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Short bowel syndrome (SBS) is a rare gastrointestinal disorder associated with intestinal failure (SBS-IF) and poor health-related outcomes. Patients with SBS-IF are unable to absorb sufficient nutrients or fluids to maintain significantly metabolic homeostasis via oral or enteral intake alone and require long-term intravenous supplementation (IVS), consisting of partial or total parenteral nutrition, fluids, electrolytes, or a combination of these. The goal of medical and surgical treatment for patients with SBS-IF is to maximize intestinal remnant absorptive capacity so that the need for IVS support may eventually be reduced or eliminated. Daily subcutaneous administration of the glucagon-like peptide 2 analog, teduglutide, has been shown to be clinically effective in reducing IVS dependence and potentially improving the health-related quality of life of patients with SBS-IF. The management of patients with SBS-IF is complex and requires close monitoring. This narrative review discusses the use of teduglutide for patients with SBS-IF in clinical practice. The screening of patient eligibility for teduglutide treatment, initiation, monitoring of efficacy and safety of treatment, adapting or weaning off IVS, and the healthcare setting needed for SBS-IF management are described, taking into consideration data from clinical trials, observational studies, and clinical experience., (© 2023 The Authors. Nutrition in Clinical Practice published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

The term "short bowel syndrome (SBS)" defines "the clinical feature associated with a remaining small bowel in continuity of less than 200 cm from the ligament of Treitz" and is characterized by malabsorption, diarrhea, fatty stools, malnutrition, and dehydration. SBS is the primary pathophysiological mechanism of chronic intestinal failure (CIF), 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 (IVS) is required to maintain health and/or growth" in a metabolically stable patient. By contrast, the reduction of gut absorptive function that does not require IVS has been termed "intestinal insufficiency or deficiency" (II/ID). The classification of SBS can be categorized as follows: anatomical (anatomy and length of the residual bowel), evolutional (early, rehabilitative, and maintenance phases), pathophysiological (SBS with or without a colon in continuity), clinical (with II/ID or CIF), and severity of CIF (type and volume of the required IVS). Appropriate and homogeneous patient categorization is the mainstay of facilitating communication in clinical practice and in research., (© 2023 American Society for Parenteral and Enteral Nutrition.)

Background: A fungal-related catheter-related bloodstream infection (CRBSI) is less frequent than those induced by bacteria. In the past, a single episode of fungal CRBSI has been used as a marker of home parenteral nutrition (HPN) failure and thus a possible indication for intestinal transplantation., Methods: Survival outcomes were assessed from a prospectively maintained database of patients initiated on HPN for underlying chronic intestinal failure between 1993 and 2018, with a censoring date of December 31, 2020. Cox regression was performed to assess predictors of mortality with univariable and multivariable analysis., Results: A total of 1008 patients were included in the study, with a total of 1 364 595 catheter days. There were 513 CRBSI events recorded in 262 patients, equating to a CRBSI rate of 0.38/1000 catheter days. A total of 38/262 (14.5%) patients had at least one episode of fungal CRBSI, whereas 216/262 (82.4%) had at least one bacterial but no fungal CRBSI. The median time between HPN initiation and the first CRBSI episode was 20.6 months (95% confidence interval, 16.5-24.1). Episodes of fungal or bacterial CRBSI and the number of CRBSI episodes were not associated with increased mortality. Overall, 15 CRBSI-related deaths were observed in the observation period (0.01 CRBSI deaths/1000 catheter days), two of these were fungal in origin., Conclusion: The occurrence of a fungal CRBSI does not increase the risk of death compared with patients who have bacterial CRBSI or those without a CRBSI event., (© 2022 American Society for Parenteral and Enteral Nutrition.)

Background: Home parenteral nutrition (HPN) is a life-saving therapy for patients with chronic intestinal failure but can be associated with a degree of psychological distress. The factors associated with the need for antidepressants (ADs) in this cohort have not yet been described., Methods: The study involved prospective data collection from patients attending an HPN clinic at a national intestinal failure referral center. Patients requiring HPN as a result of active malignancy were excluded. Patients were divided in 2 groups according to AD usage; demographic, anthropometric, socioeconomic characteristics, and intravenous supplementation (IVS) regimens were compared between groups., Results: A total of 184 patients were recruited between July 2018 and April 2019, with an overall prevalence of AD use of 41.7% (70/168 patients). Daily mean IVS volume was significantly higher among patients taking AD ("AD" group; 2125.48 ± 991.8 ml/day, "no-AD" group; 1828.54 ± 847.0 ml/day, P = .039), with the proportion of patients needing high-volume IVS (≥3000 ml/day) being 3 times higher in the AD group (20.0%(14/70 patients) vs 6.1% (6/98 patients), P = .006). The average energy IVS infusion per day was similar between the groups., Conclusion: This is the first study to demonstrate that AD use correlates with higher IVS volume rather than energy requirements in HPN patients, suggesting that high IVS volume requirements may be better associated with the patient's disease burden. Early and tailored mental health intervention may be beneficial in those with high IVS volume requirements., (© 2021 American Society for Parenteral and Enteral Nutrition.)

