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ObjectiveTo evaluate associations between neighborhood income and burden of hospitalizations for children with short bowel syndrome (SBS).Study designWe used the Pediatric Health Information System (PHIS) database to evaluate associations between neighborhood income and hospital readmissions, readmissions for central line-associated bloodstream infections (CLABSI), and hospital length of stay (LOS) for patients

IntroductionSevere forms of short bowel syndrome (SBS) resulting in chronic intestinal failure (IF) have limited therapeutic options, all of which are associated with significant morbidities. Spring-mediated distraction enterogenesis (SMDE) uses an intraluminal self-expanding spring to generate mechanical force to induce intestinal stretching and sustained axial growth, providing a promising novel approach for patients with SBS. Previous studies have established this method to be safe and effective in small and large animal models. However, SMDE has previously not been implemented in a large, clinically relevant animal model.MethodsJuvenile mini-Yucatan pigs with 75% of their small intestine resected had intraluminal springs placed after an initial adaptive period. Morphological and histological assessments were performed on SMDE segments compared to the control region of the intestine undergoing normal adaptive responses to resection.ResultsWhile the initial histologic adaptive response observed following resection was attenuated after a month, the SMDE segments instead augmented these adaptive changes. Specifically, intestinal length increased 2-fold in SMDE segments, and the widths of the epithelial, muscularis, and serosal layers were enhanced in SMDE compared with control segments of the same animal. This data suggests that morphologic intestinal adaptation may be enhanced with SMDE in the setting of SBS.DiscussionHere we demonstrate the successful and reproducible implementation of SMDE in a large animal model in the setting of prior intestinal resection, making SMDE a viable and novel approach for SBS to be explored further.

Background Aim Methods Results Conclusions Central line‐associated bloodstream infections (CLABSIs) are a potential complication for home parenteral nutrition (HPN) patients.We sought to analyse risk factors of developing HPN‐related CLABSI and assess CLABSI management in the Australian context.A retrospective observational cohort study was conducted on 34 adult patients receiving HPN via a central venous catheter (CVC) at a Queensland tertiary referral centre between 2016 and 2023. Patient charts were reviewed, and Kaplan–Meier analysis was employed to determine associations between characteristics and time to CLABSI in the first CVC using Peto‐Peto Prentice test.A total of 39 CLABSI episodes occurred in 19 patients. Patients with ≥1 CLABSI used regular opioids more than those without CLABSI (P = 0.016). Fourteen (41%, n = 14/34) patients developed a CLABSI in their first CVC. No patient or line characteristics were found to be predictive of CLABSI in their first CVC. The CLABSI rate was 1.02/1000 catheter days. Most CLABSIs were caused by Enterobacterales (22%, n = 12/55) and Candida sp. (22%, n = 12/55). Empiric antimicrobial therapy was adequate in only 25% (n = 7/28), and the median time to effective antibiotic therapy was 22.7 h (interquartile range 4.8–29.8). There were three successful CVC salvages (8%, n = 3/39).In this cohort of patients, regular opioid use was associated with increased risk of developing CLABSI. Based on our findings, an empiric antimicrobial regime of vancomycin, cefepime and caspofungin will provide adequate coverage for most HPN‐related CLABSIs in Australian IF units with a similar antimicrobial distribution and resistance pattern. [ABSTRACT FROM AUTHOR]

Background: This study aimed to gain real-world insights from healthcare professionals (HCPs) regarding the management of adult patients with short bowel syndrome and intestinal failure (SBS-IF) who received teduglutide and achieved parenteral support (PS) independence or PS volume stability for ≥12 months. Methods: This cross-sectional survey was conducted in five European countries and Canada via a self-reported questionnaire (November 2022–March 2023) among HCPs who manage patients with SBS-IF and who had prescribed teduglutide to ≥5 patients with SBS-IF receiving PS. Results: Of the 70 HCPs who completed the survey, almost all reported managing patients with SBS-IF who achieved PS independence or PS volume stability (99%, 69/70 and 97%, 68/70, respectively) and maintained the standard teduglutide dose, without changes. A total of 52 HCPs managed patients who achieved PS independence and discontinued teduglutide. Of these HCPs, 73% (38/52) anticipated that these patients would remain PS-independent, not requiring PS reintroduction. Of the remainder, 79% (11/14) estimated that ≤40% of these patients would require PS reintroduction. While many HCPs (81%, 42/52) would reintroduce teduglutide in patients who discontinued its use after achieving PS independence, none would do so for patients who discontinued teduglutide after achieving PS volume stability if a patient's condition worsened. Conclusions: This survey found that patients with SBS-IF can achieve PS independence or PS volume stability with teduglutide treatment. However, some HCPs (27%, 14/52) believe that a proportion of patients discontinuing teduglutide after achieving PS independence will require PS reintroduction. This survey suggests that teduglutide treatment should continue uninterrupted, unless clinically indicated, but this requires confirmation in future studies. [ABSTRACT FROM AUTHOR]

