Intestinal Failure (New York, N.Y.)最新文献

{"title":"INSIGHTS Intestinal Failure 2026: Fostering global collaboration and patient-centered innovation in Paris.","authors":"Francisca Joly, Lore Billiauws, Lisa Sharkey, Mihai Oltean","doi":"10.1016/j.intf.2026.100397","DOIUrl":"https://doi.org/10.1016/j.intf.2026.100397","url":null,"abstract":"","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"12 ","pages":"100397"},"PeriodicalIF":0.0,"publicationDate":"2026-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13673048/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149217701","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":"Patient perspectives on barriers to care and the perceived value of a direct-to-patient virtual learning program for chronic intestinal failure.","authors":"Elisa Fisher, Marion Winkler, Malvika Nair, Princess B Ballog, Marjorie Nisenholtz, Constantin T Yiannoutsos, Rocco Friebel, Kishore R Iyer","doi":"10.1016/j.intf.2026.100390","DOIUrl":"10.1016/j.intf.2026.100390","url":null,"abstract":"<p><strong>Background: </strong>There is a shortage of clinical expertise in chronic intestinal failure (CIF). We aimed to explore patient perspectives on the need for an evidence-based virtual tele-learning program to train patients in best practice CIF care using the ECHO™ Model. Findings were used to inform development of the Patient Intestinal Failure-ECHO (PIF-ECHO) Project.Material and Methods: Using a qualitative research design, this study included interviews with six CIF patient advocates and two virtual focus groups with nine individuals living with CIF who expressed interest in PIF-ECHO. Data were coded and analyzed using iterative thematic analysis to generate themes focused on perceived need for and value of PIF-ECHO.</p><p><strong>Results: </strong>Four main themes were identified: 1) lack of provider access and support for patients living with CIF, 2) high levels of patient responsibility for disease management, self-advocacy, and system navigation, 3) limited access to patient-facing information on best practice CIF care, and, 4) severe emotional strain. Perceived value of PIF-ECHO centered on the program's potential to improve knowledge and access to resources, which participants hypothesized would lead to improved capacity for disease self-management, and ability to advocate for their needs across healthcare settings. Findings informed the development of a PIF-ECHO logic model illustrating connections between program activities and anticipated outcomes.</p><p><strong>Conclusion: </strong>A patient-facing ECHO program for CIF has the potential to address patient-identified needs related to access to specialist expertise and patient education, ultimately improving CIF patients' ability to manage their own care and advocate for their needs across systems.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100390"},"PeriodicalIF":0.0,"publicationDate":"2026-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13476477/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148766274","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":"Weaning infants with short bowel syndrome associated intestinal failure from parenteral nutrition: An ERNICA multicentre survey.","authors":"Susan Hill, Rebecca Pulvirenti, Dominique Guimber, Sarah Macdonald, Cora Jonkers, Emmanuelle Echochard-Dugelay, Merit Tabbers, Giovanna Verlato","doi":"10.1016/j.intf.2026.100388","DOIUrl":"https://doi.org/10.1016/j.intf.2026.100388","url":null,"abstract":"<p><strong>Background: </strong>To explore and compare practices of European Intestinal Rehabilitation Centres participating in the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) intestinal failure (IF) working group when weaning short bowel syndrome (SBS)-IF infants from parenteral nutrition (PN).</p><p><strong>Material and methods: </strong>Questionnaires were distributed by email to 49 ERNICA centres. Details regarding oral/nasogastric (NG) feeding when weaning PN, use of protocols/guidelines, criteria for PN cycling/reducing infusion hours, dietary recommendations, use of synthetic glucagon-like peptide-2 (teduglutide), antibiotics for intestinal bacterial overgrowth and long-term follow-up practices were requested in children with neonatal onset SBS-IF. Descriptive statistical analysis was conducted.</p><p><strong>Results: </strong>Thirty-five/49centers from 15 countries participated. In non-thriving infants aged < 6months, 74.3% centres supplemented oral intake with NG feeding and 94.3% continued it for > 6months if needed. Local/national guidelines were used by 63% when weaning PN. Criteria for cycling PN included age (range: term-3 months), weight (3-4.5 kg), and enteral feed tolerance with 95% centres reducing PN infusion rate before disconnecting. In 88.6% centres patients were monitored until transitioned to adult care (even if weaned from PN). Dietary recommendations and antibiotic usage varied widely. Teduglutide was used in 23/25(92%) centres from 11 countries where it was approved and funded.