Journal of Pediatric Gastroenterology and Nutrition最新文献

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Predictors of antitumor necrosis factor primary nonresponse and drug durability in pediatric inflammatory bowel disease. 儿童炎症性肠病抗肿瘤坏死因子原发性无反应和药物耐受性的预测因素。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-10-01 Epub Date: 2025-07-21 DOI: 10.1002/jpn3.70160
Nicole Davidson, Grant A Morris, Molly A Wright, Guy Brock, Brendan Boyle, Jennifer L Dotson, Hilary K Michel, Ross M Maltz
{"title":"Predictors of antitumor necrosis factor primary nonresponse and drug durability in pediatric inflammatory bowel disease.","authors":"Nicole Davidson, Grant A Morris, Molly A Wright, Guy Brock, Brendan Boyle, Jennifer L Dotson, Hilary K Michel, Ross M Maltz","doi":"10.1002/jpn3.70160","DOIUrl":"10.1002/jpn3.70160","url":null,"abstract":"<p><strong>Objectives: </strong>Antitumor necrosis factor (anti-TNF) therapies are first-line therapies for children with inflammatory bowel disease (IBD) (Crohn's disease [CD], ulcerative colitis [UC] and IBD-unclassified [IBD-U]). Limited studies describing anti-TNFs durability and loss of response in children. This study evaluates predictors of primary Nonresponse and 3-year drug durability in children with IBD.</p><p><strong>Methods: </strong>This was a single-center retrospective review of patients with IBD less than 18 years old who initiated anti-TNF (infliximab or adalimumab) from January 1, 2014, to December 31, 2019. Clinical and laboratory data were recorded at the time of anti-TNF initiation, 14 weeks, 12 months, and 3 years. Predictors of primary nonresponse (discontinuation within 14 weeks) and durability were assessed.</p><p><strong>Results: </strong>A total of 456 patients initiated anti-TNF therapy (183 adalimumab and 273 infliximab). Thirty-seven (8%) patients were primary nonresponders. The 3-year drug durability for both therapies was >70%. Among patients with CD, the 3-year durability was >75% for both therapies. The 3-year durability with UC/IBD-U was 37% for adalimumab and 56% for infliximab. Predictors of primary nonresponse were an erythrocyte sedimentation rate > 55 mm/h in CD on infliximab, and baseline albumin <4 g/dL and <15.6 years at diagnosis in UC/IBD-U.</p><p><strong>Conclusions: </strong>Anti-TNF therapies had a 3-year durability of >75% in patients with CD, while the durability was lower (37%-56%) for patients with UC/IBD-U. Less than 10% of patients were considered primary nonresponders, which lends support to the long-term durability of anti-TNF therapies for pediatric IBD while keeping in mind predictive factors of Nonresponse.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"1024-1033"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675090","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}
引用次数: 0
Effect of prebiotics on gastrointestinal symptoms and quality of life in children with intestinal failure: A pilot study. 益生元对肠衰竭儿童胃肠道症状和生活质量的影响:一项初步研究
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-10-01 Epub Date: 2025-08-06 DOI: 10.1002/jpn3.70186
Rut Anne Thomassen, Janne Anita Kvammen, Beint S Bentsen, Marianne Bratlie, Siv Kjølsrud Bøhn, Hanne Farstad, Christian Kahrs, Linh Ngo, Camilla Nybø, Camilla Sæland, Erling Tjora, Rune Rose Tronstad, Ketil Størdal, Anne Charlotte Brun, Christine Henriksen
{"title":"Effect of prebiotics on gastrointestinal symptoms and quality of life in children with intestinal failure: A pilot study.","authors":"Rut Anne Thomassen, Janne Anita Kvammen, Beint S Bentsen, Marianne Bratlie, Siv Kjølsrud Bøhn, Hanne Farstad, Christian Kahrs, Linh Ngo, Camilla Nybø, Camilla Sæland, Erling Tjora, Rune Rose Tronstad, Ketil Størdal, Anne Charlotte Brun, Christine Henriksen","doi":"10.1002/jpn3.70186","DOIUrl":"10.1002/jpn3.70186","url":null,"abstract":"<p><strong>Objectives: </strong>Children with intestinal failure (IF) have a substantial disease burden, with significant gastrointestinal (GI) symptoms, abnormal stool patterns and reduced health-related quality of life (HRQOL). This study examined the effects of prebiotic supplementation on GI symptoms and HRQOL.</p><p><strong>Methods: </strong>An open-label, randomised controlled trial involving two phases. In Phase 1, children aged 1-18 years with IF received supplementation with a blend of prebiotics for 4 weeks. In Phase 2, participants were randomised to either continue supplementation or cease supplementation for 6 months to evaluate long-term effects. Primary end points included parent-reported GI symptoms and HRQOL, measured by PedsQL™ scales. Secondary end points were stool consistency and frequency, nutritional support and antibiotic use.