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The management of patients with intestinal failure due to short bowel syndrome is complex, requiring a comprehensive approach that frequently necessitates long-term, if not life-long, use of parenteral nutrition. Despite tremendous advances in the provision of parenteral nutrition over the past three decades, which have allowed significant improvements in the survival and quality of life of these patients, this mode of nutritional support carries with it significant risks to the patient, is very costly, and ultimately, does not attempt to improve the function of the remaining bowel. Intestinal rehabilitation refers to the process of restoring enteral autonomy, and thus, allowing freedom from parenteral nutrition, usually by means of dietary, medical, and occasionally, surgical strategies. While recent investigations have focused on the use of trophic substances to increase the absorptive function of the remaining gut, whether intestinal rehabilitation occurs as a consequence of enhanced bowel adaptation or is simply a result of an optimized, comprehensive approach to the care of these patients remains unclear. In Part 1 of this review, we provided an overview of short bowel syndrome and pathophysiological considerations related to the remaining bowel anatomy in these patients. We also reviewed intestinal adaptation and factors that may enhance the adaptive process, focusing on evidence derived from animal studies. In Part 2, relevant data on the development of intestinal adaptation in humans are reviewed as is the general management of short bowel syndrome. Lastly, the potential benefits of a multidisciplinary intestinal rehabilitation program in the care of these patients are also discussed.

Background/Objectives: Home parenteral support (HPS) is the core of chronic intestinal failure (IF) treatment. For legal reasons, HPS in Portugal lags behind other European countries, and only a few patients were taken care of at home by nurses. Now, the legislation has changed, allowing patient self-care. The authors report their pioneer experience as the largest Portuguese IF center, evaluating the underlying conditions leading to IF, HPS nutritional impact, HPS-related complications and survival. Methods: This is a retrospective study including IF patients who underwent HPS in a Portuguese IF center. The data included demographics, underlying conditions, IF types, HPS duration, BMI at the beginning and end of HPS/follow-up, complications, microbiological agents of infectious complications and current status (deceased or alive with/without HPS). Survival was calculated until death or September 2024. Results: A total of 23 patients (52.2% female, mean age 57.3 years), all with type III IF, were included. Short bowel syndrome (SBS) was the most common cause of IF (69.6%). Of the included patients, 78.3% received home parenteral nutrition; the others received home parenteral hydration. The mean BMI increased significantly, from 19.1 kg/m2 to 22.5 kg/m2 (p < 0.001). Two patients received Teduglutide. The most common complication was catheter-related bloodstream infection (2.5/1000 catheter days). The complications did not increase with patient self-care. At the end of follow-up, 21.7% of patients remained on HPS, 34.8% were alive without HPS, and 43.5% died. The average survival was 43.4 months. One death (4.35%) was attributable to HPS-related complications. Conclusions: The conditions underlying IF varied, with SBS being the most frequent condition. HPS improved the BMI, allowing considerable survival. Despite the complications and one attributable death, HPS was safe, even when relying on patient self-care.

Background: It has been thirty years since Bianchi introduced the technique that made intestinal lengthening possible. The last three decades have seen lengthening procedures established as vital components of intestinal rehabilitation programs. The goal of the present study was to use a systematic literature review to determine patient outcomes for the two most commonly used lengthening procedures, the Bianchi procedure and the serial transverse enteroplasty procedure (STEP). Methods: Pubmed and Embase were searched using the terms “intestinal lengthening” and “bowel lengthening.” Patient outcomes were extracted from each relevant journal article on the basis of a set proforma. The results were combined to create overall mean outcomes. Mean outcomes were also calculated separately for the Bianchi procedure and STEP. Significance was tested with the independent t -test. Results: Overall survival for the last thirty years is 83 %. However, survival for the last fifteen years has been 89 %, with no significant difference between the two procedures. The Bianchi procedure has a higher rate of weaning patients who were static on parenteral nutrition with conservative measures: 55 % versus 48 %. In addition, the Bianchi procedure was associated with a higher rate of patients receiving transplants: 10 % versus 6 %. The STEP has a higher rate of complication. Length of follow-up is significantly longer for the Bianchi procedure: 76 versus 22 months. The impact that this differential could have had on our results must be considered. Conclusions: Outcomes for intestinal lengthening procedures are very good, and increasingly so. However, further analysis is required in order to fully understand the relative strengths and weaknesses of each procedure.

