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Background: Given the growing use of home enteral nutrition (HEN), assessing the experience of consumers and caregivers is crucial to understanding the real-world subjective and objective challenges of administering HEN., Methods: After obtaining institutional review board approval, a survey was distributed to HEN consumers and caregivers between January 16, 2020, and July 16, 2021. Data collected included information regarding demographics, primary diagnosis, tube and connectors, HEN regimen, and overall HEN experience., Results: A total of 884 individuals responded to the survey: 673 (76.1%) responses by caregivers and 211 (23.9%) responses by patients. The study cohort included 566 (64%) children and 318 (36%) adults. The leading primary diagnosis of participants was developmental delay and motility disorder for children and adults, respectively. Low-profile gastric tubes were the most used (75.7% of children and 30.3% of adults). Notably, legacy connectors were utilized for more patients (46.7% children, 52.6% adults) compared to ISO-80369-3 connectors (38.9% children, 29.7% adults). HEN complications were prevalent, including enteral tube site infections and other tube-related complications, including clogging and kinking., Conclusion: This real-world data reveals that HEN complications remain prevalent. Additionally, despite introducing ISO-80369-3 connectors many years ago, most patients continue to use legacy tubes with a significant lack of knowledge about ISO-80369-3 connectors. The survey results guide HEN providers to focus on several areas to reduce complications., Competing Interests: Conflicts of interest Authors declare no relevant conflict of interest., (Copyright © 2024 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

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

Objective: This study is an assessment of home parenteral nutrition service performance and safety and efficacy outcomes in patients with benign chronic intestinal failure., Methods: This is a retrospective, non-interventional, and multicenter study. Data were collected by trained nurses and recorded in a dedicated registry (SERECARE)., Results: From January 1, 2013 to June 30, 2018, data from a total of 683 patients with benign chronic intestinal failure were entered in the registry. Patients included 208 pediatric (53.8% male; median age = 4.0 y) and 475 adult (47.6% male; median age = 59.0 y) participants. On average, patients were visited 5.4 ± 4.5 times and received 1.4 ± 0.8 training sessions. Retraining was not common and mostly due to change of therapy or change of caregiver. Of 939 complications, 40.9% were related to the central venous catheter and were mostly infectious (n = 182) and mechanical (n = 187). The rate of infectious and mechanical complications per 1000 catheter days decreased over 5 y (0.30-0.15 and 0.33 -0.19, respectively). The rate of complications per 1000 catheter days and the mean complications per patient were higher in pediatric than in adult patients. The hospitalization rate was 1.01 per patient throughout the study period. These data were similar to those registered in a previous study period (2002-2011) (n = 1.53 per patient). Changes over time in the efficacy variables were mostly small and non-significant., Conclusions: This study confirms the importance of setting up and maintaining structured registries to monitor and improve home parenteral nutrition care. Safety outcomes have improved over the years, most likely due to the underlying efficient nursing service., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: F. W. G.: personal fees from Takeda and Baxter, during the conduct of the study; A. D.: none; P. G.: grants from Baxter, outside the submitted work; U. A.: none; S. A.: none; T. C.: none; D. E.: none; M. G.: none; A. L.: personal fees from Nutricia, Nestlé, and Takeda, outside the submitted work; A. M.: none; S. M.: none; F. D. M.: none; N. R.: none; A. S. S: none; L. P.: grants from Baxter, B. Braun, Fresenius Kabi, and Takeda, outside the submitted work; and A. D. F.: personal fees from Baxter, during the conduct of the study, and personal fees from Fresenius Kabi, outside the submitted work., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Trace elements and vitamins, named together micronutrients (MNs), are essential for human metabolism. The importance of MNs in common pathologies is recognized by recent research, with deficiencies significantly impacting the outcome., Objective: This short version of the guideline aims to provide practical recommendations for clinical practice., Methods: An extensive search of the literature was conducted in the databases Medline, PubMed, Cochrane, Google Scholar, and CINAHL for the initial guideline. The search focused on physiological data, historical evidence (for papers published before PubMed release in 1996), and observational and/or randomized trials. For each MN, the main functions, optimal analytical methods, impact of inflammation, potential toxicity, and provision during enteral or parenteral nutrition were addressed. The SOP wording was applied for strength of recommendations., Results: The limited number of interventional trials prevented meta-analysis and led to a low level of evidence for most recommendations. The recommendations underwent a consensus process, which resulted in a percentage of agreement (%): strong consensus required of >90 % of votes. Altogether the guideline proposes 3 general recommendations and specific recommendations for the 26 MNs. Monitoring and management strategies are proposed., Conclusion: This short version of the MN guideline should facilitate handling of the MNs in at-risk diseases, whilst offering practical advice on MN provision and monitoring during nutritional support., Competing Interests: Conflict of interest All the authors declares no conflicts of interest., (Copyright © 2024 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

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.)

