JPGN reports最新文献

{"title":"Effects of post-hepatic portoenterostomy adjuvant therapy on liver transplantation in children with biliary atresia: A systematic review.","authors":"Bianca Ferraz de Almeida Silva, Alice Palmeira Nascimento Cerqueira, Breno Oliveira Marques, Gabriela Garcia de Carvalho Laguna, Gabrielle Dos Santos Aguiar, Luana Leal Gonzaga, Raquel Teles de Lacerda, Natalia Oliveira E Silva","doi":"10.1002/jpr3.70214","DOIUrl":"10.1002/jpr3.70214","url":null,"abstract":"<p><strong>Objectives: </strong>Biliary atresia (BA) is a cholangiopathy characterized by obstruction of the intrahepatic and extrahepatic bile ducts. Hepatic portoenterostomy (HPE) is the primary palliative treatment and there is still an urgent need to improve post-HPE management. This study aims to identify post-HPE adjuvant therapies associated with reducing or delaying the need for liver transplantation in children with BA.</p><p><strong>Methods: </strong>This systematic review was registered on the PROSPERO platform (ID: CRD42024553671). Studies were extracted from the following databases: PubMed, Web of Science, Lilacs, and Scielo. Original articles published between 2003 and 2025 in English, Portuguese, and Spanish were included, while studies involving adults, animals, or unrelated topics were excluded.</p><p><strong>Results: </strong>A total of 870 studies were identified, and after applying eligibility criteria, 16 articles were selected. The studies indicated that corticosteroids alone as adjuvant therapy were not associated with improved native liver survival. However, observational studies indicated that corticosteroid therapy combined with antibiotics, anticholestatics, and immunoglobulin may be associated with improvements in jaundice, although only two studies reported increased liver survival. Rectal budesonide demonstrated promising short and long-term results in children with nonsyndromic BA.</p><p><strong>Conclusions: </strong>The use of adjuvant therapy after HPE was associated with improvements in jaundice and cholangitis and, in some studies, with increased liver survival. However, the findings are conflicting and heterogeneous. Further research is needed, with standardized therapeutic approaches to enable more comprehensive analyses.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13394905/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148582040","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The genetic landscape of congenital diarrheas and very early onset inflammatory bowel disease in the Middle East.","authors":"Lily Gillette, Muna Al Safar, Ganeshwaran H Mochida, Casey Johnson, Stephen Babcock, Klaus Schmitz-Abe, Michael Field, Jessica Yang, Richelle Bearup, Ahmed Megahed, Mohammed Ezz El Regal Abbas, Tarek Baraket, Sherine Elziny, Ali Sobh, Gulin Eren, Fawaz AlRefaee, Rana Bitar, Mohamed Miqdady, Pankaj B Agrawal, Scott B Snapper, Jay R Thiagarajah, Jodie Ouahed","doi":"10.1002/jpr3.70217","DOIUrl":"10.1002/jpr3.70217","url":null,"abstract":"<p><strong>Objectives: </strong>Monogenic causes of congenital diarrheas and enteropathies (CoDE) and very early onset inflammatory bowel disease (VEOIBD) are mostly recessive and therefore more prevalent in populations with increased consanguinity rates. To assess the genetic basis of these disorders in a likely high-prevalence population, we established a multi-center cohort of patients across the Middle East.</p><p><strong>Methods: </strong>Patients were enrolled across four centers in the Middle East. Clinical data, including self-reported consanguinity, were collected. Trio whole exome sequencing (WES) was performed, and genomic data were processed through a standardized variant identification pipeline. Patients who inherited a rare variant consistent with the disease in a gene known to be causative for CoDE or VEOIBD were classified as having a monogenic etiology. Self-reported consanguinity and homozygosity mapping were analyzed and correlated with monogenic diagnoses.</p><p><strong>Results: </strong>WES from trios of 27 patients (13 CoDE and 14 VEOIBD) were analyzed. Higher frequency of consanguinity was self-reported by the CoDE families (11/13, 84.6%) and confirmed by homozygosity mapping than in the VEOIBD group (4/14, 28.6%). A monogenic cause of disease was identified in 10 of 13 CoDE patients (76.9%) in the following genes: <i>EPCAM</i>, <i>SPINT2</i>, <i>DGAT1, MYO5B, SPINT2</i>, and <i>STXBP2</i>. A single VEOIBD patient (7.1%) had an identifiable monogenic cause of disease in <i>SKIV2L</i>.</p><p><strong>Conclusions: </strong>Within this limited cohort, the diagnostic yield for known genes associated with CoDE was high, consistent with the primarily monogenic etiology and high consanguinity. For VEOIBD, the diagnostic yield was lower and similar to that reported in North America.