Background & Aims Second victim syndrome (SVS) describes the emotional distress and psychological burden healthcare professionals face after an unexpected procedural adverse event, impacting providers' wellness and practice. We evaluate the prevalence and impact of SVS in pediatric gastroenterology. Methods We distributed the validated electronic Second Victim Experience and Support Tool (SVEST) to faculty and fellows at our institution to analyze SVS prevalence and its impact. Results A 72.7% (fellow, n=11) and 81.3% (faculty, n=32) response rate was noted. 97.1% experienced an adverse event. 82.4% experienced psychological distress and 44.1% experienced physical distress. Half noted a detrimental impact on self-efficacy, particularly among fellows and junior faculty. 14.7% felt adequately supported, and 20.6% considered quitting. 85.3% desired additional support. Conclusions A high prevalence of SVS is noted in pediatric gastroenterology. Addressing SVS requires creating a supportive environment to help healthcare professionals cope, warranting further multi-institutional studies to develop effective interventions.

We describe biochemically and functionally validated primary bile acid malabsorption caused by novel biallelic SLC10A2 variants in a child initially diagnosed with Crohn's disease. Pediatric IBD cohort reanalysis identified PBAM-compatible genotypes, supporting selective testing when clinical features are suggestive.

BACKGROUND:Point-of-care intestinal ultrasound (IUS) is a non-invasive tool to evaluate inflammation in patients with inflammatory bowel disease (IBD). Limited studies have evaluated the role of IUS during routine clinical care. This study investigated the addition of IUS as part of routine clinic visits compared to standardized indices collected in real-world care to evaluate the role of IUS as a treat-to-target measure. METHODS:This cross-sectional study included pediatric patients (<18-years-old) with IBD who underwent IUS as part of clinical care. The primary outcome compared the accuracy of IUS with clinical indices versus clinical indices alone to predict biochemical disease activity using receiver operating characteristics. Secondary outcomes evaluated differences in bowel wall thickness (BWT) based on biochemical disease activity and level of clinical severity. RESULTS:The study included 92 patients with 136 IUS exams. The addition of IUS markers to clinical parameters improved prediction of biochemical activity in CD (AUC 0.71 versus 0.90; P = .004) and trended toward improvement in UC (AUC 0.83 versus 0.92; P = .067). Patients with active disease had higher BWT than those with quiescent disease. Median BWT for FCP activity was 4.2 mm (IQR: 2.7-5.0 mm) versus 2.0 mm (IQR: 1.6-2.7 mm; P < .001) for FCP remission (≤250µg/g). Optimal BWT thresholds to predict FCP > 250 ranged between 2.3-2.5 mm, based on disease phenotype. DISCUSSION:Integrating IUS with clinical symptoms during routine clinic visits was superior to shPCDAI alone in predicting CD activity, and may potentially be superior to PUCAI for UC. Incorporating IUS into routine visits may accelerate treatment decisions, thereby advancing an improved point-of-care treat-to-target approach.

