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Background: Although parenteral nutrition (PN) significantly improves mortality rates in pediatric short bowel syndrome (SBS), long-term PN has many possible complications and impacts quality of life. Bowel lengthening procedures (BLPs) increase the contact surface of food and the intestinal mucosa and enable the better absorption of nutrients and liquids, possibly leading to a PN decrease., Methods: We retrospectively reviewed the data of patients with short bowel syndrome who underwent BLPs in the period from January 2016 to January 2022. Overall, eight patients, four male, five born prematurely, underwent BLPs., Results: There was a significant decrease in the percentage of total caloric intake provided via PN and PN volume after the BLPs. The more evident results were seen 6 months after the procedure and at the last follow-up, which was, on average, 31 months after the procedure. Two patients were weaned off PN after their BLPs. Patients remained well nourished during the follow-up., Conclusions: The BLP led to a significant decrease in PN needs and an increase in the food intake; however, significant changes happened more than 6 months after the procedure.

Background: Short bowel syndrome (SBS) is one of the most frequent causes of intestinal failure, needing parenteral nutrition to maintain an energy-protein and water-electrolyte balance. At the Hospital Infantil de México Federico Gómez (HIMFG), the formation of two stomas is a technique used for intestinal rehabilitation, where the use of residue through the bypass technique (BT) helps to maintain gastrointestinal functionality, water-electrolyte, and nutritional stability. This study aimed to describe the technique of using intestinal residue through BT as a treatment strategy in intestinal rehabilitation and its effect on the biochemical and nutritional status of pediatric patients with SBS., Methods: An analytical and retrospective cross-sectional study was performed in patients hospitalized at HIMFG with SBS who underwent BT during their hospital stay between 2019 and 2020 and then followed up for 8 weeks., Results: A total of 10 patients were included in this study, with a mean age of 24 months; 50% were female. BT was able to reduce the inflammatory process in the liver caused by the continuous use of parenteral nutrition; enteral caloric intake increased from 25.32 kcal/kg/day to 72.94 kcal/kg/day, but it was insufficient to improve their nutritional status., Conclusions: BT is a safe and effective alternative in intestinal rehabilitation in patients with SBS to stimulate trophism and intestinal functionality, allowing a progression of enteral feeding and a decrease in the hepatic inflammatory process that occurs in these patients with prolonged parenteral nutrition., (Copyright: © 2023 Permanyer.)

Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in children. The mainstay of treatment for IF is parenteral nutrition (PN). The aim of this position paper is to review the available evidence on managing SBS and to provide practical guidance to clinicians dealing with this condition. All members of the Nutrition Committee of the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) contributed to this position paper. Some renowned experts in the field joined the team to guide with their experience. A systematic literature search was performed from 2005 to May 2021 using PubMed, MEDLINE, and Cochrane Database of Systematic Reviews. In the absence of evidence, recommendations reflect the expert opinion of the authors. Literature on SBS mainly consists of retrospective single-center experience, thus most of the current papers and recommendations are based on expert opinion. All recommendations were voted on by the expert panel and reached >90% agreement. The first part of this position paper focuses on the physiological mechanism of intestinal adaptation after surgical resection. It subsequently provides some clinical practice recommendations for the primary management of children with SBS from surgical resection until discharged home on PN., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Liraglutide, a glucagon-like peptide-1 agonist, has been shown to have beneficial effects on fecal output in short bowel syndrome (SBS) by small human studies. Its potential effects early after gut resection are not known. In this pilot observational study, we described the 1- and 6-month liraglutide effects in 19 adult patients with a new SBS diagnosis within 1 month after surgical resection. Stomal/fecal and urinary outcomes, serum/urinary electrolytes, and body composition were assessed. Both within-group differences and between-group comparisons with 20 SBS patients refusing liraglutide treatment were evaluated. The main liraglutide-related side effect was mild nausea, except in one patient, who experienced severe nausea/vomiting. The median ostomy/fecal output was significantly reduced by -550 mL/day after 6 months of treatment ( vs . -200 mL/day in untreated, p = 0.04). The number of patients reaching a ≥20% output reduction was 10/19 (52.6%) treated vs . 3/20 (15.0%) untreated patients ( p = 0.013) at 1 month and 12/19 (63.2%) vs . 6/20 (30.0%) ( p = 0.038) at 6 months, respectively. Participants with a clinically relevant output reduction at 6 months had a significantly lower baseline weight and BMI. Energy parenteral supply significantly decreased, while infused volumes, oral energy, and fluid intakes slightly decreased, though not significantly. This pilot study supports liraglutide benefits in ostomy/fecal output early after surgical gut resection in SBS patients, particularly in those with lower baseline weight values.

Objectives: Autologous intestinal reconstructive (AIR) surgery is frequently utilized in the management of pediatric short bowel syndrome (SBS). However, little is known about the long-term sequela of these procedures., Methods: We undertook a retrospective follow-up study addressing parenteral nutrition (PN) dependence, nutritional status, intestinal morbidity, and related complications in SBS patients having undergone AIR surgery (SBS-AIR, n = 19). We compared results with conservatively treated control SBS patients (SBS-C, n = 45). Eligible patients were identified from our institutional intestinal failure registry during 1985-2019., Results: After median 11.4 follow-up years, 42% of SBS-AIR patients received PN in relation to 36% in SBS-C group ( P = 0.6210), and overall PN duration was significantly longer (35.4 vs 10 months, P = 0.0004) in SBS-AIR group. Although symptoms of intestinal dysfunction improved in majority (62%) of patients after AIR surgery, their symptoms remained more frequent and severe at latest follow-up compared to SBS-C group (39% vs 5%, P = 0.0015). Although bacterial overgrowth was more frequent in SBS-AIR group (53% vs 24%, P = 0.0416), latest endoscopy findings and fecal calprotectin levels as well as occurrence of anastomotic/staple line ulcerations were comparable between groups. Histological liver steatosis (50% vs 18%, P = 0.042) and impaired bone health (26% vs 6.7%, P = 0.042) were more frequent in SBS-AIR patients., Conclusions: While AIR surgery improved gastrointestinal symptoms and transition to enteral autonomy in majority of patients, a noteworthy proportion of them continued to suffer from clinically significant intestinal dysfunction and related complications. Close long-term follow-up of pediatric AIR surgery patients is mandatory., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

