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Introduction: SBS is a rare and disabling condition. The standard management is based on diet optimization with parenteral supplementation. In addition, glucagon-like peptide-2 (GLP-2)analogs, have shown promising results as disease-modifying therapies for SBS., Areas Covered: Short bowel syndrome (SBS) is defined as a reduction in functional intestinal length to less than 200 cm, leading to intestinal failure (IF) leading to malnutrition and parenteral support dependency. This review discusses the current management of SBS-CIFpatients, the place of GLP-2 analog treatment in terms of efficacy, safety and availability, and the new perspectives opened by the use of enterohormones., Expert Opinion: Clinical trials and real-world experience demonstrated that Teduglutide   reduces dependence on parenteral support and has a place in the management of patients with SBS-CIF.  The use of Teduglutide should be discussed in patients stabilized after resection and its introduction requires the advice of an expert center capable of assessing the benefit-risk ratio. The complex, individualized management of SBS-C IF requires theexpertise of a specialized IF center which a multidisciplinary approach. The arrival of new treatments will call for new therapeutic strategies, and the question of how to introduce and monitor them will represent a new therapeutic challenge.

In intestinal resection animal models of short bowel syndrome (SBS), the remaining epithelium mounts a robust adaptive response characterized by early stem cell expansion and increased crypt depth, villus height and nutrient absorption. In humans the adaptive response is critical for resumption of oral nutrition, yet it may be variable, and underlying mechanisms are much less well understood. Current knowledge relating to the role of stem and mesenchymal niche cells in the adaptive response in animal models and in human SBS are addressed in this review., Competing Interests: Disclosure The author has no financial disclosures., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Renal impairment is a common complication in patients with short bowel syndrome with intestinal failure (SBS-IF). Glucagon-like peptide-2 analogs, such as apraglutide, have been developed as a treatment option for SBS-IF. This study assessed the potential for apraglutide overexposure in individuals with severely impaired renal function versus healthy volunteers with normal renal function. In this phase 1, open-label, multicenter, nonrandomized, parallel-group study, a single dose of apraglutide 5 mg was administered subcutaneously to individuals with severely impaired renal function (<30 mL/min/1.73 m 2 ) and healthy volunteers with normal renal function (≥90 mL/min/1.73 m 2 ). Primary pharmacokinetic endpoints were maximum observed concentration (C max ) and exposure to apraglutide (area under the curve [AUC] from time 0 to infinity [AUC inf ], and AUC from time 0 to the last quantifiable concentration [AUC last ]). Each group comprised 8 individuals. Results show that patients with severe renal impairment do not have increased apraglutide exposure. Apraglutide achieved a lower C max and AUC inf in individuals with severe renal impairment versus those with normal renal function (C max = 36.9 vs 59.5 ng/L; AUC inf = 3100 vs 4470 h · ng/mL, respectively). The respective geometric mean ratios were 0.620 and 0.693 for C max and AUC inf , and the upper bound of their 90% confidence intervals were <2, indicating patients with severe renal impairment were not overexposed to apraglutide versus those with normal renal function. Adverse events were mild or moderate in severity. Apraglutide does not require dose reduction for any degree of renal impairment and could be used in a broader patient population of renally impaired patients without dose adjustment., (© 2024 VectivBio AG. The Journal of Clinical Pharmacology published by Wiley Periodicals LLC on behalf of American College of Clinical Pharmacology.)

Objective: Fecal microbiota was investigated in adult patients with chronic intestinal failure (CIF) due to short bowel syndrome (SBS) with jejunocolonic anastomosis (SBS-2). Few or no data are available on SBS with jejunostomy (SBS-1) and CIF due to intestinal dysmotility (DYS) or mucosal disease (MD). We profiled the fecal microbiota of various pathophysiological mechanisms of CIF., Methods: Cross-sectional study on 61 adults with CIF (SBS-1 30, SBS-2 17, DYS 8, MD 6). Fecal samples were collected and profiled by 16S rRNA amplicon sequencing. Healthy controls (HC) were selected from pre-existing cohorts, matched with patients by sex and age., Results: Compared to HC, SBS-1, SBS-2 and MD patients showed lower alpha diversity; no difference was found for DYS. In beta diversity analysis, SBS-1, SBS-2 and DYS groups segregated from HC and from each other. Taxonomically, the CIF groups differed from HC even at the phylum level. In particular, CIF patients' microbiota was dominated by Lactobacillaceae and Enterobacteriaceae, while depleted in typical health-associated taxa belonging to Lachnospiraceae and Ruminococcaceae. Notably, compositional peculiarities of the CIF groups emerged. Furthermore, in the SBS groups, the microbiota profile differed according to the amount of parenteral nutrition required and the duration of CIF., Conclusions: CIF patients showed marked intestinal dysbiosis with microbial signatures specific to the pathophysiological mechanism of CIF as well as to the severity and duration of SBS., Competing Interests: Conflict of interest LP: Participation on Data Safety Monitoring Board or Advisory Board for Takeda; Consulting fees for Takeda, Northsea Therapeutics, NAPO Therapeutics, Nestlè, Lionhealth; Honoraria for lectures by Baxter. FD’A, MG, PB, ASS, ST: none., (Copyright © 2024 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

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.

Introduction: Several techniques have been described to taper the dilated small bowel to improve intestinal motility and decrease complications related to overdilated small bowel, including longitudinal intestinal lengthening and tapering, serial transverse enteroplasty, and spiral intestinal lengthening and tailoring. We propose an alternative technique designed to optimize bowel function and minimize the effects of recurrent small-bowel bacterial overgrowth in patients with short or ultra-short gut syndrome and dysfunctional anastomosis with maintenance of the actual absorptive surface., Technique: The dilated side-to-side anastomosis is identified, and the mesentery leaves from both the proximal and distal small-bowel loops are separated by using blunt dissection. The previous anastomosis is divided longitudinally with a GI stapler. Once the small-bowel transection is completed, 2 separate blind loops of intestine are created, each one with half the circumference of the dilated side-to-side anastomosis. The antimesenteric stapled line is then reinforced with an outer layer of running suture. The blind loops of the tapered small bowel are then trimmed and anastomosed in an end-to-end isoperistaltic fashion in 2 layers., Results: There were no postoperative complications. The length of the tapering ranged from 10 to 23 cm, corresponding to approximately 16% (range, 13%-20%) of the remaining small-bowel length. Three of 4 patients presented significant improvement of their symptoms and were able to have their parenteral support discontinued., Conclusions: Modified antimesenteric tapering enteroplasty is an alternative technique to improve intestinal motility and treat patients with short-bowel syndrome and dysfunctional side-to-side anastomosis without the need for further small-bowel resection. This bowel-sparing technique represents a valuable option in the armamentarium of the surgeon who manages patients with intestinal failure., (Copyright © The ASCRS 2021.)

