Tumor necrosis factor receptor-associated factor 3 (TRAF3) is an anti-inflammatory molecule that negatively regulates the non-canonical nuclear factor-κB pathway. Although TRAF3 haploinsufficiency (TRAF3 HI) can influence innate and adaptive immune cells, its effect on inflammatory bowel disease (IBD) development remains unclear. Here, we report the first case of severe early-onset IBD with a novel TRAF3 variant leading to HI, successfully treated with ustekinumab. A 6-year-old girl with a recurrent parotitis, otitis media, tonsilitis, and atopic dermatitis developed IBD involving the stomach, small intestine, and colon. At diagnosis, the immunoglobulin (Ig)G and IgA levels were relatively high, and lymphocyte subsets showed increased counts of plasmablasts, class-switch recombination B cells, and circulating T-follicular helper cells. Treatment with azathioprine and infliximab failed to maintain remission marked by several relapses accompanied by erythema nodosum and arthritis; however, ustekinumab, an anti-interleukin (IL)-12/23p40 antibody, led to long-term clinical remission, normalizing the Ig level and reducing abnormal lymphocyte counts. Whole-exome sequencing revealed a novel heterozygous mutation in TRAF3 [p.(Pro487Leufs*8)], resulting in TRAF3 under-expression. Our case may highlight the contribution of TRAF3 HI to the development of IBD and provide insights into IBD pathophysiology, suggesting the involvement of the IL-12/23-T-follicular helper cell pathway affected by genetic mutations.

Background/Aims: The incidence of perianal lesions (PL) in children with Crohn’s disease (CD) is higher in East Asia than in Western countries. Early intervention for PL is essential to prevent sphincter dysfunction and ostomy placement. In this study, we aimed to investigate the clinical features, treatment, and consequences of pediatric CD with PL. Methods: We retrospectively reviewed a cohort of children diagnosed with CD from 2010 to 2020 at a Japanese children’s hospital. Demographics, treatments, and outcomes were evaluated and compared among subgroups. Results: Among 112 pediatric patients with CD, 36 (32.1%) had experienced PL during the observational period. The median ages at diagnosis and follow-up periods were 131 and 70 months, respectively. Six (85.7%) patients in the very early-onset (VEO) group (CD diagnosed before 6 years old) and 24 (82.8%) in the older age group had PL upon diagnosis of CD (P= 0.851). Biologics were given to 94.4% of patients: infliximab (67.7%), adalimumab (58.8%), ustekinumab (44.1%), risankizumab (11.8%), and vedolizumab (5.9%). Biologics were introduced within 1 year in 89.5% and 40.0% of patients diagnosed in 2016–2020 and 2010–2016, respectively (P= 0.002). Seton was frequently used in the older age group (87.5 vs. 42.9%, P= 0.190). Ostomy was frequently required in the VEO group (42.9% vs. 0.0%, P= 0.006). Conclusions Patients with VEO-CD and PL had a notably high risk of ostomy placement. The earlier introduction of biologics and surgical interventions reduced corticosteroids use and ostomy placement in pediatric CD patients with PL.

Background/Aims: Mothers of children with very-early-onset inflammatory bowel disease (VEO-IBD) face unique challenges; however, these challenges and their consequences have not been well described. This study clarified the experiences and processes of empowerment of mothers of children with VEO-IBD. Methods: This study performed a qualitative inductive analysis using semi-structured interviews. The interview content was transcribed, generating core categories, categories, and subcategories with a focus on mothers raising children with VEO-IBD. A modified grounded theory approach was employed to inductively construct a theory from the qualitative data. Results: Fifteen mothers of children with VEO-IBD were interviewed (mean age, 43.9 ± 6.2 years). The modified grounded theory approach revealed the processes experienced by the mothers. The mothers faced various difficulties when their children developed VEO-IBD; however, their efforts to cope with these difficulties changed their situation. Furthermore, they were supported by various individuals, including family members, medical personnel, and, occasionally, families of other children with VEO-IBD. These processes strengthened and empowered the mothers. Conclusions Mothers of children with VEO-IBD who faced various difficulties were empowered through their efforts and support from family and others who understood their challenges. This process of empowerment continues throughout the development of children with VEO-IBD.

