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Background: International practice guidelines for high-stool-output (HSO) management in short bowel syndrome (SBS) are available, but data on implementation are lacking. This study describes the approach used to manage HSO in SBS patients across different global regions. Methods: This is an international multicenter study evaluating medical management of HSO in SBS patients using a questionnaire survey. Thirty-three intestinal-failure centers were invited to complete the survey as one multidisciplinary team. Results: Survey response rate was 91%. Dietary recommendations varied based on anatomy and geographic region. For patients without colon-in-continuity (CiC), clinical practices were generally consistent with ESPEN guidelines, including separation of fluid from solid food (90%), a high-sodium diet (90%), and a low-simple-sugar diet (75%). For CiC patients, practices less closely followed guidelines, such as a low-fat diet (35%) or a high-sodium diet (50%). First-line antimotility and antisecretory medications were loperamide and proton-pump inhibitors. Other therapeutic agents (e.g., pancreatic enzymes and bile acid binders) were utilized in real-world practices, and usage varied based on intestinal anatomy. Conclusion: Expert centers largely followed published HSO-management guidelines for SBS patients without CiC, but clinical practices deviated substantially for CiC patients. Determining the reasons for this discrepancy might inform future development of practice guidelines. [ABSTRACT FROM AUTHOR]

Pediatric intestinal failure (IF) is the reduction in gut function to below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth. The overall goal in treating IF is to achieve intestinal adaptation; however, the underlying mechanisms have not been fully understood. In this study, by performing single-cell RNA sequencing in pediatric IF patients, we found that decreased Kruppel-Like Factor 4 (KLF4) may serve as the hub gene responsible for the functional deficit in mature enterocytes in IF patients, leading to the downregulation of solute carrier (SLC) family transporters (e.g., SLC7A9) and, consequently, nutrient malabsorption. We also found that inducible KLF4 was highly sensitive to the loss of certain enteral nutrients: in a rodent model of total parenteral nutrition mimicking the deprivation of enteral nutrition, the expression of KLF4 dramatically decreased only at the tip of the villus and not at the bottom of crypts. By using IF patient-derived intestinal organoids and Caco-2 cells as in vitro models, we demonstrated that the supplementation of decanoic acid (DA) could significantly induce the expression of KLF4 along with SLC6A4 and SLC7A9, suggesting that DA may function as a potential therapeutic strategy to promote cell maturation and functional improvement. In summary, this study provides new insights into the mechanism of intestinal adaptation depending on KLF4, and proposed potential strategies for nutritional management using DA. [ABSTRACT FROM AUTHOR]

Background: There is increasing interest in the assessment of health‐related quality of life (QoL) in the care of patients treated with home parenteral nutrition (HPN). However, it is not known whether healthcare professionals (HCPs) have embedded QoL assessment into routine clinical practice in line with current guidelines to favour a more holistic approach to HPN care. The aim of this study was to assess knowledge, current practice and the opinions of HCPs regarding QoL in care of patients on HPN. Methods: An online survey was distributed via email to HCPs working with HPN patients throughout England, Scotland, Wales and Northern Ireland. Participants were identified using a mailing list for the British Intestinal Failure Alliance, a specialist group within the British Association for Parenteral and Enteral Nutrition. Results: The survey was completed by 67 professionals comprising 24 dietitians, 17 nurses, 14 gastroenterologists, 6 pharmacists, 5 surgeons and 1 psychologist. Of these, 54 (80%) participants agreed that the measurement of QoL is useful. In contrast, 38 (57%) of all participants, including 27 (50%) of those participants who agreed that the measurement of QoL was useful, never measured QoL. Knowledge of QoL literature was rated as poor or very poor by 27 (40%) participants. Conclusions: Despite the perceived usefulness and importance of QoL assessment, very few HCPs embed it into clinical practice. Knowledge of QoL literature and QoL tools is variable, and there is significant variability in QoL practice. This is clear in terms of the frequency of QoL assessments and heterogeneity in methodology. In contrast, there was almost unanimous agreement that the complications associated with HPN contribute to poorer QoL. There is a need for specific, evidence‐based, clinical practice guidelines detailing how to define and measure QoL in this patient population. Key points: There is significant variability in quality‐of‐life (QoL) practice for patients treated with home parenteral nutrition (HPN).Our findings suggest that very few healthcare professionals are embedding QoL assessment into clinical practice.Evidently, there appears to be a need for specific, evidence‐based, clinical practice guidelines detailing how to define and measure QoL in HPN patients. [ABSTRACT FROM AUTHOR]

Introduction: Short bowel syndrome (SBS) is a debilitating condition associated with significant morbidity and mortality. Historically, SBS patients require indefinite parenteral nutrition (PN) and endure lifelong nutritional challenges. The purpose of this study was to review the outcomes, specifically nutritional independence, of a multidisciplinary nutrition service. Methods: A retrospective analysis of SBS patients followed by our surgical nutrition service was performed. Patients without 1-year follow-up were excluded. Demographics and nutritional parameters were collected at 4 intervals: initial presentation, 1-year, 2-year, and 5-year follow-up. Short bowel syndrome anatomical subtypes identified through operative reports were characterized as end jejunostomy, jejunocolonic, or jejuno-ileocolonic with ileo-cecal valve intact. Intestinal failure was defined by the requirement of PN, while intestinal insufficiency was defined by enteral support requirement. Clinical outcomes examined included mortality, fistula closure, and nutritional independence. Results: The study cohort comprised 89 patients, 50 of whom had ≤ 100 cm intestinal length. Mean age was 57 ± 17y, 55 (62%) were female, and median initial intestinal length was 77 [60-120] cm. Short bowel syndrome was complicated by fistulas in 47 (53%) of patients. Overall mortality was 13%, and 67 (75%) were liberated from PN. A total of 58 (65%) underwent operative intervention and fistula closure was achieved in 37 of 47 (79%) patients. Conclusions: Short bowel syndrome patients can experience significant benefit under treatment by a multidisciplinary nutrition service. By incorporating surgical intervention, the majority of patients previously relegated to lifelong PN have the opportunity to become nutritionally independent within 5 years. [ABSTRACT FROM AUTHOR]