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

Objective: Short bowel syndrome (SBS) is a severe intestinal disease that causes malabsorption. Long-term parental nutrition therapy induces infection and liver failure. For the surgical management of intestinal rehabilitation, the intestinal loop lengthening method and serial transverse enteroplasty (STEP) method have been reported, although their effects have proven limited. We herein report a new surgical technique, Saeki-Spiral-Shark (3S) method for SBS using biomimetics of shark intestine., Methods: In the 3S method, a spiral valve is formed inside the intestine by external sutures. Using a 25 cm length intestinal organ model, we performed both the 3S method and STEP procedure. We then compared the length and fluid passage times of the subsequently formed intestine., Results: After the 3S method was performed, the length of the intestinal model changed to 22 cm, and after the STEP procedure, that was elongated to 30 cm. Although the water passage times did not change markedly, the semi-digestive nutritional supplement passage time slowed down in the model with the 3S method. There was slight leakage in the STEP procedure model., Conclusions: The 3S method is a unique method of treating SBS based on biomimetics. This procedure does not require an incision of the intestine, which thereby enabling clean and less-invasive surgery. We plan to conduct animal experiments in the future., (© 2022. The Author(s).)

Mesenteric ischemia is an infrequent diagnosis, although it carries substantial morbidity and mortality. In adults, the massive resection of small bowel leaving less than 150 cm of intestine results in malabsorption and diarrhoea and defines the short bowel syndrome (SBS). In this report, we present a case of emergency surgery with a near total enterectomy due to superior mesenteric ischemia with a long time of survival. KEY WORDS: Case report, General surgery, Mesenteric ischemia, Small intestine.

Background: Short bowel syndrome and its resultant nutritional deficiencies are the most common cause of intestinal failure. Significant intestinal lengthening using intraluminal springs is feasible in porcine models using an external plication technique. We hypothesize that an internal plication technique will yield significant intestinal lengthening, which may lead to future endoscopic spring placement., Methods: Uncompressed springs measuring 7.5 cm with a diameter of 1.0 cm were compressed to 2.0 cm. A gelatin-encapsulated compressed nitinol spring was inserted into the jejunal lumen of juvenile pigs and held in place with endoluminal sutures just proximal and distal to the spring-containing segment. A control segment distal to the spring was marked. Pigs were euthanized on postoperative day 7. Spring and control segments were collected for analyses., Results: There was an average lengthening by 72% of the spring segment compared to the control segment. Two out of 7 springs stayed within both sets of plications and doubled in length. Histology showed normal mucosal integrity of the spring segment and plicated areas with similar muscular thickness but increased crypt depth and villus length compared to the control segment., Conclusion: Internal plication resulted in significant bowel lengthening. Five springs had slipped through proximal, distal or both sets of plications, resulting in less lengthening than those that remained fixed. A more consistent methodology for endoluminal suturing is needed to produce more lengthening., Competing Interests: I have read the journal’s policy and the authors of this manuscript have the following competing interests: James CY Dunn, MD PhD is a co-founder of Eclipse Regenesis, a company that develops similar spring-based intestinal lengthening devices, and reported his patent on “Expandable distension device for hollow organ growth. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Background: To investigate the effects of a reversed segment of the distal small intestine to improve weight gain in an experimental short bowel syndrome (SBS) model in piglets., Methods: Twenty-four piglets underwent resection of 70% of the distal small intestine. In half of the animals a conventional anastomosis was performed, and in the other half, the distal 25 cm of the remnant jejunum was reversed before the intestinal continuity was recreated. Weight was measured daily until day 28, where the animals were euthanized. Glucagon-Like Peptide-2 (GLP-2) and Glucose-dependent Insulinotropic Peptide (GIP) was measured pre- and postoperatively at day 28., Results: The group with reversal of small intestine had a significant lower weight gain at 5.26 ± 3.39 kg (mean ± SD) compared to the control group with 11.14 ± 3.83 kg (p < 0.05). In the control group greater villus height and crypt depth was found distally, and greater muscular thickness was found proximally in the intervention group. GLP-2 and GIP levels increased significantly in the control group., Conclusions: Treatment of short bowel syndrome with a reversed jejunal segment of 25 cm had a detrimental effect on the weight gain., (© 2022. The Author(s).)

Objectives: Total colonic aganglionosis involving the small bowel is a rare form of Hirschsprung disease. We aim to analyse the long-term outcomes, digestive autonomy, and complications, to suggest recommendations for prevention and treatment., Methods: All patients born between 2000 and 2015 followed in our centre were retrospectively included. We analysed the length of aganglionosis, surgical procedures, growth, duration of parenteral nutrition (PN), enterocolitis, liver disease, intestinal transplantation., Results: Twenty-five patients were followed for a median of 10.9 years. Fifteen patients had less than 80 cm of ganglionic small bowel (SB) with a median of 20 cm. Ten patients had more than 80 cm of ganglionic sB with a median of 115 cm. The median PN duration was significantly shorter for patients with more than 80 cm: 0.9 versus 7.5 years in those with less than 80 cm (P  < 0.001). No patient with less than 80 cm was weaned off PN, except 1 who underwent intestinal transplantation. Ten patients with less than 80 cm develop enterocolitis on the excluded segment, leading to emergency entero-colectomy in 5. Liver disease was more frequent in patients with less than 80 cm (11 vs 0). Three patients required combined liver-intestine transplantation; 2 underwent an isolated intestinal transplantation., Conclusions: Digestive autonomy was possible in most patients with more than 80 cm of ganglionic SB. The more severe complication was enterocolitis. Liver disease compromised long-term survival without transplantation. Both complications should be prevented by early diversion and enterectomy of the whole aganglionic segment. Follow-up in or together with a multidisciplinary intestinal rehabilitation centre is suggested., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Short gut syndrome can lead to type 3 intestinal failure, and nutrition and hydration can only be achieved with parenteral nutrition (PN). While this is a lifesaving intervention, it carries short- and long-term complications leading to complex comorbidities, including chronic kidney disease. Through a patient with devastating inflammatory bowel disease's journey, this review article illustrates the effect of short gut and PN on kidney function, focusing on secondary hyperoxaluria and acute precipitants of glomerular filtration. In extensive small bowel resections colon in continuity promotes fluid reabsorption and hydration but predisposes to hyperoxaluria and stone disease through the impaired gut permeability and fat absorption. It is fundamental, therefore, for dietary intervention to maintain nutrition and prevent clinical deterioration (i.e., sarcopenia) but also to limit the progression of renal stone disease. Adaptation of both enteral and parenteral nutrition needs to be individualised, keeping in consideration not only patient comorbidities (short gut and jejunostomy, cirrhosis secondary to PN) but also patients' wishes and lifestyle. A balanced multidisciplinary team (renal physician, gastroenterologist, dietician, clinical biochemist, pharmacist, etc.) plays a core role in managing complex patients, such as the one described in this review, to improve care and overall outcomes.