Background: Short bowel syndrome is considered a low prevalence disease. The scant information available about intestinal failure in Latin America was the driving force to expand this registry. Methods: A prospective, multicenter observational registry was created for patients with chronic intestinal failure short bowel at specialized centers in Latin America. Demographics, clinical characteristics, nutrition assessment, parenteral nutrition management, intestinal rehabilitation, related complications, clinical outcome, and survival were analyzed. Results: From May 2020 to July 2023, 167 patients (115 adults, 52 children) from 20 centers were enrolled. For the adults, the mean age was 37.2 ± 18 years, 48% were female, and the mean follow‐up was 22.6 ± 18.3 months. The main etiology was surgical resections (postsurgical complications: 37%; ischemia: 25%); the mean intestinal length was 73 ± 55 cm. The complications were as follows: infections: 0.4/1000 catheter‐days; thrombosis: 0.24/1000 catheter‐days; liver disease: 2.6%. The outcomes were as follows: 28% were rehabilitated, 15% died, 9.6% were lost to follow‐up, 0.9% underwent transplant, and 45.6% continued follow‐up. For the children, the mean age 48 ± 52 months, 48% were female, 52% were premature. The mean follow‐up was 17.2 ± 5.6 months; the mean remaining intestinal length was 38 ± 45 cm. The leading etiologies were atresia (25%), NEC (23%), and gastroschisis (21%). The complication were as follows: infections: 2/1000 catheter‐days; thrombosis: 2.22/1000 catheter‐day; 25% developed liver disease. The outcomes were as follows: 7.7% died, 3.8% were rehabilitated, and 88.5% continued follow‐up. Conclusion: The RESTORE amendment served as a registry and educational tool for the participating teams. The aspiration is to objectively show current aspects of intestinal failure in the region and carry them to international standards. Including all Latin American countries and etiologies of chronic intestinal failure besides short gut would serve to complete this registry. [ABSTRACT FROM AUTHOR]

Background: Serial transverse enteroplasty is used to treat patients with chronic intestinal failure owing to short bowel syndrome. Current literature lacks discussion of its role for other etiologies of intestinal failure and its impact on adult patients' nutrition support needs and quality of life. Methods: We performed a case series on adults with parenteral nutrition (PN) dependence who underwent serial transverse enteroplasty at Emory University Hospital, a quaternary referral center between 2011 and 2022. Data collected included demographics, operative technique, and preoperative and postoperative PN requirements. A phone survey was administered to evaluate the impact of PN and the operation on quality of life. Results: Ten patients underwent the procedure of interest during the study period. Indications included short bowel syndrome following multiple abdominal operations or intra‐abdominal catastrophe and chronic partial bowel obstruction with dysmotility. Bowel length increased by a median of 83%. All patients were discharged home after a median hospital stay of 21 days. At 1‐year follow‐up, survival was 100%, two (20%) patients fully weaned from PN, three others (30%) reduced PN frequency, and six (60%) decreased their daily parenteral energy requirement. Two additional patients fully weaned from PN by 18 months postoperatively. Conclusion: This represents one of the largest case series of serial transverse enteroplasty in adults. Small intestinal length nearly doubled, and PN dependence was reduced in most patients. Given the low morbidity and good quality of life observed in this series, this procedure should be more widely investigated for patients with chronic intestinal failure. [ABSTRACT FROM AUTHOR]

Intestinal failure (IF) is a debilitating condition characterized by the insufficient function of the gastrointestinal tract to absorb nutrients and fluids essential for life. This review consolidates recent advancements and challenges in managing IF among adult and pediatric populations, highlighting differences in etiology, management, and outcomes. Over the recent years, significant strides have been made in the nutritional and medical management of IF, significantly reducing mortality rates and improving the quality of life for patients. Key advancements include the development and availability of glucagon-like peptide-2 (GLP-2) analogs, improved formulations of parenteral nutrition, and the establishment of specialized interdisciplinary centers. Short bowel syndrome (SBS) remains the predominant cause of IF globally. The pediatric segment is increasingly surviving into adulthood, presenting unique long-term management challenges that differ from adult-onset IF. These include the need for tailored nutritional support, management of IF-associated liver disease, and addressing growth and neurodevelopmental outcomes. The therapeutic landscape for IF continues to evolve with the development of new treatment modalities and better understanding of the condition's pathophysiology. However, disparities in treatment outcomes between children and adults suggest the need for age-specific management strategies. This review underscores the importance of a nuanced approach to IF, incorporating advancements in medical science with a deep understanding of the distinct needs. [ABSTRACT FROM AUTHOR]

Short bowel syndrome is the most common reversible cause of intestinal failure. Most of the children are started on parenteral nutrition (PN) after surgery to enable growth and allow time for intestinal adaptation. This is a process whereby the shorter length of bowel is able to achieve complete function as if the entire length of bowel is present. With management advances, most children with short bowel syndrome can discontinue PN and establish full enteral feeds. This article mainly focuses on the complications of short bowel syndrome that need to be avoided for the child to achieve intestinal adaptation, establish on enteral feeds/oral diet and achieve enteral autonomy. [ABSTRACT FROM AUTHOR]

Background: Pediatric patients with intestinal failure require long‐term parenteral nutrition owing to impaired enteral nutrition absorption. A potential complication is essential fatty acid deficiency (EFAD), resulting from decreased linoleic and α‐linolenic acid concentrations and defined by an increased triene:tetraene ratio (TTR; Mead acid:arachidonic acid). Historically, soybean oil lipid emulsion (SOLE) was the only commercially available parenteral lipid in the United States. Recently, a composite lipid emulsion (CLE) and fish oil lipid emulsion (FOLE) received US Food and Drug Administration approval. This study investigated whether lipid emulsion regimen impacts EFAD incidence in pediatric patients with intestinal failure. Methods: This study was a 10‐year retrospective cohort study of pediatric patients with intestinal failure who received parenteral SOLE, CLE, or FOLE. The primary outcome was EFAD incidence, defined as a TTR ≥ 0.2. Secondary outcomes included TTR ≥ 0.05, cholestasis incidence, lipid dose effect on EFAD incidence, and fatty acid parameter differences. Results: A total of 144 fatty acid profiles from 47 patients were reviewed. EFAD did not occur in any lipid emulsion group. There were no differences in the incidence of TTR ≥ 0.05 or cholestasis. The effect of dose could not be evaluated because of no EFAD incidence. Lastly, although each group had varied fatty acid parameters, none saw decreased essential fatty acid levels. Conclusion: This study found that, with close monitoring, the lipid emulsion regimen did not impact EFAD incidence. This suggests that FOLE and CLE do not increase EFAD risk compared with SOLE in pediatric patients with intestinal failure. [ABSTRACT FROM AUTHOR]

