 28 of 59 (48%) of perforations were identified during the procedure [26/28 (93%) endoscopically, 2/28 (7%) by fluoroscopy], and a further 5 of 59 (9%) identified within 4 hours. Overall 80% of perforations were identified within 12 hours. Among perforations identified subsequent to the procedure 19 of 31 (61%) presented with pain, 16 of 31 (52%) presented with fever, and 10 of 31 (32%) presented with abdominal rigidity or dyspnea; 30 of 59 (51%) were managed surgically, 17 of 59 (29%) managed conservatively, and 9 of 59 (15%) endoscopically; 4 of 59 (7%) patients died, all following esophageal perforation. CONCLUSIONS: Iatrogenic perforation was identified immediately in over half of cases and in 80% of cases within 12 hours. This novel data can be utilized to generate guiding principles of post-procedural follow-up and monitoring. PLAIN LANGUAGE SUMMARY: Bowel perforation following pediatric gastrointestinal endoscopy is very rare with no evidence to base post-procedure follow-up for high-risk procedures. We found that half were identified immediately with the large majority identified within 12 hours, mostly due to pain and fever.

Food intolerances in children and adolescents in Switzerland.

Adverse food reactions have become an important health concern in pediatrics. There are discrepancies between diagnosed and self-reported food allergies and intolerances, leading to food avoidance with possible psychological and nutritional implications in the latter. There is no data available so far on the number of children and adolescents, who reduce or completely avoid certain foods in their diet. The aim of this study was to determine the prevalence of children and adolescents in the Northwest of Switzerland, who omit foods from their diet because of a self-reported intolerance, as well as the prevalence of children and adolescents who eliminate well-tolerated foods for presumed health reasons. This is a cross-sectional study conducted in the Children's hospitals of Aarau and Basel (Switzerland) and in 4 pediatric private practices. A total of 2036 children and adolescents (54% male and 46% female) between 1 month and 18 years (mean age: 7.4 years) were included, of which 316 (16%) participants reported to avoid foods due to intolerance. Lactose intolerance is the most frequent one. In 55% of all cases, no medical tests had been performed to confirm such an intolerance. Avoiding tolerated foods for presumed health reasons was stated by 251 (12%) participants.  Conclusions: Clinicians should be aware of the high frequency of food avoidance in the pediatric population due to true intolerance, functional diseases, or due to lifestyle reasons. Milk is the most avoided food and half of the affected patients avoid foods without guidance by professional dieticians, therefore risking possible negative impacts on their physical and mental health. Pediatricians should perform further investigations or advise nutritional guidance if an avoidance of foods or specific diets occurs. What is Known: • The prevalence of food intolerances in children and adolescents is unknown. • Eating behavior in children is influenced by the consumption of social media. What is New: • Based on this large cross-sectional study, it was shown that 16% of children and adolescents avoid foods, half of them without any medical advice. • Food avoidance is common among this Swiss cohort of children because of different reasons: lifestyle habits, functional diseases, true intolerances with possible consequences such as micronutrient deficiencies if not properly monitored.

Evolution of International Pediatric Endoscopic Practice Changes During the Coronavirus Disease 2019 Pandemic.

OBJECTIVES: The coronavirus disease 2019 (COVID-19) pandemic has drastically altered endoscopic practices. We initially reported the international impact of COVID-19 on pediatric endoscopic practice. This follow-up study aimed to assess changes 7 months following the initial survey to delineate practice change patterns as the pandemic evolved. METHODS: Pediatric gastroenterologists who responded to the initial survey were re-surveyed seven months later using Research Electronic Data Capture (REDCap). The survey recorded information on changes in pediatric endoscopic practice patterns, including COVID-19 screening and testing processes and personal protective equipment (PPE) utilization. Additionally, endoscopists' risk tolerance of COVID-19 transmission was evaluated. RESULTS: Seventy-five unique institutions from 21 countries completed surveys from the 145 initial responses (51.7% response rate). Procedural volumes increased at most institutions (70.7%) and most were performing previously postponed cases (90.7%). Ninety-seven percent of institutions were performing pre-endoscopy screening with 78.7% testing all patients. Many institutions (34.7%) have performed procedures on COVID-19 positive patients. There was significantly less PPE reuse (P  < 0.05) and fewer institutions recommending full PPE for all endoscopies (43.2% vs 59.2%, P = 0.013). Overall, pediatric endoscopists' risk tolerance of COVID-19 transmission is low. CONCLUSIONS: This is the first survey to highlight the evolution of pediatric endoscopic practices related to the COVID-19 pandemic, underscoring the need for ongoing pandemic-related guidance for pediatric endoscopic practice.