Intestinal Failure (IF) includes the loss of functional intestinal mass and the requirement of long term Parenteral Nutrition (PN) to achieve the development and growth in childhood., Objective: To evaluate the experience in a specialized unit for pediatric patients with IF, describing the clinical cha racteristics of those admitted from November 2009 to December 2019., Patients and Method: Retros pective and descriptive review from clinical records of 24 cases that matched the inclusion criteria. The following variables were recorded: gender, neonatal history, origin unit, patient age and anthropome tric diagnosis at admission to the unit, cause of IF, hospital stay, anthropometric data and parenteral nutrition dependency at discharge. In those patients with a diagnosis of short bowel syndrome (SBS), the cause of intestinal resection and the characteristics of the intestinal remnant were identified: ana tomical classification, remnant length (defining ultra-short as < 25 cm), presence of ileocecal valve, and characteristics of the colon., Results: The median age at admission was 7.8 months. Seventeen cases were preterm. Regarding IF etiology, 10 patients presented SBS, 6 patients with Intestinal Neuromus cular Disease (INMD), 7 children with SBS associated with INMD, and 1 case of intestinal lymphan giectasia. Within the SBS etiologies found in this group, intestinal atresia (8 cases) and necrotizing enterocolitis (9 patients) were the main causes with a similar proportion. Eight patients had no ileo cecal valve. According to anatomical classification, 1 case was Type I, 8 were Type II, and 8 were Type III. Related to bowel length, 3 were ultrashort, besides being Type II; in those with > 40 cm of bowel length, 7 were Type III. Overall average hospital stay was 456.4 days. Enteral autonomy was achieved in 16 patients and 8 cases required home parenteral nutrition., Conclusions: IF requires life support, PN and prolonged hospital stay. The principal etiology of IF is SBS caused by congenital intestinal atresia and necrotizing enterocolitis. Nevertheless, the high frequency of INMD could be attributed to the local protocol analysis. Most of our patients had poor prognosis factors, however, the management by a specialized team allowed the achievement of enteral autonomy in 66.7% of cases.

Background: Various abdominal pathologies end up with surgical resection of small intestine. When the small intestine remnant is too short for adequate fluid and micronutrients absorption, short bowel syndrome is diagnosed. The disabling condition needs a multidisciplinary approach to design parenteral nutrition, care for thrombotic, hepatic and infectious complications and gradually wean the patient from parenteral nutrition. Various surgical techniques have been introduced to increase absorptive mucosa and enhance the intestinal adaptation process. Serial transverse enteroplasty and nipple valve reconstruction are among the procedures, which will be discussed in the current article., Case Presentation: Herein, we presented 5 cases of short bowel syndrome as a consequence of abdominal laparotomies, patients were referred to our center to receive parenteral nutrition and to be prepared for the final autologous gastrointestinal reconstruction or intestinal transplantation, if indicated., Conclusion: Patient's age, performance status and bowel remnant length determines the appropriate technique for autologous gastrointestinal reconstruction. Serial transverse enteroplasty is designed to increase bowel's length by creating zigzag patterns through dilated bowel loops. Presence of ileocecal valve is crucial to delay intestinal transit time and to prevent colonic bacterial transfer to ileum. Patient's with ileocecal valve loss benefit from creating an artificial valve, namely, nipple valve., (© 2021. The Author(s).)

The small intestine is the main organ for nutrient absorption, and its extensive resection leads to malabsorption and wasting conditions referred to as short bowel syndrome (SBS). Organoid technology enables an efficient expansion of intestinal epithelium tissue in vitro 1 , but reconstruction of the whole small intestine, including the complex lymphovascular system, has remained challenging 2 . Here we generate a functional small intestinalized colon (SIC) by replacing the native colonic epithelium with ileum-derived organoids. We first find that xenotransplanted human ileum organoids maintain their regional identity and form nascent villus structures in the mouse colon. In vitro culture of an organoid monolayer further reveals an essential role for luminal mechanistic flow in the formation of villi. We then develop a rat SIC model by repositioning the SIC at the ileocaecal junction, where the epithelium is exposed to a constant luminal stream of intestinal juice. This anatomical relocation provides the SIC with organ structures of the small intestine, including intact vasculature and innervation, villous structures, and the lacteal (a fat-absorbing lymphatic structure specific to the small intestine). The SIC has absorptive functions and markedly ameliorates intestinal failure in a rat model of SBS, whereas transplantation of colon organoids instead of ileum organoids invariably leads to mortality. These data provide a proof of principle for the use of intestinal organoids for regenerative purposes, and offer a feasible strategy for SBS treatment.

Background: There is little knowledge with regard to the management of intestinal failure in countries where home care services and dedicated intestinal rehabilitation centers are limited. This study presents a single-center experience of treating adult-type short bowel syndrome (SBS) with serial transverse enteroplasty (STEP)., Methods: Medical records were retrospectively reviewed from November 2009 to April 2018 on patients with adult-type SBS. All patients underwent STEP, and a representative quota sample of control patients treated with conventional measures were included. Clinico-demographic characteristics including baseline and post-treatment information about the orientation of bowel alignment and nutritional status were evaluated., Results: The mean patient age was 51.1 ± 16.2 in the STEP group and 57.6 ± 12.7 in the control group (P = .304). The median small bowel length was 60 cm (interquartile range (IQR): 40-90) in the STEP group (before the lengthening) and 90 cm (IQR: 70-100) in the control (at the initiation of intestinal rehabilitation) (P = .035). Durations of median follow-up were 18 months (IQR: 14-58) and 10 months (IQR: 3-14), respectively (P = .019). In the STEP group, the mean increase in bowel length after STEP was 37.3 ± 11.6 cm, and at their follow-up 7 patients (64%) had successfully progressed to enteral autonomy. In the control group, only 3 patients (27%) were successful. Mean time to wean parenteral nutrition was 45 ± 54 days, and the mean increase in enteral calorie intake was 1.79 ± 1.60-fold after lengthening in the STEP group., Conclusions: STEP is an easy-to-perform procedure in the surgical rehabilitation of adult-type SBS. When performed simultaneously with reconnection surgery, it may offer a cost-effective and comprehensive solution to the treatment strategy in middle income settings.