</p><p><strong>Results: </strong>Out of 47 children completing Phase 1, 43 completed Phase 2 (24 in the intervention group, 19 in the control group). After 4 weeks, 60% reported reduced GI symptoms. By the end of Phase 2, the intervention group showed no significant changes in HRQOL score, but significant GI symptom improvements compared to controls (mean paediatric quality of life inventory GI score difference of 6.9, p = 0.01). Stool frequency decreased (median -1.0 vs. 0 stools/day, p = 0.003), and stool consistency normalised more frequently in the intervention group (42% vs. 6%, p = 0.02). No significant changes were noted in nutritional support or antibiotic use.</p><p><strong>Conclusion: </strong>While HRQOL remained unchanged, short- and long-term prebiotic supplementation significantly improved GI symptoms, stool frequency and stool consistency in children with IF, indicating its potential as a therapeutic option in paediatric IF.</p><p><strong>Clinical trial registration: </strong>ClinicalTrials.gov ID: NCT04981262 https://clinicaltrials.gov/study/NCT04981262?cond=intestinal%20failure&term=prebiotics&rank=2.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"975-985"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484718/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144794746","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}
引用次数: 0
Enhancing food insecurity screening in pediatric celiac disease using a quality improvement framework. 使用质量改进框架加强儿科乳糜泻的食品不安全筛查。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-10-01 Epub Date: 2025-07-21 DOI: 10.1002/jpn3.70169
Telly Cheung, Mala Setty, Erica Riray, Bradley Green, Sharad I Wadhwani, Namrata Patel-Sanchez
{"title":"Enhancing food insecurity screening in pediatric celiac disease using a quality improvement framework.","authors":"Telly Cheung, Mala Setty, Erica Riray, Bradley Green, Sharad I Wadhwani, Namrata Patel-Sanchez","doi":"10.1002/jpn3.70169","DOIUrl":"10.1002/jpn3.70169","url":null,"abstract":"<p><strong>Objectives: </strong>Food insecurity (FI) affects more than 18 million households in the United States. Children with celiac disease (CeD) depend exclusively on the gluten-free diet, yet gaps persist in screening for food-insecure households who could benefit from interventions. We developed a system to identify and address barriers to FI screening in a pediatric clinic for CeD.</p><p><strong>Methods: </strong>Using the Model for Improvement, we conducted quality initiatives for eligible households between January 1, 2024 and January 31, 2025. We surveyed the medical team to identify barriers to FI screening and designed a key driver diagram to inform plan-do-study-act cycles. We implemented a (1) validated screening form, (2) clinic flowsheet, (3) educational module, and (4) visual aid in the electronic health records. We measured the process of FI screening and documentation. We tracked outcomes on positive screens for general and gluten-free FI.</p><p><strong>Results: </strong>Among 100 eligible children, the majority were female (63.0%), other race (55.0%), non-Hispanic (66.0%), and primary English-speakers (80.0%); and 66 received FI screening (66.0%). We identified top team-reported barriers to FI screening: language barriers, lack of FI resources, forgetting, and discomfort with asking questions. Applying the model for Improvement, we improved median FI screening from 0.0% to ≥75.0% within 12 months and sustained screening rates for ≥6 months. The average general and gluten-free FI among screened households was 19.7% and 14.8%, respectively.</p><p><strong>Conclusions: </strong>We implemented a systematic framework to address team-reported barriers, helping to increase FI screening for children with CeD. Developing interventions to address FI may improve poor outcomes among food-insecure households.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"1060-1069"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675085","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}
引用次数: 0
Commentary on acid suppression after esophageal atresia repair-Some infants do benefit. 食道闭锁修复后抑酸效果评价——部分婴儿确实受益。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-10-01 Epub Date: 2025-07-31 DOI: 10.1002/jpn3.70174
Donald George, Daniel K Robie
{"title":"Commentary on acid suppression after esophageal atresia repair-Some infants do benefit.","authors":"Donald George, Daniel K Robie","doi":"10.1002/jpn3.70174","DOIUrl":"10.1002/jpn3.70174","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"911-912"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144760383","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}
引用次数: 0
North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition position paper on the therapeutic drug monitoring in pediatric inflammatory bowel disease. 北美儿科胃肠病学、肝病学和营养学学会关于儿童炎症性肠病治疗药物监测的立场文件。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-10-01 Epub Date: 2025-07-21 DOI: 10.1002/jpn3.70158