Background and Objectives: Home parenteral nutrition (HPN) is a life sustaining therapy for patients with chronic intestinal failure. Reported outcomes for Asian HPN patients are scarce. We aim to review the clinical outcomes of adult and paediatric HPN patients in our cohort which caters for 95% of Singaporean HPN patients. Methods and Study Design: This is a retrospective review of HPN patients from an adult (2002-2017) and paediatric cohort (2011-2017) from the largest tertiary PN centres in Singapore. Patient demographics and clinical outcomes were reviewed. Results: There were 41 adult and 8 paediatric HPN patients. Mean age was 53.0(±15.1) (adults) and 8(±1.8) years-old (paediatrics). Mean duration of HPN was 2.6(±3.5) and 3.5(±2.5) years. Leading indications for adult HPN were short bowel syndrome (SBS) (n=19,46.3%), mechanical obstruction (n=9,22.0%), and gastrointestinal dysmotility disorders (GID) (n=5,12.2%). Thirteen adult (31.7%) patients had underlying malignancy, with seven (17.3%) receiving palliative HPN. Indications for HPN amongst paediatric patients was GID (n=5,62.5%) and SBS (n=3,37.5%). Central line-associated bloodstream infection (CLABSI)/1000catheter-days was 1.0(±2.1) and 1.8(±1.3). Catheter associated venous thrombosis (CAVT)/1000catheter-days was 0.1(±0.4) and 0.7(±0.8). Biochemical Intestinal Failure Associated Liver Disease (IFALD) was found in 21.9% and 87.5%. For adults, median overall survival was 90-months (4.3,175.7,95%CI), with actuarial survival of 70.7%(1-year) and 39.0%(5-years). Median survival for adult patients with malignancy was 6-months (4.2,7.7,95%CI), actuarial survival of 85.7%(3-months) and 30.7%(1-year). One adult patient died from PN related complications. No paediatric deaths were noted. Conclusions: Whilst patient numbers were modest, we report comparable complication and survival rates to other international centres in both our adult and paediatric cohorts. [ABSTRACT FROM AUTHOR]

Purpose of Review: Intestinal adaptation is the main treatment goal in short bowel syndrome to control symptoms and improve health-related quality of life. This can be achieved in part through the use of medical therapies as well as parenteral and enteral nutrition, including oral dietary choices. This review will focus on dietary and medical management of short bowel syndrome and offer insights into novel nutritional and pharmacological approaches that may benefit patients. Recent Findings: A balanced oral diet inclusive of whole plant-based foods may be beneficial in short bowel syndrome for reasons including alleviating symptoms, preventing disease-related complications, and optimizing the intestinal microbiome. Adjunctive medical therapies are often needed to improve symptoms, treat comorbid conditions, and reduce reliance on parenteral nutrition. Summary: Though treatment must be tailored to individual anatomy and symptoms, newer dietary and pharmacologic approaches may be promising for short bowel patients. Future prospective studies are needed to further understand their efficacy. [ABSTRACT FROM AUTHOR]

Teduglutide, a GLP-2 analogue, has been available in France since 2015 to treat short-bowel-syndrome (SBS)-associated chronic intestinal failure (CIF) but it remains very expensive. No real-life data on the number of potential candidates are available. The aim of this real-life study was to assess teduglutide initiation and outcomes in SBS-CIF patients. All SBS-CIF patients cared for in an expert home parenteral support (PS) center between 2015 and 2020 were retrospectively included. Patients were divided into two subpopulations: prevalent patients, already cared for in the center before 2015, and incident patients, whose follow-up started between 2015 and 2020. A total of 331 SBS-CIF patients were included in the study (156 prevalent and 175 incident patients). Teduglutide was initiated in 56 patients (16.9% of the cohort); in 27.9% of prevalent patients and in 8.0% of incident patients, with a mean annual rate of 4.3% and 2.5%, respectively. Teduglutide allowed a reduction in the PS volume by 60% (IQR: 40–100), with a significantly higher reduction in incident versus prevalent patients (p = 0.02). The two- and five-year treatment retention rates were 82% and 64%. Among untreated patients, 50 (18.2%) were considered ineligible for teduglutide for non-medical reasons. More than 25% of prevalent SBS patients were treated with teduglutide compared to 8% of incident patients. The treatment retention rate was >80% at 2 years, which could be explained by a careful selection of patients. Furthermore, this real-life study confirmed the long-term efficacy of teduglutide and showed a better response to teduglutide in incident patients, suggesting a benefit in early treatment. [ABSTRACT FROM AUTHOR]

Diseases of the liver and the digestive system can lead to malnutrition through an action of reduced food intake or nutrient use, inflammation and impaired metabolism, which result in substantial changes in body composition. Frequently, malnutrition manifests itself with weight loss and reduced muscle mass. However, weight loss and body mass index lack sensitivity to detect the loss of muscle mass and are not informative in distinguishing body water compartments and in characterizing their distribution. This issue is particularly relevant to these two disease models, which are frequently associated with fluid volume imbalances. Phase angle is a useful indicator for cell membrane integrity, water distribution between the intracellular and extracellular spaces and prediction of body cell mass as it is described by measured components of electrical impedance. Malnutrition, inflammation and oxidative stress impair electric tissue properties leading to lower values of PhA. In patients with inflammatory bowel and liver diseases, PhA was consistently found to be related to nutritional status and body composition, particularly the depletion of lean body mass and sarcopenia. It has been associated with prognosis, disease stage and severity and found to be helpful in monitoring fluid shifts and response to interventions. [ABSTRACT FROM AUTHOR]