Training in Paediatric Clinical Nutrition Across Europe: A Survey of the National Societies Network (2016-2019) of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition.

OBJECTIVES/BACKGROUND: Disease-related malnutrition is common in patients with chronic diseases and has detrimental effects, therefore, skills in nutrition care are essential core competencies for paediatric digestive medicine. The aim of this survey, conducted as part of a global survey of paediatric gastroenterology, hepatology and nutrition (PGHN) training in Europe, was to assess nutrition care-related infrastructure, staff, and patient volumes in European PGHN training centres. METHODS: Standardized questionnaires related to clinical nutrition (CN) care were completed by representatives of European PGHN training centres between June 2016 and December 2019. RESULTS: One hundred training centres from 17 European countries, Turkey, and Israel participated in the survey. Dedicated CN clinics exist in 66% of the centres, with fulltime and part-time CN specialists in 66% and 42%, respectively. Home tube feeding (HTF) andhome parenteral nutrition (HPN) programmes are in place in 95% and 77% of centres, respectively. Twenty-four percent of centres do not have a dedicated dietitian and 55% do not have a dedicated pharmacist attached to the training centre. Even the largest centres with >5000 outpatients reported that 25% and 50%, respectively do not have a dedicated dietitian or pharmacist. Low patient numbers on HTF and HPN of <5 annually are reported by 13% and 43% of centres, respectively. CONCLUSIONS: The survey shows clear differences and deficits in Clinical Nutrition training infrastructure, including staff and patient volumes, in European PGHN training centres, leading to large differences and limitations in training opportunities in Clinical Nutrition.

Overview of the Pediatric Endoscopy Quality Improvement Network Quality Standards and Indicators for Pediatric Endoscopy: A Joint NASPGHAN/ESPGHAN Guideline.

INTRODUCTION: Pediatric-specific quality standards for endoscopy are needed to define best practices, while measurement of associated indicators is critical to guide quality improvement. The international Pediatric Endoscopy Quality Improvement Network (PEnQuIN) working group was assembled to develop and define quality standards and indicators for pediatric gastrointestinal endoscopic procedures through a rigorous guideline consensus process. METHODS: The Appraisal of Guidelines for REsearch and Evaluation (AGREE) II instrument guided PEnQuIN members, recruited from 31 centers of various practice types representing 11 countries, in generating and refining proposed quality standards and indicators. Consensus was sought via an iterative online Delphi process, and finalized at an in-person conference. Quality of evidence and strength of recommendations were rated according to the GRADE (Grading of Recommendation Assessment, Development, and Evaluation) approach. RESULTS: Forty-nine quality standards and 47 indicators reached consensus, encompassing pediatric endoscopy facilities, procedures, endoscopists, and the patient experience. The evidence base for PEnQuIN standards and indicators was largely adult-based and observational, and downgraded for indirectness, imprecision, and study limitations to "very low" quality, resulting in "conditional" recommendations for most standards (45/49). CONCLUSIONS: The PEnQuIN guideline development process establishes international agreement on clinically meaningful metrics that can be used to promote safety and quality in endoscopic care for children. Through PEnQuIN, pediatric endoscopists and endoscopy services now have a framework for auditing, providing feedback, and ultimately, benchmarking performance. Expansion of evidence and prospective validation of PEnQuIN standards and indicators as predictors of clinically relevant outcomes and high-quality pediatric endoscopic care is now a research priority.

Pediatric Endoscopy Quality Improvement Network Quality Standards and Indicators for Pediatric Endoscopy Facilities: A Joint NASPGHAN/ESPGHAN Guideline.