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.

Chronic intestinal pseudo-obstruction (CIPO) is a rare, disabling disorder responsible for motility-related intestinal failure. Because it induces malnutrition, CIPO is a significant indication for home parenteral nutrition (HPN). The objective of the study was to evaluate long-term outcome of CIPO patients requiring HPN during adulthood. In total, 51 adult CIPO patients (18 men/33 women, median age at symptom occurrence 20 (0-74) years, 34/17 primary/secondary CIPO) followed up at our institution for HPN management between 1980 and 2006 were retrospectively studied for survival and HPN dependence rates using univariate and multivariate analysis. Follow-up after diagnosis was 8.3 (0-29) years. Surgery was required in 84% of patients. The number of interventions was 3 +/- 3 per patient (mean +/- s.d.), leading to short bowel syndrome in 19 (37%) patients. Actuarial survival probability was 94, 78, 75, and 68% at 1, 5, 10, and 15 years, respectively. Multivariate analysis showed that lower mortality was associated with the ability to restore oral feeding at baseline (hazard ratio (HR) = 0.2 (0.06-0.65), P = 0.008) and symptom occurrence before the age of 20 years (HR=0.18 (0.04-0.88), P = 0.03). Higher mortality was associated with systemic sclerosis (HR=10.4 (1.6-67.9), P = 0.01). Actuarial HPN dependence was 94, 75, and 72% at 1, 2, and 5 years, respectively. In this large cohort of CIPO adult patients with severe intestinal failure, i.e., those requiring HPN, we found a higher survival probability than previously reported. These results should be taken into account when considering intestinal transplantation.

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.

The aim of this study was to evaluate the clinical experience of a regional multidisciplinary intestinal failure program for children established in 2005. Data were collected from a prospective internal database. Univariate analyses were performed to compare pre- and post-treatment outcomes. Median values are reported. Forty-nine children were referred at an age of 7 months. Remnant small bowel length was 29 cm. With follow-up of 14 months, overall patient survival was 88%. Thirteen bowel-lengthening procedures were performed, thereby increasing small bowel length from 83 to 132 cm ( P < .05). Enteral autonomy was achieved in 22 patients (45%), and the caloric requirement for parenteral nutrition was decreased from 100% to 41% ( P < .01). Conjugated bilirubin was reduced from 4.1 to 0 mg/dL ( P < .05). A multidisciplinary approach to pediatric intestinal failure that prioritizes intestinal rehabilitation can achieve successful enteral feeding advancement, improved liver function, and excellent survival in intermediate-range follow-up.

The nucleotide-binding oligomerization protein 2 (NOD2) gene single nucleotide polymorphisms (SNPs) associated with Crohn's disease were recently associated with severe rejection after small-bowel transplantation (SBTx). The purpose of this study was to re-test this association and explore whether deficient innate immunity suggested by the NOD2 SNPs predisposes to intestine failure requiring isolated SBTx or combined liver-intestine failure requiring combined liver-SBTx (LSBTx). Archived DNA from 85 children with primary isolated SBTx or LSBTx was genotyped with Taqman biallelic discrimination assays. To minimize confounding effects of racial differences in minor allele frequencies (MAFs), allelic associations were tested in 60 Caucasian recipients (discovery cohort). Replication was sought in an independent cohort of 39 Caucasian pediatric and adult SBTx patients. MAF for rs2066845 and rs2066847 was similar to that seen in 538 healthy North American Caucasians. In the discovery cohort, MAF for rs2066844 was significantly higher in LSBTx (13.5 vs. 3.6%, P=0.0007, Fisher's exact test), but not in isolated SBTx recipients (2.2 vs. 3.6%, P=NS), when compared with 538 healthy Caucasians. In addition, among LSBTx recipients who received identical immunosuppression, the minor allele of rs2066844 associated with early rejection in linear regression analysis (P=0.028) (all but one of the risk alleles were found in rejectors), decreased survival (P=0.015, log-rank, Kaplan-Meier analysis), and a 20-fold greater hazard of septic death in proportional hazard analysis (P=0.030). Steroid-resistant (severe) rejection and graft loss were associated with isolated SBTx (P=0.036 and 0.082, respectively), but not with NOD2 SNPs. The association between rs2066844 and combined liver-intestine failure requiring LSBTx was significant in the replication cohort (P=0.014), and achieved greater significance in the combined cohort (P=0.00006). The NOD2 SNP rs2066844 associates with combined liver and intestinal failure in subjects with short-gut syndrome, who require combined liver-intestine transplantation, and secondarily with early rejection and septic deaths.