Short-bowel syndrome (SBS) in pediatric age is defined as a malabsorptive state, resulting from congenital malformations, significant small intestine surgical resection or disease-associated loss of absorption. SBS is the leading cause of intestinal failure in children and the underlying cause in 50% of patients on home parental nutrition. It is a life-altering and life-threatening disease due to the inability of the residual intestinal function to maintain nutritional homeostasis of protein, fluid, electrolyte or micronutrient without parenteral or enteral supplementation. The use of parenteral nutrition (PN) has improved medical care in SBS, decreasing mortality and improving the overall prognosis. However, the long-term use of PN is associated with the incidence of many complications, including liver disease and catheter-associated malfunction and bloodstream infections (CRBSIs). This manuscript is a narrative review of the current available evidence on the management of SBS in the pediatric population, focusing on prognostic factors and outcome. The literature review showed that in recent years, the standardization of management has demonstrated to improve the quality of life in these complex patients. Moreover, the development of knowledge in clinical practice has led to a reduction in mortality and morbidity. Diagnostic and therapeutic decisions should be made by a multidisciplinary team that includes neonatologists, pediatric surgeons, gastroenterologists, pediatricians, nutritionists and nurses. A significant improvement in prognosis can occur through the careful monitoring of nutritional status, avoiding dependence on PN and favoring an early introduction of enteral nutrition, and through the prevention, diagnosis and aggressive treatment of CRSBIs and SIBO. Multicenter initiatives, such as research consortium or data registries, are mandatory in order to personalize the management of these patients, improve their quality of life and reduce the cost of care. [ABSTRACT FROM AUTHOR]

The short-term effects of teduglutide (TED) for short bowel syndrome with chronic intestinal failure (SBS–IF) in patients with Crohn's disease (CD) remain unknown. The aim of this study was to investigate the effects of TED in patients with CD on home parenteral support (PS) for SBS–IF. We retrospectively investigated the medical records of patients with CD associated with SBS–IF who initiated TED between 2020 and 2021. The primary outcomes were the change in PS volume and proportion of patients with a reduction of PS volume by ≥ 20% at week 8. Secondary outcomes were the change in PS volume in patients with CD without/with colon in continuity and adverse events during the observation period. Eighteen patients with CD who underwent home PS for SBS–IF were included in this study. Two patients were excluded owing to intolerable abdominal pain or vomiting within 8 weeks (11%). Sixteen patients continued TED throughout the observation period. The median PS duration was 10.5 years. The median observation period was 22 weeks after starting TED. TED significantly reduced the PS volume from 15,825.0 mL/week to 10,700.0 mL/week (p = 0.0038), and the PS volume decreased by ≥ 20% in 7 patients (43.8%) at week 8. The PS volume was significantly reduced at week 4 (p = 0.0078) in 11 patients without colon in continuity but not in 5 patients with colon in continuity. Two patients successfully stopped home PS. No serious adverse events occurred. TED administration significantly reduced PS volume at week 8 in patients with CD associated with SBS–IF, and at week 4 in patients without colon in continuity. [ABSTRACT FROM AUTHOR]

Prior to the late 1960s, pediatric short bowel syndrome was a frequently fatal disease. Currently, pediatric interdisciplinary bowel rehabilitation centers report very high survival rates. The mortality trends, up-to-date definitions, incidence, causes, and clinical manifestations of short bowel syndrome are reviewed. Emphasis is placed upon the nutritional, medical, and surgical advances that have contributed to the dramatic improvement in outcomes for pediatric short bowel syndrome patients. Recent findings and remaining challenges are highlighted. [ABSTRACT FROM AUTHOR]

Periodical replacement of venous Hickman catheters is required for the nutritional care of patients with intestinal failure. The conventional de novo operation (DN-OP) involves inserting the catheter into a new venous tract in each replacement; however, this could result in fast consumption of functional central vessels in patients with intestinal failure. Recently, same-route operation (SR-OP) has been adopted as an alternative approach for retaining venous access. We conducted a retrospective study to compare the efficacy of Hickman catheters and the survival of venous vessels using two different operative strategies. Overall, 181 catheters were inserted, 109 using DN-OP and 72 using SR-OP. The mean catheter duration was 11.9 ± 8.8 months in the DN-OP group and 10.5 ± 5.6 months in the SR-OP group; the infection rate was 0.74 in the DN-OP group and 0.44 in the SR-OP group. The vein accesses used in these insertions (n = 113) were classified: the DN-vein group for veins accessed only by DN-OP (n = 75) and the SR-vein group for veins accessed by an initial DN-OP and subsequent SR-OPs (n = 38). Mean working duration per vein access was 12.3 ± 10.1 months in the DN-vein group and 28.2 ± 14.8 months in the SR-vein group (p < 0.001); mean infection-free duration was 11.4 ± 10.1 months in the DN-vein group and 27.7 ± 15.3 months in the SR-vein group (p < 0.001). Application of SR-OP in Hickman catheter replacement significantly extended the working duration of venous access by re-using the same venous route without compromising catheter efficacy in patients with IF having poor venous access. [ABSTRACT FROM AUTHOR]

Objective: Intestinal failure refers to a reduction in intestinal function that necessitates intravenous supplementation of macronutrients, water, or electrolytes due to the intestine's inability to absorb these substances adequately to maintain health and growth. This study aims to explore the experiences and challenges faced by patients enrolled in the intestinal rehabilitation program at Hospital Pablo Tobón Uribe in Medellín. Methodology: This qualitative study adopts a hermeneutic approach and utilizes grounded theory techniques. The sampling process involved both selective and theoretical sampling. A total of 20 semi-structured interviews were conducted, with eight interviews including contributions from family members. The data analysis commenced with open coding, followed by the grouping of codes into descriptive categories. Dimensions and properties were identified within these categories, and analytical categories were subsequently developed through axial and selective coding. This iterative process led to the emergence of the final paradigm matrix. Results: The study revealed that the healthcare system inadequately addresses the needs and expectations of patients with intestinal failure, leading to increased uncertainty about the disease's origin and future prognosis. Intestinal failure and its treatment disrupt various aspects of patients' lives, including personal, family, and work domains. Social stigmatization and rejection are prominent, underscoring the importance of support from family and close individuals in facilitating adaptation and revaluing life. Conclusions: Coping with the challenges of intestinal failure entails embracing the necessity of relying on parenteral nutrition, which is perceived as a prison that paradoxically enables survival. [ABSTRACT FROM AUTHOR]