Aim Method Results Conclusion The aim of this work was to investigate the incidence of enterocutaneous fistula (ECF), including both small bowel and colonic fistulas, in a defined population of 1.04 million during a 10‐year period and to describe aetiology, treatments, care consumption and outcome.A comprehensive search algorithm including diagnostic and procedural codes, enterostomal therapy nurse notes and in‐hospital care for >60 days yielded 1970 search hits. After reviewing medical records, 187 patients with ECF were identified.The annual incidence of ECF was 2.3 per 100 000, the incidence of ECF with intestinal failure type II was 0.9 per 100 000. Spontaneous closure of the fistula occurred in 16.0% of patients, while closure was seen in 97.3% of patients who underwent reconstructive surgery with recurrences in 6.7% and 8.3%, respectively. Cumulative ECF‐related in‐hospital care until closure or end of follow‐up was 4 (range 0–61) weeks. Eighty‐eight patients (47%) received home‐based healthcare including parenteral feeding and/or fistula wound care. The estimated overall mortality at 1, 3 and 5 years was 33.7%, 42.1% and 47.6% respectively. Mortality was mainly in patients without spontaneous closure or reconstructive surgery, and the risk of ECF‐related death was 30.2%.This study defines the population‐based incidence of ECF and reports a high overall mortality rate. Initial survivors were characterized by either spontaneous closure or eligibility for later reconstructive surgery, but with an eventual mortality rate of approximately 20%. ECF patients are high consumers of care: 55.1% needed ≥4 weeks in hospital and many received home‐based healthcare. [ABSTRACT FROM AUTHOR]

Purpose of Review: Intestinal failure (IF), a rare organic dysfunction, is characterized by a reduction in intestinal function, necessitating intravenous supplementation to sustain health and/or growth due to inadequate absorption of macro and micronutrients, water, and electrolytes. This condition is distinct from "Intestinal Insufficiency," where intravenous replacement is not immediately necessary. The purpose of this review is to evaluate the literature published in the last 5 years on drug treatment in the management of patients with intestinal failure (IF) and short bowel syndrome (SBS) and to identify strategies to minimize fluid and electrolyte loss by increasing nutrient-enterocyte contact time, improving intestinal transit time, replenishing micronutrients, and enhancing nutrient absorption. Recent Findings: We describe current treatments for rehabilitation aimed at optimizing absorption of the intestinal mucosa. We also outline recommended pharmacological adjustments of some drugs used in the treatment of commonly found comorbidities in these patients. Recent articles have demonstrated that GLP-2 analogs have become an important tool in the treatment of SBS, improving intestinal secretory control, gastrointestinal transit time, nutrient absorption, and, most importantly, positively impacting quality of life. Summary: IF is a rare disease defined by need for intravenous hydration and nutrients delivery and associated with malnutrition and high mortality rate. Medical management and treatment of IF can increase survival time and quality of life. [ABSTRACT FROM AUTHOR]

Pediatric chronic intestinal failure (PIF) is a rare and heterogeneous condition characterized by the inability of the patient's intestine to adequately absorb the required fluids and/or nutrients for growth and homeostasis. As a result, patients will become dependent on home parenteral nutrition (HPN). A MEDLINE search was performed in May 2024 with keywords "intestinal failure", "parenteral nutrition" and "pediatric". Different underlying conditions which may result in PIF include short bowel syndrome, intestinal neuromuscular motility disorders and congenital enteropathies. Most common complications associated with HPN are catheter-related bloodstream infections, catheter-related thrombosis, intestinal failure-associated liver disease, small intestinal bacterial overgrowth, metabolic bone disease and renal impairment. Treatment for children with PIF has markedly improved with a great reduction in morbidity and mortality. Centralization of care in specialist centers and international collaboration between centers is paramount to further improve care for this vulnerable patient group. A recently promising medical therapy has become available for children with short bowel syndrome which includes glucagon-like peptide 2, a naturally occurring hormone which is known to delay gastric emptying and induce epithelial proliferation. Despite advances in curative and supportive treatment, further research is necessary to improve nutritional, pharmacological and surgical care and prevention of complications associated with parenteral nutrition use. [ABSTRACT FROM AUTHOR]

Objective: To compare the phase angle (PhA) through bioelectrical impedance (BIA) of children with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by an Intestinal Rehabilitation Program, with a control group. Methods: Children under 10 years of age with IF using prolonged PN for >60 days (study group) were included. The control group consisted of healthy children without chronic pathologies, matched by sex and age. Anthropometric parameters evaluated were: weight, height, weight/ age z-score (W/A), height/age z-score (H/A), BMI, BMI/A z-score, arm circumference, triceps skinfold, subscapular skinfold, mid-arm muscle circumference. BIA parameters were resistance (R), reactance (Xc), and phase angle (PhA). Results: Twenty-eight children were included in the study group, median (IQR) age was 11 (8(27) months, 53.6 % were male. In the control group, 28 children were included, median (IQR) age was 12.5 (8(24.7) months, 50 % were male. Children from the study group had W/A z-scores and H/A z-scores significantly lower than controls. There was no significant difference between PhA in the study group and controls, [median (IQR) 4.3° (3.8;4.6) vs 4.0° (3.8;5.4) respectively, p = 0.980]. Prematurity was significantly higher in the study group than in the controls, but there was no significant correlation between gestational age at birth and PhA of the children from the study group. [ABSTRACT FROM AUTHOR]