Background: Short bowel syndrome (SBS) results from significant loss of small intestinal length. In response to this loss, adaptation occurs, with Epidermal Growth Factor Receptor (EGFR) being a key driver. Besides enhanced enterocyte proliferation, we have revealed that adaptation is associated with angiogenesis. Further, we have found that small bowel resection (SBR) is associated with diminished oxygen delivery and elevated levels of hypoxia-inducible factor 1-alpha (HIF1α)., Methods: We ablated EGFR in the epithelium and endothelium as well as HIF1α in the epithelium, ostensibly the most hypoxic element. Using these mice, we determined the effects of these genetic manipulations on intestinal blood flow after SBR using photoacoustic microscopy (PAM), intestinal adaptation and angiogenic responses. Then, given that endothelial cells require a stromal support cell for efficient vascularization, we ablated EGFR expression in intestinal subepithelial myofibroblasts (ISEMFs) to determine its effects on angiogenesis in a microfluidic model of human small intestine., Results: Despite immediate increased demand in oxygen extraction fraction measured by PAM in all mouse lines, were no differences in enterocyte and endothelial cell EGFR knockouts or enterocyte HIF1α knockouts by POD3. Submucosal capillary density was also unchanged by POD7 in all mouse lines. Additionally, EGFR silencing in ISEMFs did not impact vascular network development in a microfluidic device of human small intestine., Conclusions: Overall, despite the importance of EGFR in facilitating intestinal adaptation after SBR, it had no impact on angiogenesis in three cell types-enterocytes, endothelial cells, and ISEMFs. Epithelial ablation of HIF1α also had no impact on angiogenesis in the setting of SBS., Competing Interests: LVW has financial interests in Microphotoacoustics, Inc., which did not support this work. AKA reports receiving research support from Verastem, Selexys, Karyopharm, Cell Works, Cleave Bioscience, Glycomimetics, Abbvie and Vasculox, none of which supported this work. AKA is also the founder and owner of Targeted Therapeutics LLC and Cellatrix LLC, neither of which supported this work. All other authors have no other financial conflicts of interest. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

Pediatric Short Bowel Syndrome (SBS) can require prolonged parenteral nutrition (PN). Over the years, SBS management has been implemented by autologous gastrointestinal reconstructive surgery (AGIR). The primary objective of the present review was to assess the effect of AGIR on weaning off PN. We also evaluated how AGIR impacts survival, the need for transplantation (Tx) and the development of liver disease (LD). We conducted a systematic literature search to identify studies published from January 1999 to the present and 947 patients were identified. PN alone was weakly associated with higher probability of weaning from PN (OR = 1.1, p = 0.03) and of surviving (OR = 1.05, p = 0.01). Adjusting for age, the probability of weaning off PN but of not surviving remained significantly associated with PN alone (OR = 1.08, p = 0.03). Finally, adjusting for age and primary diagnosis (gastroschisis), any association was lost. The prevalence of TX and LD did not differ by groups. In conclusion, in view of the low benefit in terms of intestinal adaptation and of the not negligible rate of complications (20%), a careful selection of candidates for AGIR should be required. Bowel dilation associated with failure of advancing EN and poor growth, should be criteria to refer for AGIR.

We have recently established an intestinal rehabilitation unit (IRU) in Abu Ali Sina transplantation center affiliated to Shiraz University of Medical Sciences, Iran. Our intestinal failure rehabilitation and transplant program aims to provide state-of-the-art care for adult patients with different degrees of intestinal insufficiency and failure. In the IRU, we aimed to design an algorithmic approach to patients with small bowel ischemia and short bowel syndrome (SBS) based on our institutional experience in our country and based on other pioneering studies from other regions of the world., (© 2020 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

Impact Statement: Short bowel syndrome is associated with significant comorbidities and mortality. This study is important as unlike current systems, it provides a validated piglet model which mirrors anatomical, histological, and serological characteristics observed in human SBS. This model can be used to advance knowledge into mechanistic pathways and therapeutic modalities to improve outcomes for SBS patients. This study is novel in that in addition to significant reduction in the remnant bowel and noted liver disease, we also developed a method to emulate ileocecal valve resection and described gut adaptive responses which has important clinical implications in humans.

Purpose: The denervation of the intestine with benzalkonium chloride (BAC) reduces mortality and improves weight gain in rats with short bowel syndrome (SBS). Nevertheless, translating these promising findings from bench to bedside is not feasible because BAC promotes peritonitis and irreversible denervation which may be followed by an uncontrolled dilatation of the viscera. The use of botulinum toxin (BT) instead of BAC to achieve the denervation of the remaining small intestine in SBS could be an interesting option because it leads to a mild and transient denervation of the intestine., Methods: Here we evaluated the effects of the ileal denervation with BT in rats with SBS by verifying the body weight variation and intestinal morphological parameters. Four groups with 6 animals each were submitted to enterectomy with an ileal injection of saline (group E) or BT (group EBT). Control groups were submitted to simulated surgery with an ileal injection of BT (group BT) or saline (group C - control)., Results: We observed that the treatment of the remaining ileum with BT completely reversed the weight loss associated to extensive small bowel resection., Conclusion: This may provide a new promising approach to the surgical treatment of SBS.

Background: Short bowel syndrome is a harmful condition that needs experimental research., Aim: To assess the impact of the ileocecal valve removal in a model of short bowel syndrome, in order to investigate the evolution of the colon under this circumstance., Method: Fifteen Wistar rats were equitable divided into: Control (Sham), Group I (70% enterectomy preserving ileocecal valve) and Group II (70% enterectomy excluding ileocecal valve). After enterectomy was performed jejunoileal or jejunocecal anastomosis and sacrificed the animals on 30th postoperative day for histomorphometric study of the colon. During this period, was observed the clinical evolution of the animals weekly including body weight measurement., Results: Group I and II presented progressive loss of weight. In Group I was observed diarrhea, perineal hyperemia and purple color of the colon during autopsy. Histomorphometry assay showed hypertrophy and hyperplasia of colon mucosa in Group I. In Group II the colon wall was thicker due to hypertrophy and muscular hyperplasia, and in mucosa vascular proliferation and inflammatory infiltrate were intense., Conclusion: This short bowel syndrome model is relevant and achieve 100% of survival. Animal's weight loss was not altered by the presence or exclusion of the ileocecal valve. Animals with 70% of small bowel removal and presence of the ileocecal valve attained a better clinical evolution and histological colon adaptation than those without ileocecal valve.