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13395046/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148582027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessing pediatric gastroenterologists' use of artificial intelligence in clinical and professional tasks.","authors":"Ashwin Agrawal, Vineeta Guntupalli, Jonathan E Teitelbaum, Mohamed Elrais","doi":"10.1002/jpr3.70202","DOIUrl":"10.1002/jpr3.70202","url":null,"abstract":"<p><strong>Objectives: </strong>Artificial intelligence (AI) has demonstrated potential to enhance clinical efficiency. However, its real-world adoption among pediatric gastroenterologists (GIs) remains poorly characterized. The primary objective of this study was to assess how pediatric GIs are currently utilizing AI. Secondary objectives included estimating time saved, identifying commonly used tools, and evaluating the impact on burnout.</p><p><strong>Methods: </strong>An institutional review board-approved anonymous survey was distributed via the international pediatric gastroenterology listserv. The questionnaire assessed demographics, types of AI technologies utilized, and estimated time saved through their use. The survey was open from September 30, 2025, to October 28, 2025.</p><p><strong>Results: </strong>One-hundred and twenty-nine completed surveys were included in the analysis. Majority of respondents were from the United States (85.3%). AI was most frequently used for ambient AI scribes, dictation, answering clinical questions, preparing letters of medical necessity, and insurance appeals, with many reporting time savings with use of AI. In addition, 58.1% of respondents had utilized AI for some research-related purpose. AI utilization did not vary significantly across years of practice or practice type. Clinicians who utilized AI technology had higher frequency of self-reported reduced burnout. Few (28.7%) received training in AI, while the majority (84.4%) would like more education in the use of AI in practice.</p><p><strong>Conclusions: </strong>The use of AI tools by pediatric GIs is variable. Those who adopted AI reported reduced burnout and increased time savings, reflecting a positive impact on efficiency and clinician well-being. Despite limited formal training, there was a strong interest in AI education and clinical integration.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398647/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes of adalimumab biosimilar nonmedical switches in children and young adults with inflammatory bowel disease.","authors":"Daniel Himelstein, Megan McNicol, Mahmoud Abdel-Rasoul, Brendan M Boyle, Hilary K Michel, Ross Maltz","doi":"10.1002/jpr3.70212","DOIUrl":"10.1002/jpr3.70212","url":null,"abstract":"<p><strong>Objectives: </strong>Adalimumab biosimilars are as safe and effective compared to the originator. Adult patients with inflammatory bowel disease (IBD) who switched to a biosimilar have comparable outcomes, but pediatric data are limited. This study evaluates clinical outcomes of children and young adults with IBD following a nonmedical, insurance-driven switch from the adalimumab originator to a biosimilar.</p><p><strong>Methods: </strong>A single-center retrospective chart review was conducted among pediatric and young adult patients with IBD who switched from the adalimumab originator to a biosimilar between May 2023 and July 2024. Demographics, Physician Global Assessment (PGA), laboratory values, adalimumab levels, and antibodies were collected preswitch and up to 6 months postswitch. Adalimumab biosimilar continuation was assessed 6 months postswitch. McNemar's exact test, linear mixed effect models, and paired <i>t</i>-tests compared variables preswitch and postswitch.</p><p><strong>Results: </strong>Fifty patients switched to a biosimilar. Forty-two patients had PGAs pre and postswitch, and among them, 86% (36/42) of patients demonstrated stable or improved PGAs postwitch. Seventy-six percent (38/50) of patients continued on the biosimilar for at least 6 months postswitch. Of the 12 patients who discontinued adalimumab biosimilar, 42% (5/12) discontinuation was not related to the switch, while 58% (7/12) were due to the switch. Laboratory values remained stable pre and postswitch, although adalimumab levels decreased postswitch (18-15 µg/mL; <i>p</i> = 0.007).</p><p><strong>Conclusions: </strong>Switching from the adalimumab originator to a biosimilar resulted in comparable outcomes in children and young adults with IBD based on PGA, laboratory markers, and continuation of the medication.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398781/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Letter to Editor: Methodological constraints in assessing dietary and urinary molybdenum.","authors":"Nan Du, Jocelyn Silvester","doi":"10.1002/jpr3.70195","DOIUrl":"10.1002/jpr3.70195","url":null,"abstract":"","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399051/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586483","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Endoscopic findings in patients with Shwachman-Diamond syndrome: A report from the North American Shwachman-Diamond syndrome registry.","authors":"Elizabeth Korn, Jane Koo, Diana Schwarz, Meghan Haney, Juan Putra, Jaeson Kim, Leah Cheng, Sarah Steltz, Lois Schwarz, Sara Loveless, Richard Cooper, Claire Dusa, Kasiani C Myers, Akiko Shimamura, Andrew Wehrman, Amit S Grover","doi":"10.1002/jpr3.70218","DOIUrl":"10.1002/jpr3.70218","url":null,"abstract":"<p><strong>Objectives: </strong>Shwachman-Diamond syndrome (SDS) is an inherited bone marrow failure disorder, and its endoscopic phenotype is poorly defined. We sought to characterize endoscopic findings in patients with genetically confirmed SDS.