Joel Faintuch,1 Andre Dong Won Lee,2 Salomao Faintuch,3 Asher Mishaly,4 Francisco Juarez Almeida Karkow5 1Department of Gastroenterology, Sao Paulo University Medical School, Sao Paulo, SP, Brazil; 2Liver and Digestive Organs Transplantation Service, Hospital Das Clinicas, Sao Paulo, Brazil; 3Interventional Radiology, Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA; 4Private Practice, Internal Medicine, Sao Paulo, Brazil; 5Department of Clinical Nutrition, Fatima Faculty of Nutrition, Caxias do Sul, RGS, BrazilCorrespondence: Joel Faintuch, Senior Professor of Gastroenterology, Hospital das Clínicas, Avenida Eneias C. Aguiar 255, 9th Floor, Rm 9077, Sao Paulo, SP, 05403-000, Brazil, Email faintuchj@gmail.comAbstract: As recently as 1990, short bowel syndrome in infants with less than 6 cm of remaining small intestine beyond the ligament of Treitz was defined as irreversibly lethal, and withholding further treatment was considered reasonable at that time (Reference deleted, Reviewer 2 item 1). Intestinal transplantation was already available, however not highly reliable. Nowadays transplantation is associated with long-term survival, even though indications are not expanding. On the contrary, they are shrinking, particularly for children as non-transplant handling. Surgical lengthening of the remaining gut, and more recently by enterohormone supplementation to stimulate diarrhea reversal and gut rehabilitation, is permitting encouraging rates of long-term survival. The purpose of this study was to review current non-surgical interventions aiming at parenteral nutrition weaning and intestinal failure reversal in the short bowel syndrome population, with emphasis on pediatric cases.Keywords: intestinal failure, gut rehabilitation, enterohormones, GLP-2, GLP-1, food supplements, short bowel syndrome

Objective: To compare the phase angle (PhA) through bioelectrical impedance (BIA) of children with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by an Intestinal Rehabilitation Program, with a control group. Methods: Children under 10 years of age with IF using prolonged PN for >60 days (study group) were included. The control group consisted of healthy children without chronic pathologies, matched by sex and age. Anthropometric parameters evaluated were: weight, height, weight/age z-score (W/A), height/age z-score (H/A), BMI, BMI/A z-score, arm circumference, triceps skinfold, subscapular skinfold, mid-arm muscle circumference. BIA parameters were resistance (R), reactance (Xc), and phase angle (PhA). Results: Twenty-eight children were included in the study group, median (IQR) age was 11 (8–27) months, 53.6 % were male. In the control group, 28 children were included, median (IQR) age was 12.5 (8–24.7) months, 50 % were male. Children from the study group had W/A z-scores and H/A z-scores significantly lower than controls. There was no significant difference between PhA in the study group and controls, [median (IQR) 4.3° (3.8;4.6) vs 4.0° (3.8;5.4) respectively, p = 0.980]. Prematurity was significantly higher in the study group than in the controls, but there was no significant correlation between gestational age at birth and PhA of the children from the study group. Conclusion: Children with IF using prolonged PN showed lower W/A and H/A compared to the control group, but without significant difference between the PhA of children with IF compared to controls.

Background: Short bowel syndrome is a devastating malabsorptive condition and the most common cause of chronic intestinal failure (CIF). Patients need parenteral support for months or years. Ideally, it should be delivered at home, reducing limitations in everyday life activities. Summary: The Portuguese Health Directive 017/2020 was the first step in the regulation of home CIF management, and more patients are now being treated in an ambulatory setting. However, much work still needs to be performed in this area. Our country lacks a network of units capable of providing home parenteral nutrition (HPN), and only a few centers have expertise to take care of these complex patients: fluid support, oral, enteral, and parenteral nutrition; disease/HPN-related complications; pharmacologic treatment; and surgical prevention/treatment. Providing adequate transition from pediatric to adult care is a mandatory issue that should only be addressed by expert centers. Key Messages: Implementation of a national network, as well as the creation of an intestinal failure registry, with an initial focus on adult patients, will start a new era in the identification and management of these complex CIF patients.

Purpose: Patients with intestinal failure (IF) require long-term parenteral nutrition using central venous catheters (CVCs), which often require replacement. We adopted a less fallible guidewire replacement (GWR) method and verified its effectiveness and validity. Methods: We enrolled 108 cases that underwent a CVC replacement with "GWR" method with IF at our department between 2013 and 2023. We retrospectively reviewed patients' clinical details with tunneled CVC (Hickman/Broviac catheter). For the analysis, we compared for the same time period the catheter exchange method "Primary placement"; newly inserted catheter by venipuncture. Results: The success rate of catheter replacement using GWR was 94.4%. There were six unsuccessful cases. A log-rank test showed no significant difference in catheter survival between primary placement and the GWR, and the time to first infection was significantly longer in the GWR (p = 0.001). Furthermore, no significant differences were observed between the two methods until the first infection, when the exchange indication was limited to infections. In the same way, when the indication was restricted to catheter-related bloodstream infection, there was no significant difference in catheter survival between the two approaches. Conclusion: Our GWR procedure was easy to perform and stable, with a high success rate and almost no complications. Moreover, using a guidewire did not increase the frequency of catheter replacement and the infection rate. [ABSTRACT FROM AUTHOR]

Background: Chronic intestinal failure (CIF) is a heterogeneous disease that affects pediatric and adult populations worldwide and requires complex multidisciplinary management. In recent years, many advances in intravenous supplementation support, surgical techniques, pharmacological management, and intestinal transplants have been published. Based on these advances, international societies have published multiple recommendations and guidelines for the management of these patients. The purpose of this paper is to show the differences that currently exist between the recommendations (ideal life) and the experiences published by different programs around the world. Methods: A review of the literature in PubMed from 1980 to 2024 was carried out using the following terms: intestinal failure, CIF, home parenteral nutrition, short bowel syndrome, chronic intestinal pseudo-obstruction, intestinal transplant, enterohormones, and glucagon-like peptide-2. Conclusions: There is a difference between what is recommended in the guidelines and consensus and what is applied in real life. Most of the world's countries are not able to offer all of the steps needed to treat this pathology. The development of cooperative networks between countries is necessary to ensure access to comprehensive treatment for most patients on all continents, but especially in low-income countries. [ABSTRACT FROM AUTHOR]

