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BACKGROUND Chronic intestinal failure (CIF) is an ultrarare disease, with an estimated national prevalence of ∼25,000 cases. There is a suspicion of widespread lack of expertise in CIF care, but no formal assessment tool or data exist. We developed and validated a knowledge test in CIF and now report our preliminary results from testing CIF knowledge in a cohort of US gastroenterologists. METHOD We developed a 20-question knowledge test in CIF, covering four key components of IF. After internal testing, refinement, and revision, we administered the test to a convenience sample of experts and nonexperts in IF. We then deployed the validated test to a cohort of 100 US gastroenterologists. RESULTS The test had a Cronbach alpha of 0.74, suggesting a reliable test, with a threshold score to discriminate experts and nonexperts of 13.4 (maximum 20) and with a sensitivity of 81.3% and specificity of 86.4%. The overall mean score of 8.2 for the 100 US gastroenterologists was at the level of nonexperts in our convenience sample. CONCLUSION The preliminary results of our validated knowledge test in IF among a broad group of US gastroenterologists demonstrate lack of knowledge in IF.

Patients with intestinal failure often need long-term home parenteral support (PS). We aimed to determine how the underlying diagnosis, complications and survival had changed over the last 36 years in the UK's largest IF centre.978 adult home PS patient records were analysed from January 1979 until October 2016. The age, sex, underlying aetiology, complications and survival was compared over 5-year periods.Pre-1990 to 2011-2016, numbers increased from 29 to 451, the mean age of patients increased from 31 ± 16.5 to 52 ± 17.6 years. The percentage of patients with IF due to surgical complications increased (3.4%-28.8%, p 0.001)), while those with inflammatory bowel disease decreased (37.9%-22.6%, p 0.001). Complication of home PS reduced: catheter related blood stream infections (CRBSI) 71.4% to 42,2%, CVC thrombosis 34.5%-5.3%. Intestinal failure associated liver disease (IFLAD) 10.3%-1.8%. Patients with dysmotility, scleroderma and a congenital aetiology had the highest incidence of CRBSI and CVC Thrombosis. Overall survival was greater pre-1995 [HR 0.2-0.4 (p = 0.02)] most likely associated with an increase in mean age. Survival for patients without malignancy was 90%, 66%, 55%, 45%, 33% and 25% at 1,5, 10, 15, 20 and 30 years respectively. Multivariate analysis demonstrated a relationship between survival and age of starting home PS; type of home PS; presence or absence of the colon in continuity; and underlying aetiology.Demand for home PS is increasing in particular for advanced malignancy, post-surgical complications and older more co-morbid patients. Complications of home PS are reducing over the last 30 years and 10-year survival for non-malignant aetiologies improving. Survival and changes in aetiology in intestinal failure.

Objectives Short bowel syndrome (SBS) is a complex and rare condition (incidence 1200/ 100,000 live births) that requires a multidisciplinary team approach to management. In January 2019 the first European Reference Network on Rare and Inherited Congenital Anomalies (ERNICA) Intestinal Failure (IF) workshop was held. Several questions about the strategies used in managing IF associated with short bowel syndrome (SBS) were devised. The aim of our study was to collect data on the enteral feeding strategies adopted by the ERNICA centres. Methods A questionnaire (36 questions) about strategies used to introduce enteral nutrition post-operatively and start complementary food/solids in infants with SBS associated IF was developed and sent to 24 centres in 15 countries that participated in the ERNICA-IF workshop. The answers were collated and compared with the literature. Results There was 100% response rate. In infants enteral nutrition was introduced as soon as possible, ideally within 24-48 hours post-small intestinal surgical resection. In 10/24 centres, bolus feeding was used, in 9 continuous, and in 5 a combination of both. Twenty-three centres used mothers' own milk as the first choice of feed with extensively hydrolysed feed, amino acid based feed, donor human milk or standard preterm/term formula as second choice. Although twenty-two centres introduced complementary/solid food by 6 months of age, food choice varied greatly between centres and appeared to be culturally based. Conclusions There is diversity in post-surgical enteral feeding strategies among centres in Europe. Further multi-centre studies could help to increase evidence-based medicine and management on this topic.

BACKGROUND Intestinal failure-associated liver disease (IFALD) increases mortality of patients with intestinal failure (IF), but lacks effective prevention or treatment approaches. Bile acids, gut microbiota and the host have close and complex interactions, which play a central role in modulating host immune and metabolic homeostasis. Increasing evidence suggests that derangement of the bile acid-gut microbiota (BA-GM) axis contributes to the development of IFALD. AIMS To review the BA-GM axis in the pathogenesis and clinical applications of IFALD, and to explore future directions for effective disease management. METHODS We conducted a literature search on bile acid and gut microbiota in IF and liver diseases. RESULTS The BA-GM axis demonstrates a unique IF signature manifesting as an increase in primary-to-secondary bile acids ratio, disturbed enterohepatic circulation, blunted bile acid signalling pathways, gut microbial dysbiosis, and altered microbial metabolic outputs. Bile acids and gut microbiota shape the compositional and functional alterations of each other in IF; collaboratively, they promote immune dysfunction and metabolic aberration in the liver. Diagnostic markers and treatments targeting the BA-GM axis showed promising potential in the management of IFALD. CONCLUSIONS Bile acids and gut microbiota play a central role in the development of IFALD and make attractive biomarkers as well as therapeutic targets. A multitarget, individualised therapy aiming at different parts of the BA-GM axis may provide optimal clinical benefits and requires future investigation.

BACKGROUND The glucagon-like peptide 2 analogue teduglutide is an effective drug for the treatment of short bowel syndrome patients with intestinal failure (SBS-IF). This intestinotrophic peptide improves intestinal capacity for fluid and nutrient absorption through induction of mucosal growth and reduction of gastrointestinal motility. Clinical trials demonstrated the efficacy of teduglutide in reducing the need for parenteral support (PS). This study describes an SBS-IF patient population receiving teduglutide therapy in a specialized medical care setting. METHOD A retrospective analysis was performed using data of patients experiencing nonmalignant SBS-IF. They were treated with teduglutide in a multidisciplinary SBS-IF program at a single university medical center between June 2016 and June 2020. RESULTS Thirteen patients under teduglutide treatment were included in the final analysis. Mean small bowel length was 82 ± 31 cm, with 77% of patients having their colon in continuity. Over a median follow-up of 107 weeks, all patients (13 of 13, 100%) responded to the therapy with a clinically significant reduction of PS volume. Mean PS reduction increased with therapy duration and ranged from -82.5% at week 24 (n = 13) to -100% in patients (n = 5) who were treated for 144 weeks. Enteral autonomy was achieved in 12 of 13 (92%) patients. Teduglutide therapy improved stool frequency and consistency, changed dietary habits, and reduced disease-associated sleep disruptions. CONCLUSION Integrating SBS-IF patients treated with teduglutide in a proactive and tight-meshed patient care program significantly improves the clinical outcome, leading to an increased proportion of patients reaching enteral autonomy.