Avoidant/Restrictive Food Intake Disorder (ARFID) is characterized by inadequate intake due to low appetite, sensory-based avoidance, or fear of aversive consequences resulting in weight loss or growth faltering, nutrient deficiencies, supplement dependence, or psychosocial impairment. Estimated prevalence is 5.9% in adults and 4.7% in children, raising concern for nutritional and psychosocial impact. Inflammatory Bowel Disease (IBD) causes chronic GI inflammation, often prompting dietary modification to avoid symptoms. Although disordered eating was found to be elevated in youth with IBD, ARFID has not been systematically studied in pediatric IBD. Adult IBD studies report prevalence of 10–17%, underscoring the need to characterize ARFID in pediatric IBD. Building on our prior work identifying ARFID symptom prevalence in youth with IBD, we conducted a mixed-methods study in this cohort to (1) examine associations with psychological functioning and (2) explore lived experiences of affected youth reporting ARFID symptoms. Phase 1 was a cross-sectional study of patients with IBD (8–25 yrs) using validated instruments (EDY-Q; NIAS+EDE-Q) to screen for ARFID symptoms and psychosocial function (PROMIS Anxiety, Depressive Symptoms, and Global Health). Associations between ARFID symptoms and participant clinical/psychosocial variables were assessed using regression models. Phase 2 involved semi-structured interviews with a purposive subsample screening positive for ARFID, exploring food-related experiences and psychosocial context. Data were analyzed thematically. Phase 1 included 100 participants (50% female; 63% White; 61% Crohn’s disease). ARFID symptoms were identified in 28% and were significantly associated with lower PROMIS Global Health T-scores (adjusted difference -4.6, 95% CI: -8.4 to -0.75, p < 0.05), indicating poorer overall health. Phase 2 included five youth (ages 11–18; 60% male, 60% Crohn’s disease). Themes revealed sensory aversions, fear of symptom exacerbation, and protective eating behaviors reinforcing avoidance and mealtime anxiety. Youth described social withdrawal and feelings of difference, reflecting psychosocial burden. Family dynamics shaped eating behaviors, sometimes buffering and/or intensifying distress. Youth reported strategies such as carrying “safe” foods and adjusting meal timing, demonstrating both resilience and the effort required to manage ARFID within IBD. This mixed-methods study demonstrates the psychosocial burden of ARFID symptoms in youth with IBD and the need for early identification and more support. Routine ARFID screening may facilitate timely referrals to nutritional, psychological, and behavioral support. Interventions should target mealtime anxiety, sensory triggers, and family involvement while promoting adaptive eating behaviors to improve quality of life.

Inflammatory bowel disease (IBD) in children is associated with chronic gastrointestinal inflammation and increased psychosocial burden. Avoidant/Restrictive Food Intake Disorder (ARFID) is a disordered eating behavior without body image concerns characterized by inadequate intake due to low appetite, sensory-based avoidance, or fear of aversive consequences resulting in weight loss or growth faltering, nutrient deficiencies, supplement dependence, or psychosocial impairment. Data on the prevalence and clinical correlates of ARFID in pediatric IBD remain limited. Building on prior work identifying ARFID prevalence in this population, we conducted a retrospective chart review to examine clinical associations of patients with ARFID symptoms. (1) Assess associations between ARFID symptoms, disease duration, and IBD phenotype; (2) Evaluate clinical and biochemical disease activity and nutritional status; (3) Examine utilization of multidisciplinary resources in affected youth. We conducted a single-center, cross-sectional retrospective study of pediatric and young adult patients (ages 8–25 years) with IBD who screened positive for ARFID. Screening tools included the EDY-Q for ages 8–13 and the NIAS and EDE-Q for ages 14–25. Clinical data included disease activity indices short Pediatric Crohn’s Disease Activity Index (sPCDAI), Pediatric Ulcerative Colitis Activity Index (PUCAI), and Physician Global Assessment (PGA), phenotype classification (PARIS), and biochemical markers (calprotectin, ESR, CRP). Nutritional parameters assessed included nutritional status, hemoglobin, ferritin, vitamin D, and vitamin B12 levels. Utilization of multidisciplinary care was evaluated by documenting dietitian and psychologist visits within ±6 months of survey completion. Twenty-seven of 100 patients screened positive for ARFID risk: Crohn’s disease (59%), ulcerative colitis (33%), and IBD-unclassified (7%). Most Crohn’s patients had uncomplicated ileocolonic disease, while UC patients frequently had severe pancolitis. Two-thirds had disease duration >4 years. Nutritional failure was present in 33%, anemia in 30%, and vitamin D deficiency in 25%. While 62% were in clinical remission based on sPCDAI/PUCAI and 77% by PGA, Calprotectin was elevated in 63%; ESR and/or CRP in 40%. All patients saw a dietitian; most saw a psychologist. ARFID risk behaviors were identified in a substantial subset of pediatric IBD patients, even among those in clinical remission and without overt nutritional failure. This highlights the need for heightened clinical vigilance. Multidisciplinary care—including early involvement of dietitians and psychologists—appears essential for comprehensive assessment and management.