Purpose: Very few data exist on the association between metabolic dysfunction-associated steatotic liver disease (MASLD) and eating disorders. The study aimed to evaluate the presence of binge eating disorder (BED), in MASLD subjects., Methods: Demographic, clinical investigation, anthropometric measurements and laboratory were collected in 129 patients with MASLD (34.1% males; age, 53.7 years; BMI, 34.4 kg/m 2 ) addressed by general practitioners to a hospital-based unit of metabolic disorders. The risk of binge eating was tested by the binge eating scale (BES); values in the range 17-26 were considered "possible" BED, values > 26 were considered "probable" BED. Hepatic steatosis and fibrosis were tested by surrogate biomarkers and imaging (transient elastography). Calorie intake and lifestyle were self-assessed by questionnaires., Results: Possible BED was present in 17.8% of cases, probable BED in another 7.6%, and were neither associated with gender, obesity class, diabetes, features of metabolic syndrome, nor with presence and severity of hepatic steatosis and fibrosis. Also steatosis grade by CAP and fibrosis stage by liver stiffness did not correlate with BES. However, an association was present between the daily caloric intake and "possible" BED (odds ratio, 1.14; 95% confidence interval, 1.05-1.24; "probable" BED, 1.21; 1.07-1.37), after adjustment for confounders., Conclusion: Binge eating, as scored by BES, is present in a significant proportion of MASLD cases screened for metabolic disorders in a specialized center. It may impact behavioral treatment, reducing the chance of weight loss without systematic psychological support., Level of Evidence: Level III, cohort analytic study., (© 2023. The Author(s).)

Background: The benefits of immunonutrition in patients who underwent major abdominal surgery have been recently established, but the optimal combination of immunonutrients has remained unclear. The aim is to clarify this point., Methods: A systematic search of randomized clinical trials about immunonutrition in major abdominal surgery was made. A frequentist random-effects component network meta-analysis was conducted, reporting the P score and odds ratio or mean difference with a 95% confidence interval. The best components and best plausible strategies were described. The critical endpoints were morbidity and mortality rates. The important endpoints were infectious complication rate and length of stay., Results: The meta-analysis includes 87 studies and 8,375 patients. The best approach for morbidity rate, with a moderate grade of certainty, was the use of perioperative enteral/oral immunonutrition with arginine, glutamine, and polyunsaturated fatty acids (odds ratio 0.32; 0.10 to 0.98; P score of 0.93). The mortality rate was reduced by postoperative enteral immunonutrition with RNA, arginine, and polyunsaturated fatty acids (odds ratio 59; 0.29 to 1.22; P score 0.84) but with a low grade of certainty. No significant heterogeneity or incoherence is observed. The length of stay and infectious results are "at risk" for high heterogeneity or network meta-analysis incoherence. The component analysis confirmed that postoperative oral/enteral use of 2 or 3 components is crucial to reducing morbidity rate., Conclusion: The oral/enteral immunonutrition in the postoperative period, with multiple immunonutrients, can reduce the morbidity rate in patients undergoing major abdominal surgery. The effect of immunonutrition on mortality, infectious disease, and length of stay is unclear., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Introduction: Liraglutide 3.0 mg, a glucagon-like peptide-1 (GLP-1) analogue, is a medication approved for obesity treatment. This study aimed to investigate the relationship between psychiatric symptoms, including depression, anxiety, and binge eating, and their impact on therapy adherence., Methods: A clinical audit was carried out on a cohort of 54 adults with obesity treated with liraglutide 3.0 mg. We retrospectively analyzed the connection between psychiatric symptoms assessed through the State-Trait Anxiety Inventory (STAI), Beck Depression Inventory (BDI), and Binge Eating Scale (BES). Adherence to therapy was assessed by the maximum dosage (MD) and treatment duration (TD)., Results: Notably, a discontinuation rate of 59% was encountered. However, among those who continued the treatment, we observed a negative association between anxiety symptoms (STAI score) and MD, depression symptoms (BDI score) and TD, and a higher likelihood of binge eating (BES score > 17) and TD. Moreover, presence of psychiatric symptoms did not compromise drug's effectiveness in achieving weight loss, which was 4.43% (± 5.5 SD) in the whole sample and 5.3% (± 6.3 SD) in the subgroup evaluated at 12 weeks., Conclusion: We observed a high discontinuation rate in real-life clinical setting, where Liraglutide 3.0 therapy is paid out-of-pocket. While psychiatric symptoms might play a role in diminishing adherence to therapy, they do not prevent drug's effectiveness to promote weight loss. This finding underscores the potential advantages of liraglutide 3.0 mg therapy for individuals contending with obesity while simultaneously managing mental health challenges., Level of Evidence: Level V, descriptive studies., (© 2023. The Author(s).)