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.

BACKGROUND Short bowel syndrome (SBS) is a malabsorption syndrome that results from an extensive intestinal resection or repeated small bowel resections. Postoperative small bowel obstruction is a well-known complication of abdominal surgeries requiring readmission and reoperation after failed conservative management. A combination of the above factors poses a clinical challenge for surgeons due to lack of applicable treatment options. CASE REPORT A 68-year-old man underwent repetitive laparotomies and multiple small bowel resections for an incarcerated inguinal hernia, resulting in SBS. Postoperative small bowel obstruction resulting from an anastomotic stricture near the ligament of Treitz made the patient unable to sustain oral nutrition. During reoperation, insufficient jejunum length and extensive intraabdominal adhesions led us to perform a primary side-to-side duodenocolonic anastomosis, which is an unusual treatment option. After a long but uncomplicated postoperative course, the patient was able to ingest solid foods and was discharged in healthy condition with parental nutritional support. CONCLUSIONS Duodenocolostomy can be a treatment of last resort in patients with limited surgical treatment options and can lead to a significant improvement of their quality of life.

Background: Angiopoietin-like proteins (ANGPTLs) 3 and 4 are circulating factors that participate in the regulation of lipid and glucose metabolism., Subjects and Methods: We measured serum ANGPTL3 and 4 levels in 23 patients with obesity, 40 patients with obesity and type 2 diabetes mellitus (T2DM), 22 patients with anorexia nervosa (AN), 15 subjects undergoing 72-h fasting, and 12 patients with short bowel syndrome (SBS), and their changes after very-low-calorie diet (VLCD), bariatric surgery, partial realimentation, acute fasting, and parenteral nutrition in order to assess their possible role in metabolic regulations., Results: Serum ANGPTL4 levels were higher in obese subjects without/with T2DM (94.50 ± 9.51 and 134.19 ± 7.69 vs. 50.34 ± 4.22 ng/ml, p < 0.001) and lower in subjects with AN relative to healthy control subjects (38.22 ± 4.48 vs. 65.80 ± 7.98 ng/ml, p = 0.002), while serum ANGPTL3 levels demonstrated inverse tendency. Nutritional status had no effect on ANGPTL3 and 4 mRNA expression in adipose tissue. Fasting decreased ANGPTL3 and increased ANGPTL4 levels, while VLCD reduced only ANGPTL3. Bariatric surgery and realimentation of AN or SBS patients had no effect on either ANGPTL. Multiple regression analysis identified BMI as an independent predictor of ANGPTL3; and BMI and HbA 1c as independent predictors of ANGPTL4, respectively., Conclusions: Taken together, our data suggest that serum ANGPTL3 and 4 levels are influenced by nutritional status and fasting and could be involved in the metabolic disturbances present in obesity and AN.

Background and Objectives: Short bowel syndrome (SBS) is a rare and life-threatening disease. Few studies have investigated risk factors for parenteral nutrition (PN)-dependence and death in SBS. Accordingly, the aim of this study was to investigate the risk factors for PN-dependence and long-term mortality in SBS., Methods and Study Design: This retrospective study reviewed and evaluated children and adults who were diagnosed with SBS at King Chulalongkorn Memorial Hospital from October 2005 to January 2015. Age, causes of SBS, length of remnant bowel, type of anastomosis, types of nutrition support, SBS-associated complications, PN-dependence rate, duration of PN-dependence, mortality rate, and causes of death were evaluated., Results: Twenty-two adults and 19 children were reviewed. The median follow-up time was 48 months. At the end of follow-up, PNdependence rate was 51.2%. The residual colon >=50% group had a significantly lower PN-dependence rate, with a hazard ratio of 0.36 (95% CI: 0.14-0.93; p=0.03). The most frequent cause of death was infection, with the highest percentage of mortality occurring within the first 2 years after surgery. The mortality rate was 53.8% and the residual colon >=50% group had a significantly lower mortality rate, with a hazard ratio of 0.36 (95% CI: 0.14- 0.88; p=0.03)., Conclusions: PN-dependence and death occurred in about half of all patients. Residual colon >=50% was significantly associated with lower death rate and PN-dependence. The crucial role of colon in continuity as a protective factor should be investigated further in prospective studies.

Short bowel syndrome is a major cause of morbidity and mortality in children. Despite decades of experience in the management of short bowel syndrome, current therapy is primarily supportive. Definitive treatment often requires intestinal transplantation, which is associated with significant morbidity and mortality. In order to develop novel approaches to the treatment of short bowel syndrome, we and others have focused on the development of an artificial intestine, by placing intestinal stem cells on a bioscaffold that has an absorptive surface resembling native intestine, and taking advantage of neovascularization to develop a blood supply. This review will explore recent advances in biomaterials, vascularization, and progress toward development of a functional epithelium and mesenchymal niche, highlighting both success and ongoing challenges in the field.

Background: We have recently demonstrated the feasibility of lengthening the duodenum in children with short bowel syndrome and a dilated duodenum. This procedure gains additional intestinal length in a challenging area of autologous gut reconstruction. Herein, we report the successful application of this technique in an adult with ultra-short bowel syndrome., Case Report: A 25-year-old man with a history of mid-gut volvulus was referred to our center for intestinal transplant evaluation. Only a megaduodenum stump that reached as far as the third portion (30 cm of length) and the colon up to the hepatic flexure in the form of a mucous fistula was retained. A gastrostomy tube drained gastric and bilio-pancreatic secretions (output range: 2.5-4 liters/day). The time spent on parenteral nutrition (3 liters/day; 1500 calories/day) and I.V. fluid (1.5-2 liters/day) administration was 24 hours per day. The patient underwent duodenal lengthening and tapering with 7 sequential transverse applications (5 of 45 mm and 2 of 60 mm) of an endoscopic stapler on the anterior and posterior walls of the duodenum, respecting the pancreatic parenchyma and end-to-side duodeno-colonic anastomosis. The final duodenal length was 83 cm. The pre-lengthening citrulline level increased from 13.6 micromol/L to 21.6 micromol/L one year post-lengthening. After 24 month of follow-up, the time on a parenteral pump was shortened to 9 hours during the night. The volume and calorie requirements were also reduced by half., Discussion: Duodenal lengthening may be effective as part of the autologous intestinal reconstruction armamentarium in adults with short bowel syndrome.