INTRODUCTION: There is increasing international recognition of the impact of variability in endoscopy facilities on procedural quality and outcomes. There is also growing precedent for assessing the quality of endoscopy facilities at regional and national levels by using standardized rating scales to identify opportunities for improvement. METHODS: With support from the North American and European Societies of Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN and ESPGHAN), an international working group of the Pediatric Endoscopy Quality Improvement Network (PEnQuIN) used the methodological strategy of the Appraisal of Guidelines for REsearch and Evaluation (AGREE) II instrument to develop standards and indicators relevant for assessing the quality of facilities where endoscopic care is provided to children. Consensus was reached via an iterative online Delphi process and subsequent in-person meeting. The quality of evidence and strength of recommendations were rated according to the GRADE (Grading of Recommendation Assessment, Development and Evaluation) approach. RESULTS: The PEnQuIN working group achieved consensus on 27 standards for facilities supporting pediatric endoscopy, as well 10 indicators that can be used to identify high-quality endoscopic care in children. These standards were subcategorized into three subdomains: Quality of Clinical Operations (15 standards, 5 indicators); Patient and Caregiver Experience (9 standards, 5 indicators); and Workforce (3 standards). DISCUSSION: The rigorous PEnQuIN process successfully yielded standards and indicators that can be used to universally guide and measure high-quality facilities for procedures around the world where endoscopy is performed in children. It also underscores the current paucity of evidence for pediatric endoscopic care processes, and the need for research into this clinical area.

Pediatric Endoscopy Quality Improvement Network Quality Standards and Indicators for Pediatric Endoscopic Procedures: A Joint NASPGHAN/ESPGHAN Guideline.

INTRODUCTION: High-quality pediatric gastrointestinal procedures are performed when clinically indicated and defined by their successful performance by skilled providers in a safe, comfortable, child-oriented, and expeditious manner. The process of pediatric endoscopy begins when a plan to perform the procedure is first made and ends when all appropriate patient follow-up has occurred. Procedure-related standards and indicators developed to date for endoscopy in adults emphasize cancer screening and are thus unsuitable for pediatric medicine. METHODS: With support from the North American and European Societies of Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN and ESPGHAN), an international working group of the Pediatric Endoscopy Quality Improvement Network (PEnQuIN) used the methodological strategy of the Appraisal of Guidelines for REsearch and Evaluation (AGREE) II instrument to develop standards and indicators relevant for assessing the quality of endoscopic procedures. Consensus was sought via an iterative online Delphi process and finalized at an in-person conference. The quality of evidence and strength of recommendations were rated according to the GRADE (Grading of Recommendation Assessment, Development, and Evaluation) approach. RESULTS: The PEnQuIN working group achieved consensus on 14 standards for pediatric endoscopic procedures, as well as 30 indicators that can be used to identify high-quality procedures. These were subcategorized into three subdomains: Preprocedural (3 standards, 7 indicators), Intraprocedural (8 standards, 18 indicators), and Postprocedural (3 standards, 5 indicators). A minimum target for the key indicator, "rate of adequate bowel preparation," was set at ≥80%. DISCUSSION: It is recommended that all facilities and individual providers performing pediatric endoscopy worldwide initiate and engage with the procedure-related standards and indicators developed by PEnQuIN to identify gaps in quality and drive improvement.

Pediatric Endoscopy Quality Improvement Network Quality Standards and Indicators for Pediatric Endoscopists and Endoscopists in Training: A Joint NASPGHAN/ESPGHAN Guideline.