У статті надано визначення та класифікації інтестинальної недостатності в дорослих, схвалені ESPEN. Розглянуто керівництво щодо визначень і класифікацій ІН, в якому еволюція поглядів на визначення і класифікацію ІН з 1981 року по теперішній час відображає думку 26 авторів або колективів. У керівництво після дискусій і узагальнень включені основні визначення та класифікації ІН, її патофізіологічні механізми, захворювання, які визначають розвиток ІН. Показано, що ІН визначається як зниження функції кишечника нижче мінімуму, необхідного для абсорбції макронутрієнтів і/або води і електролітів, коли внутрішньовенне доповнення потрібне для підтримання здоров’я і/або росту. Розглянута функціональна класифікація, що включає 3 типи; патофізіологічна, яка розглядає ІН як п’ять патофізіологічних станів, що походять із різних гастроінтестинальних або системних захворювань; і клінічна класифікація хронічної інтестинальної недостатності, яка на основі потреби в енергії й обсягу внутрішньовенного харчування розподіляється на 16 підтипів. Показані гастроінтестинальні або системні захворювання, які можуть визначати патофізіологічні стани інтестинальної недостатності. Для оцінки органної недостатності, пов’язаної з сепсисом, була розглянута об’єктивна, проста і доступна шкала SOFA (Sequential (sespsisrelated) Organ Failure Assessment). Також представлена Лозаннська шкала гастроінтестинальної недостатності, заснована на моделі SOFA, в яку включені всі форми інтестинальних порушень.

Purpose Surgery initiates pro-inflammatory mediator cascades leading to a variably pronounced sterile inflammation (SIRS). SIRS is associated with intestinal paralysis and breakdown of intestinal barrier and might result in abdominal sepsis. Technological progress led to the development of a neurostimulator for transcutaneous auricular vagal nerve stimulation (taVNS), which is associated with a decline in inflammatory parameters and peristalsis improvement in rodents and healthy subjects via activation of the cholinergic anti-inflammatory pathway. Therefore, taVNS might be a strategy for SIRS prophylaxis. Methods The NeuroSIRS-Study is a prospective, randomized two-armed, sham-controlled, double-blind clinical trial. The study is registered at DRKS00016892 (09.07.2020). A controlled endotoxemia is used as a SIRS-mimicking model. 2 ng/kg bodyweight lipopolysaccharide (LPS) will be administered after taVNS or sham stimulation. The primary objective is a reduction of clinical symptoms of SIRS after taVNS compared to sham stimulation. Effects of taVNS on release of inflammatory cytokines, intestinal function, and vital parameters will be analyzed. Discussion TaVNS is well-tolerated, with little to no side effects. Despite not fully mimicking postoperative inflammation, LPS challenge is the most used experimental tool to imitate SIRS and offers standardization and reproducibility. The restriction to healthy male volunteers exerts a certain bias limiting generalizability to the surgical population. Still, this pilot study aims to give first insights into taVNS as a prophylactic treatment in postoperative inflammation to pave the way for further clinical trials in patients at risk for SIRS. This would have major implications for future therapeutic approaches.

Intestinal transplantation is a therapeutic treatment option for patients with irreversible intestinal failure. The presence of donor-specific antibodies (DSAs) has been associated with increased antibody-mediated rejection and allograft loss for recipients of all the solid organ transplants. This case report describes the posttransplant course in the first year of a patient who received a T-cell and B-cell flow cross-match (FXM) and complement-dependent cytotoxicity cross-match positive intestinal transplant in the presence of several class I and class II DSAs who underwent a “temporary desensitization” using the donor spleen. The temporary donor splenic transplant removed several class I and II DSAs as demonstrated by the negative subsequent T-cell FXM, the decreased mean channel shift of the positive B-cell FXM with a significant decrease in DSA mean florescence intensity post temporary splenic transplant. The patient experienced an isolated incidence of acute rejection, which responded to therapy. He had no infectious or cancerous sequelae from the immunosuppression modalities. He was able to discontinue total parenteral nutrition and gained weight after the procedure. Long-term effects are not able to be determined from this approach; hence, further research is warranted to better evaluate the real efficacy of this strategy.

Although nutritional care is a cornerstone in the management of pediatric intestinal failure (IF), little is known about feeding difficulty (FD) prevalence. The aim of this study was to determine the frequency of FD and associated factors and to characterize eating behaviours in two pediatric IF rehabilitation centres (Hôpital-Necker Enfants Malades (NEM), France and Alberta Children's Hospital (ACH), Canada).Parents of children (aged 1-18 years) on home parenteral nutrition (PN) for3 months followed at NEM and ACH completed two validated tools: Montreal Children's Feeding scale for severity of FD, Child Eating Behaviour Questionnaire and a pediatric IF-specific questionnaire for FD associated risk factors.In the entire cohort (n = 59, median 5.2 years), 15% had mild, 19% had moderate and 25% had severe FD. No FD was seen in 53% vs 11% and severe FD was seen in 20% vs. 39% of the NEM and ACH cohorts respectively (p = 0.003). Current ETF was less common at NEM vs. ACH (3% vs. 50%, p 0.001). The FD score was associated with current enteral tube feed (ETF) use (p = 0.04). Compared to healthy reference children, the NEM cohort did not differ for the enjoyment of food, whereas the ACH cohort's enjoyment was lower (p 0.0001). The ACH cohort scored higher for food avoidance behaviours: food fussiness (p 0.02), satiety responsiveness (p 0.0001), and slowness in eating (p 0.0001) while the NEM cohort was not different from healthy reference children. In the entire cohort, according to parental recall, 60% were reported to be NPO for12 weeks in the first 6 months of life, and late introduction of purees (9 months) and lumpy textures (1 year) were found in 40% and 58%, respectively. Parent-recalled ETF differed between NEM and ACH in the first 6 months of life (45% vs 76%, p = 0.03).Feeding difficulty and associated risk factors, including early ETF, prolonged NPO and delays in achieving feeding milestones were frequently reported in pediatric IF. Feeding medicalization with the use of ETF may inadvertently contribute to FD and eating disorder behavioural characteristics. This study highlights the need for FD prevention and an increased focus on establishing healthy eating. Future prospective study of FD, associated risk factors and clinical outcomes are merited.

BACKGROUND Published reports on abnormal body composition in pediatric patients with intestinal failure have been in patients with poor growth. The goal of the current study is to report the body composition of normally growing patients with intestinal failure. METHODS Children 8-18 years old with a dual-energy x-ray absorptiometry (DXA) between January 1, 2013, and July 15, 2018, were included in the study. Data were retrospectively collected from the medical charts and included demographics, residual bowel anatomy, nutrition support, height, and weight. DXA data, including total body less head bone mineral density (BMD), fat mass (FM), and fat-free mass (FFM), were collected and compared with published literature controls matched for age and sex. RESULTS Thirty-four children met inclusion criteria. Mean age at the time of DXA was 9.6 ± 1.8 years. Weight- and height-for-age z-scores were -0.4 ± 0.9 and -0.5 ± 1.0, respectively. Mean BMD z-score was -1.0 ± 1.3. Twenty-six percent of patients (n = 9) had reduced BMD. Patients with intestinal failure had higher FM (P = .02) and lower FFM (P = .02) compared with controls. CONCLUSIONS These data show that, despite reference range z-scores for height and weight, children with intestinal failure are at risk for abnormal body composition. Body composition should be routinely measured in children with intestinal failure to direct nutrition interventions.