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive disease caused by TYMP mutations and thymidine phosphorylase (TP) deficiency. Thymidine and deoxyuridine accumulate impairing the mitochondrial DNA maintenance and integrity. Clinically, patients show severe and progressive gastrointestinal and neurological manifestations. The onset typically occurs in the second decade of life and mean age at death is 37 years. Signs and symptoms of MNGIE are heterogeneous and confirmatory diagnostic tests are not routinely performed by most laboratories, accounting for common misdiagnosis. Factors predictive of progression and appropriate tests for monitoring are still undefined. Several treatment options showed promising results in restoring the biochemical imbalance of MNGIE. The lack of controlled studies with appropriate follow-up accounts for the limited evidence informing diagnostic and therapeutic choices. The International Consensus Conference (ICC) on MNGIE, held in Bologna, Italy, on 30 March to 31 March 2019, aimed at an evidence-based consensus on diagnosis, prognosis, and treatment of MNGIE among experts, patients, caregivers and other stakeholders involved in caring the condition. The conference was conducted according to the National Institute of Health Consensus Conference methodology. A consensus development panel formulated a set of statements and proposed a research agenda. Specifically, the ICC produced recommendations on: (a) diagnostic pathway; (b) prognosis and the main predictors of disease progression; (c) efficacy and safety of treatments; and (f) research priorities on diagnosis, prognosis, and treatment. The Bologna ICC on diagnosis, management and treatment of MNGIE provided evidence-based guidance for clinicians incorporating patients' values and preferences., (© 2020 SSIEM.)

Background: This survey of centers caring for patients receiving home parenteral nutrition (HPN) was conducted to assess the impact of the coronavirus disease 2019 (COVID-19) crisis on the management of these patients regarding provision of care, monitoring, regular follow-up, and any changes to service infrastructure., Methods: A survey was devised and publically published on the Research Electronic Data Capture database management system, with individual centers responding to a public link., Results: A total of 78 adult and pediatric centers worldwide contributed to the survey, representing ≥3500 patients' experiences. Centers reported infrastructure maintenance for Parenteral Nutrition (PN) bag deliveries to patients (60, 76.92%) or delivery of ancillary items (57, 73.08%), home delivery and HPN administration (65, 83.33%), and home care nurse shortages (25, 32.05%). Routine follow-up of HPN patients changed to either all telemed or mixed with emergency clinic review (70, 89.74%). In 26 centers (33.33%), HPN for newly discharged patients with benign conditions was reduced or stopped. Based on clinical history, the centers reported psychological distress for patients (52, 66.67%), with anxiety, worry, concern, and apprehension reported most frequently (37 of 52, 71.15%) but also fear (10 of 52, 19.23%), depression (5 of 52, 9.62%), and issues related to isolation/confinement (12 of 52, 23.08%)., Conclusions: The COVID-19 pandemic was reported by clinicians to have had a far-reaching adverse impact on patients receiving HPN, especially their safety in terms of provision of personal protective equipment, PN bags, available nursing staff, and psychological well-being. Healthcare systems responded to the challenge and presented new ways of working., (© 2020 American Society for Parenteral and Enteral Nutrition.)

Chronic intestinal failure (CIF) due to short-bowel syndrome (SBS) is characterized by failure to achieve optimal intestinal adaptation required to maintain oral/enteral autonomy. The conventional management strategy relies heavily on home parenteral support (PS; parenteral nutrition and/or intravenous fluids). Teduglutide, an analog of the hormone glucagon-like peptide-2, facilitates intestinal adaptation, as evidenced by reductions in PS volume in patients with SBS-associated CIF. In 2016, the European Society for Clinical Nutrition and Metabolism (ESPEN) developed guidelines for the management of adult patients with CIF, consisting of a comprehensive list of recommendations. Owing to the limited number of studies at the time of the finalization of the GRADE-method review of the available literature, teduglutide received a moderate grade of evidence (GOE) as the first choice for growth-factor treatment in patients with SBS-CIF. The GOE was also low for 7 points of recommended information to be discussed with the candidate patients. This review summarizes findings from recent studies that fill some gaps identified in the 2016 ESPEN guidelines regarding the use of teduglutide in the management of SBS-CIF. Collectively, these studies provide useful information about the probability and timing of clinical response in the individual patient. Also, recent studies report longer-term safety findings with teduglutide. These results can help physicians better manage patients with SBS-CIF by aligning clinical decision making with specific disease characteristics, setting the right expectations, and encouraging treatment adherence., (© 2019 American Society for Parenteral and Enteral Nutrition.)