Introduction : The goal for pediatric short bowel syndrome (SBS) patients is intestinal adaptation. Until recently, the medical management of pediatric SBS has centered on the prevention and treatment of complications in order to allow time for adaptation. Teduglutide, glucagon-like peptide 2 (GLP-2) analog, has recently been approved for use in pediatric SBS patients greater than 1 year of age as a novel agent to augment intestinal adaptation. Areas covered : This article reviews the pharmacology, safety, efficacy, and tolerability of GLP-2 analog teduglutide in pediatric patients greater than 1 year of age. We review all current studies and discuss teduglutide's place in pediatric SBS therapy. Expert opinion : Teduglutide marks the first successful pharmacological intervention that augments the natural process of adaptation safely and effectively in SBS pediatric patients. More studies and further development are needed to optimize its potential in other pediatric patients.

Background: Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) (NDIF). The present review revised etiology and treatments of DIF and NDIF, with special focus on the pathophysiological mechanisms, whereby NDIF develops., Methods: We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: "intestinal failure" OR "home parenteral nutrition" OR "short bowel syndrome" OR "chronic pseudo-obstruction" OR "chronic intestinal pseudo-obstruction" OR "autoimmune enteropathy" OR "long-term parenteral nutrition"., Results: We collected overall 1656 patients with well-documented etiology of IF: 1419 with DIF (86%) and 237 with NDIF (14%), 55% males and 45% females. Among DIF cases, 66% had SBS and among NDIF cases 90% had malabsorption/maldigestion., Conclusions: The improved availability of diagnostic and therapeutic tools has increased prevalence and life expectancy of rare and severe diseases responsible for IF. The present review greatly expands the spectrum of knowledge on the pathophysiological mechanisms through which the diseases not strictly affecting the intestine can cause IF. In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; in fact, through information flow within the network, the Registries could improve IF knowledge and management.

Intestinal failure (IF) is defined as the critical reduction of functional intestines below the minimum needed to absorb nutrients and fluids, so that intravenous supplementation with parenteral nutrition (PN) is required to maintain health and/or growth. Although the benefits are evident, patients receiving PN can suffer from serious cholestasis due to lack of enteral feeding and small intestinal bacterial overgrowth (SIBO). One such complication that may arise is intestinal failure-associated liver disease (IFALD). Evidences from recent studies suggest that alterations in the intestinal microbiota, as well as intraluminal bile acid driven signaling, may play a critical role in both hepatic and intestinal injury. Since Marshall first proposed the concept of the gut-liver axis in 1998, the role of gut-liver axis disorders in the development of IFALD has received considerable attention. The conversation between gut and liver is the key to maintain liver metabolism and intestinal homeostasis, which influences each other and is reciprocal causation. However, as a "forgotten organ" , intestinal microbiota on the pathogenesis of IFALD has not been well reflected. As such, we propose, for the first time, the concept of gut-microbiota-liver axis to emphasize the importance of intestinal microbiota in the interaction of gut-liver axis. Analysis and research on gut-microbiota-liver axis will be of great significance for understanding the pathogenesis of IFALD and improving the prevention and treatment measures.

Short bowel syndrome in neonates is a severe and life-threatening disease after a major loss of small bowel with or without large bowel. Intestinal adaptation, by which the organism tries to restore digestive and absorptive capacities, is entirely dependent on stimulation of the active enterocytes by enteral nutrition. This review summarizes recent knowledge about the pathophysiologic consequences after the loss of different intestinal parts and outlines the options for enteral nutrition and pharmacological therapies to support the adaptation process.

Loss of functional small bowel surface area following surgical resection for disorders such as Crohn's disease, intestinal ischemic injury, radiation enteritis, and in children, necrotizing enterocolitis, atresia, and gastroschisis, may result in short bowel syndrome, with attendant high morbidity, mortality, and health care costs in the United States. Following resection, the remaining small bowel epithelium mounts an adaptive response, resulting in increased crypt cell proliferation, increased villus height, increased crypt depth, and enhanced nutrient and electrolyte absorption. Although these morphologic and functional changes are well described in animal models, the adaptive response in humans is less well understood. Clinically the response is unpredictable and often inadequate. Here we address the hypotheses that human intestinal stem cell populations are expanded and that the stem cell niche is regulated following massive gut resection in short bowel syndrome (SBS). We use intestinal enteroid cultures from patients with SBS to show that the magnitude and phenotype of the adaptive stem cell response are both regulated by stromal niche cells, including intestinal subepithelial myofibroblasts, which are activated by intestinal resection to enhance epithelial stem and proliferative cell responses. Our data suggest that myofibroblast regulation of bone morphogenetic protein signaling pathways plays a role in the gut adaptive response after resection.

Gastrointestinal dysmotility is a common problem in a subgroup of children with intestinal failure (IF), including short bowel syndrome (SBS) and pediatric intestinal pseudo-obstruction (PIPO). It contributes significantly to the increased morbidity and decreased quality of life in this patient population. Impaired gastrointestinal (GI) motility in IF arises from either loss of GI function due to the primary disorder (e.g., neuropathic or myopathic disorder in the PIPO syndrome) and/or a critical reduction in gut mass. Abnormalities of the anatomy, enteric hormone secretion and neural supply in IF can result in rapid transit, ineffective antegrade peristalsis, delayed gastric emptying or gastroesophageal reflux. Understanding the underlying pathophysiologic mechanism(s) of the enteric dysmotility in IF helps us to plan an appropriate diagnostic workup and apply individually tailored nutritional and pharmacological management, which might ultimately lead to an overall improvement in the quality of life and increase in enteral tolerance. In this review, we have focused on the pathogenesis of GI dysmotility in children with IF, as well as the management and treatment options.