Background and Aims: Catheter-related bloodstream infection (CRBSI) is the most common complication of home parenteral nutrition (HPN) in patients with chronic intestinal failure (CIF). The aim of this study was to assess the broad range of practices of international multi-disciplinary teams involved in the care of this complication occurring in CIF patients., Design: An online questionnaire was designed and distributed to members of the European Society for Clinical Nutrition and Metabolism (ESPEN) and distributed to colleagues involved in managing patients with CIF., Results: A total of 47 responses were included from centers across 21 countries. The centers had been delivering HPN for a median 21 years (IQR 11-35) and were actively following a median 58 patients (27-120) per center for benign CIF in 80% of cases (67-95). Tunneled catheters were the most common type of central venous catheters (CVC), representing 70% (47-86) of all CVC in use. For the management of CRBSI, written procedures were provided in 87% of centers. First measures included simultaneous central and peripheral blood cultures (90%), stopping HPN infusion (74%), and administrating an antibiotic lock and systemic antibiotics (44%). Immediate removal of the CVC was more likely in case of fungal infection (78%), Staphylococcus aureus (53%), or in case of PICC catheter (52%) (all p < 0.01). After the first CRBSI, 80% of centers used preventive CVC locks (taurolidine in 84% of cases, p < 0.001). We observed a large heterogeneity in practices regarding preparation, duration, reaspiration, and volume of CVC locks, and monitoring of CRBSI (timing of blood cultures, radiological work-up)., Conclusion: In this international survey of HPN expert centers, we observed a significant consensus regarding the initial management of CRBSI and the use of secondary preventive CVC locks, while areas of variation exist. Management of CRBSI may be improved with clearer recommendations based on the micro-organism and the type of CVC, including PICC lines which are increasingly used yet insufficiently studied in HPN patients., Competing Interests: Conflicts of interest KS declares honoraria from BBraun, Fresenius Kabi, and being member of the board of the Polish Society of Enteral, Parenteral Nutrition and Metabolism. All other authors declare no conflict of interest., (Copyright © 2023. Published by Elsevier Ltd.)

Background: The benefits of immunonutrition (IM) in patients who underwent pancreatic surgery are unclear., Methods: A meta-analysis of randomized clinical trials (RCTs) comparing IM with standard nutrition (SN) in pancreatic surgery was carried out. A random-effects trial sequential meta-analysis was made, reporting Risk Ratio (RR), mean difference (MD), and required information size (RIS). If RIS was reached, false negative (type II error) and positive results (type I error) could be excluded. The endpoints were morbidity, mortality, infectious complication, postoperative pancreatic fistula (POPF) rates, and length of stay (LOS)., Results: The meta-analysis includes 6 RCTs and 477 patients. Morbidity (RR 0.77; 0.26 to 2.25), mortality (RR 0.90; 0.76 to 1.07), and POPF rates were similar. The RISs were 17,316, 7,417, and 464,006, suggesting a type II error. Infectious complications were lower in the IM group, with a RR of 0.54 (0.36-0.79; 95 CI). The LOS was shorter in IM (MD -0.3 days; -0.6 to -0.1). For both, the RISs were reached, excluding type I error., Conclusion: The IM can reduce infectious complications and LOS The small differences in mortality, morbidity, and POPF make it impossible to exclude type II error due to large RISs., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background & Aims: In 2016, ESPEN published the guideline for Chronic Intestinal Failure (CIF) in adults. An updated version of ESPEN guidelines on CIF due to benign disease in adults was devised in order to incorporate new evidence since the publication of the previous ESPEN guidelines., Methods: The grading system of the Scottish Intercollegiate Guidelines Network (SIGN) was used to grade the literature. Recommendations were graded according to the levels of evidence available as A (strong), B (conditional), 0 (weak) and Good practice points (GPP). The recommendations of the 2016 guideline (graded using the GRADE system) which were still valid, because no studies supporting an update were retrieved, were reworded and re-graded accordingly., Results: The recommendations of the 2016 guideline were reviewed, particularly focusing on definitions, and new chapters were included to devise recommendations on IF centers, chronic enterocutaneous fistulas, costs of IF, caring for CIF patients during pregnancy, transition of patients from pediatric to adult centers. The new guideline consist of 149 recommendations and 16 statements which were voted for consensus by ESPEN members, online in July 2022 and at conference during the annual Congress in September 2022. The Grade of recommendation is GPP for 96 (64.4%) of the recommendations, 0 for 29 (19.5%), B for 19 (12.7%), and A for only five (3.4%). The grade of consensus is "strong consensus" for 148 (99.3%) and "consensus" for one (0.7%) recommendation. The grade of consensus for the statements is "strong consensus" for 14 (87.5%) and "consensus" for two (12.5%)., Conclusions: It is confirmed that CIF management requires complex technologies, multidisciplinary and multiprofessional activity, and expertise to care for the underlying gastrointestinal disease and to provide HPN support. Most of the recommendations were graded as GPP, but almost all received a strong consensus., Competing Interests: Conflicts of interest The expert members of the working group were accredited by the ESPEN Guidelines Group, the ESPEN Education and Clinical Practice Committee, and the ESPEN executive. All expert members have declared their individual conflicts of interest according to the rules of the International Committee of Medical Journal Editors (ICMJE). If potential conflicts were indicated, they were reviewed by the ESPEN guideline officers and, in cases of doubts, by the ESPEN executive. None of the expert panel had to be excluded from the working group or from co-authorship because of serious conflicts. The conflict of interest forms are stored at the ESPEN guideline office and can be reviewed with legitimate interest upon request to the ESPEN executive., (Copyright © 2023 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd.. All rights reserved.)