Aim of the Study: To report the current activity of intestinal transplantation in Europe (EU) and Unites States of America (USA), underlining outcomes in the last 5 years and discussing possible trends., Patients and Methods: Data review of results was performed through analysis of ITR and UNOS registries, Eurotransplant and newsletter transplant reports, congress abstracts, international published literature, personal communications and hospital web sites., Results: The absence in Europe of a sole organization collecting donors and the presence of many low-volume centers (less than 5 cases/year) makes the difference with USA: in the last 5 years (2010-2014), 222 intestinal/multivisceral transplants have been performed in EU countries (most of them in the UK), while in USA, the number of transplants achieved 634 procedures in the same period of time. Waiting list mortality remains unacceptable in both continents. Improved short-term results, with over 80% survival at 1 year, have been achieved in the busiest transplant centers likely due to immune-induction agents, more recently to innovative cross match strategies and optimizing organ allocation, but long term outcomes are still inferior to other organ transplants. Most long-term survivors were reintegrated to society with self-sustained socioeconomic status. The economic burden for the society is high and related costs are different between USA and EU (and inside Europe between member state's health-care systems), but cost-effectiveness for intestinal transplantation still needs to be proved., Conclusion: Overall intestinal transplantation continues to develop in EU and USA together with surgical and medical rehabilitation of patients affected by short gut syndrome., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Intestinal failure is a rare life-threatening condition that results in the inability to maintain normal growth and hydration status by enteral nutrition alone. Although parenteral nutrition and whole organ allogeneic transplantation have improved the survival of these patients, current therapies are associated with a high risk for morbidity and mortality. Development of methods to propagate adult human intestinal stem cells (ISCs) and pluripotent stem cells raises the possibility of using stem cell-based therapy for patients with monogenic and polygenic forms of intestinal failure. Organoids have demonstrated the capacity to proliferate indefinitely and differentiate into the various cellular lineages of the gut. Genome-editing techniques, including the overexpression of the corrected form of the defective gene, or the use of CRISPR (clustered regularly interspaced short palindromic repeats)/Cas9 to selectively correct the monogenic disease-causing variant within the stem cell, make autologous ISC transplantation a feasible approach. However, numerous techniques still need to be further optimized, including more robust ex vivo ISC expansion, native ISC ablation, and engraftment protocols. Large-animal models can to be used to develop such techniques and protocols and to establish the safety of autologous ISC transplantation because outcomes in such models can be extrapolated more readily to humans. Stem Cells Translational Medicine 2017;6:666-676., (© 2016 The Authors Stem Cells Translational Medicine published by Wiley Periodicals, Inc. on behalf of AlphaMed Press.)

Abdominal wall transplantation (AWTX) has revolutionized difficult abdominal closure after intestinal transplantation (ITX). More important, the skin of the transplanted abdominal wall (AW) may serve as an immunological tool for differential diagnosis of bowel dysfunction after transplant. Between August 2008 and October 2014, 29 small bowel transplantations were performed in 28 patients (16 male, 12 female; aged 41 ± 13 years). Two groups were identified: the solid organ transplant (SOT) group (n = 15; 12 ITX and 3 modified multivisceral transplantation [MMVTX]) and the SOT-AWTX group (n = 14; 12 ITX and 2 MMVTX), with the latter including one ITX-AWTX retransplantation. Two doses of alemtuzumab were used for induction (30 mg, 6 and 24 h after reperfusion), and tacrolimus (trough levels 8-12 ng/mL) was used for maintenance immunosuppression. Patient survival was similar in both groups (67% vs. 61%); however, the SOT-AWTX group showed faster posttransplant recovery, better intestinal graft survival (79% vs. 60%), a lower intestinal rejection rate (7% vs. 27%) and a lower rate of misdiagnoses in which viral infection was mistaken and treated as rejection (14% vs. 33%). The skin component of the AW may serve as an immune modulator and sentinel marker for immunological activity in the host. This can be a vital tool for timely prevention of intestinal graft rejection and, more important, avoidance of overimmunosuppression in cases of bowel dysfunction not related to graft rejection., (© Copyright 2015 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Background and Objectives: Certain patients who undergo proximal jejunum resection are unable to undergo primary anastomosis and require exteriorization of the proximal jejunum. These patients usually have major problems with short bowel due to the high output of the stoma. The output of a proximal jejunostomy contains abundant amounts of enzymes and electrolytes. Therefore, it is a feasible approach to re-infuse jejunostomy output to regain homeostasis. To evaluate the effects of proximal jejunostomy output reinfusion into the distal small bowel for patients with short bowel syndrome, and to determine whether reinfusion could avoid long-term parenteral nutrition (PN)., Methods and Study Design: PN was initiated immediately after surgery. When patients started enteral nutrition, we started the proximal jejunostomy output reinfusion protocol. Proximal jejunostomy output reinfusion was performed by the patients, and continued by them after discharge. When proximal jejunostomy output reinfusion could be performed stably, PN was stopped., Results: The median length of the proximal jejunum was 20 cm and of the distal small bowel was 77.5 cm in patients who could stably receive proximal jejunostomy output reinfusion alone. Three patients did not require home PN; they only required PN during hospitalization. Four patients successfully underwent stoma takedown with intestinal anastomosis after 6-7 months without any nutritional or metabolic complications., Conclusion: Short bowel syndrome patients with an adequate length of small bowel and functional colon could avoid long-term PN by receiving reinfusion of proximal jejunostomy output into the distal small bowel.

Obesity may protect against the nutritional consequences of short bowel syndrome. We hypothesized that rats preconditioned with an obesogenic diet would have better outcomes after surgical induction of short bowel syndrome compared to rats on regular chow. Rats were fed a high-fat diet or regular rat chow for six months, and then underwent 50% proximal, 50% distal, or sham enterectomy. Food intake, weight, and body composition were monitored before and for 4 weeks after surgery. The high-fat diet consistently produced obesity (>25% body fat). All procedures induced weight loss, but there was no discernable difference between resection vs. sham resection. Rats on the high-fat diet had a greater post-resection loss of body fat compared to rats on chow (36 vs. 26 g, respectively). There was a nonsignificant trend of less lean mass loss in the former compared to the latter rats (16 vs. 33 g, respectively). Enterectomy moderated serum ghrelin, GIP, PPY, insulin, and leptin. Intestinal adaptation was not different between obese vs. non-obese rats. Rats preconditioned with the high-fat diet may have had better retention of lean body mass after a surgical procedure compared to rats on chow. The effect of 50% enterectomy was less than expected.

