JPGN reports最新文献

{"title":"Vitamin B12 deficiency in a pediatric patient with gastric obstruction and jejunal feeding dependence: A case report.","authors":"Angela H Nguyen, Dorsey Bass, Rachel E Herdes","doi":"10.1002/jpr3.70117","DOIUrl":"10.1002/jpr3.70117","url":null,"abstract":"<p><p>Pediatric B12 deficiency is most caused by insufficient dietary intake, malabsorption or autoimmune gastritis. We present a unique case of B12 deficiency in a pediatric patient with complex gastrointestinal anatomy and jejunal nutritional dependence nearly two decades after unsuccessful surgical intervention. High-dose enteral B12 supplementation via jejunostomy tube resulted in rapid resolution of the patient's neuromuscular manifestations of vitamin B12 deficiency, highlighting the importance of considering nutritional deficiencies in patients reliant on jejunal enteral nutrition and anatomic differences.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"174-178"},"PeriodicalIF":0.0,"publicationDate":"2025-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894065/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204611","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":"Unusual endoscopic remission in an anti-tumor necrosis factor refractory case of severe ulcerative colitis during upadacitinib therapy.","authors":"Carine A Halaby, Charlotte F Kim, Richard Kellermayer","doi":"10.1002/jpr3.70125","DOIUrl":"10.1002/jpr3.70125","url":null,"abstract":"","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"179-181"},"PeriodicalIF":0.0,"publicationDate":"2025-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894077/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204603","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":"Can artificial intelligence pass the test? Evaluating chatbot scores on pediatric gastroenterology board-style questions.","authors":"Annette G Roberts, Reshma Patel, Sharmilaa Babu, Matthew M Engelhard, Rachel G Greenberg, Arun Ajmera","doi":"10.1002/jpr3.70121","DOIUrl":"10.1002/jpr3.70121","url":null,"abstract":"<p><strong>Objectives: </strong>The American Academy of Pediatrics (AAP) Pediatrics Review and Education Program (PREP)® Gastroenterology (GI) Self-Assessments help pediatric gastroenterologists and trainees prepare for subspecialty board exams by providing peer-reviewed questions and critiques based on American Board of Pediatrics content specifications. These assessments test knowledge of material aligned with the pediatric gastroenterology board exams. While artificial intelligence (AI) chatbots have passed various medical board exams, their ability to pass the pediatric GI boards remains untested. This study assesses the performance of Microsoft Copilot and OpenAI ChatGPT-3.5 and 4o on the 2022-2024 AAP PREP® GI Self-Assessments.</p><p><strong>Methods: </strong>A total of 216 AAP PREP® GI Self-Assessment questions from 2022 to 2024 were entered into three AI chatbots (Microsoft Copilot, OpenAI ChatGPT-3.5, and ChatGPT-4o). Scores were compared with the passing score (> 65%) and first-time test takers' scores from the AAP for 2022-2024.</p><p><strong>Results: </strong>OpenAI ChatGPT-4o and Microsoft Copilot scored above 65% (pass) on all three PREP® GI Self-Assessments from 2022 to 2024. OpenAI ChatGPT-3.5 passed the 2023 and 2024 assessments but did not pass the 2022 assessment. The chatbots collectively scored best in anatomy, motility, and mouth and esophageal disorders, and scored poorly in physiology, pharmacology, liver, stomach and duodenum disorders.</p><p><strong>Conclusions: </strong>OpenAI ChatGPT-4o and Microsoft Copilot consistently passed the PREP® GI Self-Assessments from 2022 to 2024, showing potential for good performance on the pediatric GI boards. OpenAI ChatGPT-3.5 had limitations, passing only the 2023 and 2024 assessments. Overall, advanced AI chatbots show potential to pass the Pediatric GI board exam.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"28-35"},"PeriodicalIF":0.0,"publicationDate":"2025-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894084/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204490","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 retrospective cohort study evaluating the growth and clinical outcomes of preterm and term infants with cystic fibrosis during the first 11 years of life.","authors":"Hebah Reda, Sanjidah Ahmed, Julie Sturza, Samya Z Nasr","doi":"10.1002/jpr3.70124","DOIUrl":"10.1002/jpr3.70124","url":null,"abstract":"<p><strong>Objectives: </strong>Infants with cystic fibrosis (iwCF) have lower birth weights than others. That influences nutritional and pulmonary outcomes. It was reported that 86.0% of iwCF were born full-term and 14.0% preterm. Data on the role of gestational age in growth and clinical outcomes of people with cystic fibrosis (pwCF) is limited. Objectives of this study are to assess the impact of gestational age on the growth and clinical outcomes of pwCF at 2 and 11 years of age and pulmonary function at 6 and 11 years of age.</p><p><strong>Methods: </strong>This is a retrospective, single-center, cross-sectional study, conducted between 2008 and 2024, for pwCF at Michigan Medicine.</p><p><strong>Results: </strong>A total of 170 pwCF were enrolled, 21 were premature, and 149 were full term. There was no statistical significance between the two groups in hospitalization rate, pancreatic enzyme replacement therapy, Dornase alfa use, Pseudomonas aeruginosa colonization, and cystic fibrosis transmembrane conductance regulator modulators use. There was a statistically significant difference in Vitamin K supplement, Ursodiol, and proton pump inhibitors use, with all used more in the premature group. There was no statistical significance of weight for length (WFL) at birth. At 2 years of age, there was a statistically significant difference in WFL and in body mass index between the two groups at 11 years of age.