Background: The Covid pandemic seems to have had several detrimental effects on managing patients affected by inherited metabolic diseases (IMD), although published data about the impact of COVID-19 on patients suffering from IMD are very scarce. The scope of our work was to evaluate adherence to the vaccination plan, the side effects experienced by our adult IMD patients, and the symptoms of the SARS-CoV-2 infection., Results: Sixty-seven patients agreed to respond to a phone interview. The mean age was 36.5 (± 11.6 SD). Regarding the vaccination campaign, fifty-five patients (82%) joined it, of whom ten had received two doses and the remaining forty-five, three. Forty-two patients (76%) reported adverse events following vaccination, the most frequent being local reaction, fever, and asthenia, which lasted an average of two days and resolved without sequelae. Regarding SARS-CoV-2 infection, twenty-seven out of sixty-seven patients (40%) tested positive for the virus; seven of them were not vaccinated at the time of infection; on the other hand, twenty had already had at least two doses. Regarding the prevalence of long-Covid, as many as 12 patients (44%) reported symptoms that persisted after the nasopharyngeal swab tested negative and lasted an average of 81 (± 74 SD) days. There were no statistically significant differences in BMI of patients who contracted the infection and patients who did not (25.15 vs. 25.20, p = .861), between those who had adverse reactions to the vaccine and those who did not (24.40 vs. 25.75, p = .223), between those who had long-Covid and those who did not (25.9 vs. 27.7, p = .183). No relation was observed between metabolic inherited disease, SARS-CoV-2 infection symptoms and adverse vaccine reactions., Conclusions: The data indicate that IMD patients adhered to the vaccination campaign comparably to the general Italian population. Adverse events to the vaccine were negligible. SARS-CoV-2 infection, which occurred in most cases after receiving at least two doses of the vaccine, did not cause serious symptoms and never required hospitalisation. A non-negligible share of patients suffered from long-Covid symptoms., (© 2023. Institut National de la Santé et de la Recherche Médicale (INSERM).)

Background: Patients with inflammatory bowel disease (IBD) are at risk of malnutrition, but little is known about how IBD centres provide nutritional care., Aim: To assess how nutritional care is delivered at IBD centres across Italy., Methods: 120 IBD centres were invited to answer a web-based questionnaire., Results: 76 questionnaires (63.3%) were completed. An IBD-dedicated nutritionist is present in 27 centres (35.5%). Fifty-two centres (68.4%) have an IBD multidisciplinary team, and 22 of these include a nutritionist. In the outpatient setting, malnutrition risk is evaluated at each visit in 23 centres (30.3%), while nutritional status is assessed at each visit in 21 centres (27.6%). These assessments are performed by a gastroenterologist in almost all centres (93.4% and 88.2%, respectively) and more rarely by a nutritionist (32.9% and 36.9%), dietician (7.9% and 2.6%) or nurse (3.9% and 9.2%). The decision to offer oral nutritional support is made by a gastroenterologist alone (35.5%), a nutritionist alone (23.7%), or a team of the two (38.2%)., Conclusions: Nutritional care for IBD patients appears quite far from satisfactory in the Italian reality. Educational and structural interventions are urgently needed to improve assessment and treatment of malnutrition in everyday clinical practice., (Copyright © 2023. Published by Elsevier Ltd.)