Objective: Management of short bowel syndrome (SBS) has significantly evolved recently. We present our single-center, 25-year experience focusing on the implementation of a refined multidisciplinary SBS care protocol., Methods: This is a retrospective review of the patients with SBS treated at our tertiary center from 1988 to 2014, with either <25% short bowel remaining or duration of parenteral nutrition (PN) >3 months. Patients with primary intestinal motility disorders were excluded. Clinical characteristics, including intestinal anatomy, markers of cholestasis, and catheter-related infections (CRIs), were analyzed. The implementation of a refined modern uniform management protocol in 2009 divided the cohort into 2 subgroups, whose outcomes are compared., Results: Forty-eight patients with SBS were identified (median gestational age 33 weeks). Of them, 22 were born between 2009 and 2014. The main causes of SBS were necrotizing enterocolitis (46%) and midgut volvulus (23%). Median remaining small bowel length was 36 cm. The overall survival was 23 of 26 (88%) before 2009 and 21 of 22 (95%) thereafter, whereas none were transplanted. Duration of PN shortened from a median of 15 to 6 months (P = 0.0015) in the latter cohort, whereas frequency of autologous intestinal reconstruction surgery (31%) remained unchanged. Frequency of neonatal cholestasis was similar in both groups (75%), but cleared in all after 2009. Before 2009, 2 patients died of progressive cholestatic liver failure. The CRI rates decreased from 1.7 to 0.7 per 1000 catheter-days between 2000-2008 and 2009-2014, respectively (P = 0.0178)., Conclusions: Uniform refined multidisciplinary approach decreased the duration of PN and CRI rates with high transplant-free survival and avoidance of liver failure, although the frequency of transient neonatal cholestasis remained unchanged.

Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty has been increasingly used as the surgical treatment of choice for children with refractory short bowel syndrome, but there have been few reports about the adult patients. This report describes the case of a 71-year-old man with a short bowel after distal gastrectomy with Billroth II reconstruction for gastric cancer, followed by extensive intestinal resection. The serial transverse enteroplasty operation was performed and lengthened the small intestine from 49 to 67 cm. The patient tolerated the procedure well and weaned off total parenteral nutrition. Liver function also improved. This case shows that the serial transverse enteroplasty procedure increases intestinal length. This procedure should be considered a surgical option for adult patients with extreme short bowel syndrome.

Purpose: To evaluate the current model of small bowel resection and intestinal transplantation in pigs., Methods: Forty two Large White pigs were distributed in five groups: G1(n=6), G2(n=6) and G3(n=6) were submitted to 80%,100% and 100% plus right colon resection respectively and G4(n=7) and G5(n=5) to 100% SBR plus IT without and with immunosuppression based on Tacrolimus and Mycophenolic acid. Evaluation included weight control, clinical status, biochemical analysis and endoscopies for graft biopsies. Follow-up in G1 and 2 was 84 days, while in G3, four and five was ± three weeks., Results: G1 increased weight suggesting adaptation while G2 and 3 loused weight and inadequate adaptation. G4 and 5 died of acute cellular rejection (ACR) and sepses respectively. Overall survival in G1, 2, 3, 4 and 5 at 30 days was 100, 100, 0 and 20 %, respectively. Medium survival in G4 and 5 was 14 and 16 days., Conclusions: The resection of 80% of small intestine in pigs is not suitable for short bowel syndrome induction. Intestinal transplantation with the proposed immunosuppression protocol was effective in prevent the occurrence of severe acute rejection, but inappropriate to increase recipients survival.

Background: Endogenous glucagon-like peptide-2 (GLP-2) levels and intestinal adaptation are reduced in distal-intestinal resection animal models of short bowel syndrome (SBS) that lack remnant ileum. We hypothesized that exogenous GLP-2 would improve intestinal adaptation in a distal-intestinal resection neonatal piglet model of SBS., Methods: In all, 35 piglets were randomized to 2 treatment and 3 surgical groups: control (sham), 75% mid-intestinal resection (JI), and 75% distal-intestinal resection (JC). Parenteral nutrition (PN) commenced on day 1 and was weaned as enteral nutrition (EN) advanced. IV GLP-2 (11 nmol/kg/d) or saline was initiated on day 2. Piglets were maintained for 14 d. Clinical, functional, morphological, and histological outcomes were obtained., Results: JC-GLP-2 piglets had fewer days on PN (10.0 ± 0.6 vs. 13.8 ± 0.2), more days on EN (4.0 ± 0.6 vs. 0.2 ± 0.2), a higher percentage of EN at termination (92 ± 5 vs. 52 ± 10%), fewer days of diarrhea (8.0 ± 0.7 vs. 12.3 ± 0.4), increased intestinal length (19 ± 4 vs. -5 ± 3%), and deeper jejunal crypts (248 ± 21 vs. 172 ± 12 μm), compared with saline piglets., Conclusion: GLP-2 therapy improves clinical, morphological, and histological outcomes of intestinal adaptation in a distal-intestinal resection model of SBS. Since this anatomical subtype represents the majority of clinical cases of neonatal SBS, these results support a potential role for GLP-2 therapy in pediatric SBS.

Short bowel syndrome (SBS) is characterized by a significant reduction in the effective intestinal surface by an anatomical or functional loss of the small intestine. It mainly occurs after extensive bowel resection, intestinal intrinsic disease or surgical bypass. The main complications are malabsorption, maldigestion, malnutrition, dehydratation and, potentially, lethal metabolic lesions. The treatment is based on appropiate, individualized nutritional support; however, the most recent outcomes on bowel transplantation (BT) and a great rate of survivors achieving complete digestive autonomy and able to carry out activities according to their age allow for considering BT as the first choice therapy in patients with irreversible intestinal failure in whom poor prognosis with parenteral nutrition is foreseen. In this paper the most outstanding aspects of SBS are revised., (Copyright AULA MEDICA EDICIONES 2014. Published by AULA MEDICA. All rights reserved.)