Pediatric intestinal failure (PIF) is a relatively rare disease entity that requires focused interdisciplinary care and specialized nutrition management. There has long been a lack of consensus in the definition of key terms related to PIF because of its rarity and a plethora of small studies rather than large trials. As such, the American Society for Parenteral and Enteral Nutrition (ASPEN) PIF Section, composed of clinicians from a variety of disciplines caring for children with intestinal failure, is uniquely poised to provide insight into this definition void. This document is the product of an effort by the Section to create evidence-based consensus definitions, with the goal of allowing for appropriate comparisons between clinical studies and measurement of long-term patient outcomes. This paper has been approved by the ASPEN Board of Directors.

Background: This study characterized the gut microbiota and its diet‐related metabolic activity in children with intestinal failure (IF) on parenteral nutrition (PN) compared with healthy controls and in relation to disease characteristics. Methods: Serial fecal samples were collected from 15 IF patients (n = 68) and from 25 healthy children (n = 25). The fecal microbiota, and short chain fatty acids (SCFA) were measured. Results: The microbiota of patients with IF had a lower bacterial load (p = 0.003), Shannon diversity index (p

Introducción: actualmente, la insuficiencia y la falla intestinal están presentes en cerca del 60 % de los pacientes en unidades de cuidado intensivo en el mundo, siendo más frecuente la presencia de insuficiencia. El objetivo de esta revisión es presentar la evidencia actual sobre la relación de la insuficiencia, la falla intestinal y su influencia en el desarrollo de la malnutrición hospitalaria. Métodos: se realizó una búsqueda en Medline y PubMed de las publicaciones que incluyeran las palabras clave “insuficiencia intestinal”, “falla intestinal”, “soporte nutricional”, “malnutrición hospitalaria”, de artículos en inglés y español. Resultados: la presencia de falla e insuficiencia intestinal aumenta la probabilidad de malnutrición en el paciente en estado crítico debido a que la absorción de nutrientes se ve disminuida y, por ende, lo hace más vulnerable a desarrollar una mayor pérdida de peso, lo que aumenta su morbimortalidad. Además, la estancia hospitalaria de los pacientes es más prolongada, y genera altos costos al sistema de salud. Esto se debe a que frecuentemente se presentan infecciones y complicaciones asociadas con la desnutrición y la pérdida de productividad, lo que disminuye la calidad de vida, con efectos negativos sobre el entorno social. Conclusiones: la insuficiencia y falla intestinal son situaciones que pueden generarse de manera bidireccional. Las diferencias en la clasificación de la falla intestinal según las diversas asociaciones afectan el manejo de los pacientes. Palabras clave: insuficiencia intestinal, falla intestinal, soporte nutricional, malnutrición hospitalaria.

Since the first clinical description in 1952, immunoglobulin replacement therapy remains the mainstay of treatment of patients with X-linked agammaglobulinemia (XLA). However, this therapy only replaces IgG isotype and does not compensate for the loss of Bruton tyrosine kinase in non-B-lymphocytes. Patients may still therefore develop complications despite current standard of care. Here, we describe an XLA patient with persistent chronic norovirus infection, refractory to treatment and causing intestinal failure. The patient underwent haematopoietic stem cell transplantation, curing XLA and allowed clearance of norovirus prior to humoral immunoreconstitution, suggesting non-humoral immunodeficiency in these patients.

Introduction Short bowel syndrome (SBS) is a rare, highly disabling, life-threatening condition due to extensive intestinal resections, characterized by diarrhea, malabsorption, and malnutrition. SBS is the main cause of intestinal failure (SBS-IF). The primary therapy for SBS-IF is intravenous supplementation (IVS) of nutrients. The pharmacological therapy aims to improve the remnant bowel function, leading to the decrease of IVS requirement. Areas covered This review provides a safety perspective and discusses unmet clinical needs on pharmacotherapy for SBS, ranging from symptomatic agents traditionally used off-label to manage hypersecretion and diarrhea, to curative drugs with selective intestinotrophic properties. Real-world evidence on symptomatic drugs is lacking. Data on teduglutide - the first-in-class glucagon-like peptide-2 (GLP-2) receptor agonist approved in SBS - are mainly derived from clinical trials, with several unsettled safety issues, including the risk of malignancies. Expert opinion Defining the long-term safety of drugs used for SBS is a priority; a unified list of commonly used drugs with consolidated proof of effectiveness is needed to harmonize the symptomatic pharmacological approach to SBS. GLP-2 receptor agonists are a promising curative pharmaco-therapeutic approach, although long-term safety and effectiveness deserve further real-world assessment. Pharmacovigilance and global data sharing are crucial to support safe prescribing in SBS.

Our aim was to analyze the outcomes in children with short-bowel syndrome (SBS), parenteral nutrition dependence (PND), and intestinal failure-associated liver disease (IFALD) treated in our Intestinal Rehabilitation Program (IRP) during 2007-2018. We retrospectively reviewed charts of 135 patients with SBS-PND at the time of enrollment in IRP; of these, 89 (66%) had IFALD, defined as conjugated bilirubin (CB) of ≥2 mg/dl at enrollment and/or abnormal liver biopsy showing stage 2-4 fibrosis. Outcomes included resolution of CB, enteral autonomy, laboratory parameters (platelets, aspartate aminotransferase to platelet ratio index), growth trends, transplant rates, and mortality. Of the 89 patients, 74 had elevated CB at enrollment; the other 15 had normalized CB but had fibrosis on liver biopsy. Thirty-eight patients had liver biopsies: 36 (95%) had fibrosis, including 21/36 with bridging fibrosis/cirrhosis. The median proportion of residual small bowel was 23% (interquartile range, 13%-38%) of the expected length for age and median, daily energy requirement by PN was 100%. Two received a transplant, three died (one posttransplant), and the remaining 85 survived; 69 (81%) achieved enteral autonomy. Seventy-three (99%) of the 74 patients with hyperbilirubinemia normalized their CB with medical treatment. In a subset of eight of 89 patients with initial platelet count of

Summary Introduction Patients with chronic intestinal failure (IF) require home parenteral nutrition (HPN). Central venous access is needed for prolonged use of PN, usually via a long term central venous access device (CVAD). Post insertion there may be mechanical complications with a CVAD such as catheter rupture or tear. Repair of damaged CVADs is possible to avoid risks associated with catheter replacement in patients with IF. However, catheter related blood stream infections (CRBSI) are a concern when CVAD's are accessed or manipulated. Aims To investigate the success of repair of CVADs in patients with IF on HPN, related to repair longevity and incidence of CRBSI following repair. Method Nutrition team records of CVAD repairs carried out in patients with IF were reviewed retrospectively for the period April 2015 to March 2019. Results Nutrition Clinical Nurse Specialists carried out 38 repairs in 27 patients. Male n = 5, female n = 22; mean age 55 years. Catheter longevity before first repair (n = 27): median 851 days, IQR 137–1484 days. 30/38 (78.9%) of repairs were successful lasting ≥30days. Hospital admission was avoided in 76% of cases. 4 patients in the failed repair group underwent catheter re-insertion where 4 had a further, subsequently successful, repair, an overall success rate of 89.4% (34/38). 30-day CRBSI rate was 0.09/1000 catheter days in repaired catheters. In comparing costs, there is a potential cost saving of 2766GBP for repair compared to replacement of damaged CVADs. Conclusion Repair of tunnelled CVADs in patients with IF is successful and safe with no increased risk of CRBSI. Significant cost savings may be made.

