Journal of Pediatric Gastroenterology and Nutrition最新文献

{"title":"Mount St. Helen's sign: A case for caution.","authors":"Ahmad Salah Sami, Christian Hochhalter, Sabina Mir","doi":"10.1002/jpn3.12461","DOIUrl":"10.1002/jpn3.12461","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"535-536"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932122","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intestinal ultrasound at diagnosis of pediatric inflammatory bowel disease compared to endoscopy.","authors":"Hamza Hassan Khan, Martha M Munden, Leslie H Spence, Richard H Jones, Jordan Whatley, Carmine Suppa","doi":"10.1002/jpn3.12444","DOIUrl":"10.1002/jpn3.12444","url":null,"abstract":"<p><strong>Objectives: </strong>Intestinal ultrasound (IU) has emerged as an alternative to detect bowel wall inflammation. The aim of this study was to compare IU findings to clinical disease, fecal calprotectin (FC), and endoscopic findings in newly diagnosed pediatric inflammatory bowel disease (IBD) patients.</p><p><strong>Methods: </strong>This study was a 1-year, single-center, prospective study. Any pediatric patient undergoing colonoscopy could be recruited. Following ileo-colonoscopy, subjects were divided into two groups: patients diagnosed with IBD and patients without IBD. Participants had an IU within 1 month. Endoscopists and radiologists were blinded to each other. The IU findings were compared with clinical disease activity, FC, and endoscopic findings.</p><p><strong>Results: </strong>A total of 50 subjects were enrolled in the study; 29 (58%) were females, median age was 13.5 years, and 25 (50%) were diagnosed with IBD. IU sensitivity was 76%, specificity 84%, positive predictive value (PPV) 83%, and negative predictive value (NPV) 78%. For detection of moderate to severe disease, sensitivity, specificity, PPV, and NPV were 91.3%, 86.21%, 84%, and 92.6%, respectively. A significant correlation was noted between IU and FC, Mayo score, and Simple Endoscopic Score (0.513, 0.565, and 0.731, respectively). Pediatric Ulcerative Colitis Activity Index and Pediatric Crohn's Disease Activity Index scores had Pearson correlations of 0.070 and -0.159, respectively.</p><p><strong>Conclusions: </strong>IU can be considered a screening tool for pediatric IBD. It has reasonable sensitivity, specificity, PPV, and NPV, particularly for moderate-to-severe disease. The severity noted on IU correlated with FC and endoscopic disease activity but did not correlate with clinical disease activity.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"440-445"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142882440","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"FISPGHAN statement on the global public health impact of metabolic dysfunction-associated steatotic liver disease.","authors":"Tania Mitsinikos, Marion M Aw, Robert Bandsma, Marcela Godoy, Samar H Ibrahim, Jake P Mann, Iqbal Memon, Neelam Mohan, Nezha Mouane, Gilda Porta, Elvira Verduci, Stavra Xanthakos","doi":"10.1002/jpn3.12399","DOIUrl":"10.1002/jpn3.12399","url":null,"abstract":"<p><p>As rates of obesity rise worldwide, incidence of metabolic dysfunction-associated steatotic liver disease (MASLD), formerly referred to as nonalcoholic fatty liver disease, is increasing, worsening the burden of healthcare systems. The council of the Federation of International Societies for Pediatric Gastroenterology, Hepatology, and Nutrition (FISPGHAN) identified the topic of MASLD epidemiology, treatment, and prevention as a global priority issue to be addressed by an expert team, with the goal to describe feasible and evidence-based actions that may contribute to reducing MASLD risk. The FISPGHAN member societies nominated experts in the field. The FISPGHAN council selected and appointed members of the expert team and a chair. The subtopics included in this manuscript were chosen through a consensus of the experts involved. We review the epidemiology, natural history, and screening and management. We further expand to relevant public health measures aimed at MASLD prevention, including identifying interventions that could reduce risk factors (environmental and iatrogenic), optimize maternal and newborn health, and support healthier lifestyles for older children and adolescents on a local, national, and international scale. While recognizing that various aspects of population health and public policy can shape MASLD risk, we also review what we can do on an individual level to support our patients to reduce the significant burden of this ever rising disease in pediatrics.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"397-407"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142895392","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Publication bias in studies on biologic therapy for children with inflammatory bowel disease.","authors":"Ariel Weil, Gili Focht, Ohad Atia","doi":"10.1002/jpn3.12433","DOIUrl":"10.1002/jpn3.12433","url":null,"abstract":"<p><strong>Objective: </strong>The utilization of biologic drugs in children with inflammatory bowel disease (IBD) surged following the publications of positive results in randomized controlled trials and real-world studies. We aimed to explore the extent of publication bias associated with these findings.</p><p><strong>Methods: </strong>Two reviewers assessed all abstracts evaluating the efficacy or safety of biologics presented at the annual European Society for Pediatric Gastroenterology Hepatology and Nutrition and North American Society for Pediatric Gastroenterology conferences from 2015 to 2019. Abstracts were classified as \"positive\" or \"negative.