Background: Autologous gastrointestinal reconstructive surgery (AGIR) has become a key component of intestinal rehabilitation programs. However, the best surgical option for short bowel syndrome (SBS) remains unknown. This paper presents our experience using combined procedures as primary treatment. Methods: We collected data on SBS patients who underwent surgery from 2008 to 2021 in two tertiary European Centres. Combined procedures were defined as more than one technique used on the same patient. Charts were reviewed for demographics, type of procedures, complications, and outcomes. Data are presented as median and IQR. Wilcoxon signed rank was used for all paired analyses. Results: Twenty‐one children (12 females) underwent combined procedures. Preoperative median small bowel length was 20 cm (IQR: 15–35 cm); after lengthening, it was 35.5 cm (IQR: 30.75–50.50 cm) (P < 0.001). Combined procedures were simultaneous in 15 patients and sequential in 6. At a median of 9.2 years (IQR: 7.55–9.78 years) follow‐up, complications were three bowel obstructions after strictures of anastomosis and two wound infections. Two patients achieved enteral autonomy, and others followed a weaning home parenteral nutrition regimen with a median of 4 nights off (IQR: 3–4 nights) starting with a median of 7 nights (IQR: 7–7 nights). Conclusions: Combined AGIR techniques are practical and safe in SBS treatment when tailored to meet patients' needs, combining lengthening, tailoring, and reducing transit time procedures. Therefore, combined AGIR may be considered a resource in intestinal rehabilitation units' armamentarium. [ABSTRACT FROM AUTHOR]

Intestinal failure is defined as the inability to absorb the minimum of macro and micronutrients, minerals and vitamins due to a reduction in gut function. In a subpopulation of patients with a dysfunctional gastrointestinal system, treatment with total or supplemental parenteral nutrition is required. The golden standard for the determination of energy expenditure is indirect calorimetry. This method enables an individualized nutritional treatment based on measurements instead of equations or body weight calculations. The possible use and advantages of this technology in a home PN setting need critical evaluation. For this narrative review, a bibliographic search is performed in PubMed and Web of Science using the following terms: 'indirect calorimetry', 'home parenteral nutrition', 'intestinal failure', 'parenteral nutrition', 'resting energy expenditure', 'energy expenditure' and 'science implementation'. The use of IC is widely embedded in the hospital setting but more research is necessary to investigate the role of IC in a home setting and especially in IF patients. It is important that scientific output is generated in order to improve patients' outcome and develop nutritional care paths. [ABSTRACT FROM AUTHOR]

Multiply high-output enterocutaneous fistulas (ECF) is a tragic postoperative complication. This report describes complex treatment of patient with multiple enterocutaneous fistulas after bariatric surgery, including a comprehensive preoperative preparation for 3 months (sepsis control, nutritional support and wound care) and reconstructive surgery (laparotomy, distal gastrectomy, resection of the small bowel with fistulas, Roux-gastrojejunostomy, transversostomy). [ABSTRACT FROM AUTHOR]

This guideline will inform physicians, nurses, dieticians, pharmacists, caregivers and other home parenteral nutrition (HPN) providers, as well as healthcare administrators and policy makers, about appropriate and safe HPN provision. This guideline will also inform patients requiring HPN. The guideline is based on previous published guidelines and provides an update of current evidence and expert opinion; it consists of 71 recommendations that address the indications for HPN, central venous access device (CVAD) and infusion pump, infusion catheter and CVAD site care, nutritional admixtures, program monitoring and management. Meta-analyses, systematic reviews and single clinical trials based on clinical questions were searched according to the PICO format. The evidence was evaluated and used to develop clinical recommendations implementing Scottish Intercollegiate Guidelines Network methodology. The guideline was commissioned and financially supported by ESPEN and members of the guideline group were selected by ESPEN. [ABSTRACT FROM AUTHOR]

Background: Most data on anemia in children with intestinal failure (IF) have been obtained from studies in which the data were collected at a single point in time. We aimed to identify the frequency of anemia and factors associated with hemoglobin levels in children with IF during their course of home parenteral nutrition. Methods: We performed a longitudinal cohort study of patients with IF followed up at a pediatric intestinal rehabilitation center. Outcome variables were hemoglobin levels and prevalence of anemia during the follow‐up period. The exposure variables were age, duration of parenteral nutrition, chronic disease, and serum concentrations of C‐reactive protein, iron, copper, selenium, vitamins A, D, B12, and folic acid. Results: Twenty‐five children with a median time of receiving parenteral nutrition of 40.7 months were included. A median (and interquartile range) of 40.7 (25.2–58) hemoglobin measurements were performed per patient. Mean (SD) hemoglobin was 10.7 (1.8) g/dL at baseline and 11.6 (0.9) g/dL in the last observation (paired t test, P = 0.07); 32% of patients had mean hemoglobin values below the lower limit for age. In a multivariable predictive model, having C‐reactive protein >1 mg/dL was associated with a decrease of 0.57 g/dL in hemoglobin (95% CI, −0.90 to −0.24, P = 0.01), and an increase of 1 mg/L in vitamin A concentration was associated with the increase of 0.93 g/dL in Hb level (95% CI, 0.24–1.61; P = 0.008). Conclusion: Anemia affects almost one‐third of children with IF and its frequency decreases during the follow‐up period. Hemoglobin levels are associated with inflammatory response and serum micronutrient concentrations. [ABSTRACT FROM AUTHOR]

Background: In research on pediatric chronic intestinal failure, heterogeneity in reported definitions and outcomes exists. This leads to a risk of reporting bias and impossibility of evidence synthesis. Also, reported outcomes should be relevant to both healthcare providers and patients and their parents. Therefore, the aim of this study is to create a core outcome set (COS) to be used in studies on pediatric chronic intestinal failure. Methods: Candidate outcomes were selected from a recent systematic review. A three‐round Delphi study among key stakeholders and a consensus meeting with an expert panel were undertaken to achieve consensus on the COS. Results: Seventy‐two stakeholders (79%) completed all three rounds of the Delphi process. Ninety‐eight outcomes were assessed, and five new outcomes were added after the first round. Ten outcomes were included in the final COS: weaning from parenteral nutrition, growth, mortality, central line–related infection, central line longevity, sepsis not related to central line infection, central line–related thrombosis, intestinal failure–associated liver disease, (serious) adverse events, and health‐related quality of life. Conclusion: This pediatric chronic intestinal failure COS consists of 10 outcomes important for all key stakeholders. Usage of this set in future research should minimize outcome heterogeneity and enhance the value of evidence synthesis. This will lead to better management in this field of rare gastrointestinal conditions. [ABSTRACT FROM AUTHOR]