Background: Spontaneous mesenteric hematoma is a rare condition that is diagnosed when clinical and pathological findings do not identify an obvious causative disease. Various treatment options for spontaneous mesenteric hematoma exist; however, there are no clear treatment criteria. Herein, we report a case of spontaneous mesenteric hematoma that was successfully treated surgically and discuss the optimum treatment strategy based on similar cases. Case presentation: A 63-year-old man with abdominal persisting for 3 days presented to our hospital after going into shock without any triggers. The patient had a history of atrial fibrillation, stroke, and an aneurysm, and was receiving antithrombotic therapy. Abdominal contrast-enhanced computed tomography revealed a mass structure within the sigmoid mesentery, which was suspected to be a hematoma. The patient was admitted to the hospital for follow-up observation after initial infusion and vital stabilization. However, the following day, the patient developed acute generalized peritonitis with necrosis of the sigmoid colon; therefore, emergency Hartmann's surgery was performed. Intraoperative and histopathological examinations revealed no evidence of bleeding. Conclusion: Spontaneous mesenteric hematomas tend to be associated with intestinal necrosis and may require surgical treatment with bowel resection owing to the difficulty in identifying the responsible vessel. Moreover, our results suggest that the presence of antithrombotic therapy may be an important factor affecting spontaneous mesenteric hematoma development. [ABSTRACT FROM AUTHOR]

Teduglutide is a glucagon-like-peptide-2 analogue that reduces the need for parenteral support in patients with short bowel syndrome (SBS). Nevertheless, data about long-term therapy with teduglutide in children are still scarce. Our objective was to describe the real-life experience with teduglutide in children with SBS over the last 5 years in Spain. This was a national multicentre and prospective study of paediatric patients with intestinal failure (IF) treated with teduglutide for at least 3 months. The data included demographic characteristics, medical background, anthropometric data, laboratory assessments, adverse events, and parenteral nutrition (PN) requirements. Treatment response was defined as a > 20% reduction in the PN requirement. The data were collected from the Research Electronic Data Capture (REDCap) database. Thirty-one patients from seven centres were included; the median age at the beginning of the treatment was 2.3 (interquartile range (IQR) 1.4–4.4) years; and 65% of the patients were males. The most frequent cause of IF was SBS (94%). The most common cause of SBS was necrotizing enterocolitis (35%). The median residual bowel length was 29 (IQR 12–40) cm. The median duration of teduglutide therapy was 19 (IQR 12–36) months, with 23 patients (74%) treated for > 1 year and 9 treated for > 3 years. The response to treatment was analysed in 30 patients. Twenty-four patients (80%) had a reduction in their weekly PN energy > 20% and 23 patients (77%) had a reduction in their weekly PN volume > 20%. Among the responders, 9 patients (29%) were weaned off PN, with a median treatment duration of 6 (IQR 4.5–22) months. The only statistically significant finding demonstrated an association between a > 20% reduction in the weekly PN volume and a younger age at the start of treatment (p = 0.028). Conclusions: Teduglutide seems to be an effective and safe treatment for paediatric patients with IF. Some patients require a prolonged duration of treatment to achieve enteral autonomy. Starting treatment with teduglutide at a young age is associated with a higher response rate. What is Known: • Glucagon-like peptide-2 (GLP-2) plays a crucial role in the regulation of intestinal adaptation in short bowel syndrome (SBS). Teduglutide is a GLP-2 analog that reduces the need for parenteral support in patients with SBS. • Data about long-term therapy with teduglutide in children in real life are still scarce. What is New: • Most pediatric patients with SBS respond in a satisfactory manner to teduglutide treatment. The occurrence of long-term adverse effects is exceptional. • Starting treatment with the drug at a young age is associated with a greater response rate. [ABSTRACT FROM AUTHOR]

Life expectancy of children with chronic intestinal failure (CIF) on home parenteral nutrition has greatly improved. Children are now able to grow into adulthood which requires transfer from pediatric to adult health care. A guideline for structured transition is lacking and the demand for a more standardized care for this patient group is necessary. Therefore, we investigated the perceptions of health care professionals from various disciplines working in this specific field, concerning effective interventions regarding transition to adult health care. To create a standardized protocol which provides practical guidance for health care professionals in order to bridge the gap between pediatric and adult health care and to facilitate successful transition of children with chronic intestinal failure. A survey consisting of 20 interventions for transition was sent out to members of the Intestinal Failure working group of European Reference Network for Rare Inherited Congenital (gastrointestinal and digestive) Anomalies (ERNICA) and the Network of Intestinal Failure and Intestinal Transplant in Europe (NITE) group - European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) healthcare professionals in 48 medical centers in various countries. Next to 20 interventions, an open-ended question to fill in any other suggestion with respect to most effective intervention was included. Interventions scoring higher than 80% by the participants were included in the protocol. Interventions scoring between 50% and 80% and other own suggestions were discussed during a consensus meeting and included when consensus, defined as unanimous agreement, was reached. Interventions scoring as effective by < 50% of participants were excluded directly. A total of 80 healthcare professionals from 33 medical centers (participation rate 69%) participated. The protocol consisted of modifiable components expected to be targets of interventions. The most important key outcomes of the survey were: 1) assessment of patient's transition readiness and provision of knowledge to the patient by the pediatric team, 2) involvement of parents in the transition process, and 3) collaboration between the pediatric and adult chronic intestinal failure team. In addition it is advised that the transition process should start 1–2 years before transfer. A nurse specialist working in both services should form a bridge. All interventions must be tailor-made and based on the maturity of the patient. This study provides a protocol describing transition of children with chronic intestinal failure from pediatric to adult care. This international protocol will serve as practical guidance for pediatric chronic intestinal failure which will provide a more structured, optimal transition process. It is advised to use this protocol as a formal checklist that can be placed in the patient's chart to review and track the transition process by CIF team members. Future research investigating transition readiness of CIF patients is needed. [ABSTRACT FROM AUTHOR]