\" Time to publication was analyzed using Kaplan-Meier curve and groups were compared using the log-rank test. A Cox proportional model was utilized to determine the likelihood of publication.</p><p><strong>Results: </strong>Out of 209 included abstracts, only 130 (62%) were published as full manuscripts. The median time to publication was 2.8 years (interquartile range = 0-8.2). In the univariate Cox model, the likelihood of publication was four times higher for abstracts reporting positive results (hazard ratio = 4.4 [95% confidence interval, CI = 2.3-8.5]). The probabilities for publication at 1, 3, and 5 years after the conference were 32%, 59%, and 66% for abstracts with significantly positive results in favor of biologic treatment compared to 10%, 22%, and 25% for those with negative results (p < 0.001). In multivariable model, positive results (odds ratio = 6.4 [95% CI = 2.5-16.4]) were significant associated with publication rate.</p><p><strong>Conclusion: </strong>Only 62% of abstracts presented in medical conferences regarding biologics in pediatric IBD are eventually published as full manuscripts, and those reporting positive results were more likely to be published and at an earlier time. Clinicians, guideline groups, and medical authorities dealing with drug approval, need to be aware of potential publication bias of published studies when employing evidence-based management strategies.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"426-432"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142837203","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Burkitt lymphoma in a child with rectal bleeding.","authors":"Stephanie W Hum, Rebecca Citrin, Petar Mamula, Vera De Matos, Ronen Stein","doi":"10.1002/jpn3.12453","DOIUrl":"10.1002/jpn3.12453","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"533-534"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term growth and nutrition outcomes in children following intestinal transplantation.","authors":"Ahmad Miri, Angela K Iverson, Nathan Law, Junghyae Lee, Ruben E Quiros Navarrete, Emille M Reyes-Santiago, Warapan Nakayuenyongsuk, David F Mercer, Luciano M Vargas, Shaheed Merani, Wendy J Grant, Alan N Langnas, Ruben E Quiros-Tejeira","doi":"10.1002/jpn3.12455","DOIUrl":"10.1002/jpn3.12455","url":null,"abstract":"<p><strong>Objective: </strong>Intestinal transplantation (ITx) has become an accepted option for children with serious complications from intestinal failure and parenteral nutrition (PN) dependence. We aimed to assess long-term growth and nutritional outcomes in these patients. We also assessed factors influencing nutritional status and ability to wean off tube feedings (TFs) after ITx.</p><p><strong>Methods: </strong>We looked retrospectively into post-ITx growth parameters, nutrition treatment, and micronutrient status for children who survived for 5 or more years after ITx. One hundred thirty-three children between 1993 and 2014 were involved. Descriptive data and growth parameters were collected over 15 years after ITx. We also analyzed influencing factors, including the presence of permanent stoma, prolonged use of steroids, partial gastrectomy at the time of ITx, developmental delay, concurrent visceral transplant, and graft rejection episodes.</p><p><strong>Results: </strong>There was an increase in the height z-scores over the 15-year period post-ITx (p < 0.001). There was a downward trend in body mass index (BMI) z-scores over the 15-year post-ITx period. Isolated intestinal transplant patients showed a better height z-score compared to multivisceral transplant (p = 0.04). The height and BMI z-scores for patients on steroids were not significantly different from the z-scores for steroid-free patients (p = 0.72, 0.99, respectively). There was no significant change in height and BMI z-scores based on prednisolone dose: ≤0.2 mg/kg (p = 0.76); >0.2 mg/kg (p = 0.52). Patients were more likely to require supplemental TF up to 15 years post-ITx when they had partial gastrectomy (p < 0.001), permanent ostomy (p = 0.009), or developmental delay (p < 0.001).</p><p><strong>Conclusions: </strong>There was improved long-term linear growth post-ITx. Developmental delay, partial gastrectomy, and a permanent ostomy are likely to delay TF wean post-ITx.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"490-497"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11874248/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932099","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Determining the time to cholangiocarcinoma in pediatric-onset PSC-IBD.","authors":"Batul Kaj-Carbaidwala, Johan Fevery, Douglas G Adler, Annika Bergquist, Lissy de Ridder, Mark Deneau, Corinne Gower-Rousseau, Roger W Chapman, Kate D Lynch, Catherine A M Stedman, David C Wilson, Uzma Shah, Lipika Goyal, Harland S Winter, Jochen K Lennerz","doi":"10.1002/jpn3.12443","DOIUrl":"10.1002/jpn3.12443","url":null,"abstract":"<p><p>Primary sclerosing cholangitis (PSC) is a risk factor for cholangiocarcinoma. When a child is diagnosed with both PSC and inflammatory bowel disease (IBD), evidence-based information on counseling families and risk management of developing cholangiocarcinoma is limited. In this case series (PubMed/collaborators), we included patients with PSC-IBD who developed cholangiocarcinoma and contacted authors to determine an event curve specifying the time between the second diagnosis (IBD or PSC) and a diagnosis of cholangiocarcinoma. Review of n = 175 studies resulted in a cohort of n = 21 patients with pediatric-onset PSC-IBD-cholangiocarcinoma. The median time to development of cholangiocarcinoma was 6.95 years from the second diagnosis. Despite the small number, 38% of cholangiocarcinoma developed within the first 2 years, and 47% of patients developed cholangiocarcinoma in the transition period to adult care (age 14-25). Our findings highlight the importance of screening that extends over the so-called transition period from pediatric to adult care.