Background: Improved survival rates for children with intestinal failure (IF) have resulted in an increased population of children receiving long‐term parenteral nutrition (PN). Our objective was to determine burden on caregivers of children with IF receiving long‐term PN. Methods: We performed a cross‐sectional study of caregivers of children with IF receiving long‐term PN in our intestinal rehabilitation program. A healthy comparison group matched on age of the child was enrolled. All participants completed standardized questionnaires, including the Parental Stress Index – Short Form (PSI‐SF), Hospital Anxiety and Depression Scale (HADS), and PedsQL Family Impact Module (PedsQL FIM). Univariate analysis was completed using a Student t test and chi‐square, with an alpha value of <0.05 considered significant. Results: Thirty‐eight caregivers of children with IF and 29 caregivers of healthy children consented, with response rates of 89% and 96.5%, respectively. Our study demonstrated increased stress for caregivers compared with comparison parents (PSI‐SF total score of 83 [SD = 26.8] vs 62.9 [SD = 13.5]; P < 0.01). Caregivers had increased anxiety (HADS anxiety score of 9.3 [SD = 4.8] vs 6.7 [SD = 3.2]; P = 0.02) and higher depression scores (HADS depression score of 6.3 [SD = 4.3] vs 4.1 [SD = 2.6]; P = 0.02) compared with the comparison group. Caregivers of children with IF demonstrated decreased health‐related quality of life (HRQoL) (reduced PedsQL FIM total score of 50.6 [SD = 18.2] vs 84.1 [SD = 20.5]; P < 0.01). Conclusions: Our results demonstrated significant burden of care in caregivers of children with IF receiving long‐term PN, with elevated stress, anxiety, and depression and decreased HRQoL. [ABSTRACT FROM AUTHOR]

Background: Sodium dodecyl sulfate (SDS) detergent is widely used in tissue decellularization to produce scaffolds for tissue engineering. Despite its strong decellularization, this substance has relatively high toxicity and causes changes in tissue composition. Sodium lauryl ether sulfate (SLES) is a new poly anionic detergent that is less toxic than SDS but weaker than it. The present study aimed to decellularize the intestinal tissue using SDS and SLES solutions, forming a cell scaffold, and examining scaffolds obtained from this tissue. Methods: Eighteen male Sprague-Dawley rats were divided into three groups. The intestines of all rats were removed after anesthesia. In the first group (controls), rats’ intestines were placed in a 10% formalin solution. In the second group, intestines were decellularized using an SLES solution. In the third group animals’ intestines were decellularized using an SDS solution. To evaluate decellularization, samples were stained with hematoxylin-eosin staining and Alcian blue staining for glycosaminoglycans (GAGs), and Masson’s trichrome for collagen fibers. A confocal Raman microscope was used to compare collagen, lipid, GAG, and genetic content. Results: Hematoxylin-eosin staining showed that the nucleus and DNA were removed in the decellularized scaffolds by SDS or SLES. The SLES group, compared to the SDS group, showed fewer changes in the epithelial tissue, and muscle layers in both scaffolds were well preserved. The results of confocal Raman microscopy showed that tryptophan, lipid, glycogen, and protein were broken down by both detergents; however, the residual amount of glycogen was the same in both substances, but disulfide bonds of proteins, hydroxyproline, and lipids in the decellularized intestine with SLES were mostly preserved. Conclusion: Both substances were suitable for intestinal decellularization and removed the overall structure of intestinal tissue, but SLES retained collagen and GAG content better than SDS. [ABSTRACT FROM AUTHOR]

Key Clinical Message Amyloidosis is a heterogeneous disease characterized by tissue deposition of abnormally folded fibrillary proteins that can manifest itself by a wide variety of symptoms depending on the affected organs. GI involvement among amyloidosis patients is common. Its clinical manifestation often presents with nonspecific symptoms such as weight loss, diarrhea, and malabsorption. With no specific treatment existing for GI amyloidosis, therapy focuses on impeding amyloid deposition and managing the patients' symptoms with supportive measures. Here, we present an AL‐amyloidosis patient with GI involvement and intestinal failure (IF) who was successfully treated with the glucagon‐like peptide‐2 (GLP‐2) analogue teduglutide. Over the course of treatment with teduglutide, the patient was able to achieve independence from parenteral nutrition and experienced a significant improvement in quality of life (QoL) as stool frequency and consistency improved, urinary output was stabilized and body weight as well as body composition improved over the course of teduglutide therapy. With no longer being exposed to the burden and associated risks of parenteral nutrition, we were able to reduce the potential morbidity and mortality rate as well as to improve the patient's overall QoL. Intestinal tissue biopsy workup revealed a histopathological correlate for the clinical response; Congo‐Red‐positive intestinal depositions almost completely disappeared within 6 months of teduglutide therapy. Implementing intestinotrophic GLP‐2 analogue teduglutide may enrich the spectrum of treatment options for amyloidosis patients with IF who are dependent on parenteral support.

Background: The efficacy of fish oil-based lipid emulsion (FOLE) in treating intestinal failure-associated liver disease and other cholestasis in children has been established. However, it has been found to be ineffective in some patients. This study aimed to identify factors negatively influencing FOLE administration. Methods: This study included 21 patients treated with FOLE, who were less than 100 days old, and had direct bilirubin (D. Bil) levels ≥1.0 mg/dL. Differences in patient backgrounds between patients who eventually had a reduction in jaundice and those who did not, as well as factors affecting treatment duration in the reduction group, were investigated. Results: All four patients of the non-reduction group died of cholestasis and severe comorbidities. The D. Bil level at treatment initiation was significantly higher in the no-reduction group than in the reduction group (p = 0.006). The duration of FOLE administration in the reduction group was negatively correlated with gestational age (ρ = −0.603, p = 0.01). In contrast, a positive correlation was observed between age (ρ = 0.708, p = 0.002) and the D. Bil level at treatment initiation (ρ = 0.490, p = 0.046). Conclusions: FOLE treatment may be ineffective in patients with severe comorbidities. Preterm birth, high D. Bil levels, and older age at treatment initiation may be factors that result in prolonged treatment duration. Level of evidence: III

Intestinal failure (IF) is characterized by a critical reduction in functional gut mass below the minimum needed for optimal growth in children. It requires parenteral nutrition (PN) and home-PN (HPN), which is challenging in terms of meeting nutritional needs according to age, growth velocity, clinical situation, and rapid changes in fluid and electrolyte requirements. Due to these complex requirements, age-adapted multi-chamber bags (MCBs) are important additions to the nutrition armamentarium. The launch of composite fish oil (FO)-containing intravenous lipid emulsions (ILEs) heralded the development of MCBs containing these ILEs in combination with a crystalline amino acid solution adapted for pediatric use. The safety and efficacy of lipid and amino acid components in this context have been widely documented in numerous published studies. This narrative manuscript includes a review of the articles published in PudMed, Embase, and Google Scholar up to June 2023 for the age groups of term infants to children and adolescents. Preterm infants with their highly specific demands are not included. It aims to offer an overview of the clinical experience regarding the use of a composite FO-based ILE and a developed specific amino acid solution.