</p><p><strong>Conclusion: </strong>A variety of nutritional strategies were used to wean PN in SBS-IF infants even in specialist centres using guidelines. NG-tube feeding was used alongside oral intake in most centres, but other practices differed widely. Multicentre studies investigating which practices are associated with the best outcome for infants with SBS-IF are needed to develop evidence-based guidelines.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100388"},"PeriodicalIF":0.0,"publicationDate":"2026-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13476471/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148766475","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":"Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) due to a de novo ACTG2 mutation: A neonatal case report.","authors":"Tommaso Amato, Michele Maria Cantagalli, Marco Di Mitri, Riccardo Coletta, Antonino Morabito","doi":"10.1016/j.intf.2026.100386","DOIUrl":"10.1016/j.intf.2026.100386","url":null,"abstract":"<p><strong>Background: </strong>Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital visceral myopathy characterized by severe gastrointestinal dysmotility and bladder dysfunction, most commonly associated with <i>ACTG2-</i>mutations.</p><p><strong>Case report: </strong>We report a preterm neonate with prenatally detected megacystis and a de novo heterozygous <i>ACTG2</i> variant (c.593G>T; p.Gly198Val). Postnatally, the patient developed progressive abdominal distension and feeding intolerance. Imaging demonstrated dilated bowel loops and microcolon. Exploratory laparotomy on day 5 confirmed dilated ileum, microcolon, and massive megacystis without mechanical obstruction. Gastrostomy and ileostomy were performed. The patient required parenteral nutrition followed by gradual enteral feeding with stool recirculation. Clean intermittent catheterization and antibiotic prophylaxis were initiated.</p><p><strong>Conclusion: </strong>MMIHS should be suspected in cases of fetal megacystis. Early genetic diagnosis and multidisciplinary management, including intestinal failure rehabilitation and urological care, are essential to improve outcomes.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100386"},"PeriodicalIF":0.0,"publicationDate":"2026-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13446039/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148687507","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":"Filling the gaps: Information sources and needs of patients with intestinal failure and their caregivers.","authors":"Marie L Neumann, Katie E Corcoran, Jessica Y Allen, Swapna R Kakani","doi":"10.1016/j.intf.2026.100385","DOIUrl":"10.1016/j.intf.2026.100385","url":null,"abstract":"<p><strong>Background: </strong>Health information-seeking is a central strategy used by patients and caregivers to cope with chronic illness. This study investigated the sources of disease-specific information utilized by adult patients with IF and caregivers of pediatric IF patients to identify existing information needs.</p><p><strong>Materials and methods: </strong>Using a community-driven research design, we developed a cross-sectional questionnaire to identify information sources and needs. The survey was disseminated via relevant patient support organizations, personal networks, and disease-specific social media support groups. Descriptive and univariate analyses were conducted to identify common information sources and needs and assess differences by respondent type.</p><p><strong>Results: </strong>A total of 283 respondents, consisting of adult patients with a history of IF (n = 167) and caregivers of pediatric patients with a history of IF (n = 116), completed the survey. Only half were receiving care via an intestinal rehabilitation program. The most frequently used sources of IF-specific information were specialist providers or teams (64%) and social media support groups (52%); a significantly larger share of patients than caregivers reported general practitioners as an information source (30% vs. 13%, p = 0.001). Top information needs pertained to 1) better understanding the current state of the field, 2) optimizing day-to-day life with IF and improving quality of life, and 3) supporting mental health and fostering a sense of connection.</p><p><strong>Conclusion: </strong>Within this sample of engaged and resource-seeking participants, we found respondents utilized multiple information sources and expressed a range of information needs. We offer several suggestions for addressing these needs.