Background and Aims: To investigate the incidence and the severity of COVID-19 infection in patients enrolled in the database for home parenteral nutrition (HPN) for chronic intestinal failure (CIF) of the European Society for Clinical Nutrition and Metabolism (ESPEN)., Methods: Period of observation: March 1st, 2020 March 1st, 2021., Inclusion Criteria: patients included in the database since 2015 and still receiving HPN on March 1st, 2020 as well as new patients included in the database during the period of observation. Data related to the previous 12 months and recorded on March 1st 2021: 1) occurrence of COVID-19 infection since the beginning of the pandemic (yes, no, unknown); 2) infection severity (asymptomatic; mild, no-hospitalization; moderate, hospitalization no-ICU; severe, hospitalization in ICU); 3) vaccinated against COVID-19 (yes, no, unknown); 4) patient outcome on March 1st 2021: still on HPN, weaned off HPN, deceased, lost to follow up., Results: Sixty-eight centres from 23 countries included 4680 patients. Data on COVID-19 were available for 55.1% of patients. The cumulative incidence of infection was 9.6% in the total group and ranged from 0% to 21.9% in the cohorts of individual countries. Infection severity was reported as: asymptomatic 26.7%, mild 32.0%, moderate 36.0%, severe 5.3%. Vaccination status was unknown in 62.0% of patients, non-vaccinated 25.2%, vaccinated 12.8%. Patient outcome was reported as: still on HPN 78.6%, weaned off HPN 10.6%, deceased 9.7%, lost to follow up 1.1%. A higher incidence of infection (p = 0.04), greater severity of infection (p < 0.001) and a lower vaccination percentage (p = 0.01) were observed in deceased patients. In COVID-19 infected patients, deaths due to infection accounted for 42.8% of total deaths., Conclusions: In patients on HPN for CIF, the incidence of COVID-19 infection differed greatly among countries. Although the majority of cases were reported to be asymptomatic or have mild symptoms only, COVID-19 was reported to be fatal in a significant proportion of infected patients. Lack of vaccination was associated with a higher risk of death., Competing Interests: Conflict of interest statements LP: Participation on a Data Safety Monitoring Board or Advisory Board for Takeda, Consulting fees for Takeda, Northsea, NAPO. SL: Participation on a Data Safety Monitoring Board or Advisory Board for Baxter, Takeda, NorthSea, VectivBio; Grants or contracts from any entity for Baxter, Takeda; Consulting fees for VectivBio, Takeda, Northsea; Support for attending meetings and/or travel for Takeda; Payment or honoraria for lectures for Takeda, Fresenius. PG: none. LS: none. PO: none. NW: none. RT: Royalties or licenses for Royalties for designing the Simple Evaluation of Food Intake® (SEFI®) (Knoë, le Kremlin Bicêtre, France); Consulting fees for Nestlé Health Science; Payment or honoraria for lectures for Baxter, BBraun, Fresenius-Kabi, Nutricia; Support for attending meetings for Nutricia, NHC. PS: none. LE: none. PO: none. L D’A: none. AT: none. ND: Leadership of Pediatric Nutrition Association of Thailand Society of Parenteral Enteral Nutrition of Thailand. ASZ: Payment or honoraria for lectures for Siegfried; Consulting for Takeda; Support for attending meetings for Abbott and Nestlè. MF: Payment or honoraria for lectures for Fresenius Kabi, B Braun, Baxter. GV: none. MIS: none. MT: none. ERB: none. NVC: Payment or honoraria for lectures for Takeda, Nutricia; Payment for expert testimony, Support for attending meetings and Participation on a Data Safety Monitoring Board for Takeda. AL: Consulting fees, Support for attending meetings, Participation on a Data Safety Monitoring Board or Advisory Board for Nestlè; Participation on a Data Safety Monitoring Board or Advisory Board for Takeda; Payment or honoraria for lectures for baxter. LC: none. MA: none. EO: none. AGM: none. AVG: none. VP: honoraria for lectures for Thai Otsuka Pharmaceutical Co., Ltd., Abbott Laboratories Ltd., Nestle (Thai) Ltd., Fresenius Medical Care (Thailand) Ltd., Baxter Healthcare (Thailand) Co., Ltd., Mega Lifesciences PTY Ltd., Novo Nordisk Thailand. MSM: Grants or contracts from any entity for Fresenius Kabi, Nestle, Realfood Blends, VectivBio, Rockfield, Zealand; Consulting fees, Northsea; Participation on a Data Safety Monitoring Board for EndoBarrier. M D-S: none. TV: Grants or contracts from any entity for Vectiv Bio, Takeda; Consulting fees for Vectiv Bio, Zealand Pharma, Takeda, Baxter, Hamni, NorthSea Therapeutics; Payment or honoraria for lectures for Vectiv Bio, Takeda, Baxter; Support for attending meetings for Takeda, Vectiv Bio, Zealand Pharma, Fresenius Kabi; Receipt of equipment, materials, drugs for VectivBio. ZK: Support for attending meetings for Abbott, Fresenius, Nutricia, Nestle, Takeda; Leadership for Croatian Medical Association- The President. FP: none. LM: none. LCE: Consulting fees, Payment or honoraria for lecture and Support for attending meetings for Takeda. UA: Payment or honoraria for lectures for Takeda, Baxter; Support for attending meetings and Participation on a Data Safety Monitoring for Takeda. MK: none. MMJ: none. AC: none. DW: none. GO: none. CC: none. JS: Grants or contracts from any entity and for BBraun, FreseniusKabi, Nestle; Payment or honoraria for lectures for BBraun, OlimpLabs, FreseniusKabi, Baxter, Nestle; Support for attending meetings for FreseniusKabi. FWG: none. CS: none. MBD: none. DS: none. SL: none. SK: Payment or honoraria for lectures, for Abbott, Abela Farm, Danone/Nutricia, Fresenius, GM Pharma, Nestle, Nestle Nutrition Institute, Oktal Pharma, Shire/Takeda; Non-restricted grant delivered to the hospital from BioGaia. BC: none. GM: none. MHMdC: Grants or contracts, Consulting fees, Payment or honoraria, Support for attending meetings, Participation on a Data Safety Monitoring for lectures for Takeda Pharmaceutical Brazil. EPJ: none. FJ: none. DJ: none. GL: none. AZM: none. MT: none. DZ: none. MK: Payment or honoraria for manuscript writing and educational events for Nutricia, FreseniusKabi. ASS: none. GB: none., (Copyright © 2023 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