Lina M Felipez, Sabina Ali, Edwin F de Zoeten, Anne M Griffiths, Sandra C Kim, Ashish S Patel, Joel R Rosh, Jeremy Adler
{"title":"North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition position paper on the therapeutic drug monitoring in pediatric inflammatory bowel disease.","authors":"Lina M Felipez, Sabina Ali, Edwin F de Zoeten, Anne M Griffiths, Sandra C Kim, Ashish S Patel, Joel R Rosh, Jeremy Adler","doi":"10.1002/jpn3.70158","DOIUrl":"10.1002/jpn3.70158","url":null,"abstract":"<p><p>Inflammatory bowel diseases (IBD) require effective therapies to prevent morbidity and maintain quality of life. The introduction of biologic agents, beginning with monoclonal antibodies targeting tumor necrosis factor (TNF) alpha, has launched a new era of advancements that have markedly improved short- and long-term outcomes of Crohn's disease and ulcerative colitis. Along with these improvements, there have been challenges to address in optimizing use of biologic therapies in children with IBD. Young children may have rapid drug clearance, and growing children have changing medication needs related to changes in body size, metabolism, and development. For these and other reasons, one size (one dose) does not fit all. Therapeutic drug monitoring (TDM), which involves measurement of drug concentration in serum usually, typically at the predose trough, has emerged as a valuable tool for optimizing dosing and preventing pharmacokinetic failure. This society paper reviews the use of TDM, including target ranges during induction and maintenance therapy for anti-TNF agents and for emerging biologics. This report has been compiled by pediatric gastroenterologists on behalf of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition IBD committee after extensive review of the current literature. The purpose of this clinical position statement is to provide guidance to clinicians in the use of TDM to optimize the treatment of children with IBD.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"1100-1117"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484717/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682754","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}
引用次数: 0
Frailty in children with chronic liver disease: Growing evidence for an underexplored topic. 患有慢性肝病的儿童虚弱:越来越多的证据表明这是一个未被充分探索的话题。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-10-01 Epub Date: 2025-07-16 DOI: 10.1002/jpn3.70042
Angelo Di Giorgio, Claudia Mandato, Eberhard Lurz
{"title":"Frailty in children with chronic liver disease: Growing evidence for an underexplored topic.","authors":"Angelo Di Giorgio, Claudia Mandato, Eberhard Lurz","doi":"10.1002/jpn3.70042","DOIUrl":"10.1002/jpn3.70042","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"907-910"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144649791","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}
引用次数: 0
Splenic stiffness measurement by transient elastography for predicting varices in children with portal hypertension. 瞬态弹性图测量脾刚度预测门脉高压患儿静脉曲张。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-09-28 DOI: 10.1002/jpn3.70215
Janvi Sirwani, Samarendra Mahapatro, Aditi Kumar, Ranjan Patel, Manas Kumar Panigrahi
{"title":"Splenic stiffness measurement by transient elastography for predicting varices in children with portal hypertension.","authors":"Janvi Sirwani, Samarendra Mahapatro, Aditi Kumar, Ranjan Patel, Manas Kumar Panigrahi","doi":"10.1002/jpn3.70215","DOIUrl":"https://doi.org/10.1002/jpn3.70215","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the diagnostic accuracy of splenic stiffness measurement (SSM) using spleen-dedicated vibration-controlled transient elastography (VCTE) for predicting varices and varices needing treatment (VNT) in children with clinically evident portal hypertension (CEPH).</p><p><strong>Methods: </strong>In this single-centre prospective cross-sectional study, children aged 6 months to 14 years with portal hypertension meeting the criteria for CEPH were eligible. SSM was measured using the VCTE with a spleen-specific (100 Hz) probe. Esophagogastroduodenoscopy (EGD) was performed to detect varices and risk stratification. Diagnostic performance was assessed using receiver operating characteristic analysis and contingency tables.