Background: Short bowel syndrome (SBS) is a condition that results from inadequate intestinal absorptive capacity, usually after the loss of functional intestine. We have previously developed a severe model of SBS in zebrafish that demonstrated increased intestinal adaptation (IA) and epithelial proliferation in SBS zebrafish. However, many children with SBS do not have this extreme intestinal loss. Therefore, in this study, we developed a variation of this model to evaluate the effects of increasing intestinal length on IA and the complications of SBS., Materials and Methods: After Institutional Animal Care and Use Committee approval, adult male zebrafish were assigned to three groups: sham (n = 30), S1-SBS (n = 30), and S3-SBS (n = 30). Sham surgery included ventral laparotomy alone. S1-SBS surgery consisted of laparotomy with creation of a proximal stoma at S1 (jejunostomy equivalent) and ligation at S4. S3-SBS surgery had stoma creation at S3 (ileostomy equivalent) and the same ligation. Fish were harvested at 14 d. Markers of IA were measured from proximal intestinal segments, and the liver was analyzed for development of hepatic steatosis., Results: At 14 d, S3-SBS fish lost less weight than S1-SBS and had increased markers of IA compared with sham fish, which were decreased compared with S1-SBS fish. S3-SBS fish had decreased proximal intestinal inflammation compared with S1-SBS fish. S1-SBS fish developed extensive hepatic steatosis. Although S3-SBS fish have increased hepatic steatosis compared with sham fish, it is decreased compared with S1-SBS., Conclusions: Longer remnant intestine decreases the extent of IA, inflammation, and hepatic steatosis in a zebrafish model of SBS., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Pediatric intestinal failure occurs when gut function is insufficient to meet the nutrient and hydration needs of the growing child. The commonest cause is short bowel syndrome with maldigestion and malabsorption following massive bowel loss. The remnant bowel adapts during the process of intestinal rehabilitation. Management promotes the achievement of enteral autonomy while mitigating the risk of comorbid disease. The future of care is likely to see expansion of pharmacologic methods for augmenting bowel adaptation, tissue engineering techniques enabling immune suppression-free autologous bowel transplant, and the development of electronic health record tools for efficient, collaborative study and care improvement., Competing Interests: Disclosures The authors have nothing to disclose., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Some temporary double enterostomies (DES) or entero-atmospheric fistulas (EAF) have high output and are responsible for Type 2 intestinal failure. Intravenous supplementations (IVS) for parenteral nutrition and hydration compensate for intestinal losses. Chyme reinfusion (CR) artificially restores continuity pending surgical closure. CR treats intestinal failure and is recommended by European Society for Clinical Nutrition and Metabolism (ESPEN) and American Society for Parenteral and Enteral Nutrition (ASPEN) when possible. The objective of this study was to show changes in nutritional status, intestinal function, liver tests, IVS needs during CR, and the feasibility of continuing it at home. A retrospective study of 306 admitted patients treated with CR from 2000 to 2018 was conducted. CR was permanent such that a peristaltic pump sucked the upstream chyme and reinfused it immediately in a tube inserted into the downstream intestine. Weight, plasma albumin, daily volumes of intestinal and fecal losses, intestinal nitrogen, and lipid absorption coefficients, plasma citrulline, liver tests, and calculated indices were compared before and during CR in patients who had both measurements. The patients included 185 males and 121 females and were 63 ± 15 years old. There were 37 (12%), 269 (88%) patients with EAF and DES, respectively. The proximal small bowel length from the duodeno-jejunal angle was 108 ± 67 cm ( n = 232), and the length of distal small intestine was 117 ± 72 cm ( n = 253). The median CR start was 5 d (quartile 25-75%, 2-10) after admission and continued for 64 d (45-95), including 81 patients at home for 47 d (28-74). Oral feeding was exclusive 171(56%), with enteral supplement 122 (42%), or with IVS 23 (7%). Before CR, 211 (69%) patients had IVS for nutrition (77%) or for hydration (23%). IVS were stopped in 188 (89%) 2 d (0-7) after the beginning of CR and continued in 23 (11%) with lower volumes. Nutritional status improved with respect to weight gain (+3.5 ± 8.4%) and albumin (+5.4 ± 5.8 g/L). Intestinal failure was cured in the majority of cases as evidenced by the decrease in intestinal losses by 2096 ± 959 mL/d, the increase in absorption of nitrogen 32 ± 20%, of lipids 43 ± 30%, and the improvement of citrulline 13.1 ± 8.1 µmol/L. The citrulline increase was correlated with the length of the distal intestine. The number of patients with at least one liver test >2N decreased from 84-40%. In cases of Type 2 intestinal failure related to DES or FAE with an accessible and functional distal small bowel segment, CR restored intestinal functions, reduced the need of IVS by 89% and helped improve nutritional status and liver tests. There were no vital complications or infectious diarrhea described to date. CR can become the first-line treatment for intestinal failure related to double enterostomy and high output fistulas., Competing Interests: The authors declare no conflicts of interest.

Short bowel syndrome (SBS) is a rare malabsorptive disorder as a result of the loss of bowel mass mostly secondary to surgical resection of the small intestine. Other causes are vascular diseases, neoplasms or inflammatory bowel disease. The spectrum of the disease is widely variable from single micronutrient malabsorption to complete intestinal failure, depending on the remaining length of the small intestine, the anatomical portion of intestine and the function of the remnant bowel. Over the last years, the management of affected patients has remarkably improved with the increase in patients' quality of life and survival, mainly thanks to advances in home-based parenteral nutrition (PN). In the last ten years new treatment strategies have become available together with increasing experience and the encouraging results with new drugs, such as teduglutide, have added a new dimension to the management of SBS. This review aims to summarize the knowledge available in the current literature on SBS epidemiology, pathophysiology, and its surgical (including intestinal lengthening procedures and intestinal transplantation) and medical management with emphasis on the recent advances. Moreover, this review attempts to provide the new understanding and recent approaches to SBS complications such as sepsis, catheter thrombosis, and intestinal failure-associated liver disease., (Copyright © 2019 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Background & Aims: In short-bowel syndrome (SBS), inadequate intestinal adaptation is responsible for the majority of complications, including sepsis, liver failure, and death. In this study, we sought to further delineate the adaptive response to identify potential therapeutic targets., Methods: We performed a 75% small-bowel resection (SBR) or sham operation on C57Bl/6J wild-type (WT), lipocalin-2 (LCN2) -/- , and interleukin 22 (IL22) -/- mice. Exogenous IL22 was administered to SBR WT mice. Cecal fecal matter from SBR WT and SBR LCN2 -/- mice were transplanted into germ-free mice. Intestinal permeability, inflammation, proliferation, and the microbiome were evaluated 1 week after surgery. CD4 + IL22 + laminal propria lymphocytes were sorted by flow cytometry. Naïve T cells were polarized to T-helper cells with or without LCN2., Results: A 75% SBR in a mouse re-creates the increased intestinal permeability, enterocyte proliferation, and intestinal dysbiosis seen in SBS. LCN2 expression increases after 75% SBR, and this increase can be abrogated with broad-spectrum antibiotic treatment. LCN2 -/- mice have less intestinal inflammation, increased IL22 expression, and greater adaptation as evidenced by less intestinal permeability, increased carbohydrate enzyme expression, less weight loss, and less dysbiosis after 75% SBR than WT mice. The proinflammatory and anti-adaptive effects of LCN2 can be transferred to germ-free mice via a fecal transplant. Administration of exogenous IL22 improves adaptation and restores the normal microbiome after 75% SBR in WT mice., Conclusions: LCN2 promotes inflammation and slows intestinal adaptation through changes in the microbiome and IL22 inhibition in a mouse SBS model. Strategies to reduce LCN2 may offer novel therapeutic approaches to enhance adaptation in SBS., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Despite growing literature characterizing the fecal microbiome and its association with health and disease, few studies have analyzed the microbiome of the small intestine. Here, we examine what is known about the human small intestinal microbiota in terms of community structure and functional properties. We examine temporal dynamics of select bacterial populations in the small intestine, and the effects of dietary carbohydrates and fats on shaping these populations. We then evaluate dysbiosis in the small intestine in several human disease models, including small intestinal bacterial overgrowth, short-bowel syndrome, pouchitis, environmental enteric dysfunction, and irritable bowel syndrome. What is clear is that the bacterial biology, and mechanisms of bacteria-induced pathophysiology, are enormously broad and elegant in the small intestine. Studying the small intestinal microbiota is challenged by rapidly fluctuating environmental conditions in these intestinal segments, as well as the complexity of sample collection and bioinformatic analysis. Because the functionality of the digestive tract is determined primarily by the small intestine, efforts must be made to better characterize this unique and important microbial ecosystem., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Short bowel syndrome / intestinal failure (SBS/IF) is a rare and debilitating disease process that mandates a multidisciplinary approach in its management. Inflammatory bowel disease (IBD), in particular Crohn's disease (CD), predisposes patients to development of SBS/IF. This review discusses SBS/IF from the perspective of IBD, with an emphasis on prevention and treatment in the setting of CD. The aims of this review are to emphasize the unique treatment goals of the newly diagnosed SBS/IF patient, and highlight the role of both medical and surgical therapies in the management of IBD-related SBS/IF, including intestinal transplantation., (Copyright © 2019 Elsevier Inc. All rights reserved.)