INTRODUCTION: High-quality pediatric endoscopy requires reliable performance of procedures by competent individual providers who consistently uphold all standards determined to assure optimal patient outcomes. Establishing consensus expectations for ongoing monitoring and assessment of individual pediatric endoscopists is a method for confirming the highest possible quality of care for such procedures worldwide. We aim to provide guidance to define and measure quality of endoscopic care for children. METHODS: With support from the North American and European Societies of Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN and ESPGHAN), an international working group of the Pediatric Endoscopy Quality Improvement Network (PEnQuIN) used the methodological strategy of the Appraisal of Guidelines for REsearch and Evaluation (AGREE) II instrument to develop standards and indicators relevant for assessing the quality of endoscopists. Consensus was sought via an iterative online Delphi process and finalized at an in-person conference. The quality of evidence and strength of recommendations were rated according to the GRADE (Grading of Recommendation Assessment, Development, and Evaluation) approach. RESULTS: The PEnQuIN working group achieved consensus on 6 standards that all providers who perform pediatric endoscopy should uphold and 2 standards for pediatric endoscopists in training, with 7 corresponding indicators that can be used to identify high-quality endoscopists. Additionally, these can inform continuous quality improvement at the provider level. Minimum targets for defining high-quality pediatric ileocolonoscopy were set for 2 key indicators: cecal intubation rate (≥90%) and terminal ileal intubation rate (≥85%). DISCUSSION: It is recommended that all individual providers performing or training to perform pediatric endoscopy initiate and engage with these international endoscopist-related standards and indicators developed by PEnQuIN.

Pediatric Endoscopy Quality Improvement Network Pediatric Endoscopy Reporting Elements: A Joint NASPGHAN/ESPGHAN Guideline.

INTRODUCTION: High-quality procedure reports are a cornerstone of high-quality pediatric endoscopy as they ensure the clear communication of procedural events and outcomes, guide patient care and facilitate continuous quality improvement. The aim of this document is to outline standardized reporting elements that achieved international consensus as requirements for high-quality pediatric endoscopy procedure reports. METHODS: With support from the North American and European Societies of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN and ESPGHAN), an international working group of the Pediatric Endoscopy Quality Improvement Network (PEnQuIN) used Delphi methodology to identify key elements that should be found in all pediatric endoscopy reports. Item reduction was attained through iterative rounds of anonymized online voting using a 6-point scale. Responses were analyzed after each round and items were excluded from subsequent rounds if ≤50% of panelists rated them as 5 ("agree moderately") or 6 ("agree strongly"). Reporting elements that ≥70% of panelists rated as "agree moderately" or "agree strongly" were considered to have achieved consensus. RESULTS: Twenty-six PEnQuIN group members from 25 centers internationally rated 63 potential reporting elements that were generated from a systematic literature review and the Delphi panelists. The response rates were 100% for all three survey rounds. Thirty reporting elements reached consensus as essential for inclusion within a pediatric endoscopy report. DISCUSSION: It is recommended that the PEnQuIN Reporting Elements for pediatric endoscopy be universally employed across all endoscopists, procedures and facilities as a foundational means of ensuring high-quality endoscopy services, while facilitating quality improvement activities in pediatric endoscopy.

Percutaneous Endoscopic Gastrostomy in Children: An Update to the ESPGHAN Position Paper.

BACKGROUND: The European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) position paper from 2015 on percutaneous endoscopic gastrostomy (PEG) required updating in the light of recent clinical knowledge and data published in medical journals since 2014. METHODS: A systematic review of medical literature from 2014 to 2020 was carried out. Consensus on the content of the manuscript, including recommendations, was achieved by the authors through electronic and virtual means. The expert opinion of the authors is also expressed in the manuscript when there was a lack of good scientific evidence regarding PEGs in children in the literature. RESULTS: The authors recommend that the indication for a PEG be individualized, and that the decision for PEG insertion is arrived at by a multidisciplinary team (MDT) having considered all appropriate circumstances. Well timed enteral nutrition is optimal to treat faltering growth to avoid complications of malnutrition and body composition. Timing, device choice and method of insertion is dependent on the local expertise and after due consideration with the MDT and family. Major complications such as inadvertent bowel perforation should be avoided by attention to good technique and by ensuring the appropriate experience of the operating team. Feeding can be initiated as early as 3 hours after tube placement in a stable child with iso-osmolar feeds of standard polymeric formula. Low-profile devices can be inserted initially using the single-stage procedure or after 2-3 months by replacing a standard PEG tube, in those requiring longer-term feeding. Having had a period of non-use and reliance upon oral intake for growth and weight gain-typically 8-12 weeks-a PEG may then safely be removed after due consultation. In the event of non-closure of the fistula the most successful method for closing it, to date, has been a surgical procedure, but the Over-The-Scope-Clip (OTSC) has recently been used with considerable success in this scenario. CONCLUSIONS: A multidisciplinary approach is mandatory for the best possible treatment of children with PEGs. Morbidity and mortality are minimized through team decisions on indications for insertion, adequate planning and preparation before the procedure, subsequent monitoring of patients, timing of the change to low-profile devices, management of any complications, and optimal timing of removal of the PEG.