Purpose: Pediatric gastrointestinal (GI) endoscopy significantly contributes to the diagnosis and management of GI diseases in children. Global data on pediatric GI endoscopy in the Asia-Pacific region are limited. We aimed to report the findings of a regional survey on pediatric endoscopy in the Asia-Pacific region. Methods: A questionnaire-based survey involving GI endoscopy centers in 13 Asia-Pacific countries (June to November 2021). The questionnaires included annual procedure volumes (from basic diagnostics to advanced therapeutic endoscopy), endoscopists, sedation procedures, and national training programs. Results: A total of 162 GI endoscopy centers completed the survey. All centers performed basic endoscopies (esophagogastroduodenoscopy and ileocolonoscopy); however, 45.1% and 59.1% of the centers performed less than 50 esophagogastroduodenoscopies and ileocolonoscopies per year, respectively. Small bowel evaluation (capsule endoscopy or balloon-assisted enteroscopy) was performed in 59.3% of the centers. Foreign body removal, polypectomy, and percutaneous endoscopic gastrostomy were performed in 89.5%, 85.8%, and 52.5% of centers, respectively. Endoscopic hemostatic interventions, which are lifesaving procedures, included glue injection (30.9%), hemostasis of nonvariceal bleeding (65.4%), and endoscopic variceal ligation (70.4%). Pediatric GI endoscopy is performed not only by pediatric gastroenterologists but also by adult gastroenterologists in 21–50% of centers for many kinds of procedures. Sedation was provided by anesthesiologists in 65.4% of the centers. Most centers offer both adult and pediatric endoscopy training. Conclusion The study highlights regional disparities in pediatric GI endoscopy services. It emphasizes the need for expanded pediatric GI training and improved access to therapeutic endoscopy, particularly for life-saving procedures.

Purpose: Pediatric gastrointestinal (GI) endoscopy significantly contributes to the diagnosis and management of GI diseases in children. Global data on pediatric GI endoscopy in the Asia-Pacific region are limited. We aimed to report the findings of a regional survey on pediatric endoscopy in the Asia-Pacific region. Methods: A questionnaire-based survey involving GI endoscopy centers in 13 Asia-Pacific countries (June to November 2021). The questionnaires included annual procedure volumes (from basic diagnostics to advanced therapeutic endoscopy), endoscopists, sedation procedures, and national training programs. Results: A total of 162 GI endoscopy centers completed the survey. All centers performed basic endoscopies (esophagogastroduodenoscopy and ileocolonoscopy); however, 45.1% and 59.1% of the centers performed less than 50 esophagogastroduodenoscopies and ileocolonoscopies per year, respectively. Small bowel evaluation (capsule endoscopy or balloon-assisted enteroscopy) was performed in 59.3% of the centers. Foreign body removal, polypectomy, and percutaneous endoscopic gastrostomy were performed in 89.5%, 85.8%, and 52.5% of centers, respectively. Endoscopic hemostatic interventions, which are lifesaving procedures, included glue injection (30.9%), hemostasis of nonvariceal bleeding (65.4%), and endoscopic variceal ligation (70.4%). Pediatric GI endoscopy is performed not only by pediatric gastroenterologists but also by adult gastroenterologists in 21–50% of centers for many kinds of procedures. Sedation was provided by anesthesiologists in 65.4% of the centers. Most centers offer both adult and pediatric endoscopy training. Conclusion The study highlights regional disparities in pediatric GI endoscopy services. It emphasizes the need for expanded pediatric GI training and improved access to therapeutic endoscopy, particularly for life-saving procedures.

Purpose: Pediatric gastrointestinal (GI) endoscopy significantly contributes to the diagnosis and management of GI diseases in children. Global data on pediatric GI endoscopy in the Asia-Pacific region are limited. We aimed to report the findings of a regional survey on pediatric endoscopy in the Asia-Pacific region. Methods: A questionnaire-based survey involving GI endoscopy centers in 13 Asia-Pacific countries (June to November 2021). The questionnaires included annual procedure volumes (from basic diagnostics to advanced therapeutic endoscopy), endoscopists, sedation procedures, and national training programs. Results: A total of 162 GI endoscopy centers completed the survey. All centers performed basic endoscopies (esophagogastroduodenoscopy and ileocolonoscopy); however, 45.1% and 59.1% of the centers performed less than 50 esophagogastroduodenoscopies and ileocolonoscopies per year, respectively. Small bowel evaluation (capsule endoscopy or balloon-assisted enteroscopy) was performed in 59.3% of the centers. Foreign body removal, polypectomy, and percutaneous endoscopic gastrostomy were performed in 89.5%, 85.8%, and 52.5% of centers, respectively. Endoscopic hemostatic interventions, which are lifesaving procedures, included glue injection (30.9%), hemostasis of nonvariceal bleeding (65.4%), and endoscopic variceal ligation (70.4%). Pediatric GI endoscopy is performed not only by pediatric gastroenterologists but also by adult gastroenterologists in 21–50% of centers for many kinds of procedures. Sedation was provided by anesthesiologists in 65.4% of the centers. Most centers offer both adult and pediatric endoscopy training. Conclusion The study highlights regional disparities in pediatric GI endoscopy services. It emphasizes the need for expanded pediatric GI training and improved access to therapeutic endoscopy, particularly for life-saving procedures.