In children, short-bowel syndrome (SBS) accounts for two-thirds of the cases of intestinal failure, and motility disorders and congenital mucosal diarrheal disorders account for the remaining one-third. Children with SBS are supported primarily by parenteral nutrition, which is the single-most important therapy contributing to their improved prognosis. More than 90% of children with SBS who are cared for at experienced intestinal rehabilitation programs survive, and roughly 60% to 70% undergo intestinal adaptation and achieve full enteral autonomy. This article focuses on the predictors of pediatric intestinal adaptation and discusses the pathophysiology and clinical management of children with SBS., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Nucleotide-binding oligomerization domain-containing protein 2 ( NOD2 ) gene mutations are a risk factor for Crohn's disease and also associated with worse outcome in short bowel syndrome (SBS) patients independent of the underlying disease. The aim of this study was to analyze the effect of Nod2 deficiency on barrier function and stool microbiome after extensive ileocecal resection in mice. Male C57BL6/J wild-type (WT) and Nod2 -knockout (KO) mice underwent 40% ileocecal resection. Sham control mice received simple transection of the ileum. Clinical outcome was monitored daily. Barrier function was measured with Ussing chambers using FITC-4-kDa-Dextran flux, transmucosal electrical resistance, and dilution potentials. Immunofluorescence of claudin-2 was studied. Composition of the stool microbiome was assessed by 16S rRNA gene sequencing. Resected Nod2 -KO mice had impaired clinical outcome compared with resected WT mice. This was accompanied by increased stool water contents and increased plasma aldosterone. Histomorphological adaptation was independent of Nod2 . Barrier function studies revealed impaired sodium to chloride permeability and altered claudin-2 localization in the absence of Nod2 . Resection induced decreases of bacterial diversity and a shift of bacteriodetes-to-firmicutes ratios. Ileum and cecum resection-induced increase in proteobacteria was absent in Nod2 -deficient mice. Verrucomicrobia were temporarily increased in Nod2 -KO mice. Nod2 deficiency functionally impairs adaptation to short bowel syndrome via a lesser increase of epithelial sodium pore permeability, altered epithelial barrier function, and the microbiome. NEW & NOTEWORTHY NOD2 gene mutations are associated with the development of severe short bowel syndrome and intestinal failure. The influence of Nod2 mutations on intestinal adaptation in experimental short bowel syndrome has not been studied yet. Here, we provide data that Nod2 deficiency worsens clinical outcome and functional adaptation under SBS conditions in mice, indicating that NOD2 is required for successful adaptation after ileocecal resection.

Introduction: Patients with short bowel syndrome (SBS) are dependent on parenteral nutrition (PN) while their bowel attempts to compensate. Our objective was to create an SBS disease severity score to predict the probability of achieving enteral autonomy (EA)., Methods: A retrospective cohort study of children with SBS managed by our Intestinal Rehabilitation Program was completed. Data abstracted included demographic, bowel anatomy, and outcome variables including conjugated bilirubin (CB) and enteral nutrition (EN) tolerated 6 months postoperatively. Univariate analysis and Cox proportional hazards (CPH) model were performed. A score was created based on weighting of coefficients. An α-value of < 0.05 was considered significant., Results: One hundred thirty-nine patients were analyzed (61% males). Ninety-five (68%) achieved EA. Patients possessing >50% residual small bowel (hazard ratio [HR] 2.68 [95% confidence interval {CI} 1.60-4.49], P < 0.001), ileocecal valve intact (HR 0.61 [95% CI 0.37-1.01], P < 0.055), and >50% enteral tolerance at 6 months (HR 5.70 [95% CI 2.77-11.74] P < 0.001) were positively associated with EA. CB >34 µmol/L (2 mg/dL) was negatively associated with EA (HR 0.42 [95% CI0.27-0.66], P < 0.001). A severity score was created by weighting CPH parameter estimates (small bowel length >50%, ileocecal valve intact, CB <34 µmol/L, and EN >50% for a maximum score of 8), and disease severity strata were developed (severe [25.7% EA], moderate [52.9% EA], and mild [97.1% EA])., Conclusion: We propose a pediatric SBS disease severity score that predicts probability of EA. The score allows prognostication of individual patients and could assist research by adjusting outcome reporting or stratifying recruitment., (© 2019 American Society for Parenteral and Enteral Nutrition.)

The aim of our study was to assess the presence and degree of intestinal leakage in subjects suffering from short bowel syndrome (SBS) and its modification by parenteral nutrition. To this end we assessed circulating levels of selected makers of intestinal permeability including zonulin, fatty acid binding protein 2 (FABP-2), citrulline and glucagon-like peptide 2 (GLP-2). We also measured lipopolysaccharide binding protein (LBP) as a marker of circulating levels of lipopolysaccharide acting through the CD14 molecule. Eleven SBS and 10 age- and BMI-matched control subjects were included into the study. The effect of parenteral nutrition was assessed after 14 days, 6 and 12 months from its initiation, respectively. At baseline, SBS patients had increased gut permeability as measured by zonulin (47.24+/-2.14 vs. 39.48+/-1.20 ng/ml, p=0.006) and LBP (30.32+/-13.25 vs. 9.77+/-0.71 microg/ml, p<0.001) compared to healthy controls. Furthermore, SBS subjects had reduced FABP-2, unchanged citrulline and increased sCD14 and GLP-2 relative to control group. Throughout the whole study period the administered parenteral nutrition had no significant effect on any of the studied parameters. Taken together, our data show that patients with short bowel syndrome have increased intestinal permeability that is not affected by parenteral nutrition.