ObjectiveSalvage surgery for patients with highly advanced or relapsed epithelial ovarian cancer (EOC) complicated by bowel obstruction and resulting in short bowel syndrome (SBS) constitutes a therapeutic dilemma. Our aim was to evaluate surgical and clinical outcome in these highly palliative situations.MethodsWe evaluated all patients with EOC who underwent salvage extraperitoneal en bloc intestinal resection with terminal ileostomy or jejunostomy resulting in SBS and total parenteral nutrition owing to bowel obstruction between May 2003 and January 2012 in our institution.ResultsThirty-seven patients were identified (median age, 58 years; range, 22–71 years), 3 (8.1%) with primary and 34 (91.6%) with relapsed EOC. Five patients (13.5%) were platinum sensitive. Median residual intestinal length was 70 cm (range, 10–180 cm); 21 patients (56.8%) had a residual intestinal length less than 1 m. Operative 30-day mortality and major morbidity rates were 10% and 51%, respectively. Median overall survival was 5.6 months (range, 0.1–49 months). One-year and 2-year overall survival rates were 18.3% (95% confidence interval, 5.1%–31.5%) and 8.1% (95% confidence interval, 0%–18.0)%, respectively. Within a median follow-up period of 5 months (range, 0.2–49 months), 4 patients (10.8%) are still alive. No significant differences in survival were seen between patients with or without major complications, tumor residuals, or residual intestinal length of less than 1 m versus greater than 1 m.ConclusionsSalvage palliative surgery in EOC due to bowel obstruction resulting in SBS and in need of long-life total parenteral nutrition is associated with high morbidity rates and low overall survival. These surgeries should ideally be performed only in a multidisciplinary setting with adequate infrastructure and possibility of home care support. Conservative management should be the route of action in the absence of acute abdomen or intestinal perforation.

Background Short bowel syndrome is a multisystemic disorder that results from the loss of a significant amount of small bowel. The goal of treatment in these patients is to achieve complete enteral autonomy while minimizing complications. Our unit has 30 years of experience in the management of short gut patients. During the past decade, our results have improved significantly, especially in children with severe short bowel syndrome. This brief communication looks at the algorithm presently used in our unit. Methods In this communication, the principles in management of short bowel syndrome in our unit are discussed. In addition, our algorithm is published for the first time. A brief summary of our results is provided. Results Twenty-seven children were enrolled from 2000 to 2009. In this cohort, two patients died because of significant liver disease: one after having two liver and bowel transplants. Overall, survival stands at 92%. All had autologous gastrointestinal reconstruction, and 19 patients underwent bowel lengthening (longitudinal intestinal lengthening and tailoring). The median residual length of bowel of this subgroup at first operation was 25 cm in those who had their gut measured. Two patients were lost to follow-up. Two patients remain on supplemental total parenteral nutrition (TPN), with an overall 91% of surviving patients off TPN at a median of 6 months after reconstruction. Conclusions We believe this improvement is related to the development—over many years—of a structured pathway for managing these patients.