Background: Patients with chronic intestinal failure (CIF) are at increased risk of developing renal impairment. The aim of this study was to evaluate the occurrence of chronic kidney disease (CKD) in patients dependent on home parenteral nutrition (HPN) and assess risk factors for renal impairment, including patients with all mechanisms of CIF. Methods: This was a cohort study of patients initiated on HPN between March 1, 2015, and March 1, 2020, at a national UK IF Reference Centre. Patients were followed from their first discharge with HPN until HPN cessation or the end of follow‐up on December 31, 2021. Results: There were 357 patients included in the analysis. Median follow‐up time was 4.7 years. At baseline, >40% of patients had renal impairment, with 15.4% fulfilling the criteria for CKD. Mean estimated glomerular filtration rate (eGFR) decreased significantly during the first year after initiation of HPN from 93.32 ml/min/1.73 m2 to 86.30 ml/min/1.73 m2 at the first year of follow‐up (P = 0.002), with sequential stabilization of renal function. Increased age at HPN initiation and renal impairment at baseline were associated with decreased eGFR. By the end of follow‐up, 6.7% patients developed renal calculi and 26.1% fulfilled the criteria for CKD. Conclusion: This is the largest study of renal function in patients receiving long‐term HPN. After the first year following HPN initiation, the rate of decline in eGFR was similar to that expected in the general population. These findings should reassure patients and clinicians that close monitoring of renal function can lead to good outcomes. [ABSTRACT FROM AUTHOR]

Background: Chronic intestinal failure (CIF) refers to the long‐lasting reduction of gut function below the minimum necessary to absorb macronutrients, water, and/or electrolytes. Patients with CIF likely develop various forms of malnutrition and dehydration, yet studies that focus primarily on body composition are lacking. Therefore, this study aimed to evaluate the body composition of adult patients with CIF. Methods: This retrospective descriptive cohort study was performed at the Radboud University Medical Center, a tertiary referral center for CIF treatment in the form of home parenteral nutrition. We collected available bioelectrical impedance analysis (BIA) data from routine care between 2019 and 2023. The primary outcome was body composition, which was evaluated by assessing body mass index (BMI), fat‐free mass index (FFMI), and fat percentage (fat%). Results: Overall, 147 adult patients with CIF were included with a median (interquartile range) age of 58 (25–68) years; 69% were female. The mean (SD) BMI was 22.1 (4.3) kg/m2, FFMI was 14.2 (1.9) kg/m2 in females and 17.0 (2.0) kg/m2 in males, and fat% was 33.7% (6.8%) in females and 24.6% (6.4%) in males. 63% had an FFMI below references, and 48% had a high fat%. Conclusion: This study found that most adult patients with CIF have an unfavorable body composition characterized by a high fat% and low FFMI despite having a normal mean BMI. These results highlight the necessity for in‐depth nutrition assessment, including BIA measurement. Moreover, future studies should focus on exercise interventions to increase FFMI and improve body composition and function. [ABSTRACT FROM AUTHOR]

Background: Home parenteral nutrition (HPN) is a life‐saving therapy required for the management of type III intestinal failure, one of the rarest organ failures. It requires a multidisciplinary approach to manage the complexity of the underlying medical, surgical, and nutrition issues, but the current levels of healthcare funding in Australia are unknown. This study aimed to quantify the caseload, staffing, and capacity of existing HPN centers nationally. Methods: This was a cross‐sectional survey inviting centers known to provide HPN care. The survey was designed to capture metrics related to the national framework for the delivery of HPN. These centered on staffing levels, patient load, capacity to audit key outcomes, and service challenges. Results: A total of 24 (89%) of 27 invited centers responded to the survey. There were 17 (71%) adult centers and 7 (29%) pediatric centers. Adult centers managed a median of 12 (interquartile range [IQR]: 6–25) patients vs 16 (IQR: 9–17) in pediatric centers. Several centers did not have dedicated funding for core team members. The total funded clinician time each week per patient was 7 min (IQR: 0–12 min) in adult centers and 14 min (IQR: 10–21 min) in pediatric centers. Fewer than half of centers reported having sufficient resources to regularly audit key metrics. Conclusion: The availability of dedicated expertise to manage the highly complex needs of people living with type III intestinal failure is lacking in Australia. Current funding of HPN services falls well short of being sufficient to meet the requirements outlined in the national quality framework. [ABSTRACT FROM AUTHOR]

The Short Bowel Syndrome (SBS) Registry (NCT01990040) is a multinational real-world study evaluating the long-term safety of teduglutide in patients with SBS and intestinal failure (SBS-IF) in routine clinical practice. This paper describes the study methodology and baseline characteristics of adult patients who have (ever-treated) or have never (never-treated) received teduglutide. A total of 1411 adult patients (679 ever-treated; 732 never-treated) were enrolled at 124 sites across 17 countries. The mean (standard deviation [SD]) age at enrollment was 55.4 (15.46) years, and 60.2% of patients were women. Crohn's disease was the most common cause of major intestinal resection in both ever-treated (34.1%) and never-treated patients (20.4%). A similar proportion of ever-treated and never-treated patients had a prior history of colorectal polyps (2.7% vs. 3.6%), whereas proportionally fewer ever-treated patients reported a history of colorectal cancer (1.8% vs. 6.2%) or any malignancy (17.7% vs. 30.0%) than never-treated patients. Never-treated patients received a numerically greater mean (SD) volume of parenteral nutrition and/or intravenous fluids than ever-treated patients (12.4 [8.02] vs. 10.1 [6.64] L/week). Ever-treated patients received a mean teduglutide dosage of 0.05 mg/kg/day. This is the first report of patient baseline characteristics from the SBS Registry, and the largest cohort of patients with SBS-IF to date. Overall, ever-treated and never-treated patients had similar baseline characteristics. Differences between treatment groups may reflect variations in patient selection and degree of monitoring. [ABSTRACT FROM AUTHOR]