Purpose: Pediatric gastrointestinal (GI) endoscopy significantly contributes to the diagnosis and management of GI diseases in children. Global data on pediatric GI endoscopy in the Asia-Pacific region are limited. We aimed to report the findings of a regional survey on pediatric endoscopy in the Asia-Pacific region. Methods: A questionnaire-based survey involving GI endoscopy centers in 13 Asia-Pacific countries (June to November 2021). The questionnaires included annual procedure volumes (from basic diagnostics to advanced therapeutic endoscopy), endoscopists, sedation procedures, and national training programs. Results: A total of 162 GI endoscopy centers completed the survey. All centers performed basic endoscopies (esophagogastroduodenoscopy and ileocolonoscopy); however, 45.1% and 59.1% of the centers performed less than 50 esophagogastroduodenoscopies and ileocolonoscopies per year, respectively. Small bowel evaluation (capsule endoscopy or balloon-assisted enteroscopy) was performed in 59.3% of the centers. Foreign body removal, polypectomy, and percutaneous endoscopic gastrostomy were performed in 89.5%, 85.8%, and 52.5% of centers, respectively. Endoscopic hemostatic interventions, which are lifesaving procedures, included glue injection (30.9%), hemostasis of nonvariceal bleeding (65.4%), and endoscopic variceal ligation (70.4%). Pediatric GI endoscopy is performed not only by pediatric gastroenterologists but also by adult gastroenterologists in 21–50% of centers for many kinds of procedures. Sedation was provided by anesthesiologists in 65.4% of the centers. Most centers offer both adult and pediatric endoscopy training. Conclusion The study highlights regional disparities in pediatric GI endoscopy services. It emphasizes the need for expanded pediatric GI training and improved access to therapeutic endoscopy, particularly for life-saving procedures.

Purpose: Pediatric gastrointestinal (GI) endoscopy significantly contributes to the diagnosis and management of GI diseases in children. Global data on pediatric GI endoscopy in the Asia-Pacific region are limited. We aimed to report the findings of a regional survey on pediatric endoscopy in the Asia-Pacific region. Methods: A questionnaire-based survey involving GI endoscopy centers in 13 Asia-Pacific countries (June to November 2021). The questionnaires included annual procedure volumes (from basic diagnostics to advanced therapeutic endoscopy), endoscopists, sedation procedures, and national training programs. Results: A total of 162 GI endoscopy centers completed the survey. All centers performed basic endoscopies (esophagogastroduodenoscopy and ileocolonoscopy); however, 45.1% and 59.1% of the centers performed less than 50 esophagogastroduodenoscopies and ileocolonoscopies per year, respectively. Small bowel evaluation (capsule endoscopy or balloon-assisted enteroscopy) was performed in 59.3% of the centers. Foreign body removal, polypectomy, and percutaneous endoscopic gastrostomy were performed in 89.5%, 85.8%, and 52.5% of centers, respectively. Endoscopic hemostatic interventions, which are lifesaving procedures, included glue injection (30.9%), hemostasis of nonvariceal bleeding (65.4%), and endoscopic variceal ligation (70.4%). Pediatric GI endoscopy is performed not only by pediatric gastroenterologists but also by adult gastroenterologists in 21–50% of centers for many kinds of procedures. Sedation was provided by anesthesiologists in 65.4% of the centers. Most centers offer both adult and pediatric endoscopy training. Conclusion The study highlights regional disparities in pediatric GI endoscopy services. It emphasizes the need for expanded pediatric GI training and improved access to therapeutic endoscopy, particularly for life-saving procedures.

Purpose: To evaluate prolonged esomeprazole use in Japanese pediatric patients for reflux esophagitis (RE) maintenance therapy and prevention of gastric (GU) and/or duodenal ulcers (DU) while using non-steroidal anti-inflammatory drugs (NSAIDs) or low-dose aspirin (LDA). Methods: This multicenter, open-label, parallel-group, phase III study (NCT03553563) included patients who were administered esomeprazole according to body weight (10 mg/day [Groups 1 and 3] and up to 20 mg/day [Groups 2 and 4] for patients weighing 10–20 kg and ≥20 kg, respectively). Efficacy outcomes for Groups 1 and 2 (maintenance therapy for healed RE) and Groups 3 and 4 (prevention of long-term NSAID/LDA use-associated GU/DU) were the presence/absence of RE relapse and GU/DU recurrence, respectively. Results: Esomeprazole as maintenance therapy was associated with a low RE recurrence rate, independent of body weight or dosage. Recurrence rates of RE were 0.0% and 5.3% for Groups 1 and 2, respectively. In patients previously diagnosed with GU and/or DU due to long-term NSAID/LDA use, the recurrence rates of GU/DU during weeks 0–32 were 11.1% and 0.0% in Groups 3 and 4, respectively. Conclusion Long-term use of 10- or 20-mg, once-daily esomeprazole demonstrated a favorable benefit-risk balance in preventing RE and suppressing recurrence of GU and/or DU secondary to NSAID or LDA therapy in Japanese pediatric patients. No new safety concerns were identified. Esomeprazole may be a viable option for managing RE and preventing GU and DU in Japanese pediatric patients.