Aim: To study the effects of mesenchymal stem cell (MSC) therapy on the prevention of acute rejection and graft vs host disease following small bowel transplantation., Methods: In our transplantation center, 6 isolated intestinal transplants have been performed with MSC therapy since 2009. The primary reasons for transplants were short gut syndrome caused by surgical intestine resection for superior mesenteric artery thrombosis (n = 4), Crohn's disease (n = 1) and intestinal aganglionosis (n = 1). Two of the patients were children. At the time of reperfusion, the first dose of MSCs cultured from the patient's bone marrow was passed into the transplanted intestinal artery at a dose of 1000000 cells/kg. The second and third doses of MSCs were given directly into the mesenteric artery through the arterial anastomosis using an angiography catheter on day 15 and 30 post-transplant., Results: The median follow-up for these patients was 10.6 mo (min: 2 mo-max: 30 mo). Three of the patients developed severe acute rejection. One of these patients did not respond to bolus steroid therapy. Although the other two patients did respond to anti-rejection treatment, they developed severe fungal and bacterial infections. All of these patients died in the 2(nd) and 3(rd) months post-transplant due to sepsis. The remaining patients who did not have acute rejection had good quality of life with no complications observed during the follow-up period. In addition, their intestinal grafts were functioning properly in the 13(th), 25(th) and 30(th) month post-transplant. The patients who survived did not encounter any problems related to MSC transplantation., Conclusion: Although this is a small case series and not a randomized study, it is our opinion that small bowel transplantation is an effective treatment for intestinal failure, and MSC therapy may help to prevent acute rejection and graft vs host disease following intestinal transplantation.

Background/objectives: Chronic radiation enteritis (RE) has been reported in up to 20% of patients receiving pelvic radiotherapy and can lead to intestinal failure (IF), accounting for 3.9% of new registrants for home parenteral nutrition (HPN) in the UK annually. Our aim is to report nutritional and survival outcomes for patients with RE referred to a national IF unit., Subjects/methods: A retrospective study of all new admissions over a 13-year period at the Intestinal Failure Centre, Manchester, UK. Data are presented as median (range)., Results: Twenty-three (3.8%) of 611 patients were admitted with IF secondary to RE. The primary site of malignancy was genitourinary in 17 (74%) patients. Radiotherapy was administered 9.5 (1-42) years previously. Patients underwent 2 (1-5) laparotomies prior to intestinal failure unit (IFU) admission. Twelve (52%) patients were admitted with intestinal obstruction and 11 (48%) with intractable weight loss and/or high output fistulae/stomas. Additional conditions contributing to IF were noted in 11 (48%) patients. Twenty-two (96%) patients had 2 (1-5) laparotomies prior to IFU referral. At discharge, 5 (22%) patients resumed oral diet without the need for artificial nutrition support, 3 (13%) required enteral feeding and 13 (56%) commenced HPN. The 10-year survival of the patient cohort was 48.2%., Conclusions: Surgical intervention is infrequently required, whereas the majority of patients with IF secondary to RE require long-term HPN. The judicious use of surgery in selected patients, coupled with an aggressive medical strategy to detect and treat contributing factors, and optimal enteral feeding may allow a modest proportion of patients with IF secondary to RE to achieve independence from PN.

Advances in the medical and surgical treatments of intestinal failure have led to a decrease in the number of transplants over the past decade. In 2012, 152 candidates were added to the intestinal transplant waiting list, a new low. Of these, 64 were listed for intestine-liver transplant and 88 for intestinal transplant alone or with an organ other than liver. Historically, the most common organ transplanted with the intestine was the liver; this practice decreased substantially from a peak of 52.9% in 2007 to 30.0% in 2012. Short-gut syndrome, which encompasses a large group of diagnoses, is the most common etiology of intestinal failure. The pretransplant mortality rate decreased dramatically over time for all age groups, from 51.0 per 100 wait-list years in 1998-1999 to 6.7 for patients listed in 2010-2012. Numbers of intestinal and intestine-liver transplants steadily decreased from 198 in 2007 to 106 in 2012. By age, intestinal transplant recipients have changed substantially; the number of adult recipients now approximately equals the number of pediatric recipients. Graft survival has improved over the past decade. Graft failure in the first 90 days after transplant occurred in 15.7% of 2011-2012 intestinal transplant recipients, compared with 21% in 2001-2002., (© Copyright 2013 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Objectives: Intestinal atresia is a common cause of intestinal obstruction in neonates. The predominant cause of late complications in these children is prolonged dependence on parenteral nutrition (PN). Our aims were to analyze the outcomes for patients with intestinal atresia at our institution, and to see how these changed with the implementation of an intestinal rehabilitation program (IRP)., Methods: This is a retrospective cohort study. The patient population is all children with intestinal atresias (118 patients) treated at our institution from July 2000 to June 2010, 20 of whom became PN dependent., Results: Survival to hospital discharge was 95% for all patients, and 100% for those who had isolated atresia. Twenty of 118 patients (17%) were PN dependent beyond initial hospital discharge. At discharge, their median intestinal length was 22.5 cm, and they required PN for a mean of 88.5% of energy needs. Of these 20 patients, 2 died, 2 received transplants, and 2 transferred away for transplantation. The remaining 14 joined the IRP. Their mean energy requirement from PN is presently 10%, down from 87% at IRP enrollment, and 10 patients fully weaned off PN. Eleven of the 14 children had hyperbilirubinemia, with mean direct bilirubin of 7.5 mg/dL. All resolved their cholestasis during an average of 12 weeks., Conclusions: These results compare favorably with those reported in earlier periods. With programs such as the IRP, patients with short bowel secondary to intestinal atresia can show improvement in liver function and nutritional parameters, and discontinue PN, avoiding the need for transplantation.

Kidney transplantation is the treatment of choice for end-stage renal disease whereas indications for intestinal transplantation are currently restricted to patients with irreversible small bowel failure and severe complications of total parenteral nutrition (mostly shortage and infection of venous accesses, major electrolyte disturbances and liver failure). Enteric hyperoxaluria is secondary to certain intestinal diseases like intestinal resections, chronic inflammatory bowel disease and other malabsorption syndromes and can lead to end-stage renal disease requiring kidney transplantation. We report two patients suffering from renal failure due to enteric hyperoxaluria (secondary to extensive intestinal resection) in whom we elected to replace not only the kidney but also the intestine to prevent recurrence of hyperoxaluria in the transplanted kidney., (© Copyright 2013 The American Society of Transplantation and the American Society of Transplant Surgeons.)