Non-invasive monitoring of intestinal failure (IF) associated liver disease is an ongoing challenge in children with IF. Our objective was to develop a combined algorithm of clinical, transient elastography (TE) and biochemical parameters to identify liver fibrosis in this population. A retrospective cohort study of IF patients followed by our intestinal rehabilitation program between November 2015 to October 2019. Patients with a liver biopsy and TE were included. Demographic and liver function tests were collected. Fibrosis on liver biopsies was graded using the modified Scheuer score. Decision tree based algorithms classified low (F0–F1) versus high (F2–F4) fibrosis scores based on a combination of TE, biochemical and demographic parameters, using 6-fold classification error, sensitivity and specificity cross-validation (CV) scores. 42 patients (74% male, median age 7.6 (4.6; 42.7) months) were evaluated. Median length of PN therapy was 182 (121; 556) days. High fibrosis was present in 40.5% with a median TE of 12.1 (6.7; 12.9) kPa in high fibrosis children. An algorithm, based on cut-off values for TE of 11.3 kPa and AST of 40 U/L, and grouping of the underlying etiology resulted in a correct classification of 88.1% of the pathology scores; with sensitivity 0.82 (95% CI 0.57; 0.96), specificity 0.92 (95% CI 0.74; 0.99), positive predictive value 0.88 (95% CI 0.64; 0.96) and negative predictive value 0.88 (95% CI 0.73; 0.96). The CV classification error was 28.6%, CV sensitivity 72.2% and CV specificity 75.5%. This algorithm shows promising results that could simplify non-invasive monitoring of liver fibrosis in children with IF. Validation in additional IF cohorts is needed. [ABSTRACT FROM AUTHOR]

• 44% of patients achieved enteral autonomy (EA) following autologous gastrointestinal reconstructive (AGIR) surgery. • Percentage of expected of small bowel length (≥15%) before AGIR is a strong predictor of achievement of EA. • The AGIR technique employed did not influence weaning from parenteral nutrition. • Aetiology of short bowel syndrome (gastroschisis) can affect outcomes and a tailored, multi-disciplinary approach is recommended. The implementation of multidisciplinary care and improvements in parenteral nutrition (PN) in patients with short bowel syndrome (SBS) have led to better outcomes and higher survivability. Autologous gastrointestinal reconstructive (AGIR) surgery can reduce the duration on PN and lead to earlier enteral autonomy (EA). Our aim was to investigate the effect of SBS aetiology and other predictors on the achievement of enteral autonomy following AGIR surgery. Retrospective review of all patients undergoing AGIR surgery in two tertiary paediatric surgical units, between 2010 and 2021. Continuous data is presented as median (range). Twenty-seven patients underwent 29 AGIR procedures (20 serial transverse enteroplasties (STEP), 9 longitudinal intestinal lengthening and tailoring (LILT)) at an age of 6.6 months (1.5 – 104.5). EA rate was 44% at 13.6 months after surgery (1 – 32.8). AGIR procedures achieved an increase in small bowel length of 70% (pre-operative 46.5 vs 77 cm, p = 0.003). No difference was found between STEP and LILT (p = 0.84). Percentage of expected small bowel length (based on the child's weight) was a strong predictor of EA (bowel length >15% – EA 80% vs bowel length ≤15% – EA 17%, p = 0.008). A diagnosis of gastroschisis showed a negative non-significant correlation with the ability to achieve EA (25% vs 60%, p = 0.12). Overall survival rate was 96%. AGIR surgery is an important tool in the multidisciplinary management of children with SBS. Percentage of expected small length and aetiology of SBS are likely predictors of achievement of EA in patients undergoing AGIR surgery. Retrospective Case-Series [ABSTRACT FROM AUTHOR]

Background: Short bowel syndrome is the most common cause of intestinal failure (IF) in infants. We aimed to evaluate growth, nutrition intakes, and predictors of weaning from parenteral nutrition (PN) of infants with IF. Methods: Clinical parameters, nutrition intakes, body weight and length z‐scores were compared monthly from the 1st to 12th and at 18 and 24 months among infants receiving PN and those weaned. Logistic regression analysis was conducted to explore the predictors of weaning. Results: We included 23 infants (10/23 weaned). Median [range: minimum; maximum] birth weight and gestational age were 1620 [590; 3490] g and 31 [24; 39] weeks, respectively. All infants showed growth retardation with similar median delta weight z‐score from birth to discharge: −1.48 [−1.92; −0.94] in not‐weaned and −1.18 [−2.70; 0.31] in weaned infants (P = 0.833) and a subsequent regain after the discharge: 0.20 [−3.47; 3.25] and 0.84 [−0.03; 2.58], respectively (P = 0.518). No differences in length z‐score were found. After the sixth month, infants weaned from PN received lower PN energy and protein intakes compared with those not‐weaned. Infants weaned from PN showed lower PN dependency index (PNDI%) from 5 months onward (45% for weaned and 113% for not‐weaned infants at 5 months: P < 0.001). The Belza score, a predictor of enteral autonomy computed at 6 months, is associated with being weaned from PN within 24 months (odds ratio: 1.906; P = 0.039). Conclusion: Infants weaned and not‐weaned showed similar growth patterns. Our findings support the clinical relevance of Belza score and PNDI% as predictors of weaning from PN. [ABSTRACT FROM AUTHOR]