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 and Aim: Chronic intestinal failure (IF) is a rare but life-altering condition, care delivery of which is complex. The ATLAS Programme was initiated in 2016 to increase disease awareness and address inconsistencies in delivery of care across Europe. We describe the results of a non-interventional study that aimed to explore how adult patients with chronic IF are managed across Europe., Materials and Methods: This mixed-methods, non-interventional, cross-sectional study comprised a desk-based landscape assessment (Phase 1), qualitative interviews (Phase 2), and an online quantitative survey (Phase 3) completed by healthcare professionals (HCPs) involved in the management of adult patients with chronic IF during the period November 2020 to January 2021. Data were collected from 12 European countries. Survey data were anonymised and pooled for analysis at European and country level. Responses were summarised as frequencies, ranks and percentage., Results: The quantitative survey was carried out on 119 HCPs across an estimated 58 centres. Gastroenterology was the most frequent specialty of respondents (45%). Three-quarters of HCPs (N = 119) reported that their department/unit had a multidisciplinary team for the management of patients with chronic IF. HCPs reported improving quality of life (QoL) to be the most important goal of treatment (39%), followed by reducing mortality (25%), intestinal rehabilitation (20%) and reducing morbidity (9%). Similarly, 63% of HCPs responded that improved QoL was the most important treatment goal from the perspective of their patients. Overall, 87% of HCPs reported that patients with chronic IF routinely receive home parenteral nutrition (HPN) in their country, which was more common in Western versus Eastern Europe. Meeting treatment goals (53%) and achieving better levels of support with HPN (44%) were reported as the main challenges faced by HCPs in the management of patients with chronic IF. A general lack of disease awareness of chronic IF among HCPs (46%), and insufficient accredited patient referral centres (41%) were considered the most important areas for improvement., Conclusions: HCPs specialising in treating chronic IF considered that improvement in QoL is needed for their patients. They reported a low level of awareness of chronic IF among non-specialist HCPs., Competing Interests: Declaration of Competing Interest PG was an employee of Takeda at the time of the study, with share and stock options in Takeda. SL has received consulting fees from NorthSea Therapeutics, Takeda and VectivBio; payment or honoraria for lectures, presentations, speakers' bureaus, manuscript writing or educational events from Baxter, Fresenius Kabi and Takeda; has participated in data safety monitoring boards or advisory boards for Takeda and VectivBio. PBJ has received honoraria for speaking events and has participated in data safety monitoring boards or advisory boards for Takeda. LP has received consulting fees from Baxter, NorthSea Therapeutics and Takeda. ER declares no competing interest. SS has been awarded institutional grants from B. Braun, Nestlé Health Science, Takeda, VectivBio and Zealand; received consulting fees from Axium MTech, Nestlé Health Science, Nutrisens, Takeda and VectivBio; received payment for honoraria for lectures, presentations, speakers' bureaus, manuscript writing or educational events from Axium MTech, Biocodex, Cardinal Health, Fresenius Kabi, Grand Fontaine, Homeperf, Merck, Nestlé Health Science, Nutricia, Takeda and Theradial; has received support for attending meetings/travel from Danone and Takeda, as well as participating in data safety monitoring boards or advisory boards for Baxter and Takeda., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: Acute mesenteric ischaemia (AMI) is a condition with high mortality. This survey assesses current attitudes and practices to manage AMI worldwide., Methods: A questionnaire survey about the practices of diagnosing and managing AMI, endorsed by several specialist societies, was sent to different medical specialists and hospitals worldwide. Data from individual health care professionals and from medical teams were collected., Results: We collected 493 individual forms from 71 countries and 94 team forms from 34 countries. Almost half of respondents were surgeons, and most of the responding teams (70%) were led by surgeons. Most of the respondents indicated that diagnosis of AMI is often delayed but rarely missed. Emergency revascularisation is often considered for patients with AMI but rarely in cases of transmural ischaemia (intestinal infarction). Responses from team hospitals with a dedicated special unit (14 team forms) indicated more aggressive revascularisation. Abdominopelvic CT-scan with intravenous contrast was suggested as the most useful diagnostic test, indicated by approximately 90% of respondents. Medical history and risk factors were thought to be more important in diagnosis of AMI without transmural ischaemia, whereas for intestinal infarction, plasma lactate concentrations and surgical exploration were considered more useful. In elderly patients, a palliative approach is often chosen over extensive bowel resection. There was a large variability in anticoagulant treatment, as well as in timing of surgery to restore bowel continuity., Conclusions: Delayed diagnosis of AMI is common despite wide availability of an adequate imaging modality, i.e. CT-scan. Large variability in treatment approaches exists, indicating the need for updated guidelines. Increased awareness and knowledge of AMI may improve current practice until more robust evidence becomes available. Adherence to the existing guidelines may help in improving differences in treatment and outcomes., Competing Interests: Declaration of competing interest Benjamin Hess: Has no conflicts of interest. Martin Cahenzli: Has no conflicts of interest. Alastair Forbes: Received speaker or consultancy fees Fresenius-Kabi, BBraun. Rosa Burgos: Received speaker or consultancy fees from Nestlé, Takeda, Abbott and Nutricia. Federico Coccolini: Has no conflicts of interest. Olivier Corcos: Has no conflicts of interest. Mette Holst: Has no conflicts of interest. Øivind Irtun: Received speaker fees from Takeda and Nutricia. Stanislaw Klek: Received speaker or consultancy fees Fresenius-Kabi, BBraun, Baxter, Nestlé, Nutricia. Loris Pironi: Received speaker or consultancy fees from Takeda, Baxter, Northsea Henrik Rasmussen: Has no conflicts of interest. Mireille J Serlie: Received grants from Mediq Tefa bv, ZonMw (Dutch Organisation for Health Research), Dutch Diabetes Foundation. Ronan Thibault: Received speaker or consultancy fees from Aguettant, Baxter, BBraun, Boehringer Ingelheim, Fresenius-Kabi, Nestlé Health Science, Nutricia, Roche, Servier; and royalties for the design of the Simple Evaluation of Food Intake® (SEFI®) (Knoë, le Kremlin Bicêtre, France). Simon Gabe: Received speaker or consultancy fees from Takeda, Fresenius Kabi. Annika Reintam Blaser: Received speaker or consultancy fees from Fresenius Kabi, Nestlé, VIPUN Medical and Nutricia., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Introduction: Pregnancy in patients with chronic intestinal failure (CIF) is a relatively rare occurrence but is an important contemporary topic given both the increasing use of home parenteral nutrition (HPN) and the demographics of patients with CIF., Method: An opinion-based survey was produced in a multidisciplinary manner, which was then distributed internationally, via the European Society for Clinical Nutrition and Metabolism network, using a web-based survey tool for healthcare professionals with a specialist interest in the management of CIF., Results: Seventy specialists from 11 countries completed the survey. Fifty-four per cent of the respondents reported some experience of managing pregnancy in patients with CIF. However, 60% stated that they did not feel that it was their role to discuss the topic of pregnancy with their patients, with fewer than 10% stating that they routinely did so. Respondents felt that an individualised approach was required when considering alterations to parenteral support prior to conception, during pregnancy and in the postnatal period. Most respondents also felt there was no increased risk of catheter-related blood stream infections, while catheter-related thrombosis was deemed to be the most significant HPN-related complication for pregnant women., Conclusion: This study reports a variable experience, knowledge and confidence of healthcare professionals when considering pregnancy in patients with CIF. The risk of HPN-related complication was felt to be greater during pregnancy, with an individualised approach being the preferred route for most aspects of care. The findings support the need for an international registry and subsequent consensus guidelines for the management of pregnancy in CIF., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