</p><p><strong>Results: </strong>Ninety-three consecutive children with CEPH underwent EGD, and successful SSM acquisition was achieved in 90 cases. The median (interquartile range) age of presentation was 6 (3-10) years. EGD identified varices in 79 (87.8%) and VNT in 57 (63.3%). Children with varices had higher median SSM values than those without (37.9 vs. 17.9 kPa; p <  0.001). Among those with varices, SSM values were higher in high-risk than in low-risk cases (42.9 vs. 21.9 kPa; p <  0.001). SSM demonstrated good accuracy for predicting varices (area under the curve: 0.86, 95% confidence interval: 0.76-0.97) with a sensitivity of 75.9% (65.0%-84.9%) and specificity of 90.9% (58.7%-99.8%) at a threshold of 31.1 kPa. For VNT, accuracy was 0.838 (0.702-0.877), with a sensitivity of 87.7% (76.3%-94.9%) and specificity of 72.7% (54.5%-86.7%) at the SSM cut-off of 31.4 kPa.</p><p><strong>Conclusion: </strong>In children with CEPH, spleen-dedicated VCTE estimated SSM to have a reasonably high accuracy in predicting varices and VNT. Integrating SSM into clinical practice could assist in the screening and management of portal hypertension in children.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186035","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}
引用次数: 0
Changes in intestinal microbiota in pediatric cystic fibrosis patients after 6 months of elexacaftor-tezacaftor-ivacaftor therapy. 儿童囊性纤维化患者6个月后肠道微生物群的变化。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-09-28 DOI: 10.1002/jpn3.70216
Isabel Gutiérrez-Díaz, Jose R Gutiérrez-Martinez, Ruth García-Romero, Saioa Vicente-Santamaría, Agustin De La Mano-Hernández, Encarnación Torcuato-Rubio, Maria Garriga-Garcia, Carmen Martin-Fernández, Natalia Baston-Paz, Clara Simon-Bernaldo de Quiros, Juan J Díaz-Martín, Susana Delgado-Palacios, David González-Jiménez
{"title":"Changes in intestinal microbiota in pediatric cystic fibrosis patients after 6 months of elexacaftor-tezacaftor-ivacaftor therapy.","authors":"Isabel Gutiérrez-Díaz, Jose R Gutiérrez-Martinez, Ruth García-Romero, Saioa Vicente-Santamaría, Agustin De La Mano-Hernández, Encarnación Torcuato-Rubio, Maria Garriga-Garcia, Carmen Martin-Fernández, Natalia Baston-Paz, Clara Simon-Bernaldo de Quiros, Juan J Díaz-Martín, Susana Delgado-Palacios, David González-Jiménez","doi":"10.1002/jpn3.70216","DOIUrl":"https://doi.org/10.1002/jpn3.70216","url":null,"abstract":"<p><strong>Objectives: </strong>Cystic fibrosis (CF) is frequently associated with gastrointestinal problems, including intestinal microbiota dysbiosis. Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, such as elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA), have demonstrated improvements in lung function, abdominal symptoms, and quality of life in patients with CF. However, the impact of these modulators on the intestinal microbiota in the pediatric population remains incompletely understood. The objective of this study was to characterize the changes in the intestinal microbiota of pediatric patients with CF after 6 months of treatment with ELX/TEZ/IVA.</p><p><strong>Methods: </strong>Thirty-one patients with CF, aged 6-18 years, were recruited. Stool samples were collected before the initiation of treatment and approximately 6 months thereafter. Microbiota analysis was performed using 16S rRNA gene amplicon sequencing. Statistical analyses were employed to evaluate changes in alpha and beta diversity and variations in the relative abundance of different bacterial taxa. Clinical variables such as concomitant use of azithromycin and probiotics were considered.</p><p><strong>Results: </strong>After 6 months of treatment, no significant changes in the alpha diversity were observed. However, alterations in bacterial composition were detected. A decrease in the abundance of potentially pathogenic bacteria, such as Enterobacteriaceae members (Escherichia/Shigella) was observed. The abundance of genus Blautia increased. Differential analysis according to antibiotic and probiotic consumption revealed specific changes in microbiota composition.</p><p><strong>Conclusions: </strong>ELX/TEZ/IVA therapy for 6 months induces changes in the intestinal microbiota composition of pediatric patients with CF, characterized by a reduction in potentially harmful bacteria and an increase in potentially beneficial bacteria. These findings suggest a modulation towards a healthier intestinal microbiota profile.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186039","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}
引用次数: 0
Diagnostic approaches for infants with cholestatic liver diseases: Position paper and perspectives of the Federation of International Societies of Pediatric Gastroenterology, Hepatology, and Nutrition. 婴儿胆汁淤积性肝病的诊断方法:国际儿科胃肠病学、肝病学和营养学学会联合会的立场文件和观点。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-09-22 DOI: 10.1002/jpn3.70207
Huey-Ling Chen, Sarah A Taylor, Way Seah Lee, Mirta Ciocca, Mohamed A El-Guindi, Surender K Yachha, Rima Fawaz, Veronica Botero, Suporn Treepongkaruna, Emmanuel Gonzales, Nedim Hadžić