The incidence of inflammatory bowel disease (IBD) has significantly increased in developing countries over the last decade with a rising prevalence among the pediatric population. Very early-onset IBD (VEOIBD), defined as IBD in children younger than 6 years, requires a comprehensive diagnostic approach including clinical history, physical examination, laboratory tests, endoscopy, and genetic evaluation. Literature indicates that 8–20% of pediatric patients diagnosed with VEOIBD have identifiable genetic causes, often involving monogenic mutations related to inborn errors of immunity (IEI), namely those that play a role in intestinal barrier function, innate and adaptive immunity, and autoinflammatory disorders. Identifying these mutations can offer insights into potential therapeutic targets. This study examines our cohort of VEOIBD and early-onset IBD patients referred to the Stanford immunology clinic, with a focus on the prevalence of monogenic diseases and their clinical implications. Among the 31 pediatric patients referred for immunologic evaluation at Stanford, targeted genetic testing for primary immune deficiency revealed pathogenic mutations in 7 patients (22%). These included mutations in DUOX2, NOD2, CTLA4 (2), CARD11, and NEMO (Table I). The median age of IBD onset was 6.25 years, with a slight female predominance. Crohn’s disease was more prevalent than ulcerative colitis in this VEOIBD cohort. As a note, one 11-year-old female diagnosed with Crohn’s was found to be a carrier for LRBA, inherited from her asymptomatic mother. Patients with identified monogenic causes showed significant improvement with targeted therapies, such as abatacept for CTLA-4 haploinsufficiency. Table 1.PatientGenderAge of Onset IBD-like Symptoms (year)UC/Crohn’sPathogenic MutationAMale1Crohn'sDUOX2BMale1.5GVHD vs NEMO colitis/Crohn'sNEMOCFemale11CombinedCTLA4DFemale1Crohn'sCTLA4EFemale11Crohn'sNOD2FMale6-6.5UCAS20GFemale11Crohn'sNOD2Other:HFemale11Crohn'sCarrier for LRBA Predicting which patients have an underlying monogenic disease remains challenging, emphasizing the importance of genetic evaluation. Genetic screening plays a crucial role in the management of VEOIBD, providing insights and guiding treatment decision. Targeted therapies should be considered whenever available. The effective management of these complex conditions relies on multidisciplinary teams, underscoring their pivotal role in comprehensive care.