Blastocystis in Swiss children: a practical approach.

Blastocystis is a parasite with a worldwide distribution and a varying prevalence in different countries. The pleomorphic nature of the protozoon and the lack of understanding a possible pathogenesis have led to confusion regarding its clinical significance. The aim of the study was to shed light on clinical characteristics of pediatric patients in Swiss children with a positive stool sample for Blastocystis, in order to provide recommendations for a practical approach for the clinician to know whom, when, and how to test. This is a retrospective study of pediatric patients, whose stool has been tested positive for Blastocystis in the last 10 years in northern Switzerland. A total of 4047 stool samples, belonging to 1887 different patients, were analyzed; 240 stool samples (of 160 patients) were tested positive for Blastocystis. On average, 2.2 (CI 1.98-2.35) stool samples per patient were analyzed, of which 1.48 (CI 1.36-1.61) were positive for Blastocystis. In 63% abdominal pain was the leading symptom, while in 17.5% it was an accidental finding without symptoms. There was a high significance in correlation of abdominal pain and chronicity (p < 0.0001) but none in diarrhea (p = 0.082) nor nausea/vomiting or other symptoms and chronicity. Followed by Entamoeba coli (8%), 26.3% of the patients with Blastocystis had a co-infection with another parasite, mostly Endolimax nana (13%).Conclusion: Carriage of Blastocystis is common; therefore, only children/teenagers at risk for a symptomatic Blastocystis infection should be tested. There is a good correlation between Blastocystis and chronic abdominal pain. Children with abdominal symptoms persisting over 4 weeks should have two different stool samples analyzed. No screening after travels/immigration is recommended.What is Known:• Blastocystis has a worldwide distribution.• The clinical significance is unclear.What is New:• Based on retrospective data, we recommend to only test children/teenagers with chronic abdominal pain for Blastocystis.• Two different stool samples should be examined by microscopy; serological investigations are not warranted.

Favorable impact of long-term exercise on disease symptoms in pediatric patients with inflammatory bowel disease.

BACKGROUND: Evidence is growing that both short- and long-term physical exercise have the potential to positively impact on the physiological system related to inflammatory indices, though, such patterns are unknown for pediatric patients with Inflammatory Bowel Disease (IBD). The aim of the present intervention study was to investigate the influence of a single bout and chronic moderate-intensity exercise on IBD-related inflammatory indices and exercise capacity among pediatric individuals with IBD and healthy controls. METHOD: Twenty-one pediatric patients with IBD, split into a "remission-group" (IBD-RE; n = 14) and an "active disease group" (IBD-AD; n = 7), were compared to 23 age matched healthy controls (HC). All participants completed a single bout of exercise at baseline and an 8-week exercise intervention. Before and after the single bout of exercise IBD-related inflammatory indices (erythrocyte sedimentation rate (ESR), albumin, C-reactive protein (CRP), cortisol, hemoglobin, hematocrit, thrombocytes and leukocytes) were assessed. RESULTS: At baseline, after a single bout of exercise, inflammation (albumin, hemoglobin, erythrocytes, hematocrit and leukocytes) increased in all three groups IBD-AD, IBD-RE and HC. CRP and thrombocytes were only elevated in IBD-AD and IBD-RE, compared to HC. After a longer-term exercise intervention, ESR, CRP and thrombocytes significantly decreased in all groups. The longer-term exercise intervention did not decrease acute immunopathologic responses after a single bout of exercise, compared to baseline. CONCLUSION: Whereas a single bout of exercise increases albumin, erythrocytes and leukocytes, longer-term moderate-intensity exercise reduced inflammatory markers in pediatric patients with IBD. Children and teenagers with IBD should be encouraged to engage in regular moderate-intensity exercise activities, as such activities may contribute to inflammation suppression and improved disease management.