Background and Aims: Patients receiving home parenteral nutrition (HPN) deserve a high-quality and patient-centered care. Patient-centered care can be delivered only if the patient's priorities and concerns are known. Therefore, the aim is to identify the top 3 most important outcome indicators according to patients' perspectives and the differences between several centers, HPN regimen, and HPN experience., Methods: A questionnaire, based on previously developed outcome indicators, was translated into the mother tongue using forward-backward translation and distributed to adult HPN patients with benign disease in March 2013. To identify differences, a Kruskal-Wallis or Mann-Whitney test was performed with GraphPad Prism (significance level <.05) when applicable., Results: Nine centers over 8 countries (300 patients) participated. The top 3 outcome indicators for patients were (1) incidence of catheter-related infection (CRI), (2) survival, and (3) quality of life (QoL). Between the participating centers, significant differences on rating were found for 5 outcome indicators (catheter obstruction, .015; weight, .002; energy, .010; fear, <.001; and independence, .010). The independence outcome indicator (.050) was considered less important for experienced (>2 years HPN) vs less experienced patients. For this outcome indicator, patients' view also differed significantly based on number of HPN days per week (.0103)., Conclusion: A cohort of HPN patients identified incidence of CRI, survival, and QoL as the most important outcome indicators for their care; however, there were significant differences between the participating centers. For one outcome indicator (independence), there were significant differences based on experience and regimen., (© 2014 American Society for Parenteral and Enteral Nutrition.)

Background: Long-term home parenteral nutrition (HPN) may induce liver disorders. Transient elastography (TE) has been proposed as a noninvasive alternative to liver biopsy analysis for assessment of the progression of hepatic fibrosis to cirrhosis. The goal of this study was to compare values from TE measurements to biopsy-determined stages of histologic fibrosis in patients receiving HPN., Methods: In this multicenter prospective study, patients receiving long-term HPN (≥ 6 months) who required a liver biopsy for clinical reasons were included. TE (FibroScan) values for each patient were compared with the degree of hepatic fibrosis measured from biopsy specimens based on the Brunt classification. TE values were also correlated to biochemical and histologic cholestasis. Two noninvasive indices for predicting liver fibrosis (APRI and FIB-4) were also evaluated., Results: Thirty patients were included in this study (mean age, 42.1 years; 63% male). The mean duration of HPN was 100.7 months; 25 patients had a short bowel and 13 had an intestinal stoma. Biochemical cholestasis was described in 22 patients. Liver histologic features varied among these patients. There was no correlation between the values of TE and the stages of histologic fibrosis, but TE values were significantly correlated to serum bilirubin level and the severity of histologic cholestasis as well as APRI and FIB-4 scores., Conclusions: In patients with long-term HPN, TE failed to assess the degree of hepatic fibrosis. This could be due to the heterogeneity of liver histologic features observed in these patients and the presence of chronic cholestasis., (© 2014 American Society for Parenteral and Enteral Nutrition.)

The morphologic and cytoproliferative patterns of the duodenal mucosa of two adult patients, one of whom had a short bowel, were evaluated after more than 2 months of postoperative total parenteral nutrition and 2 and 12 months after the resumption of oral alimentation. Morphometric analysis was performed on routinely processed duodenal biopsies. Cell proliferation was evaluated by means of in vitro bromodeoxyuridine uptake. The results were compared with those obtained in five healthy controls. After parenteral nutrition, patients showed significantly lower villus height and crypt depth than those of controls and a normal bromodeoxyuridine labeling index. After 2 months of refeeding, villus and crypt returned to normal, and the labeling index was increased. After 12 months of oral refeeding, labeling index, villus height, and crypt depth were similar to those of controls. The patient with the short bowel showed a number of cells per unit length of villus and crypt significantly greater than those of the controls and of the patient who underwent shorter intestinal resection. In human duodenal mucosa, (1) hypoplasia develops after long-term total parenteral nutrition; (2) mucosal recovery occurs through an increased cell proliferation after oral refeeding; and (3) extensive small bowel resection determines the development of relative hyperplasia.

To establish the effect of chemically defined formula diets on pancreatic mass, 58 male Wistar rats, weighing approximately 200 g, were fed orally one of the following diets for 20 days: standard diet; partially hydrolyzed diet; elemental diet. The diets were isocaloric and provided 73 cal/day per rat. At the end of the experiment, the rats were killed and pancreas removed to assess wet weight and DNA and RNA content. Compared to the controls, the elemental diet caused a reduction in pancreatic wet weight (p less than 0.005), pancreatic DNA (p less than 0.001), and RNA content (p less than 0.001). In the group fed partially hydrolyzed diet, only pancreatic DNA content showed an highly significant reduction when compared with the reference group (p less than 0.001), whereas the value of RNA failed to reach the statistical significance. We found an increase of the ratio mg RNA/mg DNA in the partially hydrolyzed and elemental diet groups. These results suggest that long-term nutrition with the partially hydrolyzed diet, and more strongly with the elemental diet, may induce pancreatic hypoplasia.

Malnutrition in gastrointestinal disease can result from several pathogenetic mechanisms. The resulting clinical and laboratory features of malnutrition vary according to the specific pathogenetic factors involved. Many of the causes of malnutrition in gastroenterology are similar to those found in other acute and chronic diseases. However, certain disorders, specific to gastroenterology, may alter nutritional indices independent of the usual causes of malnutrition. These include small bowel bacterial overgrowth and protein-losing enteropathy. The impact of these disorders on the indexes of nutritional status is discussed.