Inflammatory Bowel Disease (IBD) is a chronic inflammation of the gut, where symptoms are perceived to be influenced by diet. Avoidant/Restrictive Food Intake Disorder (ARFID) in IBD is driven by fear of pain or diarrhea, sensory sensitivity or lack of interest in eating. This can lead to malnutrition and emotional distress, compromising overall health and disease management. The prevalence of ARFID in the general population is considered to be 2-3% and was found to be more common in adult IBD patients (10-17%); however, its occurrence in pediatric populations remains poorly understood, revealing a critical knowledge gap. While there is an increased prevalence of eating disorders (ED) in adolescents with IBD, studies reporting ARFID prevalence in this demographic are lacking. This study aims to assess the prevalence of ARFID in youth with IBD, providing data to support clinical screening guidelines to enhance patient outcomes. A cross-sectional cohort study was conducted at Stanford Medicine Children’s Health with patients aged 8-25 with IBD. Participants completed the Nine-Item ARFID Screen (NIAS)/Youth-NIAS (Y-NIAS) and Eating Disorder Examination-Questionnaire (EDE-Q) via Stanford REDCap (Table 1). NIAS and Y-NIAS scores were consolidated into a single score. NIAS subscales were considered positive at or above the following cutoffs: NIAS-picky eating ≥10, NIAS-appetite ≥9, and/or NIAS-fear ≥10. EDs were ruled out with an EDE-Q < 2.3. ARFID prevalence was considered using two methods: 1) a NIAS cutoff of ≥24 and 2) for patients aged 14-25 with EDE-Q < 2.3 and at least one positive NIAS subscale. Patient characteristics were collected from surveys and medical chart reviews, with descriptive statistics reported using R version 4.3. The cohort included 88 participants: 47% aged 18-25, 53% female, 66% White/European, 63% with Crohn’s disease (CD), and a median disease duration of 4 years (IQR 2-7 years). Over half (56%) reported a history of dietary modification, including Specific Carbohydrate Diet (24%), CD Exclusion Diet (10%), Dairy-Free (25%), and Gluten-Free (24%). The prevalence of ARFID was 15% based on NIAS scores (Table 2), with at least one positive NIAS subscale noted in 35% of participants. Lastly, among those aged 14-25, 26% screened positive for ARFID. ARFID prevalence in our pediatric IBD population aligns with adult IBD findings. Literature suggests that a positive score on the picky eating subscale (25%) distinguishes ARFID from other ED. This subscale was most prevalent among patients aged 8-25, a trend persistent even after adjusting for other ED in the 14-25 age group. Further analyses will explore patient well-being measures and conduct semi-structured interviews to assess the influences of eating behaviors on mental health and quality of life. Table 1. Validated Instruments Table 2. NIAS and EDE-Q Scores*NIAS/Y-NIAS were compiled into a single NIAS score†Denominator corresponds to the number of participants aged 14-25 (n=61)

A significant complication of Crohn's disease (CD) is intestinal fibrosis, which narrows the bowel lumen to form a stricture. Creeping fat (CF) is the wrapping of mesenteric adipose tissue around diseased bowel, of which the role in CD stricture progression is unclear. By constructing a human single-cell CD fibroblast atlas, we identified CF-derived, CTHRC1+ fibroblasts enriched for Yes-associated protein (YAP)/transcriptional co-activator with PDZ-binding motif (TAZ) signatures and localized to a fibrotic CF-bowel wall interface within the stricture. We further showed that analogous Cthrc1+ mouse fibroblasts derive from mesenteric adipose tissue stromal cells, infiltrate fibrotic bowel, and deposit extracellular matrix in a YAP/TAZ-dependent manner in a mouse model of intestinal fibrosis. Our findings identify CF as a key source of pro-fibrotic fibroblasts and raise the possibility of improving future clinical management of stricture progression by targeting not only the bowel but also CF.

BACKGROUND:Disseminated mycobacterium poses a significant risk for patients with NEMO deficiency. Hematopoietic stem cell transplant (HSCT) corrects the NEMO defect in hematopoietic cells thus treating the immunodeficiency. METHODS:We present a patient with NEMO deficiency who successfully underwent HSCT despite a disseminated Mycobacterium szulgai infection. RESULTS:Despite successful engraftment and resolution of the mycobacterium infection, he developed inflammatory disease leading to his death. CONCLUSION:HSCT does not cure all aspects of the NEMO protein defect and posttransplant inflammatory diseases involving nonhematopoietic cell types may manifest clinically.