{"title":"Diagnostic approaches for infants with cholestatic liver diseases: Position paper and perspectives of the Federation of International Societies of Pediatric Gastroenterology, Hepatology, and Nutrition.","authors":"Huey-Ling Chen, Sarah A Taylor, Way Seah Lee, Mirta Ciocca, Mohamed A El-Guindi, Surender K Yachha, Rima Fawaz, Veronica Botero, Suporn Treepongkaruna, Emmanuel Gonzales, Nedim Hadžić","doi":"10.1002/jpn3.70207","DOIUrl":"https://doi.org/10.1002/jpn3.70207","url":null,"abstract":"<p><p>Cholestasis in infancy is the most common manifestation of liver disease in children, with some patients progressing to cirrhosis or liver failure necessitating transplantation. Neonatal cholestasis remains a diagnostic challenge, as it requires differentiation of cholestatic infants from a large number of jaundiced newborns with benign causes. The first step is to diagnose patients with biliary atresia (BA) as early as possible to ensure timely surgery-Kasai portoenterostomy (KPE). Universal newborn screening using stool color cards or direct bilirubin measurements have been shown to identify patients before the onset of symptoms. Multiple diagnostic modalities, including clinical history, physical examination, laboratory tests, emerging biomarkers, imaging studies, and liver histopathology, can facilitate the decision for intraoperative cholangiography and potential corrective surgery. Advances in diagnostic testing, particularly genetic sequencing, have greatly enhanced our ability to evaluate and manage infants with cholestasis. Given highly variable resources and access to these new diagnostic modalities, local flexibility and adaptability should be implemented within each institution and medical care system to foster seamless collaboration between primary care physicians and specialized centers with expertise in genetic diagnosis, KPE, and liver transplantation. This report provides updates on the evaluation of neonatal cholestasis, including insights into screening, diagnosis, and genetic testing, along with future perspectives.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145113536","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}
引用次数: 0
Pediatric liver transplant outcomes: A comparative analysis of steatotic donor grafts. 儿童肝移植结果:脂肪变性供体移植的比较分析。
IF 2.6 3区 医学
Journal of Pediatric Gastroenterology and Nutrition Pub Date : 2025-09-22 DOI: 10.1002/jpn3.70213
Ahmad Anouti, Hamza Dahshi, Madhukar S Patel, Thomas G Cotter, Julie K Heimbach, Sara Hassan
{"title":"Pediatric liver transplant outcomes: A comparative analysis of steatotic donor grafts.","authors":"Ahmad Anouti, Hamza Dahshi, Madhukar S Patel, Thomas G Cotter, Julie K Heimbach, Sara Hassan","doi":"10.1002/jpn3.70213","DOIUrl":"https://doi.org/10.1002/jpn3.70213","url":null,"abstract":"<p><strong>Objectives: </strong>Hepatic steatosis impacts the quality of grafts, affecting transplant outcomes. Rising obesity rates and subsequent donor graft steatosis further influence the organ shortage crisis in pediatric liver transplantation (LT). Our study aimed to evaluate how donor steatosis modulates the outcomes of pediatric LT.</p><p><strong>Methods: </strong>We analyzed the United Network of Organ Sharing database for transplanted donor grafts from January 01, 2004, to April 30, 2024. We stratified pediatric (≤18 years) LT recipients into steatotic grafts, subdivided into <30% and ≥30%. Graft failure was assessed using Kaplan-Meier curves, and Cox proportional hazards models, with Lasso regression identifying key predictive variables. Gradient-boosting decision tree was used to assess the level of likelihood importance for post-LT survival.</p><p><strong>Results: </strong>Five hundred and ninety-five pediatric LT recipients were included; 62 (10.4%) received donors with steatosis levels ≥30%. Survival rates for steatotic grafts ≥30% were 93.5% at 1 year, 89.9% at 5 years, and 84.4% at 10 years, compared to 94.7%, 89.5%, and 85.2% respectively, among steatotic grafts <30% (p = 0.72, p = 0.92, and p = 0.92). Donor age (adjusted hazard ratio [aHR]: 1.01, 95% confidence interval [CI]: 1.01-1.03), donation after cardiac death (DCD) (aHR: 10.68, 95% CI: 3.27-34.86), and recipient life support (aHR: 1.95, 95% CI: 1.19-3.20) were associated with an increased risk of mortality.</p><p><strong>Conclusion: </strong>Steatotic grafts in pediatric patients had acceptable outcomes. Predictors of mortality in steatotic grafts, including donor age, DCD, and recipient life support, underscore the complex interplay of multiple factors in post-LT outcomes.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145113578","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}
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