Summary Aim Home parenteral nutrition (HPN) is one of the most demanding medical therapies as it is the only option for patients for intestinal failure (IF). No unequivocal policy on how to start and progress with HPN has ever been presented. The IF Center at the Stanley Dudrick's Memorial Hospital in Skawina is one of the biggest centers in Poland, celebrating its twentieth birthday last year. It offered the unique chance to present how to create and grow the IF center, increasing the quality of care. The above became the aim of this study. Methods A retrospective analysis of all medical records of HPN patients from the University Hospital and Stanley Dudrick's Memorial Hospital in Skawina. Patient profile, clinical course and treatment outcomes were assessed. The evolution of all aspects of HPN is presented. A brief historical perspective has been added to better illustrate the center's growth and transformation. Results 608 patients (363 female, 245 male, mean age 55.55 year) from all over Poland were treated between December 1999 and December 2019. The most frequent indication for HPN was mechanical obstruction (277, 45.7%), followed by short bowel syndrome (SBS, 208, 34.3%) and intestinal fistula (46, 7.59%). The most common primary disease was cancer (n = 267), followed by mesenteric ischemia (n = 104), and surgical complications (n = 62). 314 patients (51.8%) died. 73 (12.04%) were successfully weaned off PN. The catheter infection rate reached 0.39/1000 catheter days. Conclusions the founding of the HPN center and its further development is possible provided that there is team of dedicated people, supported by hospital base. The real growth opportunity is guaranteed by the reimbursement. It is necessary to adapt to the changing circumstances.

Introduction Central line-associated bloodstream infections (CLABSIs) lead to significant morbidity and mortality in children with intestinal failure (IF). Ethanol lock prophylaxis (ELP) greatly reduces CLABSI frequency with minimal side effects. However, in the United States, a recently approved orphan drug designation for dehydrated alcohol has greatly increased 70% ethanol cost from about $10/day to $1000/day. We examined the cost-effectiveness of ELP in relation to these changes. Methods We simulated a previously developed IF Markov model over 1 year. Costs were measured in 2020 US dollars and effectiveness in quality-adjusted life-years (QALYs). CLABSI rate with and without ELP was estimated from the largest available comparative observational study. The primary outcome was incremental cost-effectiveness ratio (ICER) between treatments. Secondary outcomes included CLABSI frequency. Sensitivity analyses on all model parameters were performed. Results In the base model, children with IF not using ELP accumulated $131,815 in costs and 0.32 QALYs per patient compared with $437,884 and 0.33 QALYs per patient in those using ELP. The ICER was nearly $17 million/QALY gained. ELP resulted in a 40% reduction in CLABSI frequency. ELP became cost-effective at $68/day and cost-saving at $63/day. Sensitivity analysis identified no other plausible parameter variation to reach the benchmark of $100,000/QALY gained. Conclusions At the current price, ELP is not cost-effective for CLABSI prevention in children with IF in the United States. This study highlights the critical need for the approval of an affordable lock therapy option to prevent CLABSIs in these children.

Background: The direct effect of the COVID‐19 pandemic on patients with intestinal failure (IF) has not been described. Methods: We conducted a nationwide study of U.K. IF centres to evaluate the infection rates, presentations and outcomes in patients with types 2 and 3 IF. Results: A total of 45 patients with IF contracted COVID‐19 between March and August 2020; this included 26 of 2191 (1.2%) Home Parenteral Nutrition (HPN)‐dependent adults and 19 of 298 (6.4%) adults hospitalized with type 2 IF. The proportion of patients receiving nursing care for HPN administration was higher in those with community‐acquired COVID‐19 (66.7%) than the proportion in the entire HPN cohort (26.1%; p

BACKGROUND & AIMS: Home parenteral nutrition (HPN) is a life-saving therapy for patients with chronic intestinal failure but can be associated with a degree of psychological distress. The factors associated with the need for antidepressants (AD) in this cohort have not yet been described.METHODS: The study involved prospective data collection from patients attending HPN clinic at a national intestinal failure referral centre. Patients requiring HPN as a result of active malignancy were excluded. Patients were divided in two groups according to AD usage; demographic, anthropometric, socioeconomic characteristics and IVS regimens were compared between groups.RESULTS: A total of 184 patients were recruited between July 2018 and April 2019, with an overall prevalence of AD use of 41.7%. Daily mean IVS volume was significantly higher among patients taking AD ('AD' group; 2125.48±991.8 ml/day, 'no-AD' group; 1828.54±847.0 ml/day, P = 0.039) with the proportion of patients needing high volume IVS (≥3000ml/day) being three-times higher in the AD group (20.0% vs. 6.1%, P = 0.006). The average energy IVS infusion per day was similar between the two groups. Hypnotic use (25.7% vs. 5.1%, P

Aim High-output enterocutaneous fistulas (ECFs) are an established cause of intestinal failure. Parenteral nutrition (PN) remains the gold standard for nutritional management but is complex, expensive and associated with significant complications. Chyme reinfusion (CR) has been reported by multiple centres as a viable option for nutritional management that improves nutritional status, provides the capacity to cease PN and is cost-effective. The aim of this paper is to describe the first use of a novel pump device (The Insides System™) by an independent centre in Australia for the nutritional management of a patient with high-output ECF. Method CR was performed on a 66-year-old woman with a high-output ECF. The device consists of two main components: a centrifugal pump that sits inside the stoma appliance and a battery-powered driver that is magnetically coupled externally onto the pump. The device allows for bolus CR at a rate of infusion that is manually controlled by the patient based on comfort, volume and effluent viscosity. Results CR provided adequate nutritional support, with successful cessation of PN. Effective use of the device was learnt easily by the patient with minimal demands on nursing assistance. Side effects of CR (diarrhoea, abdominal cramping) were overcome by the patient's ability to manually adjust the reinfusion rate. Conclusion Our experience with the novel Insides System™ device showed promising results in maintaining nutritional status as well as providing a minimally invasive, easy to use and low-cost system for CR. CR should be considered as a viable alternative for the nutritional management of patients with a high-output ECF.