Introduction: Pediatric acute-onset neuropsychiatric syndrome (PANS) is an immune-mediated disease characterized by abrupt onset neurobehavioral changes. Inflammatory bowel disease (IBD) includes ulcerative colitis (UC) and Crohn's disease (CD), chronic conditions characterized by gastrointestinal inflammation. We describe eight individuals with both PANS and IBD. Methods: All individuals with both IBD and PANS were identified from Stanford Immune Behavioral Health Clinic, Cedars-Sinai Medical Center Pediatric Inflammatory Bowel Disease Program, and Dartmouth Neuroimmune Psychiatric Disorders (NIPD) Clinic. Data were collected by chart review. Results: Eight cases of PANS with IBD were identified. Five were male. The mean age of onset was 9.3 years for PANS and 15.6 years for IBD. PANS preceded development of IBD in 7 of 8 cases by a mean of 8.4 years. Seven patients (88%) had a first-degree relative with an immune-mediated disease, including 5 with psoriasis or psoriatic arthritis. Five patients themselves had arthralgias or arthritis (63%). All 5 cases where PANS preceded IBD treatment sufficiently for analysis were free of major behavioral relapses after IBD was managed. Conclusion: The triad of PANS, joint complaints, and family history of autoimmunity, including psoriasis, may represent a subset of PANS at heightened risk for IBD and additional immune-mediated disorders. For children with this triad, clinicians should have a low threshold to evaluate for gastrointestinal inflammation with biomarkers like hemoglobin, CRP, fecal calprotectin, and diagnostic endoscopy when indicated. PANS symptoms may improve with effective treatment of IBD. The high prevalence of joint complaints in our cohort and psoriasis in first-degree family members suggests this subset of PANS may share immune mechanisms with psoriasis and arthritis. Treatment strategies used in IBD and arthritis should be studied for potential application in PANS.

Abstract BACKGROUND Infliximab (IFX) is the only FDA-approved intravenous therapy for the treatment of pediatric inflammatory bowel disease (IBD). The gut-selective anti-integrin vedolizumab (VDZ) has been increasingly used as second-line therapy. However, the comparative effectiveness of IFX vs. VDZ to treat biologic-naïve children with IBD is currently unknown. AIMS 1. Compare the real-world effectiveness of IFX vs. VDZ in bio-naïve pediatric patients with IBD at 12 months. 2. Study the safety of IFX and VDZ in the pediatric population. METHODS A single-center retrospective cohort study was conducted at an academic children’s hospital between February 2015 and August 2023. We included bio-naive mild-moderate IBD patients, <23 years old, who started IFX or VDZ as their first advanced therapy. Patients with a confirmed diagnosis of IBD based on Porto criteria with baseline and at least 6-month follow-up data were included. Exclusion criteria were designed to limit the study population to patients who would typically be eligible for vedolizumab treatment according to the current standard of care, including: prior biologic use; steroid-refractory acute-severe colitis; stricturing or penetrating Crohn’s disease (CD); or perianal disease; and extraintestinal manifestations. The primary outcome was steroid-free clinical remission (SFCR) at 12 months, defined by the PUCAI <10 or ShPCDAI <10 and off corticosteroids. Biochemical remission (BR) was defined as fecal calprotectin (FCP) <250 μg/g. Descriptive statistics included the Chi-square and the Mann-Whitney U test. RESULTS Among the 100 included patients, 73 in IFX group vs. 27 in VDZ group, the mean age was 13 ± 4 years old, males were 68% (IFX) vs. 41% (VDZ) (P = 0.01). 49% had CD, 48% had UC, and 3% had unclassified IBD (IBDU). IFX- and VDZ-treated patients had similar Paris classifications. Baseline albumin levels (3.7 vs. 4.2 g/L, P<0.01) and steroid use (58% vs. 74%, P = 0.13) were lower in IFX vs. VDZ-treated patients. 12-month SFCR was 72% (49/68) and 71% (15/21) in IFX- and VDZ-treated patients, respectively (NS). Among SFCR at 12 months, BR was 59.62% (IFX) vs. 76.47% (VDZ), P = 0.21. Adverse events were rated as mild to moderate and occurred rarely in 8.22% (6/73) of IFX patients and in 7.41% (2/27) of VDZ patients (P = 0.89). These included an infusion reaction (1 in each group), elevated liver enzymes (1 in each group), infection (1), folliculitis (1), eczema (1), , and psoriasis (1). CONCLUSION We found both IFX and VDZ to have similar effectiveness in achieving SFCR and BR at 12 months when used as first-line agents for patients with uncomplicated mild-moderately active IBD. Prospective, larger head-to-head studies are needed for further validation. IFX group vs. VDZ group at the baseline. IFX group vs. VDZ group for Steroid Free Clinical Remission Patients at 12 months.