Introduction: Patients with chronic intestinal failure (IF) have a low degree of physical activity, decreased muscle mass, and decreased muscle strength, leading to a high risk of sarcopenia. We aimed to test the prevalence of sarcopenia by the use of SARC‐F and EWGSOP and to investigate the association between the two at baseline and after 12 weeks of an exercise intervention. Methods: Thirty‐one patients with chronic IF completed 12 weeks of three weekly home‐based individualized exercise sessions. Body composition was measured by bioimpedance analysis and physical function by handgrip strength (HGS) and timed up‐and‐go (TUG). Sarcopenia was assessed by SARC‐F and EWGSOP. Multiple regression analysis was used to test for the association between the two tools. Results: The prevalence of sarcopenia measured by EWGSOP was 59%. This prevalence did not change after the intervention. At baseline, 38.8% of patients were screened as at risk for sarcopenia by SARC‐F. This decreased to 29.0% after the intervention (P < 0.001). A statistically significant increase was achieved in muscle mass (P = 0.017) and muscle mass index (P = 0.016). Furthermore, both TUG (P = 0.033) and HGS (P = 0.019) improved. Conclusions: Sarcopenia is prevalent in patients with chronic IF. EWGSOP finds more patients to be at risk of sarcopenia than SARC‐F but was not sufficiently sensitive to measure changes induced by the physical intervention. The significant change in SARC‐F may illustrate that patients, themselves, find an improvement in self‐perceived health. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is a rare gastrointestinal condition that is defined as having less than 200 cm of remaining small intestine. SBS results from extensive surgical resection and is associated with a high risk for intestinal failure (IF) with a need for parenteral support (PS). Depending on the region of intestinal resection, three different main anatomy types can be distinguished from each other. In this review, we synthesize the current knowledge on the role of the colon in the setting of SBS-IF with a colon-in-continuity (SBS-IF-CiC), e.g., by enhancing the degree of intestinal adaptation, energy salvage, and the role of the microbiota. In addition, the effect of the disease-modifying treatment with glucagon-like peptide-2 (GLP-2) analogs in SBS-IF-CiC and how it differs from patients without a colon will be discussed. Overall, the findings explained in this review highlight the importance of preservation of the colon in SBS-IF. [ABSTRACT FROM AUTHOR]

The differences in outcomes after weaning off intravenous support (IVS) for chronic intestinal failure (IF) are unclear. Adult IF patients who are weaned off IVS at a tertiary care center (June 2019–2022) were included in this study, and nutritional and functional markers were assessed before, during, and after weaning. Short bowel syndrome (SBS) was present in 77/98 of the IF patients, with different outcomes according to the final anatomy. The body weight and the BMI increased during IVS in those with a jejunocolonic (JC) anastomosis (p < 0.001), but weight loss was significant during follow-up (p < 0.001). Malnutrition was present in >60%, with a reduced muscle mass, which was found using bioelectrical impedance analysis (BIA), in >50% of SBS-JC patients. Although reduced hand-grip strength and sarcopenia were less common, the muscle quality, or phase angle (BIA), decreased during follow-up, also correlating with serum albumin and muscle mass (p ≤ 0.01). The muscle quality and albumin were low in the patients restarting IVS, which was only the case with ≤60 cm of small bowel. Closer follow-up and earlier treatment with teduglutide (TED) should be considered in these patients, as none of the TED-treated patients were malnourished or sarcopenic. Studies on the potential benefits of nutritional and physical interventions for low muscle mass and associations with outcomes are needed in chronic IF patients. [ABSTRACT FROM AUTHOR]

Aim: The effect of negative pressure wound therapy (NPWT) on the pathogenesis and outcome of enteroatmospheric fistulation (EAF) in the septic open abdomen (OA) is unclear. This study compares the development and outcome of EAF following NPWT with that occurring in the absence of NPWT. Methods: Consecutive patients admitted with EAF following abdominal sepsis at a National Reference Centre for intestinal failure between 01 January 2005 and 31 December 2015 were included in this study. Patients were divided into two groups based on those that had been treated with NPWT and those that had not (non‐NPWT) and characteristics of their fistulas compared. Clinical outcomes concerning nutritional autonomy at 4 years and time to fistula development, size of abdominal wall defect and complete fistula closure were compared between groups. Results: A total of 160 patients were admitted with EAF following a septic abdomen (31‐NPWT and 129‐non‐NPWT). Median (range) time taken to fistulation after OA was longer with NPWT (18 [5–113] vs. 8 [2–60] days, p = 0.004); these patients developed a greater number of fistulas (3 [2–21] vs. 2 [1–10], p = 0.01), involving a greater length of small bowel (42.5 [15–100] cm vs. 30 [3.5–170] cm, p = 0.04) than those who did not receive NPWT. Following reconstructive surgery, nutritional autonomy was similar in both groups (77% vs. 72%) and a comparable number of patients were also fistula‐free (100% vs. 97%). Conclusions: Negative pressure wound therapy appears to be associated with more complex and delayed intestinal fistulation, involving a greater length of small intestine in the septic OA. This did not, however, appear to adversely affect the overall outcome of intestinal and abdominal wall reconstruction in this study. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is a chronic disease whose natural history requires a changing array of management strategies over time. Chief amongst these is the chronic use of parenteral nutrition (PN) to ensure adequate nutritional intake. With time and appropriate management, approximately half of all SBS patients will successfully regain a functional, baseline level of intrinsic bowel function that will allow for them to achieve PN independence. However, the other half of SBS patients will progress into chronic intestinal failure which warrants a change in therapy to include more aggressive medical and potentially surgical measures. This review examines the evolving treatment strategies involved in the management of SBS as well as intestinal failure. [ABSTRACT FROM AUTHOR]