Reducing soybean lipid emulsion (SLE) dose may prevent parenteral nutrition-associated cholestasis (PNAC) but effects on growth and neurodevelopment are unknown. The purpose of this study was to evaluate the effect of reduced dose SLE on growth and neurodevelopment. Surgical neonates at 4 centers were randomized to standard SLE (3 g/kg/day) or reduced SLE (1 g/kg/day) over a 12-week period. Bilirubin levels and growth parameters were measured baseline and weekly while on study. The effects of time and group on direct bilirubin and growth were evaluated with a linear mixed effects model. Neurodevelopmental outcomes were assessed at 12- and 24-months corrected gestational age. Twenty-one individuals were randomized (standard dose = 9, reduced dose = 12). Subjects in the reduced dose group had slower rates of direct bilirubin increase and overall levels decreased earlier than those in the standard dose group. There was a trend toward a faster direct bilirubin decrease in the reduced dose group (p = 0.07 at day 84). There were no differences in the rates of change in weight (p = 0.352 at day 84) or height Z-scores (p = 0.11 at day 84) between groups. One subject in the reduced dose group had abnormal neurodevelopmental testing at 24 months. Surgical neonates randomized to a reduced dose of SLE had improved trends in direct bilirubin levels without clinically significant differences in overall growth and neurodevelopment. Randomized Controlled Trial. II. • Reduced soybean lipid emulsion dose may prevent the development of PNAC in surgical neonates but the effects on long term growth and neurodevelopment are not known • Here we demonstrate that surgical neonates receiving reduced dose soybean lipid emulsion had improved bilirubin trends without significant differences in growth or neurodevelopment [ABSTRACT FROM AUTHOR]

Background: Selection of central venous catheter (CVC) lock solution impacts catheter mechanical complications and central line–associated bloodstream infections (CLABSIs) in pediatric patients with intestinal failure. Disadvantages of the current clinical standards, heparin and ethanol lock therapy (ELT), led to the discovery of new lock solutions. High‐risk pediatric patients with intestinal failure who lost access to ELT during a recent shortage were offered enrollment in a compassionate use trial with 4% tetrasodium EDTA (T‐EDTA), a lock solution with antimicrobial, antibiofilm, and antithrombotic properties. Methods: We performed a descriptive cohort study including 14 high‐risk pediatric patients with intestinal failure receiving 4% T‐EDTA as a daily catheter lock solution. CVC complications were documented (repairs, occlusions, replacements, and CLABSIs). Complication rates on 4% T‐EDTA were compared with baseline rates, during which patients were receiving either heparin or ELT (designated as heparin/ELT). Results: Patients initiated 4% T‐EDTA at the time they were enrolled in the compassionate use protocol. Use of 4% T‐EDTA resulted in a 50% reduction in CVC complications, compared with baseline rates on heparin/ELT (incidence rate ratio: 0.50; 95% CI, 0.25–1.004; P = 0.051). Conclusion: In a compassionate use protocol for high‐risk pediatric patients with intestinal failure, the use of 4% T‐EDTA reduced composite catheter complications, including those leading to emergency department visits, hospital admissions, additional procedures, and mortality. This outcome suggests 4% T‐EDTA has benefits over currently available lock solutions. [ABSTRACT FROM AUTHOR]

Jejunocolic anastomosis (JCA) is a surgical procedure that can result in various complications. Depending on how much of the jejunum remains, these patients are often defined as having intestinal failure (IF) because of short bowel syndrome (SBS). One of the significant complications among these patients is the formation of calcium oxalate renal stones. It has been estimated that 24% of patients with a JCA develop symptomatic renal stones. This narrative review aims to explore the nursing and dietary management strategies for individuals with oxalate stones following JCA. This includes providing adequate hydration, limiting dietary intakes of fat and oxalate, and optimising dietary intake of fluid and calcium whilst discussing the evidence for dietary magnesium, vitamin C and citric acid. [ABSTRACT FROM AUTHOR]

Background and Aim Methods Results Conclusions Patients with intestinal failure (IF) have abnormal intestinal anatomy, secretion, and dysmotility, which impairs intestinal homeostatic mechanisms and may lead to small intestinal bacterial overgrowth (SIBO). We conducted a systematic review and meta‐analysis to determine the prevalence of SIBO in patients with IF and to identify risk factors for SIBO.MEDLINE (PubMed) and Embase electronic databases were searched from inception to December 2023 for studies that reported the prevalence of SIBO in IF. The prevalence rates, odds ratio (OR), and 95% confidence intervals of SIBO in IF and the risk factors for SIBO in IF were calculated using random effects model.Final dataset included nine studies reporting on 407 patients with IF. The prevalence of SIBO in IF was 57.5% (95% CI 44.6–69.4), with substantial heterogeneity in this analysis (I2 = 80.9, P = 0.0001). SIBO prevalence was sixfold higher in patients with IF who received parenteral nutrition (PN) compared with IF patients not on PN (OR = 6.0, 95% CI 3.0–11.9, P = 0.0001). Overall, the prevalence of SIBO in patients with IF using PPI/acid‐suppressing agents (72.0%, 95% CI 57.5–83.8) was numerically higher compared with IF patients not using these agents (47.6%, 95% CI 25.7–70.2).This systematic review and meta‐analysis suggests that there is an increased risk of SIBO in patients with IF and that PN, and potentially, the use of PPI/acid‐suppressing agents is risk factors for SIBO development in patients with IF. However, the quality of evidence is low and can be attributed to lack of case–control studies and clinical heterogeneity seen in the studies. [ABSTRACT FROM AUTHOR]