The ability to provide parenteral support represents a revolutionary change in medical therapy for patients with temporary and inadequate intestinal absorptive capacity or for patients with chronic intestinal failure due to digestive diseases. Nevertheless, due to the rarity of intestinal failure, a de facto policy of "discrimination by organ failure treatment" exists in many countries whereby this problem is under-recognized and under-treated. With the increasing recognition of the pathophysiological consequences of intestinal resection and the occurrence of new pro-adaptive treatments for patients suffering from short bowel syndrome, this review reflects on the history of developments in this area and discusses current practice and future directions of the field.

It is not known whether Teduglutide can allow patients with Short bowel syndrome, previously dependent on continuous or periodic intravenous (IV) magnesium, to attain oral autonomy with or without supplementation. Here, we report on two patients previously dependent on continuous or intermittently administered IV magnesium to achieve autonomy from IV, one with and one without oral supplementation that was previously ineffective in both patients., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

Background & Aims: Teduglutide, a glucagon-like peptide 2 (GLP-2) analog, is an approved medication specific for short bowel syndrome patients with chronic intestinal failure (SBS-IF). Due to its intestinotrophic properties, it improves intestinal absorption of fluids and nutrients, which was shown to reduce the need for parenteral support in clinical trials. The present report aims to describe the experience of teduglutide's effects in routine medical care with focus on clinical and nutritional effects., Methods: Data of adult SBS-IF patients, treated with teduglutide between Sept. 2014 and May 2017 within a structured multidisciplinary program to enhance intestinal rehabilitation, were analyzed retrospectively from a single university medical center., Results: In total, 27 patients were treated with teduglutide. Parenteral nutrition independency was achieved in 4/19 (21%) patients analyzed, with two remaining on intravenous fluids. A clinically significant reduction of parenteral volume was observed in 15/19 patients (79%) with onset between 1 and 45 weeks. Significant parenteral support reductions were observed, ranging from about -20% in patients treated for 3 months to about -45% in patients treated for 2 years. This was accompanied by an increase in parenteral nutrition-free days. We also report on a clinically relevant and significant effect of teduglutide-mediated improvement of stool frequency and consistency. Furthermore, nutritional status subgroup analysis revealed long-term stability in body weight, albumin levels and body composition albeit parenteral support reduction. Structural effects of teduglutide treatment were observed on small intestinal mucosa with significantly increased villus height, crypt depth and plasma citrulline levels., Conclusions: Teduglutide can be applied to anatomically and clinically heterogeneous SBS-IF patients and results in an adaptive response with variable time and effect range in routine medical care. Teduglutide-induced functional and structural changes bring on a gradual reduction of parenteral support at no cost to body composition and suggest an improved intestinal function with compensatory effect on nutritional status., (Copyright © 2018 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Introduction : Short bowel syndrome represents the leading etiology that causes intestinal failure both in children and adults. Total parenteral nutrition support has dramatically improved the prognosis for these patients but, if related irreversible complications occur, the alternative is represented by surgery and/or transplantation. Areas covered : Autologous gastrointestinal reconstructive procedures are a feasible, alternative approach with good long-term outcome data inexperienced surgical centers. Expert opinion : Ongoing innovative efforts have driven the surgical options for successful autologous reconstructive surgery: bowel elongation/tapering techniques (LILT, STEP, and the new SILT) together with the 'reversed bowel segment' procedure are now recognized procedures and all must be tailored to the individual patient needs to obtain the optimal result in terms of enteral autonomy. Background laboratory experimentation with new procedures e.g. options for bowel dilation techniques and distraction-induced enterogenesis, may provide additional management and treatment modalities.

Patients with severely orally decompensated malsaborption due to surgical rescetion suffer from short bowel syndrome (SBS). The resultant decrease in intestinal surface area after rescetion of parts of the intestines leads to chronic functional intestinal insufficiency or even complete failure (cIF) which requires parenteral fluid and nutrient restoration for establishment of metabolc stability. Since the introduction of glucagon-like peptide (GLP)-2 analogues such as teduglutide functional rehabilitation of the remaining gastrointestinal tract has become a causal treatment option. Teduglutide functionally and effectively enhances intestinal absorption of fluid as well micro- and macronutrients allowing a reduction of parenteral nutrition and/or volume with even complete weaning off in some patients. This results increased metabolic stability, oral autonomy, quality of life and putatively less complications., Competing Interests: U.-F. Pape gibt an, von Shire Pharma und Fresenius Kabi finanzielle Unterstützung bei Forschungsvorhaben erhalten zu haben sowie von Shire Pharma Honorare für Vortragsleistungen und die Teilnahme an Advisory-Boards bezogen zu haben.S. Maasberg gibt an, von Shire Pharma Honorare für die Teilnahme an Advisory-Boards erhalten zu haben., (© Georg Thieme Verlag KG Stuttgart · New York.)

Mortality associated with liver disease has been observed in patients with short bowel syndrome (SBS); however, its mechanism remains unclear, but bile acid (BA) dysmetabolism has been proposed as a possible cause. The farnesoid X receptor (FXR) is the key regulator of BA synthesis. Here, we showed that, in a rat model of short bowel resection associated with liver disease (SBR-ALD), the BA composition of hepatic tissues reflected a larger proportion of primary and secondary unconjugated BAs, whereas that of the colon contents and serum showed an increased ratio of secondary unconjugated BAs. Both hepatic and intestinal regulation of BA synthesis was characterized by a blunted hepatic FXR activation response. The mRNA expression levels of cholesterol 7a-hydroxylase ( CYP7A1 ), sterol 12a-hydroxylase ( CYP8B1 ), and sterol 27 hydroxylase ( CYP27A1 ), the key enzymes in BA synthesis, were upregulated. After intervention with the FXR agonist GW4064, both the liver histology and serum transaminase activity were improved, which demonstrated the attenuation of SBR-ALD. The BA compositions of hepatic tissue, the colon contents, and serum recovered and were closer to those of the sham group. The expression levels of hepatic FXR increased, and its target genes were activated. Consistent with this, the expression levels of CYP7A1 , CYP8B1 , and CYP27A1 were downregulated. Ileum tissue FXR and its target genes were slightly elevated. This study showed that the FXR agonist GW4064 could correct BA dysmetabolism to alleviate hepatotoxicity in SBR animals. GW4064 intervention resulted in a decrease in fecal bile excretion and elevated plasma/hepatic conjugated BA levels. GW4064 increased the reabsorption of conjugated BAs by inducing apical sodium-dependent bile salt transporter expression in the ileum. Concomitantly, FXR activation in the presence of GW4064 decreased BA production by repressing the expression of key synthetases, including CYP7A1 , CYP8B1 , and CYP27A1 . These findings provide a clinical research direction for the prevention of liver disease in patients with SBS. NEW & NOTEWORTHY This study assessed the impact of treatment with GW4064, a farnesoid X receptor agonist, on the development of short bowel resection (SBR) associated with liver disease in a rat model of SBR. GW4064 was able to correct bile acid dysmetabolism and alleviate hepatotoxicity in SBR animals.