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"450-454"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142864595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical effects of pediatric commercial food-based formulas: A systematic review.","authors":"Judy-April Murayi, Elizabeth Evenson, Chelsea Britton, Alison Gehred, Praveen S Goday","doi":"10.1002/jpn3.12450","DOIUrl":"10.1002/jpn3.12450","url":null,"abstract":"<p><p>Blenderized tube feeding (BTF) uses a feeding tube to deliver blended whole foods directly to the gastrointestinal (GI) system and has had renewed interest over the last two decades. This was initially delivered in the form of homemade BTF (HBTF) and led to the development of commercial food-based formula (CFBF). The safety and clinical outcome data for CFBF are limited. From our systematic review of the clinical benefits of pediatric CFBF, we found that families are very satisfied with CFBF despite the paucity of research. Most included studies evaluated both HBTF and CFBF, with only a few evaluating CFBF alone. The limited data suggests that CFBF may improve upper and lower GI symptoms such as nausea, vomiting, retching, constipation, and diarrhea; however, the effects of CFBF on growth have been mixed.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"501-509"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142895373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetic profiling of Wilson disease reveals a potential recurrent pathogenic variant of ATP7B in the Jordanian population.","authors":"Fareed Khdair Ahmad, Dunia Aburizeg, Yaser Rayyan, Tarek A Tamimi, Salma Burayzat, Abdullah Ghanma, Maha Barbar, Bilal Azab","doi":"10.1002/jpn3.12446","DOIUrl":"10.1002/jpn3.12446","url":null,"abstract":"<p><strong>Objectives: </strong>Wilson disease (WD) is an autosomal-recessive disorder that disrupts copper homeostasis. ATPase copper transporting beta (ATP7B) gene is implicated as the disease-causing gene in WD. The common symptoms associated with WD include hepatic, neurological, psychiatric, and ophthalmic manifestations. The genetic landscape of WD is under-investigated in the Middle East and has never been studied in Jordan. We aimed to investigate the genetic profile of several unrelated Jordanian families with one or more patients affected by WD.</p><p><strong>Methods: </strong>Twenty-four Jordanian families with WD underwent clinical evaluation and genetic profiling by whole-exome and Sanger sequencing.</p><p><strong>Results: </strong>Surprisingly, the same variant (ATP7B:c.3551C>T;p.Ile1184Thr) was identified, for the first time, exclusively in the homozygous state, in eight consanguineous unrelated families. Before our study, the previous classification of this variant was either of uncertain significance (VUS) or likely pathogenic (LP). Interestingly, the patients harboring this variant displayed variable clinical manifestations on both the intra- and interfamilial levels, as previously described in cases with WD. The age of diagnosis, hepatic manifestations, neuropsychiatric involvements, and Kayser-Fleischer ring occurrence varied significantly in terms of existence and severity among the recruited individuals. Following our investigation, based on clinical data and co-segregation analysis, we re-classified the variant ATP7B:c.3551C>T;p.Ile1184Thr from VUS/LP to pathogenic, for the first time. Besides, our genetic analysis helped in resolving diagnostic ambiguity by either establishing or ruling out the diagnosis of WD.</p><p><strong>Conclusion: </strong>Since the identified variant (ATP7B:p.Ile1184Thr) was discovered in multiple unrelated families, we create an avenue for the potential consideration of this variant as a recurrent, or possibly a founder variant, in the Jordanian population. Our work sheds light, for the first time, on the molecular underpinnings of WD in Jordan and compiles the WD-causing variants in the Middle East. Ultimately, the findings of our work can guide designing region-specific targeted genetic testing of WD in Jordan and provide valuable insights to direct clinical decisions for atypical WD presentations.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":"80 3","pages":"471-481"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143537328","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The expanded Child Tax Credit was associated with improved BMI and metabolic outcomes in low-income children.","authors":"Paul Po-Ting Lu, Amy R Mahar, Hal Sitt, Janet Rosenbaum, Renee Bargman, Thomas Wallach","doi":"10.1002/jpn3.12445","DOIUrl":"10.1002/jpn3.12445","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"498-500"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11875967/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142864619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}