This guideline will inform physicians, nurses, dieticians, pharmacists, caregivers and other home parenteral nutrition (HPN) providers, as well as healthcare administrators and policy makers, about appropriate and safe HPN provision. This guideline will also inform patients requiring HPN. The guideline is based on previous published guidelines and provides an update of current evidence and expert opinion; it consists of 71 recommendations that address the indications for HPN, central venous access device (CVAD) and infusion pump, infusion catheter and CVAD site care, nutritional admixtures, program monitoring and management. Meta-analyses, systematic reviews and single clinical trials based on clinical questions were searched according to the PICO format. The evidence was evaluated and used to develop clinical recommendations implementing Scottish Intercollegiate Guidelines Network methodology. The guideline was commissioned and financially supported by ESPEN and members of the guideline group were selected by ESPEN., Competing Interests: Conflicts of interest The expert members of the working group were accredited by the ESPEN Guidelines Group, the ESPEN Education and Clinical Practice Committee, and the ESPEN executive. All expert members have declared their individual conflicts of interest according to the rules of the International Committee of Medical Journal Editors (ICMJE). If potential conflicts were indicated, they were reviewed by the ESPEN guideline officers and, in cases of doubts, by the ESPEN executive. None of the expert panel had to be excluded from the working group or from co-authorship because of serious conflicts. The conflict of interest forms are stored at the ESPEN guideline office and can be reviewed by ESPEN members with legitimate interest upon request to the ESPEN executive., (Copyright © 2022 © 2023 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd.. All rights reserved.)