Introduction: Home parenteral nutrition (HPN) prescriptions should be individualised in adults with chronic intestinal failure (IF). The aims of the review were to explore HPN requirements and available guidelines and to determine whether adults (≥ 18 years) receive recommended parenteral nutrient doses. Methods: Online databases searches identified empirical evidence (excluding case‐reports), reviews and guidelines (Published 2006–2024 in English language). Additional reference lists were hand‐searched. Older studies, cited in national guidelines were highlighted to map evidence source. Two reviewers screened 1660 articles independently, with 98 full articles assessed and 78 articles included (of which 35 were clinical studies). Citation tracking identified 12 older studies. Results: A lack of evidence was found assessing parenteral macronutrient (amounts and ratios to meet energy needs), fluid and electrolyte requirements. For micronutrients, 20 case series reported serum levels as biomarkers of adequacy (36 individual micronutrient levels reported). Studies reported levels below (27 out of 33) and above (24 out of 26) reference ranges for single micronutrients, with associated factors explored in 11 studies. Guidelines stated recommended parenteral dosages. Twenty‐four studies reported variable proportions of participants receiving HPN dosages outside of guideline recommendations. When associated factors were assessed, two studies showed nutrient variation with type of HPN administered (multichamber or individually compounded bags). Five studies considered pathophysiological IF classification, with patients with short bowel more likely to require individualised HPN and more fluid and sodium. Conclusions: This review highlights substantial evidence gaps in our understanding of the parenteral nutritional requirements of adult receiving HPN. The conclusions drawn were limited by temporal bias, small samples sizes, and poor reporting of confounders and dose. Optimal HPN nutrient dose still need to be determined to aid clinical decision‐making and further research should explore characteristics influencing HPN prescribing to refine dosing recommendations. Key points: There are substantial evidence gaps in our understanding of the parenteral nutritional requirements of adults with chronic intestinal failure (IF) receiving home parenteral nutrition (HPN).Clinical guidelines report daily recommended parenteral dosages for macronutrients, fluid, electrolytes and micronutrients; these are reported as total or per kilogram body weight or minimum and/or maximum amounts in the case of lipid and glucose. Not all are specific to HPN.Studies that report HPN composition, suggest a wide variation in individual nutrient content is prescribed, with evidence that varying proportions of clinical cohorts receive nutrient amounts above or below the recommended dosages stated in guidelines. The reasons for this have not been fully explored, beyond the small body of evidence suggesting HPN requirements are associated with pathophysiological IF cause.Further research is required to determine the HPN requirements of those with chronic IF to support clinical decision‐making. [ABSTRACT FROM AUTHOR]

Loss of venous access is threatening for patients with intestinal failure (IF) under long-term parenteral nutrition (PN). We aimed to identify the incidence of central venous catheter (CVC) complications, compare different devices, and analyze interventional recanalizing procedures to restore the patency of occluded CVCs. For this retrospective cohort study, patient data from a prospective IF database spanning 16 years was analyzed at a tertiary referral center. Catheter dwell times (CDTs) were distinguished by Kaplan–Meier survival analysis and subgroup analyses were performed for different CVC types (tunneled/port catheters). Specific complications (occlusion, catheter-related infection (CRI), displacement, and material defect) were analyzed. Explantation rates and CDTs were compared. Overall, 193 CVCs in 77 patients with IF under PN could be enrolled (62.524 "CVC-days"). Broviac type "B" was found to be significantly superior to type "A" regarding occlusion, CRI, and material defects (log-rank test: p = 0.05; p = 0.026; p = 0.005 respectively). Port catheters were displaying the highest incidence of CRI (2.13 events/1000 catheter days). Interventional catheter recanalization was performed 91 times and significantly increased the CDT from a median of 131 days (IQR: 62; 258) to 389 days (IQR: 262; 731) (Mann-Whitney-U-test: p= <0.001) without increasing complications. Different complication rates and CDT were seen depending on CVC type. Tunneled catheters were significantly superior concerning CRI. Interventional catheter recanalization is a viable alternative to fibrinolytics to restore CVC patency, but long-term patency data is scarce. [ABSTRACT FROM AUTHOR]

Fecal microbiota was investigated in adult patients with chronic intestinal failure (CIF) due to short bowel syndrome (SBS) with jejunocolonic anastomosis (SBS-2). Few or no data are available on SBS with jejunostomy (SBS-1) and CIF due to intestinal dysmotility (DYS) or mucosal disease (MD). We profiled the fecal microbiota of various pathophysiological mechanisms of CIF. Cross-sectional study on 61 adults with CIF (SBS-1 30, SBS-2 17, DYS 8, MD 6). Fecal samples were collected and profiled by 16S rRNA amplicon sequencing. Healthy controls (HC) were selected from pre-existing cohorts, matched with patients by sex and age. Compared to HC, SBS-1, SBS-2 and MD patients showed lower alpha diversity; no difference was found for DYS. In beta diversity analysis, SBS-1, SBS-2 and DYS groups segregated from HC and from each other. Taxonomically, the CIF groups differed from HC even at the phylum level. In particular, CIF patients' microbiota was dominated by Lactobacillaceae and Enterobacteriaceae , while depleted in typical health-associated taxa belonging to Lachnospiraceae and Ruminococcaceae. Notably, compositional peculiarities of the CIF groups emerged. Furthermore, in the SBS groups, the microbiota profile differed according to the amount of parenteral nutrition required and the duration of CIF. CIF patients showed marked intestinal dysbiosis with microbial signatures specific to the pathophysiological mechanism of CIF as well as to the severity and duration of SBS. [ABSTRACT FROM AUTHOR]