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100385"},"PeriodicalIF":0.0,"publicationDate":"2026-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13382461/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148612180","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 memorial tribute to Dr Theresa Byrne, PhD, RD.","authors":"Maria Karimbakas, Kishore Iyer","doi":"10.1016/j.intf.2026.100384","DOIUrl":"10.1016/j.intf.2026.100384","url":null,"abstract":"","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100384"},"PeriodicalIF":0.0,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13351155/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148428422","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":"Motor and cognitive outcomes in pediatric intestinal failure: A longitudinal cohort study.","authors":"Anna Gold, Catherine Patterson, Christina Belza, Dylan Johnson, Bianca C Bondi, Alaine Rogers, Paul W Wales, Yaron Avitzur, Stephanie So","doi":"10.1016/j.intf.2026.100383","DOIUrl":"10.1016/j.intf.2026.100383","url":null,"abstract":"<p><strong>Background: </strong>Improved survival in pediatric intestinal failure (IF) necessitates a focus on neurodevelopmental outcomes. Existing cross-sectional research demonstrates increased risk of developmental delay. This novel study explores longitudinal motor and cognitive outcomes, and associated risk factors, in a single cohort across infancy to early school age.</p><p><strong>Material and methods: </strong>Retrospective review of children admitted to an IF program < 4 months of age between 2011 and 2016, who completed: motor assessment between 4 and 15 months corrected age (CA), motor and cognitive assessment between 12 and 32 months CA and 4-8 years. Descriptive analysis of medical, socio-demographic variables and assessment scores. Outcomes were assessed using linear mixed methods, with multi-variable analyses to explore main effects and significant interactions with time.</p><p><strong>Results: </strong>Study population included 41 children (56% male), median (IQR) gestational age 33 (28.5, 35.5) weeks and length of first year hospitalization 209 (140.8, 255) days. Five children (12%) had central nervous system (CNS) co-morbidities. Gross motor scores were below average at pre-school time-points, with significant improvement by school age; whereas fine motor and cognitive scores significantly decreased at 26-32 months CA, compared to largely average scores at 12-15-months CA and school age. Main effects predicting poorer scores included: birth weight, early motor delay, CNS co-morbidity, auditory impairment, NEC diagnosis, surgeries, sepsis, and prolonged hospitalization.</p><p><strong>Conclusions: </strong>Children with IF demonstrate developmental vulnerably in infancy and preschool years, with promising motor and cognitive gains observed by early school age. Identification of key risk factors can guide IF teams in providing targeted therapeutic interventions, mitigating longer term developmental risks.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100383"},"PeriodicalIF":0.0,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13332454/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148393295","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":"Clinical outcomes of teduglutide therapy in children with short bowel syndrome-associated intestinal failure: A single-center experience.","authors":"Mariah Mukasa, Hadar Moran-Lev, Lindsey Hornung, Samuel A Kocoshis, Stephanie B Oliveira, Michael A Helmrath, Paul W Wales, Conrad R Cole","doi":"10.1016/j.intf.2026.100382","DOIUrl":"10.1016/j.intf.2026.100382","url":null,"abstract":"<p><strong>Background: </strong>Short bowel syndrome-associated intestinal failure (SBS-IF) requires long-term parenteral nutrition (PN), increasing the risk of complications such as central line associated bloodstream infections, sepsis and liver disease. Teduglutide, a glucagon-like peptide-2 (GLP-2) analog, has been shown to enhance intestinal adaptation and reduce PN dependence, but long-term real-world data in children are limited.</p><p><strong>Objectives: </strong>To describe the medium-term clinical outcomes and safety profile of teduglutide treatment in children with short bowel syndrome-associated intestinal failure (SBS-IF).</p><p><strong>Methods: </strong>A retrospective cohort study was conducted at a single tertiary center. There were 41 children with SBS-IF treated with Teduglutide among the 161children who were managed at the center between 2018 and 2022. Data on PN requirements, growth parameters, and treatment duration were analyzed. The time to PN weaning was assessed using a Cox proportional hazards model.</p><p><strong>Results: </strong>At a median follow-up of 29.2 months, 21 patients (51.2%) were successfully weaned off PN, and 24 patients (65%) achieved at least a 20% reduction in PN requirements. The median time to complete PN weaning was 7.3 months. Baseline variables associated with time to PN discontinuation included the percentage of PN-derived calories at treatment initiation and residual small bowel length. No treatment-related adverse events were reported, although 12% of patients required increased PN support over time.