</p><p><strong>Conclusion: </strong>Our study demonstrated a statistically significant difference in preterm WFL percentile compared to term pwCF at 2 years of age, with preterm pwCF having a lower average WFL percentile (<i>p</i> = 0.04). We found no difference between the groups with respect to pulmonary function testing, medication, and nutrition supplementation use.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"167-173"},"PeriodicalIF":0.0,"publicationDate":"2025-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894058/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204474","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":"Interferon-gamma blocking as a promising treatment for severe liver dysfunction in secondary hemophagocytic lymphohistiocytosis after liver transplantation.","authors":"Chen Chen, Tao Zhou, Jing Jin, Yi Luo, Lijing Shen, Qiang Xia, Yongbing Qian","doi":"10.1002/jpr3.70126","DOIUrl":"10.1002/jpr3.70126","url":null,"abstract":"<p><p>Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that can occur after solid organ transplantation but remains underrecognized in this setting. The diagnosis is often delayed due to overlapping clinical manifestations with infection, rejection, or malignancy, and management becomes particularly challenging when accompanied by severe hepatic dysfunction. Here, we describe a pediatric patient who developed Epstein-Barr virus (EBV)-associated secondary HLH following liver re-transplantation for biliary atresia. On postoperative Day 10, the patient presented with high fever, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and marked hyperferritinemia, accompanied by rapidly worsening liver injury. Bone marrow biopsy confirmed hemophagocytosis. Given the severe hepatic impairment and persistent inflammation, the patient received three doses of emapalumab (1 mg/kg), a monoclonal antibody blocking interferon-γ, combined with corticosteroids, intravenous immunoglobulin, and antimicrobial prophylaxis. The treatment led to a rapid decline in ferritin levels, reversal of liver dysfunction, and complete clinical recovery without opportunistic infections. The patient has remained stable with normal liver function and negative EBV DNA for over 2 years of follow-up. This case demonstrates that early interferon-γ blockade can safely and effectively reverse HLH-related liver injury after transplantation, offering a promising therapeutic strategy for the management of secondary HLH complicated by severe hepatic dysfunction.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"14-18"},"PeriodicalIF":0.0,"publicationDate":"2025-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894085/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204602","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":"Peripheral blood cytokine levels in paediatric-onset inflammatory bowel disease: A systematic review and meta-analysis.","authors":"Sabine Jansson, Andrea Ehrström, Charlotte Ulrikka Rask, Johan Burisch, Michael Eriksen Benros, Jakob Seidelin, Mikkel Malham, Vibeke Wewer","doi":"10.1002/jpr3.70116","DOIUrl":"10.1002/jpr3.70116","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to summarise findings on blood cytokine levels in patients with paediatric-onset inflammatory bowel disease (PIBD) compared to healthy controls.</p><p><strong>Methods: </strong>This systematic review followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and was registered at Prospero (CRD42024579684). A literature search was performed on 1 August 2024 in PubMed, EMBASE, Web of Science and Scopus. We included studies that reported levels of cytokines in plasma or serum. Pooled effect sizes of mean values were calculated using Hedges' <i>g</i> for any cytokine reported by two or more studies.</p><p><strong>Results: </strong>The search revealed 5529 papers. Twenty-three articles reporting on 58 cytokines were included, totalling 1018 patients with PIBD and 634 healthy controls. Meta-analysis was feasible for three cytokines: interleukin (IL)-1β, IL-6 and tumour necrosis factor (TNF)-α. IL-6 levels were elevated in PIBD (<i>n</i> = 482, standardised mean difference [SMD]: 1.71; 95% confidence interval [CI]: 1.00-2.43), although with high heterogeneity (<i>I</i> ²: 96.69%). The effect size for patients with Crohn's disease (<i>n</i> = 336) was SMD 1.93 (95% CI: 0.85-3.00, <i>I</i> ²: 97.81%), and for patients with ulcerative colitis (<i>n</i> = 85) the SMD was 1.58 (95% CI: 0.34-2.83, <i>I</i> ²: 93.98%). In contrast, levels of IL-1β (SMD: 1.07, 95% CI: -1.01 to 3.14, <i>I</i> ²: 93.8%) and TNF-α (SMD: 0.22; 95% CI: -0.17 to 0.60; <i>I</i> ²: 77.0%) did not differ significantly between patients and controls. Additionally, the associations with the remaining 55 cytokines not eligible for meta-analysis were reviewed.</p><p><strong>Conclusions: </strong>IL-6 was increased in the peripheral blood of patients with PIBD. Further proteomic studies, including correlation analyses of clinical data, are encouraged.