Background: The milk fat globule membrane (MFGM) contains various bioactive components which have been shown to maintain gut barrier integrity. This study aimed to evaluate the protective effects of MFGM on intestinal barrier function and its possible mechanisms in a rat model of short bowel syndrome (SBS)., Materials and Methods: Five-week-old male Sprague-Dawley rats were divided into 3 groups (n = 8 per group), consisting of Sham group and rats submitted to massive small-bowel resection then supplemented with either water (SBS) or 1.5g/kg/d MFGM (SBS+MFGM) by daily gavage. Rats were sacrificed on day 15 postoperation. Intestinal adaptation, gut permeability, bacterial translocation (BT), expression of tight junction proteins, mucin 1 (MUC1), and nucleotide-binding oligomerization domain leucine-rich repeat and pyrin domain-containing protein 3 (NLRP3) pathway in the ileum were evaluated., Results: Both SBS+MFGM and SBS groups exhibited lower body weight and higher ileum villus height than Sham group, but no difference was detected between each other. SBS group had significantly higher intestinal permeability and BT rate than other groups (P < .05). Compared with SBS rats, SBS+MFGM group showed higher expression of tight junction proteins and MUC1, lower expression of NLRP3 and caspase-1 in the ileum, as well as lower interleukin (IL)-1β but higher IL-18 levels in ileum tissue., Conclusions: Supplementation of MFGM helps to modulate NLRP3 inflammasome activation and enhances gut barrier integrity in rats after massive small-bowel resection, which provides experimental support for potential applications of MGFM in intestinal barrier dysfunction, although further studies are needed., (© 2018 American Society for Parenteral and Enteral Nutrition.)

Objective: Patients with short bowel syndrome (SBS) receiving long-term parenteral nutrition (PN) are at risk for intestinal failure-associated liver disease (IFALD). The aim of the present study was to evaluate dynamic changes of liver fibrosis and steatosis within 12 mo by transient elastography (TE), including controlled attenuation parameter (CAP) in a cohort of patients with SBS receiving long-term PN., Methods: Twenty-five adult patients with SBS and PN requirement for ≥3 mo consecutively were included and prospectively followed. Liver stiffness by FibroScan (Echosens, Paris, France) and CAP measurement were done at study entry and after 12 mo. Clinical parameters, as well as data on underlying bowel disease and nutrition composition, were collected. Bioelectrical impedance analysis was performed in all patients., Results: FibroScan and CAP did not show any significant differences after 12 mo (5.2 kPa [2.8-16.2 kPa]; 223dB/m [101-366 dB/m]) compared with study entry (5.3 kPa [2.7-12.3 kPa]; 237dB/m [100-344 dB/m]). There was no significant correlation between FibroScan and CAP and elevated transaminase levels. CAP significantly correlated with triacylglyceride levels (r = 0.411; P = 0.042) and body mass index (r = 0.468; P = 0.016). Patients with a remnant small bowel <100cm showed a significantly higher stiffness value by FibroScan than those having a remnant length ≥100cm (6.1 versus 4.7 kPa; P = 0.028)., Conclusion: In the present study cohort, prevalence of advanced fibrosis or cirrhosis was low (<10%) without significant dynamic within the 12-mo follow-up. Short intestinal remnant length <100cm appeared to be a risk factor for development of fibrosis., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: Some patients with short bowel syndrome (SBS) develop hyperphagic behavior. Such an increase in food intake stimulates intestinal adaptation and limits dependence on parenteral nutrition (PN). The aim of this study was to determine the factors modulating food consumption in patients with SBS., Methods: The associations between oral energy intake (OEI) and anthropometric, metabolic, nutritional, and intestinal absorption-related characteristics were determined in a monocentric cohort of patients with SBS on PN with a stable nutritional status. Body composition was assessed by dual x-ray absorptiometry. Data were retrospectively collected from clinical records., Results: After screening, 38 adult patients with a SBS on PN were included in this study. OEI ranged from 577 to 4054kcal/d. OEI correlated positively with weight, fat-free mass, handgrip strength, and resting energy expenditure (REE) and negatively with free triiodothyronine and C-reactive protein using Spearman correlation. Fat-free mass and thyroid-stimulating hormone remained positively correlated with OEI independently of all other parameters in a multilinear regression model., Conclusions: Fat-free mass is a strong predictor of OEI in patients with SBS on PN and without debilitating gastrointestinal symptoms. Increasing fat-free mass could be a way to stimulate OEI in these patients. Further studies are needed to assess this assumption., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: People diagnosed with intestinal failure (IF) as a result of short bowel syndrome are dependent on home parenteral nutrition (HPN). Measuring nutritional status is essential for monitoring treatment. The present study aimed to determine the agreement and feasibility of three methods bioelectrical impedance analysis (BIA), ultrasound and air displacement plethysmography (ADP) for measuring body composition in people receiving HPN., Methods: Body composition data were collected from patients attending an IF clinic., Results: There were 50 participants recruited and data were collected for BIA (n = 46), ultrasound (n = 49) and ADP (n = 9). Numbers for ADP were much lower because of a lack of participant uptake. Fat-free mass (FFM) measured by BIA and ultrasound in comparison to ADP was found to have good intraclass correlation (ICC) 0.791 (95% confidence interval (CI) CI  -0.21 to 0.96) and a moderate ICC 0.659 [95% (CI) -0.27 to 0.92], respectively. Fat mass (FM) measured by both BIA and ultrasound in comparison to ADP was found to have moderate ICC 0.660 (95% CI -0.28 to 0.92) and poor ICC -0.005 (95% CI -0.73 to 0.65), respectively., Conclusions: Compared to ADP, BIA indicated moderate to good agreement for measuring body composition, whereas ultrasound indicated far less agreement, particularly when measuring FM. The lack of uptake of ADP suggests that participants found the Bodpod (COSMED Srl, Shepperton, UK) unfavourable. Considering that ultrasound has limited agreement and ADP was not the preferred option for participants, BIA shows some potential. However, the difference between ADP and BIA was larger for FM compared to FFM, which needs to be considered in the clinical setting., (© 2018 The Authors. Journal of Human Nutrition and Dietetics published by John Wiley & Sons Ltd on behalf of British Dietetic Association.)