Summary Background Use of HPN in paediatrics in the UK has increased rapidly over the last 20 years but the prevalence of HPN has been challenging to define. Clinicians in the UK have noted an evolving complexity of cases and perceive improved outcomes and increased acceptability of long-term PN. These factors combined have the potential to increase the burden on existing paediatric gastroenterology services in the UK. Methods A national database was interrogated to define the prevalence of HPN in children in the UK and to explore outcomes for patients receiving HPN. Results Since 2015, 525 children were notified to the database; of these patients, mortality was In 2019, 389 children received HPN in the UK; this is nearly double the number last reported in 2012 and is a prevalence of 30 per million children. Short bowel syndrome is the largest category of these patients. However, a poorly defined group including those with multisystem disease has increased 10 fold since 2012 and is now the second largest category. Conclusions Long term HPN in childhood is safe and associated with good survival and low risk of the need for intestinal transplantation.

OBJECTIVES With improved survival of children with intestinal failure (IF), it is important to examine the impact on long-term physical function, physical activity (PA), and fatigue and identify clinical factors that may be predictive of impairment. METHODS Cross-sectional study in children with IF on parenteral nutrition (PN) compared with healthy age- and sex-matched controls (HCs). Assessments included: Paediatric Quality of Life (PedsQL) Physical Function subscale, PedsQL Multi-Dimensional Fatigue Scale, and PA Perceived Benefits and Barriers scale. PA was measured using an accelerometer. Medical data was collected by chart abstraction. RESULTS Participants included 21 children with IF (14 girls), median age 8.33 (interquartile range [IQR] 6.96-11.04) years and 33 HCs (20 boys), 8.25 (6.67-10.79) years. In those with IF, 13 (62%) were born prematurely with a median of 15 (7.5-24.5) in-patient hospitalizations. There was a significant difference (P = 0.033) in mean steps/day in children with IF (9709 +/- 3975) compared with HCs (13104 +/- 5416), and a correlation between moderate-to-vigorous PA and gestational age (r = 0.642, P = 0.010). Child and parent proxy scores indicate poorer physical function and greater fatigue in the IF group, along with a correlation between greater fatigue (r = -0.538, P = 0.012), poorer physical function (r = -0.0650, P = 0.0001) in children with more hospitalizations. Barriers to PA include "I am tired" and "I am worried about my line." CONCLUSIONS Children with IF present with lower levels of PA and physical function and greater fatigue compared with their peers. Ongoing development of medical and rehabilitation intervention strategies is vital to optimize outcomes.

OBJECTIVE The aim of the study was to assess overall and disease-specific health-related quality of life (hrQOL) in patients with pediatric intestinal failure (PIF) and caregivers and elucidate differences from healthy and chronic gastrointestinal (GI) illness cohorts. METHODS Cross-sectional study of patients with PIF and their caregivers managed at a multidisciplinary intestinal rehabilitation program using the PedsQL Generic Core and the Gastrointestinal Symptoms Module to assess generic and disease-specific hrQOL, respectively. These data were compared to established healthy and chronic GI disease controls. RESULTS A total of 53 patients (mean age 6.2 ± 3.9 years) and their caregivers were studied. Patients reported lower generic hrQOL than healthy children (73.0 vs 83.84, P

Intestine transplant can be life-saving and can improve quality of life for patients with intestinal failure. Medical and surgical advances in treatment of intestinal failure over the past 10 to 15 years have resulted in fewer patients being added to the waiting list for intestine transplant alone or for intestine transplant in combination with liver transplant (and sometimes other organs). Consequently, fewer transplants are being performed. The numbers of listings and transplants fell to new lows in 2019. The number of programs performing transplants in at least one patient in 2019 was the lowest in the last decade, equal to 2014, at 15. Graft failure plateaued over the past decade, but early graft loss has increased in the past 2 years, notably in recipients of a combined liver and intestine allograft. Five-year patient survival for transplants in 2012-2014 varied little by graft type.

Intestinal failure is broadly understood as the failure of the gut to achieve adequate absorption of macronutrients and/or fluids and electrolytes. Enterocutaneous fistula, an abnormal connection between the gastrointestinal tract and skin, is one aetiology for intestinal failure. In this article we explore the critical knowledge within a clear framework for managing this challenging condition. We expand on the role of the multidisciplinary team and specialized tertiary centres to enhance the clinical application of this knowledge in practice. Throughout this article we highlight specific tips and pitfalls in the management of these patients.

Background Intestinal failure (IF) is a rare but severe form of organ failure. The condition is defined as body’s inability to absorb adequate fluids, macronutrients and minerals for growth and development, so that intravenous supplementation is necessary. A broad spectrum of diseases, trauma and complications of surgery might eventually end up with intestinal failure. Nowadays, intestinal failure patients are preferably cared for in intestinal rehabilitation units (IRU). Autologous gastrointestinal reconstruction (AGIR) refers to non-transplant operative management of IF patients designed to improve enteral tolerance and gut absorptive capacity. Case presentation Herein we present five cases with complications of surgeries due to peptic ulcer bleeding, blunt abdominal trauma, obesity and gastric tumor. The surgeries were complicated by anastomotic leak, peritonitis and fistula formation. By adopting multidisciplinary decisions and special care for each complication, all the five patients were successfully managed and discharged. Discussion and conclusions As presented, re-anastomosis in presence of abdominal contamination will probably fail. In patients with intestinal failure, PN should start as soon as possible to increase the success rate of future surgeries and prevent potential need for intestinal transplantation. We suggest referring patients with complicated outcomes of gastrointestinal surgeries to the IRUs to reduce morbidity and mortality.

Position paper of the Federal Association of German Hospital Pharmacists, the nutrition committees of the German and Austrian Societies of Pediatrics and Adolescent Medicine and the working group on intestinal failure of the Society of Pediatric Gastroenterology and Nutrition. In 2019 the German Federal Institute for Drugs and Medical Devices (Bundesinstitut fur Arzneimittel und Medizinprodukte, BfArM) launched a “doctors letter” (Rote-Hand-Brief) recommending light protection of all parenteral nutrition (PN) solutions during administration for preterm infants, term infants and children less than 2 years of age. The aim of the present paper is to review the available evidence and provide evidence-based recommendations for hospital and home PN light protection in children. The Federal Association of German Hospital Pharmacists, the nutrition committees of the German and Austrian Societies of Pediatrics and Adolescent Medicine and the working group on intestinal failure of the Society of Pediatric Gastroenterology and Nutrition recommend in accordance with the recommendations of the ESPGHAN, ESPEN, ESPR and CESPEN guidelines on pediatric parenteral nutrition: 1. Lipid emulsions and/or vitamins containing PN solutions should be protected from light during administration (bags and tubing) to prevent the generation of oxidants. 2. There is no conclusive evidence for recommending light protection for PN solutions without lipid emulsions and without vitamins during administration (solutions containing, e.g. amino acids, glucose, electrolytes, or trace elements). In addition, in home PN non-light-protected spiral tubing may be used because validated light-protected spiral tubing is currently not available, given that spiral tubing improves the range of motion and safety in infants and young children.