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"77-89"},"PeriodicalIF":0.0,"publicationDate":"2025-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894046/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204598","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":"Over-the-wire insertion of a naso-duodenal feeding tube in a critically ill infant.","authors":"Hashim Abdul-Khaliq, Jochen Pfeifer","doi":"10.1002/jpr3.70122","DOIUrl":"10.1002/jpr3.70122","url":null,"abstract":"","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"182-183"},"PeriodicalIF":0.0,"publicationDate":"2025-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894050/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204634","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":"When manual disimpaction isn't enough: Case report and review of neostigmine's role in refractory constipation management.","authors":"Mojdeh Mostafavi, Sarah Lee, Victoria Martin","doi":"10.1002/jpr3.70115","DOIUrl":"10.1002/jpr3.70115","url":null,"abstract":"<p><p>Severe refractory constipation can lead to fecal retention requiring hospitalization, nasogastric cleanout, or manual disimpaction, and in some cases, surgery. Neostigmine, a reversible acetylcholinesterase inhibitor, is commonly used for pseudo-obstruction but rarely for refractory constipation. Here, we report a 21-year-old male with chronic constipation and likely undiagnosed autism spectrum disorder hospitalized for severe fecal retention, unresponsive to nasogastric polyethylene glycol. Manual disimpaction removed distal stool but left significant proximal stool burden. A carefully monitored trial of intravenous neostigmine, 4 mg in divided doses, resulted in substantial stool passage without bradycardia or other adverse effects. This case highlights neostigmine as a safe and effective adjunct to manual disimpaction. Its use may improve outcomes and reduce the need for surgical intervention in select patients with complex gastrointestinal motility disorders.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"55-58"},"PeriodicalIF":0.0,"publicationDate":"2025-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894083/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204649","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":"Autoantibody signatures in children with celiac disease, juvenile idiopathic arthritis, and polyautoimmunity.","authors":"Nan Du, Denis Chang, Madison Wong, Jay R Thiagarajah, Erin Janssen, Lauren A Henderson, Krishnan Raghunathan, Jocelyn A Silvester","doi":"10.1002/jpr3.70119","DOIUrl":"10.1002/jpr3.70119","url":null,"abstract":"<p><strong>Objective: </strong>To determine if multiplex autoantibody arrays can identify novel biomarker signatures in children with one or multiple autoimmune diseases (polyautoimmunity).</p><p><strong>Methods: </strong>Plasma collected from children (18 years or younger) in the Boston Children's Hospital Precision Link Biobank for Health Discovery between January 2007 and June 2021 was analyzed using a microarray with 120 autoantigens associated with various autoimmune diseases to assess for both immunoglobulin G (IgG) and A (IgA) autoantibodies. To determine if disease-specific, we binary classified those with polyautoimmunity and the most common autoimmune diseases in our cohort (autoimmune thyroid disease, type 1 diabetes, or celiac disease [CeD]). For each comparison within and between groups, the autoantibody intensity was releveled to the control and a linear model was fit.</p><p><strong>Results: </strong>Plasma was analyzed from 114 children with either CeD (<i>n</i> = 31), juvenile idiopathic arthritis (<i>n</i> = 20), polyautoimmunity (<i>n</i> = 31), or no autoimmune disease (<i>n</i> = 32). Overall, children with polyautoimmunity had higher levels of specific autoantibodies relative to the other groups. Children with CeD and polyautoimmunity expressed higher levels of H/K ATPase IgA and IgG and MDA5 IgG compared to those with CeD alone.</p><p><strong>Conclusions: </strong>Use of multiplex antigen arrays in patients with and without polyautoimmunity is a valuable tool to identify known and potentially novel disease-antigens. Further study is needed to determine the role of H/K ATPase autoantibodies in monitoring of CeD patients.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"118-126"},"PeriodicalIF":0.0,"publicationDate":"2025-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894067/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204472","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":"Maralixibat for the treatment of severe xanthomas in two children with Alagille syndrome: Case reports.","authors":"Geetanjali Bora, Ruben E Quiros-Tejeira, Bernadette Vitola","doi":"10.1002/jpr3.70105","DOIUrl":"10.1002/jpr3.70105","url":null,"abstract":"<p><p>Alagille syndrome (ALGS) is a rare, autosomal dominant disorder which presents with a broad range of clinical manifestations, including cholestatic pruritus. A unique manifestation of ALGS is the presence of xanthomas in 24%-42% of patients, which can lead to liver transplantation. Maralixibat, an ileal bile acid transporter (IBAT) inhibitor, has demonstrated improvements in both cholestatic pruritus and xanthomas in clinical trials. We report here on the use of maralixibat in two patients with ALGS and unusual manifestations of xanthomatosis, including one patient with airway xanthomas and a second patient with severe, diffuse xanthomas. In both cases, almost complete resolution of severe, debilitating xanthomas and clinically meaningful improvements in pruritus and serum bile acid levels were observed after up to 1 year of treatment with maralixibat. These cases support the utilization of maralixibat for the management of ALGS beyond cholestatic pruritus.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"7 1","pages":"1-5"},"PeriodicalIF":0.0,"publicationDate":"2025-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12894057/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146204593","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}