Validation of the simplified PIBD-classes criteria: A single-center retrospective study.

Background: Pediatric inflammatory bowel disease (PIBD) subtypes are classified according to the PIBD-classes criteria, comprising 23 items. These criteria were later simplified to 19 diagnostic items. Inflammatory bowel diseases (IBDs) are classified as ulcerative colitis (UC), atypical UC, IBD-unclassified (IBD-U), Crohn's disease (CD), or isolated colonic CD.

Long-Term Course and Prognostic Factors in Pediatric Ulcerative Proctitis: A Multicenter Cohort Study.

Background: Although ulcerative proctitis (UP) in children is considered relatively mild, some patients have proximal disease extension and require immunosuppressive treatment. We investigated clinical characteristics and course of refractory UP in a multicenter pediatric cohort.

Methods: Analyzing data obtained between 2013 and 2022 at 10 institutions specializing in pediatric inflammatory bowel disease, we elucidated natural history and factors predicting a need for immunosuppressive UP treatment.

First case report of dichorionic diamniotic twins with chronic enteropathy associated with the SLCO2A1 gene.

We report the case of twins diagnosed with chronic enteropathy associated with the SLCO2A1 gene (CEAS) based on characteristic ulcer findings, which required 8 years to diagnose. Both twins had similar symptoms, including anemia and growth failure but the gastrointestinal tract was not evaluated initially because of mild symptoms that were considered consistent with psychological etiology. The endoscopic findings of the firstborn child showed spiral ulcer scars and pseudodiverticulum formation without Helicobacter pylori infection or eosinophilic infiltration in the duodenum.

Correction: Impact of the Coronavirus Disease 2019 Pandemic on Pediatric Gastrointestinal Endoscopy: A Questionnaire-based Internet Survey of 162 Institutional Experiences in Asia Pacific.

[This corrects the article on p. 291 in vol. 26, PMID: 38025493.

Prognosis of pediatric ulcerative colitis after infliximab failure: A multicenter registry-based cohort study.

Background And Aim: Even with increasing numbers of biologic agents available for management of ulcerative colitis (UC), infliximab (IFX) retains an important place in treatment of pediatric patients with this disease. As few reports have addressed outcomes in pediatric UC patients who had to discontinue IFX, we examined clinical course and prognosis after IFX failure in pediatric UC.

Methods: A prospective cohort study of pertinent cases enrolled in the Japanese Pediatric Inflammatory Bowel Disease Registry between 2012 and 2020 was conducted to determine outcomes for pediatric UC patients who received IFX but required its discontinuation during follow-up (IFX failure).

Impact of the Coronavirus Disease 2019 Pandemic on Pediatric Gastrointestinal Endoscopy: A Questionnaire-based Internet Survey of 162 Institutional Experiences in Asia Pacific.

Purpose: The impact of coronavirus 2019 (COVID-19) on gastrointestinal (GI) endoscopy procedures in adults has been reported, with a drastic reduction in the number of procedures. However, there are no sufficient data regarding the impact on pediatric GI endoscopy. Here, we aimed to report that impact in the Asia-Pacific region.

Utility of a rapid assay for prostaglandin E-major urinary metabolite as a biomarker in pediatric ulcerative colitis.

Prostaglandin E-major urinary metabolite (PGE-MUM) is a urinary biomarker reflecting ulcerative colitis (UC) activity. This prospective observational study aimed to evaluate the usefulness of PGE-MUM via rapid chemiluminescent enzyme immunoassay in detecting endoscopic remission (ER) and histologic remission (HR) in pediatric UC (6-16 years) in comparison with fecal calprotectin (FCP). ER and HR were defined as Mayo endoscopic score (MES) of 0 and Matts' histological grades (Matts) of 1 or 2, respectively.

Safety and efficacy of vedolizumab in pediatric patients with ulcerative colitis: multicenter study in Japan.

Background: Vedolizumab (VDZ) is a humanized monoclonal antibody that binds to α4β7 integrin expressed in T-lymphocytes and is gut selective. Few studies have evaluated the safety and efficacy of VDZ in pediatric ulcerative colitis (UC) patients, especially from Asia.

Methods: A longitudinal multicenter retrospective study was conducted at 10 Japanese tertiary medical institutions.

A case of early-onset idiopathic chronic pancreatitis associated with a loss-of-function TRPV6 p.R483Q variant successfully treated by pancreatic duct stenting.

Several pancreatitis-related genetic variants have been identified. Recently, the association of loss-of-function variants in the transient receptor potential cation channel subfamily V member 6 (TRPV6) gene and early-onset non-alcoholic chronic pancreatitis (CP) has been reported. However, detailed clinical presentation of the cases carrying TRPV6 variants remains largely unknown.

Clinical outcome of ulcerative colitis with severe onset in children: a multicenter prospective cohort study.

Background: As best practices for treating children with severe-onset ulcerative colitis remain controversial in the era of biologic agents, we prospectively investigated treatments and outcomes in a multicenter cohort.