Background: There are no in vivo methods to measure adaptation in neonatal short bowel syndrome (SBS). We evaluated citrulline (Cit) levels in neonatal piglet surgical models of SBS., Methods: Piglets underwent 75% mid-intestinal resection with jejunoileal anastomosis (JI), 75% distal resection of ileum with jejunocolic anastomosis (JC) or sham surgery. Jugular and gastric catheters were inserted for parenteral and enteral nutrition. On D7, small intestine length and weight were measured, jejunum collected for histopathology and Cit level determined., Results: JI (n = 5) compared to JC (n = 5) had increased small intestinal length (JC - 17.5 cm; JI +22.0 cm; p = 0.02) and mass (JC 43.1 mg/cm/kg; JI 51.3 mg/cm/kg; p = 0.02), while Cit did not differ (JI 801.0 µM; JC 677.7 µM; p = 0.90). Including non-resected shams (n = 4), Cit correlated with length (R 2  = 0.48; p = 0.006), but not for SBS alone (R 2  = 0.11; p = 0.4), mass (R 2  = 0.05; p = 0.5). A second experiment compared change in Cit levels from baseline to D7. Levels declined in sham (n = 8) and JC (n = 10) (sham - 110.1 µM; JC - 56.6 µM; p = 0.17), regardless of intestinal lengthening (sham 29.9 cm; JC - 10.4 cm; p = 0.002)., Conclusion: Citrulline levels predict large differences in intestinal length and 'identify' SBS. However, citrulline cannot discriminate between adaptation in JI and JC, nor predict intestinal lengthening.

Introduction: Short bowel syndrome (SBS) is a leading cause of intestinal failure (IF). Home parenteral nutrition (HPN) remains the standard treatment, with small intestinal transplantation reserved for cases with severe complications to HPN. There have recently been significant developments in growth factor therapy. We aimed to develop a greater contemporary understanding of our SBS-IF subset., Method: We performed a retrospective observational study of a prospectively maintained HPN audit database in October 2017. Intestinal anatomical details and parenteral requirements were recorded. Each case was assessed for eligibility for growth factor therapy using recently published trials., Results: Of 273 patients receiving HPN, 152 (55.7%) had type three IF as a result of SBS (SBS-IF), with a mean duration of HPN of 61 months (range 4-416). Mean length of small intestine was 98 cm. Furthermore, 114 (41.8%) patients had an end jejunostomy (SBS-J), 18 (6.6%) had an end ileostomy, and 7.3% of patients had all or part of the colon-in-continuity. Crohn's disease was the most common underlying pathology. Univariate analysis for the whole HPN cohort demonstrated SBS-IF and a longer duration of HPN to be associated with higher PN energy requirements, p ≤ 0.0001. Of all, 73 (48%) patients with SBS-IF were deemed suitable for GLP-2 analogue therapy, with co-morbidity being the most frequent cause of non-suitability (29.1%)., Conclusion: We describe a large U.K. HPN cohort using ESPEN pathophysiological and clinical severity classification. The majority of patients with SBS-IF had a jejunostomy and relatively few had colon-in-continuity. Co-morbidity is the most common contra-indication to GLP-2 analogue therapy., Clinical Relevancy: GLP-2 analogues are emerging as an important treatment for patients with short bowel syndrome. Our study explores patient suitability in a large HPN cohort managed in a national IF centre. Furthermore, the international variation in the pathophysiology of SBS-IF varies significantly, which can have a bearing on PN requirements and outcomes when GLP-2 analogues are used.

Background: The main cause of intestinal failure is short bowel syndrome (SBS). The management goal for children with SBS is to promote intestinal adaptation while preserving growth and development with the use of parenteral nutrition (PN)., Objectives: This study evaluated the intestinal absorption rate in children with SBS, focusing on the role of the remnant colon. In addition, the relation between intestinal absorption rate, citrulline concentration, and small bowel length was studied., Methods: Thirty-two children with SBS on PN were included. They were divided into 3 groups according to the European Society for Clinical Nutrition and Metabolism (ESPEN) anatomical classification system: type 1 SBS (n = 9), type 2 (n = 13), and type 3 (n = 10). Intestinal absorption rate was assessed by a stool balance analysis of a 3-d collection of stools. Plasma citrulline concentrations were measured and the level of PN dependency was calculated., Results: The total energy absorption rate did not differ significantly between the 3 groups: 68% (61-79% ) for type 1, 60% (40-77%) for type 2, and 60% (40-77%) for type 3 ( P = 0.45). Children with type 2 or 3 SBS had significantly shorter small bowel length than children with type 1: 28 cm (19-36 cm) and 16 cm (2-29 cm), respectively, compared with 60 cm (45-78 cm) ( P = 0.04). Plasma citrulline concentrations were lower in type 3 SBS but not significantly different: 15 µmol/L (11-25 µmol/L) in type 1, 14 µmol/L (7-21 µmol/L) in type 2 , and 9 µmol/L (6-14 µmol/L) in type 3 ( P = 0.141). A multivariate analysis confirmed the role of the remnant colon in providing additional energy absorption., Conclusion: This study demonstrated the importance of the colon as a salvage organ in children with SBS. Plasma citrulline concentrations should be interpreted according to the type of SBS. Efforts should focus on conservative surgery, early re-establishment of a colon in continuity, and preserving the intestinal microbiota., (Copyright © American Society for Nutrition 2019.)

Introduction: Short bowel syndrome (SBS) is the major cause of chronic intestinal failure (IF), defined as 'the reduction of gut function 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'. Areas covered: Spontaneous intestinal adaptation, including increased hormonal secretion, development of hyperphagia and gut microbiota dysbiosis, occurs 2 years after resection, improving intestinal absorption and decreasing PN dependency. Hormonal treatments, promoting intestinal hyperadaptation, have been proposed in patients with SBS with chronic IF. Clinical studies showed teduglutide to increase urine production and reduce the need for parenteral support volume in these patients. According to the latest ESPEN Guidelines, if a growth factor treatment is considered, the GPL2 analog, teduglutide, should be the first-choice treatment. Expert opinion: These therapies underline the importance of patient monitoring at home and the complexity for HPN adaptation. A multidisciplinary approach should be a gold standard.

Short bowel syndrome (SBS) is defined as having less than 200 cm of functional small bowel. Malabsorptive diarrhoea and dehydration are difficult to manage despite medical therapy and dietary manipulations. Evidence shows that supplementing the diet with High Amylase Resistant Starch (HARS) can reduce diarrhoea from a number of causes including gastroenteritis. It is hypothesised HARS will decrease stool output via the production of short chain fatty acids and the resultant increased water reabsorption. This study aimed to determine if the addition of HARS can reduce diarrhoea in patients with SBS., Methods: Patients with SBS with colon in continuity were recruited from the intestinal rehabilitation clinic at Austin Health. The study was a 2 week crossover trial. Each participant completed the control and the intervention (addition of 50 g HARS to usual diet). Total daily stool weight and number of bowel actions per day were compared between groups using paired t-tests., Results: Eight adults (58% male, mean age 55.7 yrs) were recruited. Five participants completed the trial. Total daily stool weight was reduced in all participants when consuming HARS. Mean daily stool output was significantly decreased 1049 ± 519 g/d to 804 ± 585 g/d (p = 0.023). Number of bowel actions per day showed a trend to reduction., Conclusion: This study gives some support to the hypothesis that the addition of HARS into the diet of patients with short bowel syndrome reduces stool output. Longer trials are required to confirm the effect on nutritional/hydration status., (Crown Copyright © 2018. Published by Elsevier Ltd. All rights reserved.)