Short-bowel syndrome (SBS) is defined as a state of malabsorption after resection or loss of a major portion of the bowel due to congenital or acquired factors. This article presents an overview on the recent management of pediatric SBS. The pediatric SBS population is very heterogeneous. The incidence of SBS is estimated to be 24.5 per 100,000 live births. The nutritional, medical, and surgical therapies available require a comprehensive evaluation. Thus, multidisciplinary intestinal rehabilitation programs (IRPs) are necessary for the management of these complex patients. The key points of focus in IRP management are hepato-protective strategies to minimize intestinal failure-associated liver disease; the aggressive prevention of catheter-related bloodstream infections; strategic nutritional supply to optimize the absorption of enteral calories; and the management and prevention of small bowel bacterial overgrowth, nephrocalcinosis, and metabolic bone disease. As the survival rate of children with SBS currently exceeds 90%, the application of small bowel transplantation has been evolving. The introduction of innovative treatments, such as combined therapy of intestinotrophic hormones, including glucagon-like peptide-2, may lead to further improvements in patients' quality of life.

We report the case of treatment of a 30-year-old woman with an impaired passage in the duodenojejunal segment of the gastrointestinal tract due to Ladd’s syndrome. Occlusion of the superior mesenteric artery was developed as a result of incomplete rotation of midgut in dysembryogenesis. Mesenteric blood circulation was compensated by collaterals formed in the antenatal period. Ladd’s operation was performed to treat acute intestinal failure, the duodenojejunal passage was restored. The patient was discharged on the 11th postoperative day in a good condition with the main diagnosis «Ladd’s syndrome». In the presented case report, the impaired passage in the gastrointestinal tract in the middle age patient was traced from childhood. Detailed examination of medical history, correct interpretation of MSCT findings allowed to form an accurate diagnosis and perform effective surgical treatment.

Summary Background This practical guideline is based on the ESPEN Guidelines on Chronic Intestinal Failure in Adults. Methodology ESPEN guidelines have been shortened and transformed into flow charts for easier use in clinical practice. The practical guideline is dedicated to all professionals including physicians, dieticians, nutritionists, and nurses working with patients with chronic intestinal failure. Results This practical guideline consists of 112 recommendations with short commentaries for the management and treatment of benign chronic intestinal failure, including home parenteral nutrition and its complications, intestinal rehabilitation, and intestinal transplantation. Conclusion This practical guideline gives guidance to health care providers involved in the management of patients with chronic intestinal failure.

Introduction: Intestinal failure-associated liver disease (IFALD) refers to hepatic dysfunction that results from prolonged parenteral nutrition (PN) use. IFALD is multifactorial in origin and remains a major cause of morbidity and mortality. Prior to 2004, IFALD was associated with mortality as high as 90% in infants who remained on PN greater than 1 year. The advent of new strategies for intravenous lipid emulsion (ILE) administration and improved catheter care now allow many patients to remain on PN and recover from this once fatal condition. Several additional treatment modalities are often used to further improve outcomes for IFALD patients and they are reviewed here.Areas covered: The etiology of IFALD is presented, as well as the rationale behind the use of ILEs that contain fish oil. Other management strategies are addressed, including the effects of several pharmacologic and nutritional interventions.Expert opinion: Like its etiology, the management of IFALD is multifactorial. Prompt recognition of patients at risk, avoiding macronutrient excess, and preventing central line associated bloodstream infections will improve outcomes. In patients who develop IFALD, the use of fish oil monotherapy seems to be efficacious. The most effective intervention, however, continues to be discontinuation of PN and achieving full enteral feedings.

High output entero-cutaneous fistulas may lead to intestinal failure with parenteral nutrition (PN) as the gold standard treatment to prevent dehydration and malnutrition. However in case of entero-atmospheric fistula (EAF) with the distal limb of the fistula accessible, chyme reinfusion (CR), a technique that restores artificially digestive continuity can be performed until the surgical repair. Our aim was to study the efficacy of CR in EAF regarding nutritional status, intestinal function, PN weaning and liver tests.Retrospective study of 37 patients admitted for EAF and treated by CR from 1993 to 2017. Delays were expressed in median (25%-75% quartiles) and other data on mean ± SD.Location of EAF: jejunum (29), ileum (8). The length of the upstream intestine was estimated in 21 patients: 19 had a bowel length150 cm of which 16 had less than 100 cm. During CR, mean digestive losses decreased from 1734 ± 578 to 443 ± 487 ml/24 h (p0.000001), nitrogen absorption increased from 45.3 ± 18.6 to 81.8 ± 12.9% of ingesta (p0.001). The percentage of patients with plasma citrulline20 µmol/l decreased from 71 to 10%. PN was stopped in all patients within 3 (0-14) days after CR initiation, 2 patients required an intravenous hydration and 20 had an additional enteral support. The nutritional status improved: albumin (33.1 ± 5.1 g/L vs 28.4 ± 6.5, p0.001), NRI (decrease of the number of patients at risk of severe malnutrition from 22 to 10 (p0.001)). The number of patients who had one or several liver tests abnormalities (2 N) decreased from 94 to 41% (p0.001).When the efferent part of the small bowel is accessible, CR is a safe and inexpensive method that restores bowel function. In most cases, it makes it possible to stop PN and helps to improve the nutritional status until surgical reconstruction.

Acute intestinal failure (AIF), “reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, requiring parenteral nutrition”, is common, but very often neglected part of multiorgan dysfunction syndrome (MODS) in the critically ill patients. It is now increasingly being recognized as a cause of prolonged ICU and hospital stay and poor outcome. Multidisciplinary team approach, systematic approach to management with treatment of sepsis, early mobilization and enteral feeding with prokinetics if required, control of intra-abdominal pressure and surgical intervention, when mandated, can help treat AIF and improve patient outcomes. How to cite this article: Chandankhede SR, Kulkarni AP. Acute Intestinal Failure. Indian J Crit Care Med 2020;24(Suppl 4):S168–S174.

Background Caregivers of children with intestinal failure (IF) face difficult decisions without a clear best alternative. Providers assist in decision-making but often lack knowledge of caregiver perspectives. Using decision-making around anemia treatment as a focal point, we explored how caregivers of children with IF prefer to make decisions. Our goal was to offer insight to guide providers as they assist in decision-making. Methods We conducted 12 half-hour semistructured interviews with parents of children with IF. Interview questions addressed general decision-making and specifics of iron supplementation, including key factors and stakeholders in decision-making. Interviews were transcribed verbatim. Two investigators coded the transcripts and inductively derived themes. Results Four themes were identified regarding decision-making. They involved the search for reliable, accurate, and positive information; the role of caretakers on the medical team; the relationships between caretakers and the medical team; and effective communication. Themes around anemia treatment included: identification of anemia by bloodwork; proactive supplementation; individualized regimens; prioritizing safety and convenience. Conclusions Understanding caregiver perspectives regarding anemia treatment in pediatric IF identifies opportunities for systematic quality improvement.