</p><p><strong>Methods: </strong>Retrospective registry study of 45 patients with biallelic Shwachman-Bodian-Diamond syndrome mutations and evaluable endoscopic procedures. Clinical, laboratory, imaging, endoscopic reports, and biopsy findings were extracted from patient medical records.</p><p><strong>Results: </strong>Among 45 patients with genetically confirmed SDS, 102 endoscopic procedures were performed, most commonly esophagogastroduodenoscopy (EGD, 58.8%). The leading indication was diarrhea and/or steatorrhea (23.6%). Abnormal biopsy findings were most frequent in patients with dysphagia/dyspepsia or gastrointestinal bleeding and were strongly associated with elevated C-reactive protein and abnormal hepatic enzymes. Most pre-procedural imaging studies were normal (63.4%). Pre-hematopoietic stem cell transplant (HSCT) biopsies were largely normal, with occasional mild acute or chronic inflammation in the esophagus, stomach, and duodenum. In contrast, post-HSCT biopsies showed more diverse abnormalities, including inflammation, basal crypt apoptosis, and graft-versus-host disease (GVHD)-associated changes. Central pathology review also identified rare findings such as adenoviral infection and mild or equivocal GVHD in post-transplant samples.</p><p><strong>Conclusions: </strong>Endoscopic evaluation in patients with SDS demonstrates heterogeneous histopathologic findings, with diagnostic yield influenced by clinical indication, laboratory abnormalities, and transplant status. Routine endoscopy in asymptomatic or nonspecifically symptomatic patients may have limited value, whereas targeted evaluation based on symptoms or supportive laboratory findings may improve diagnostic yield. These findings provide updated insight into gastrointestinal manifestations of SDS and may help guide clinical decision-making regarding endoscopic evaluation in this rare disease.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399004/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Molybdenum-Closing the gap.","authors":"Erika Sievers","doi":"10.1002/jpr3.70196","DOIUrl":"10.1002/jpr3.70196","url":null,"abstract":"","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398600/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reply-Green pediatrics beyond endoscopy: Toward systemic sustainability in celiac care.","authors":"Mario Brusco, Sara Trivellini, Francesco Valitutti","doi":"10.1002/jpr3.70204","DOIUrl":"10.1002/jpr3.70204","url":null,"abstract":"","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399574/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A joint transdisciplinary approach for paediatric inflammatory bowel disease: Integrating nursing, dietetic and psychological support.","authors":"King-Chi Yau, Laura Tesser, Kay Crook","doi":"10.1002/jpr3.70216","DOIUrl":"10.1002/jpr3.70216","url":null,"abstract":"<p><p>Paediatric inflammatory bowel disease (pIBD) is a long-term, immune-mediated condition characterised by fluctuating gastrointestinal symptoms, nutritional risk and psychosocial difficulties. Although multidisciplinary team (MDT) support is considered the gold standard, there is considerable variation in how it is delivered, and it remains common for healthcare services to rely on separate discipline-specific appointments within the MDT. Here, we describe a transdisciplinary model for pIBD that integrates nursing, dietetic and psychological expertise within a joint appointment. Through the methodology of reflective practice, we identify several potential benefits, including more holistic symptom management, enhanced access to specialist input, reduced stigma around psychological support and improved cross-disciplinary learning. Potential implementation challenges include implementation feasibility, role ambiguity and demands on some service users. To the best of our knowledge, this paper represents an innovative model of care for pIBD.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13399047/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Green pediatrics beyond endoscopy: Toward systemic sustainability in celiac care.","authors":"Valentina Pucinischi, Giovanni Di Nardo, Maurizio Mennini","doi":"10.1002/jpr3.70205","DOIUrl":"10.1002/jpr3.70205","url":null,"abstract":"","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398365/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148586469","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}