Background: The proadaptive effects of glucagon‐like peptide‐2 (GLP‐2) include stimulation of intestinal mucosal growth as well as intestinal blood flow and angiogenesis. We have recently reported that daily subcutaneous injections of glepaglutide, a long‐acting GLP‐2 analog, improved intestinal absorptive function in patients with short bowel syndrome (SBS). As secondary and exploratory end points, the effects of glepaglutide on intestinal morphology and perfusion are reported. Methods: The following assessments were done in 18 patients with SBS in a randomized, crossover, dose‐finding, phase 2 trial before and after three weeks of treatment with glepaglutide: plasma citrulline and mucosa biopsies to assess changes in (1) intestinal morphology by immunohistochemistry and (2) gene expressions associated with absorption, proliferation, and markers of tight‐junction integrity by quantitative polymerase chain reaction. Intestinal perfusion was assessed in stoma nipples by laser speckle contrast imaging and quantitative fluorescence angiography with indocyanine green. Results: In the 1‐ and 10‐mg dose groups, glepaglutide significantly increased plasma citrulline by 15.3 µmol/L (P = 0.001) and 15.6 µmol/L (P = 0.001), respectively. Trends toward an increase in villus height, crypt depth, and epithelium height were seen in the same groups. No significant changes were seen in gene expressions or intestinal perfusion. Conclusion: The increase in plasma citrulline and the morphological improvements may partly account for improvement in the intestinal absorptive function. However, the finding of a stability in perfusion after three weeks of treatment with glepaglutide may have been preceded by a more profound acute‐phase increase in intestinal perfusion at treatment initiation. [ABSTRACT FROM AUTHOR]

Systemic oxalosis is a condition in which calcium oxalate crystals deposit into various bodily tissues. Although this may occur as the result of a rare primary syndrome in which an error of glyoxylate metabolism causes an overproduction of oxalate, it is more often seen as a secondary process characterized by increased enteric oxalate absorption. Here, we describe a patient with short bowel syndrome on long‐term parenteral nutrition support who developed a unique manifestation of systemic oxalosis, leading to deposition of oxalate crystals within the bone marrow contributing to pancytopenia. In this report, in addition to reviewing the literature on this presumably rare manifestation of oxalosis, we also discuss its pathogenesis in the setting of short bowel syndrome and its management, including prevention. [ABSTRACT FROM AUTHOR]

Background: The most common cause of intestinal failure (IF) in childhood remains short bowel syndrome (SBS), where bowel mass is significantly reduced due to a congenital atresia or resection and parenteral nutrition (PN) needed. Home PN has improved outcome and quality of life, but the long-term therapeutic goal is to achieve enteral autonomy whilst avoiding long term complications. This paper is aimed at discussing nutritional strategies available to clinicians caring for these patients. Methods: A literature search was performed from 1992 to 2022 using Pubmed, MEDLINE and Cochrane Database of Systematic Reviews, and recent guidelines were reviewed. In the absence of evidence, recommendations reflect the authors' expert opinion. Results: Consensus on the best possible way of feeding children with IF-SBS is lacking and practice varies widely between centres. Feeding should commence as soon as possible following surgery. Oral feeding is the preferred route and breast milk (BM) the first milk of choice in infants. Donor BM, standard preterm or term formula are alternatives in the absence of maternal BM. Extensively hydrolysed or amino acid-based feeds are used when these are not tolerated. Solids should be introduced as soon as clinically appropriate. Children are encouraged to eat by mouth and experience different tastes and textures to avoid oral aversion. Aggressive weaning of PN and tube (over-) feeding are now discouraged. Conclusions: To date, uniform agreement on the optimal type of feed, timing of food introduction and feeding regime used is lacking and great difference in practice remains. There is need for more research to establish common treatment protocols. [ABSTRACT FROM AUTHOR]

Background: Human intestinal organoids (HIOs), when transplanted into immunocompromised mice (tHIOs), demonstrate significant growth and maturation. While both male and female mice are reported to be viable hosts for these experiments, a direct comparison of sex-related differences in tHIO structure and development has not been performed. Aims: We sought to identify host sex-related differences in tHIO engraftment, morphology, and epithelial and mesenchymal development. Methods: HIOs were generated in vitro and transplanted beneath the kidney capsule of NSG male and female mice. tHIOs were harvested at 8–9 weeks. Anthropometric measurements were captured. tHIOs were divided in half and histology or RT-qPCR performed. Morphology was evaluated and epithelial architecture graded on a scale of 1 (absence of crypts/villi) to 4 (elongated crypt-villus axis). RT-qPCR and immunofluorescence microscopy were performed for epithelial and mesenchymal differentiation markers. Results: Host survival and tHIO engraftment were equivalent in male and female hosts. tHIO weight and length were also equivalent between groups. The number of lumens per tHIOs from male and female hosts was similar, but the mean lumen circumference was larger for tHIOs from male hosts. tHIOs from male hosts were more likely to demonstrate higher grades of epithelial development. However, both groups showed similar differentiation into secretory and absorptive epithelial lineages. Markers for intestinal identity, mesenchymal development, and brush border enzymes were also expressed similarly between groups. Conclusions: While male host sex was associated with larger tHIO lumen size and mucosal maturation, tHIOs from both groups had similar engraftment, growth, and epithelial and mesenchymal cytodifferentiation. [ABSTRACT FROM AUTHOR]

Purpose: Mac-2 binding protein glycosylation-modified isomer (M2BPGi) is a new marker for hepatic fibrosis progression. We examined the relationship between serum M2BPGi levels and liver histological findings in intestinal failure (IF) patients without IF-associated liver disease (IFALD). Methods: This study included IF patients without IFALD followed at our hospital. All patients underwent routine liver biopsies per protocol every 1–2 years. We examined M2BPGi levels and histological findings in relation to aspartate aminotransferase (AST) to platelet ratio index, fibrosis-4 index, and AST/ALT ratio. Liver fibrosis was evaluated based on the METAVIR score. Results: Total 18 liver biopsies out of eight patients were included. The median age was 11.5 years. Mean M2BPGi was 0.44 cutoff index (COI) in patients with F0 fibrosis, 0.78 COI in patients with F1 fibrosis and 1.63 COI in patients with F2 fibrosis. Mean M2BPGi was significantly higher in patients with F2 versus F1 or F0 fibrosis (P < 0.016 and P < 0.028, respectively). M2BPGi levels were more strongly correlated with fibrosis stage than with other conventional fibrosis markers. Conclusion: Serum M2BPGi is a novel marker of liver fibrosis in patients with IF. It is useful for follow-up prior to IFALD. Serum M2BPGi levels can support the interpretation of liver status. [ABSTRACT FROM AUTHOR]