In response to the novel COVID-19 pandemic, rapid and unprecedented public infection control measures were undertaken by all four nations of the UK culminating in ‘lockdown’ with the majority of the population being asked to stay at home other than for a few designated essential activities. In addition, identified vulnerable members of the population were required to participate in ‘enhanced social distancing’ or ‘shielding’, remaining strictly housebound, dependent on outside assistance for essential items and isolating from members within their household. This was proposed for 12 weeks in the first instance.1–4 Necessity for shielding was considered on the basis of relative burden of chronic disease and known risk factors for severe COVID-19 infection; however, young age conferred a protective association with infection.5 Although central government described the principles of enhanced distancing measures, the framework for the degree of measures employed and to which distinct patient groups was largely devolved to national expert bodies. As such, multiple national expert bodies considered adults with long-term intestinal failure (IF) with an ongoing need for home parenteral nutrition (HPN), as significantly vulnerable enough to warrant ‘shielding’.6 7 The Royal College of Paediatrics and Child Health (RCPCH), in consultation with multiple paediatric specialty groups, published advice on the principles of ‘shielding’ for children. The advice outlined the unique challenges faced by families and carers delivering socially distanced care to dependent children who have specific conditions, and also the impact of ‘shielding’ on children.8 The RCPCH and the British Society for Paediatric Gastroenterology Hepatology and Nutrition (BSPGHAN) endorsed ‘shielding’ for a number of key chronic gastrointestinal conditions, with a stratified approach in some conditions such as inflammatory bowel disease. The consensus of the BSPGHAN Intestinal Failure Working Group (BIFWG) was that children and young people receiving HPN should participate in shielding.8 …

Small bowel obstruction (SBO) is 3-4% of all abdominal acute diseases. Small bowel obstruction due to bezoars is rare (2-4%), and is detected mainly in patients with predisposing risk factors: gastrointestinal motility disorders, psychiatric diseases, enzymatic insufficiency, previous bariatric surgery, diabetes mellitus and hypothyroidism complicated by gastroparesis. The leading role in the verification of small bowel obstruction in patients without surgical interventions on the abdominal organs should be given to computed tomography. According to the literature, the mortality from SBO due to bezoar may reach 30%. We present an unusual clinical case of recurrent small bowel obstruction with severe intestinal disease and septic shock induced by multiple phytobezoars and complicated with severe pseudomembranous colitis. Moreover, in our opinion, laparoscopic access can be used both for diagnostic and therapeutic purposes in patients with acute small bowel obstruction.

Background: Patients with short-bowel syndrome and intestinal failure (SBS-IF) require parenteral support (PS) and may need long-term home-care support. This survey assessed the impact of care provision on adult caregivers of adult patients receiving PS for SBS-IF. Methods: An online, cross-sectional survey of caregivers of adults with a self-reported physician diagnosis of SBS-IF was conducted in France, Germany, Italy, the UK, and USA. Impact on caregivers was evaluated using the 18-item Caregiver Strain Index (CSI), the Work Productivity and Activity Impairment Questionnaire: Specific Health Problem (WPAI:SHP), and self-reporting impact questionnaires. Results: Caregivers (N = 121; aged 51 ± 13.7 years; 59% women) provided assistance for a mean of 9.9 ± 12.53 years; 77% were providing care 7 days per week. Patients (51 ± 16.4 years; 56% women) of caregivers were typically family members: spouse/partner (61%), adult son/daughter (19%), or parent (10%). Caregivers reported experiencing some strain (CSI score 4 ± 3.4). Among 71 of 73 employed caregivers, the WPAI:SHP assessment showed that caregivers missed 7% ± 12.7% of work hours in the preceding week and were present but not productive at work 37% ± 23.1% of the time; 28% of caregivers reported a reduced number of working hours because of caregiving. Many caregivers reported limitations in recreational activities (53%), and ≥30% had difficulty spending time with family and friends. Caregivers (87%) also reported worrying about the patient's health. Conclusions: Caregivers of adult patients with SBS-IF experience negative daily personal impacts and loss of productivity arising from their caregiving responsibilities.

Background: Treatment with glucagon-like peptide-2 (GLP-2) analogs improve intestinal adaptation in patients with short bowel syndrome–associated intestinal failure (SBS-IF) and may reduce parenteral support requirements. Apraglutide is a novel, long-acting GLP-2 analog designed for once-weekly dosing. This trial investigated the safety and efficacy of apraglutide in patients with SBS-IF. Methods: In this placebo-controlled, double-blind, randomized, crossover phase 2 trial, eight adults with SBS-IF were treated with once-weekly 5-mg apraglutide doses and placebo for 4 weeks, followed by once-weekly 10-mg apraglutide doses for 4 weeks, with a washout period of 6–10 weeks between treatments. Safety was the primary end point. Secondary end points included changes from baseline in urine volume output compared with placebo, collected for 48 h before and after each treatment period. Results: Common treatment-related adverse events (AEs) were mild to moderate and included polyuria, decreased stoma output, stoma complications, decreased thirst, and edema. No serious AEs were considered to be related to apraglutide treatment. The safety profile was comparable for the lower and higher doses. Treatment with once-weekly 5- and 10-mg apraglutide doses significantly increased urine volume output by an adjusted mean of 714 ml/day (95% CI, 490–939; P

Intestinal failure (IF) patients are dependent on central venous access to receive parenteral nutrition. Longstanding central venous catheters are associated with life-threatening complications including infections and thromboses resulting in multiple line exchanges and the development ofprogressive central venous stenosis or occlusion. The Haemodialysis Reliable Outflow (HeRO) graft is an arterio-venous device that has been successfully used in haemodialysis patients with ‘end-stage vascular access’. We describe a case series of HeRO graft use in patients with IF and end-stage vascular access. Four HeRO grafts were inserted into IF patients with end-stage vascular access to facilitate or support intestinal transplantation. In all patients the HeRO facilitated immediate vascular access, supporting different combinations of parenteral nutrition, intravenous medications, fluids or renal replacement therapy with no bloodstream infections. In a highly complex group of IF patients with central venous stenosis/occlusion limiting conventional venous access or at risk of life-threatening catheter-related complications, a HeRO® graft can be a feasible alternative.

Background Pediatric intestinal rehabilitation (PIR) programs are associated with improved outcomes in children with intestinal failure but remain heterogeneous nationally. This study characterizes PIR program components to aid those seeking to establish or expand a program. Methods Members of the Children's Hospital Association reporting a PIR program to the US News and World Report completed a 14-item questionnaire using the Qualtrics Online Survey Software. Programs were categorized as small or large (≤50 vs >50 patients) and new or established (≤10 vs >10 years). Results Seventy-one programs were identified and 61 surveys were returned for a response rate of 86%. Majority of programs had gastroenterology, surgery, nutrition, nursing, and social work services involved. Large programs (n = 34; 59%) were more likely to serve as referral centers; have greater participation by nursing, social work, and primary care; have more dedicated time by gastroenterology, surgery, nursing, nutrition, and social work; have more frequent meetings; and have various funding sources (P Conclusion Critical components of a PIR program include gastroenterology, surgery, and nutrition services with strong nursing and social work support. These data document the components of modern PIR programs, though further studies on the relationship between program structure and patient outcomes are warranted.