</p><p><strong>Conclusions: </strong>Teduglutide reduced PN dependence in children with SBS-IF, with over half achieving enteral autonomy. These findings support its role in intestinal rehabilitation and emphasize the need for individualized treatment strategies based on baseline clinical characteristics associated with response.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100382"},"PeriodicalIF":0.0,"publicationDate":"2026-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13273769/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148280664","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":"De novo activating GUCY2C variant with prenatal bowel dilatation, neonatal functional obstruction, and reversible intestinal failure: A case report.","authors":"Michele Maria Cantagalli, Tommaso Amato, Sofia Chioccioli, Riccardo Coletta, Antonino Morabito","doi":"10.1016/j.intf.2026.100376","DOIUrl":"10.1016/j.intf.2026.100376","url":null,"abstract":"<p><strong>Background: </strong>Activating variants in GUCY2C increase cyclic GMP signaling in enterocytes, driving chloride rich luminal secretion and impaired sodium absorption. Affected neonates can present with prenatal bowel dilatation and severe postnatal fluid losses that mimic mechanical obstruction.</p><p><strong>Case report: </strong>We describe a term male infant with fetal ultrasound and MRI evidence of markedly dilated bowel loops and severe polyhydramnios. Fetal testing identified a de novo heterozygous GUCY2C missense variant (c.2732 A>G). After birth, profuse intestinal secretion combined with profound intestinal hypotonia led to acute functional obstruction and massive ileal dilatation, prompting urgent laparotomy and diverting ileostomy. No mechanical stenosis was found; the ileocecal valve and colon were patent. Histology showed eosinophilic enteritis with prominent ganglionitis and preserved ganglion cells. The infant required prolonged parenteral nutrition with gradual advancement of enteral feeds and fecal recycling through the distal mucous fistula to achieve enteral autonomy by 2 years of age. He is currently 3 years old.</p><p><strong>Conclusion: </strong>GUCY2C mediated secretory diarrhea may be complicated by severe dysmotility and inflammatory enteric neuropathy, requiring early decompression and prolonged intestinal rehabilitation before recovery.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100376"},"PeriodicalIF":0.0,"publicationDate":"2026-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13273893/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148280695","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":"Neonatal growth with fish oil or mixed oil lipid emulsions in intestinal failure associated liver disease.","authors":"Emily A Gutzwiller, Katie A Huff","doi":"10.1016/j.intf.2026.100381","DOIUrl":"10.1016/j.intf.2026.100381","url":null,"abstract":"<p><strong>Background: </strong>Treatment strategies for intestinal failure associated liver disease (IFALD) include use of fish-oil lipid emulsion (FO-ILE) or mixed-oil emulsion containing soy, medium chain triglyceride, olive, and fish oils (SO,MCT,OO,FO-ILE). FO-ILE is limited to 1 g/kg/day, either limiting overall calorie provision or shifting calories toward carbohydrate predominance. The objective of this project was to compare the growth of neonates with IFALD receiving either SO,MCT,OO,FO-ILE or FO-ILE.</p><p><strong>Material and methods: </strong>A retrospective chart review was conducted of neonates with IFALD receiving SO,MCT,OO,FO-ILE or FO-ILE. Growth metrics, nutritional data, and lab values were analyzed using the Mann-Whitney or t-test with a p-value < 0.05 used for significance.</p><p><strong>Results: </strong>We included 46 patients (22 SO,MCT,OO,FO-ILE; 24 FO-ILE). Both groups had similar weight gain (SO,MCT,OO,FO-ILE 24.6 vs FO-ILE 31.6 g/day; p = 0.121), although the FO-ILE patients had increased weight z-scores over time (SO,MCT,OO,FO-ILE: -0.11 vs FO-ILE: 0.09 p = 0.032). The FO-ILE group received fewer calories (SO,MCT,OO,FO-ILE:112 vs FO-ILE:100.7 kcal/kg/day; p = 0.004), less lipid (SO,MCT,OO,FO-ILE: 2.1 vs FO-ILE 1.0 g/kg/day; p < 0.001), and more carbohydrate (SO,MCT,OO,FO-ILE: 9.6 vs FO-ILE: 12.8 mg/kg/min; p < 0.001).</p><p><strong>Conclusion: </strong>Neonates receiving FO-ILE had similar weight gain to those receiving SO,MCT,OO,FO-ILE while receiving fewer overall calories and parenteral lipid. The FO-ILE group did have increased weight z-score over time. Further investigation is needed to understand the influence of these differences on additional short-term and long-term outcomes.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"11 ","pages":"100381"},"PeriodicalIF":0.0,"publicationDate":"2026-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13276606/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148298782","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}