Methods: Using a Web-based data registry maintained in Japan between October 2012 and March 2020, we compared management and treatment outcomes in an S1 group defined by a Pediatric Ulcerative Colitis Activity Index of 65 or more points at diagnosis with those in an S0 group defined by an index value below 65.

Results: Three hundred one children with ulcerative colitis treated at 21 institutions were included, with follow-up for 3.

Case of hypereosinophilic syndrome with gastrointestinal involvement showing tissue eosinophil cytolysis.

Hypereosinophilic syndrome (HES), which is characterized by eosinophilia in the peripheral blood, often causes various organ disorders, including those of the gastrointestinal (GI) tract. The eosinophils play a key role in inflammation in eosinophilic GI disorders (EGIDs), including HES with GI involvement. Here, we report a case of HES with GI involvement that showed major basic proteins (MBPs) deposition in the absence of marked eosinophilic infiltration in the mucosa of the GI tract.

Intussusception Caused by Percutaneous Endoscopic Gastrostomy With Jejunal Extension in Patients With Severe Motor and Intellectual Disabilities.

The risk of intussusception related to percutaneous endoscopic gastrostomy with jejunal extension (PEG-J) in patients with severe motor and intellectual disabilities (SMID) remains unknown. In a cross-sectional study, a review of 26 patients (mean age, 11.6 ± 6.

Phenotypic characteristics of pediatric inflammatory bowel disease in Japan: results from a multicenter registry.

Background/aims: There are few published registry studies from Asia on pediatric inflammatory bowel disease (IBD). Registry network data enable comparisons among ethnic groups. This study examined the characteristics of IBD in Japanese children and compared them with those in European children.

Current role of colonoscopy in infants and young children: a multicenter study.

Background: To evaluate the role of colonoscopy in infants and young children and clarify the distribution of colonoscopy-requiring diseases in this age group.

Methods: Cohorts of colonoscopies performed at three children's hospitals in Japan between April 2011 and March 2016 including infants and children younger than six years of age were retrospectively reviewed.

Results: In total, 453 colonoscopies were performed in 276 infants and young children.

Plasma Corticotropin-Releasing Factor Receptors and B7-2⁺ Extracellular Vesicles in Blood Correlate with Irritable Bowel Syndrome Disease Severity.

Extracellular vesicles (EVs) are composed of bilayer membranes that are released by different cell types and are present in bodily fluids, such as blood, urine, and bile. EVs are thought to play a key role in intracellular communication. Based on their size and density, EVs are classified into small, medium, or large EVs.

Clinical Safety and Utility of Pediatric Balloon-assisted Enteroscopy: A Multicenter Prospective Study in Japan.

Objectives: The benefit of balloon-assisted enteroscopy (BAE) had been recently documented in pediatric patients, but previous reports are based on single institution experiences. We evaluated the feasibility of pediatric BAE in 8 tertiary referral hospitals throughout Japan.

Methods: This was a prospective, multi-institutional study.

Sexually dimorphic metabolic responses mediated by CRF receptor during nutritional stress in mice.

Background: Chronic stress is a major contributor in the development of metabolic syndrome and associated diseases, such as diabetes. High-fat diet (HFD) and sex are known modifiers of metabolic parameters. Peptide hormones corticotropin-releasing factor (CRF) and urocortins (UCN) mediate stress responses via activation and feedback to the hypothalamic-pituitary-adrenal (HPA) axis.

Gastric corticotropin-releasing factor influences mast cell infiltration in a rat model of functional dyspepsia.

Functional gastrointestinal disorders (FGIDs) are characterized by dysregulated gut-brain interactions. Emerging evidence shows that low-grade mucosal inflammation and immune activation contribute to FGIDs, including functional dyspepsia (FD). Stress plays an important role in the onset of FD symptoms.

Zinc monotherapy for young children with presymptomatic Wilson disease: A multicenter study in Japan.

Background And Aim: Few studies of zinc monotherapy for presymptomatic Wilson disease have focused on young children. We therefore evaluated long-term efficacy and safety of zinc monotherapy for such children and established benchmarks for maintenance therapy.

Methods: We retrospectively and prospectively examined children under 10 years old with presymptomatic Wilson disease who received zinc monotherapy from time of diagnosis at 12 participating pediatric centers in Japan.

Prostaglandin E-major Urinary Metabolite as a Biomarker for Pediatric Ulcerative Colitis Activity.

Objectives: Prostaglandin E-major urinary metabolite (PGE-MUM) is a useful biomarker for adult ulcerative colitis (UC) activity. In the present study, we evaluated whether PGE-MUM can also be a biomarker of pediatric UC activity and compared its efficacy in predicting UC activity with that of C-reactive protein and erythrocyte sedimentation rate.

Methods: Twenty-nine pediatric patients with UC (8-18 years) and 29 healthy age- and sex-matched subjects were enrolled.