The role of total parenteral nutrition (TPN) in cancer patients is controversial, but it may be a treatment option for some patients with indolent but advanced small intestinal neuroendocrine neoplasms (SI-NENs). The aim of this study is to investigate whether home TPN was associated with long-term survival and to assess the indications, duration and complications of TPN in patients with advanced SI-NENs. Patients with advanced SI-NENs who received home TPN were retrospectively included. Electronic records were reviewed for clinical information. Five patients receiving home TPN were identified out of 1011 patients with SI-NENs in our center. The median duration of TPN administration was 12 mo. Small bowel obstruction was the most common reason for TPN initiation. TPN-related complications included two catheter infections, one thrombosis and one episode of TPN-related transaminitis. At the last follow-up, three patients had died and two were alive. The median survival was 12 mo. Overall estimated 1-yr probability of survival on home TPN by Kaplan-Meier analysis was 40%. In conclusion, home TPN may be a treatment option in highly selected advanced SI-NEN patients with severe gastrointestinal tract dysfunction. The initiation of home TPN is associated with long-term survival (≥1 yr), and complication rates appear acceptable.

Pediatric intestinal failure (IF) is the critical reduction of intestinal mass or function below the amount necessary for normal growth in children. Short-bowel syndrome (SBS) is the most common cause of IF in infants and children and is caused by intestinal resection. Enteral autonomy and freedom from parenteral nutrition is the mainstay of nutrition management in SBS. The goal is to achieve intestinal adaptation while maintaining proper growth and development. Treatment is variable, and there remains a paucity of evidence to draw well-informed conclusions for the care of individuals in this complex population. Physiological principles of enteral nutrition and practical recommendations for advancing the diet of patients with pediatric SBS are presented. Emerging trends in nutrition management, such as the growing interest in blending diets and the impact on SBS, are reviewed. Finally, the influence of the microbiome on enteral tolerance and small bowel bacterial overgrowth are considered.

OBJECTIVE Gastrointestinal dysmotility is common in patients with pediatric intestinal failure (PIF), leading to delays in advancement of enteral nutrition (EN). Few studies have been published regarding the safety and efficacy of cisapride for improvement of enteral tolerance and ability to wean parenteral nutrition. Our objective was to describe a single center experience on the use of cisapride in patients with PIF. METHODS Retrospective chart review of patients was performed. Demographic, intestinal anatomy, and outcome data were collected. Percentage of EN before initiation of cisapride, progression of EN at 3 and 6 months, and ability to wean parenteral nutrition after initiation of cisapride were calculated. RESULTS Prokinetics were used in 61 of 106 patients (56.6%); 29 of 60 patients (48.3%) failed to progress EN on other prokinetics and started on cisapride. Before cisapride the progress of EN plateaued for a mean of 42.3 (standard deviation [SD] 60.2) days. The rate of feed progression was 0.14% (SD 0.19)/day pre-cisapride and 0.69%/day (SD 0.31) after cisapride initiation (P

Aim Type 2 intestinal failure (IF) is characterized by the need for longer-term parenteral nutrition (PN). During this so-called bridging-to-surgery period, morbidity and mortality rates are high. This study aimed to evaluate to what extent a multidisciplinary IF team is capable to safely guide patients towards reconstructive surgery. Methods A consecutive series of patients with type 2 IF followed up by a specialized IF team between January 1st, 2011, and March 1st, 2016, was analyzed. Data on their first outpatient clinic visit (T1) and their last visit before reconstructive surgery (T2) was collected. The primary outcome was a combined endpoint of a patient being able to recover at home, have (partial) oral intake, and a normal albumin level (> 35 g/L) before surgery. Results Ninety-three patients were included. The median number of previous abdominal procedures was 4. At T2 (last visit prior to reconstructive surgery), significantly more patients met the combined primary endpoint compared with T1 (first IF team consultation) (66.7% vs. 28.0% (p < 0.0001), respectively); 86% had home PN. During “bridging-to-surgery,” acute hospitalization rate was 40.9% and acute surgery was 4.3%. Postoperatively, 44.1% experienced a major complication, 5.4% had a fistula, and in-hospital mortality was 6.5%. Of the cohort, 86% regained enteral autonomy, and when excluding in-hospital mortality and incomplete follow-up, this was 94.1%. An albumin level < 35 g/L at T2 and weight loss of > 10% at T2 compared with preadmission weight were significant risk factors for major complications. Conclusion Bridging-to-surgery of type 2 IF patients under the guidance of an IF team resulted in the majority of patients being managed at home, having oral intake, and restored albumin levels prior to reconstructive surgery compared with their first IF consultation.

Background Fibrosis is the final common pathway to intestinal failure in Crohn’s disease, but no medical therapies exist to treat intestinal fibrosis. Activated myofibroblasts are key effector cells of fibrosis in multiple organ systems, including the intestine. AXL is a receptor tyrosine kinase that has been implicated in fibrogenic pathways involving myofibroblast activation. We aimed to investigate the AXL pathway as a potential target for the treatment of intestinal fibrosis. Methods To establish proof of concept, we first analyzed AXL gene expression in 2 in vivo models of intestinal fibrosis and 3 in vitro models of intestinal fibrosis. We then tested whether pharmacological inhibition of AXL signaling could reduce fibrogenesis in 3 in vitro models of intestinal fibrosis. In vitro testing included 2 distinct cell culture models of intestinal fibrosis (matrix stiffness and TGF-β1 treatment) and a human intestinal organoid model using TGF-β1 cytokine stimulation. Results Our findings suggest that the AXL pathway is induced in models of intestinal fibrosis. We demonstrate that inhibition of AXL signaling with the small molecule inhibitor BGB324 abrogates both matrix-stiffness and transforming growth factor beta (TGF-β1)–induced fibrogenesis in human colonic myofibroblasts. AXL inhibition with BGB324 sensitizes myofibroblasts to apoptosis. Finally, AXL inhibition with BGB324 blocks TGF-β1-induced fibrogenic gene and protein expression in human intestinal organoids. Conclusions The AXL pathway is active in multiple models of intestinal fibrosis. In vitro experiments suggest that inhibiting AXL signaling could represent a novel approach to antifibrotic therapy for intestinal fibrosis such as in Crohn’s disease.

Protein‐losing enteropathy and chyle leakage may lead to severe malnutrition in heart transplantation for failing Fontan. Nutritional management may be challenging from defining nutrient needs to diagnosis of malnutrition enteropathy, and expertise is necessary. Body composition and hematological nutritional indices may help define malnutrition severity and guide nutritional strategy.