Pediatric gastrointestinal endoscopy: European Society of Gastrointestinal Endoscopy (ESGE) and European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) Guideline Executive summary.

This Executive summary of the Guideline on pediatric gastrointestinal endoscopy from the European Society of Gastrointestinal Endoscopy (ESGE) and the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) refers to infants, children, and adolescents aged 0 - 18 years. The areas covered include: indications for diagnostic and therapeutic esophagogastroduodenoscopy and ileocolonoscopy; endoscopy for foreign body ingestion; endoscopic management of corrosive ingestion and stricture/stenosis; upper and lower gastrointestinal bleeding; endoscopic retrograde cholangiopancreatography, and endoscopic ultrasonography. Percutaneous endoscopic gastrostomy and endoscopy specific to inflammatory bowel disease (IBD) have been dealt with in other Guidelines and are therefore not mentioned in this Guideline. Training and ongoing skill maintenance will be addressed in an imminent sister publication.

Psychological wellbeing and physical activity in children and adolescents with inflammatory bowel disease compared to healthy controls.

BACKGROUND: Children and adolescents with inflammatory bowel disease (IBD) report impairments in daily activities, social interactions and coping. Findings regarding psychological functioning are inconsistent, while limited information is available on objectively assessed physical activity (PA). The aims of the present study were therefore to compare anthropometric dimensions, blood values, psychological functioning and PA of children and adolescents with IBD with healthy controls. METHODS: Forty-seven children and adolescents took part in the study. Of these, 23 were diagnosed with IBD (mean age: 13.88 years, 44% females). The IBD group was divided into a medically well adjusted "remission-group" (n = 14; IBD-RE) and a group with an "active state" of disease (n = 8; IBD-AD). Healthy controls (n = 24; HC) were age- and gender-matched. Participants' anthropometric data, blood values and objective PA were assessed. Further, participants completed questionnaires covering socio-demographic data and psychological functioning. RESULTS: Participants with IBD-AD showed higher erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) values, haemoglobin, and leukocyte values. IBD-AD had poorer psychological functioning and lower PA (average steps per day) compared to IBD-RE and HC. No mean differences were found between IBD-RE and HC. CONCLUSIONS: The pattern of results suggests that effective medical treatment of IBD in children and adolescents is associated with favorable physiological parameters, psychological dimensions and PA. Psychological counselling of children and adolescents in an active state of IBD seem to be advised in addition to standard treatment schedules. TRIAL REGISTRATION: NCT NCT02264275 ; Registered 8 October 2014.

Extraintestinal Manifestations of Pediatric Inflammatory Bowel Disease: Prevalence, Presentation, and Anti-TNF Treatment.

BACKGROUND: There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD). METHODS: Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively. RESULTS: A total of 55 patients (16.7%) experienced 1-4 EIM (39 Crohn disease, 12 ulcerative colitis, and 4 IBD-unclassified patients). At IBD onset, presence of EIM was more frequent than in the adult population (8.5% vs 5.0%, P = 0.014). EIM were more frequent in Crohn disease when compared to ulcerative colitis/IBD-unclassified (22.5% vs 10.3%, P = 0.003). The most prevalent EIM were peripheral arthritis (26/329, 7.9%) and aphthous stomatitis (24/329, 7.3%). Approximately 27.6% of all EIM appeared before IBD diagnosis. Median time between IBD diagnosis and occurrence of first EIM was 1 month (-37.5-149.0). Thirty-one of the 55 patients (56.4%) were treated with 1 or more anti-tumor necrosis factor (TNF) agents. IBD patients with EIM were more likely to be treated with anti-TNF compared to those without (56.4% vs 35.0%, P = 0.003). Response rates to anti-TNF depended on underlying EIM and were best for peripheral arthritis (61.5%) and uveitis (66.7%). CONCLUSIONS: In a cohort of pediatric patients with IBD, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings may translate into an increased awareness of underlying IBD, thereby decreasing diagnostic delay. Anti-TNF for the treatment of certain EIM is effective, although a substantial proportion of new EIM may present despite ongoing anti-TNF therapy.