Management of severe Short Bowel Syndrome (SBS) is still one of the largest challenges of the medicine. Vast majority of the short bowel patients are children, the conditions that lead to this possible outcome most often are necrotizing enterocolitis (NEC), small intestinal volvulus as a result of intestinal malrotation, gastroschisis and the "apple peel" syndrome. Therefore, paediatricians and paediatric surgeons face this challenge most often. The nontransplant treatment appears to be effective using surgical procedure to increase absorptive surface and to reduce the transit time, but in some cases these procedures are enough to weaning of TPN. The aim of this review was to summarize the modern non-stransplant surgical management of short bowel syndrome., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Necrotizing enterocolitis (NEC) remains the most threatening gastrointestinal complication of prematurity leading to high mortality, morbidity and cost. Common complications of NEC include neurodevelopmental delay, failure to thrive, gastrointestinal problems including strictures and adhesions, cholestasis, short bowel syndrome with or without intestinal failure that can be difficult to manage. Infants who develop NEC benefit from close follow-up for early diagnosis and treatment of complications. Those who present with severe complications such as intestinal failure benefit from a multidisciplinary approach involving careful assessment and treatment. Studies done so far are limited in providing a long-term prognosis. Here we review some of these complications. More studies with a longer follow-up period are needed to better understand the later comorbidities that develop in babies with NEC., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Introduction: Short bowel syndrome (SBS) is a dramatic clinical condition in both children and adults; the residual bowel length is not sufficient to avoid intestinal failure, with subsequent malnutrition and growth retardation, and intravenous support is required to provide the nutrients normally coming from the intestine. Apart from the primary disease, the medical status can be worsened by complications of intestinal failure: if there are irreversible, the prognosis is poor unless a successful intestinal rehabilitation is achieved. Areas covered: The rescue of the remnant small bowel requires a multidisciplinary expertise to achieve digestive autonomy. The use of intestinal trophic factors has shown encouraging results in improving the intestinal adaptation process. Whenever the residual bowel length is inadequate, in a well-selected population weaning parenteral nutrition (PN) off could be attempted by surgery through lengthening procedures. A further subset of patients, with total and irreversible intestinal failure and severe complications on PN, may have an indication to intestinal transplantation. This procedure is still affected by poor long-term results. Expert commentary: Novel approaches developed through a multidisciplinary team work, such as manipulation of microbiota or tissue bioengineering, should be added to current therapies to treat successfully SBS.

Necrotizing enterocolitis (NEC) is an important complication for premature newborns. Infants who survive NEC have a greater possibility of poor long-term physiological and neurodevelopmental growth. The objective of this paper is to give a comprehensive description of the long-term consequences of NEC. Despite the rise in incidence of NEC there is a scarcity of data regarding long-term outcomes of these infants that can be divided into two groups. The first group includes gastrointestinal complications that could occur in relation to the bowel disease, the surgical treatment and quality of the residual bowel. These complications are strictures and short bowel syndrome (SBS). Intestinal strictures are a common occurance after recovery from NEC that should be investigated with a contrast study in case of suspicious clinical findings of bowel obstruction or before reversal ostomy. After this diagnostic investigation, if a stricture is detected in a symptomatic patient, resection of the affected loop of bowel with anastomosis is required. SBS is the result of a massive intestinal resection or of a dysfunctional residual bowel and it can occur in a fourth of patients affected by NEC. The second group includes neurodevelopmental impairment and growth. Neurodevelopmental outcomes of patients after NEC recovery have not been widely reported. Infants with NEC is a population of patients at high risk for adverse neurodevelopmental outcomes whose cause can be multifactorial and linked to perinatal events, severity of disease, surgical treatment and its complications and hospitalization. Understanding the morbidity of NEC with a longterm follow-up would aid neonatologists and pediatric surgeons to make informed decisions in providing care for these patients. Further research on this topic is needed., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Objective: Intestinal failure (IF) is a complex disease that is gaining significance and attention throughout the world. In Mexico, there are no available data on this condition. The aim of this study was to determine the frequency and characteristics of patients with IF type II and III hospitalized at a tertiary referral center in our country., Patients and Methods: A cross-sectional study was carried out from August 2016 to July 2017. Adult patients hospitalized in noncritical areas with a recent diagnosis of IF type II or III according to the European Society for Clinical Nutrition and Metabolism classification were included. Demographic, anthropometric, nutritional therapy, biochemical, and clinical characteristics were registered. Nutritional risk was determined by the Nutritional Risk Score 2002., Results: During the study, 4144 patients were admitted to noncritical areas; 21 (5/1000) of these patients were included. The mean age of the patients was 51±18.8 years, and the mean BMI was 17.6±5.5 kg/m. Fifteen (71.5%) patients were diagnosed with IF type II and six (28.5%) with IF type III. The primary diagnosis was surgical complications in seven (33.3%) of the cases and the principal pathophysiological mechanism was short bowel syndrome in nine (42.8%) patients. The most frequent (37%) clinical classification was D2: more than 20 kcal/kg/day and 1001-2000 ml/day and parenteral nutrition and PN2: 1001-2000 ml/day (52.3%) based on the modified European Society for Clinical Nutrition and Metabolism clinical classification., Conclusion: In this study, a high frequency of IF was found; surgical complications and short bowel syndrome were the main mechanisms involved, and D2 was the most frequent clinical category.

Background: Regulation of energy balance in patients with short bowel syndrome (SBS) is disturbed due to lack of significant part of the intestine. The goal of the research was to analyse the plasma concentrations of selected regulatory peptides - ghrelin, visfatin, and irisin - in children with SBS., Methods: To achieve this aim we recruited study group consisted of 28 children with SBS fed parenterally for at least two weeks, mean age 14 ± 5 months and mean standardised body mass index (SDS-BMI) -1.26 ± 0.84. The control group was represented 25 healthy children of matching age and SDS-BMI. The plasma concentrations of peptides (ghrelin, visfatin, and irisin) were determined using immunoassays, and liver enzymes (AST, ALT, GGT) using an auto-analyser., Results: We observed lower visfatin and ghrelin levels in the study group as compared to controls (both P <0.0001). The lowest total ghrelin concentration was observed in SBS children after ileal resection (P = 0.0016). Irisin concentration did not differ between the groups. Most of the SBS children showed elevated liver enzymes activities at the first measurement and during one-year follow-up., Conclusion: Our findings showed that plasma ghrelin and visfatin themselves may play a role in the course of SBS, while a lack of disturbance in irisin might imply that it is neither playing any role nor it is affected by SBS itself.