Background & Aims: Home parenteral nutrition (HPN) is the primary treatment for chronic intestinal failure (CIF) due to non-malignant disease and is increasingly used in patients with a diagnosis of cancer. This project engaged with patients, family members and healthcare professionals to ascertain what questions they want researched., Methods: This study followed the five-stage process of the James Lind Alliance that involved (1) setting up a steering group, (2) carrying out an initial survey to gather participants' questions, (3) data processing, (4) an interim priority setting survey and (5) final priority setting workshop. Surveys were translated and back translated into Italian, Danish and French., Results: The project was delivered by an international steering committee with representation from Denmark, Italy, the United Kingdom and United States consisting of three patients, six healthcare professionals and facilitated by University researchers. For the first survey, 633 questions were submitted by 292 respondents from 12 countries. There were 79 questions removed as out of scope or already in the published literature. Responses were collated into two interim surveys of 41 questions for benign CIF and 13 questions for HPN and cancer. In the second survey, 216 respondents prioritised their top ten questions. The ordering from the cancer and HPN survey was taken as definitive; top priorities were quality of life, survival, when to commence HPN, using HPN with anti-cancer treatments, access barriers, measuring benefit and ethical implications. For CIF with benign disease, 18 questions were discussed in two workshops attended by 13 patients and 7 healthcare professionals. The questions were ranked using a modified nominal group technique; the top research priorities were prevention and treatment of liver disease, improving central infusion lines, oral absorption, avoiding long-term negative consequences, vascular access, side effects, line infections, decreasing stoma output, quality of life and sleep., Conclusions: Priorities identified will assist researchers to focus on research questions important to patients, family members and healthcare professionals., Competing Interests: Declaration of competing interest AMS, SB, DJ, CF, LW and SL report an investigator initiated grant from Takeda into their departments during the conduct of the study. JM reports a Health Education England (HEE)/National Institute for Health Research (NIHR) ICA Predoctoral Clinical Academic Fellowship and a Royal Marsden Partners Research Fellowship. Unrelated to this study: SL reports grants into his department from Takeda and Baxter and consulting fees from Takeda, Baxter, Zealand, and VectivBio, LP reports consulting fees from Takeda, Baxter and Northsea Therapeutics, CW reports consulting fees from VectioBio, payments for presentations from Fresenius Kabi and Inspiration Healthcare and conference registration fees from Fresenius Kabi, payment for being on a clinical governance board from B Braun, and BJ reports an honoraria for speaking from Snow Companies., (Copyright © 2022 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Hypoxia is a critical condition that governs survival, self-renewal, quiescence, metabolic shift and refractoriness to leukemic stem cell (LSC) therapy. The present study aims to investigate the hypoxia-driven regulation of the mammalian Target of the Rapamycin-2 (mTORC2) complex to unravel it as a novel potential target in chronic myeloid leukemia (CML) therapeutic strategies. After inducing hypoxia in a CML cell line model, we investigated the activities of mTORC1 and mTORC2. Surprisingly, we detected a significant activation of mTORC2 at the expense of mTORC1, accompanied by the nuclear localization of the main substrate phospho-Akt (Ser473). Moreover, the Gene Ontology analysis of CML patients' CD34+ cells showed enrichment in the mTORC2 signature, further strengthening our data. The deregulation of mTOR complexes highlights how hypoxia could be crucial in CML development. In conclusion, we propose a mechanism by which CML cells residing under a low-oxygen tension, i.e., in the leukemia quiescent LSCs, singularly regulate the mTORC2 and its downstream effectors.

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.)

mitochondrial neuro-gastrointestinal encephalomyopathy (MNGIE) is a rare genetic disorder characterized by thymidine phosphorylase (TP) enzyme defect. The absence of TP activity induces the imbalance of mitochondrial nucleotide pool, leading to impaired mitochondrial DNA (mtDNA) replication and depletion. Since mtDNA is required to ensure oxidative phosphorylation, metabolically active tissues may not achieve sufficient energy production. The only effective life-saving approach in MNGIE has been the permanent replacement of TP via allogeneic hematopoietic stem cell or liver transplantation. However, the follow-up of transplanted patients showed that gut tissue changes do not revert and fatal complications, such as massive gastrointestinal bleeding, can occur. The purpose of this study was to clarify whether the reintroduction of TP after transplant can recover mtDNA copy number in a normal range. Using laser capture microdissection and droplet-digital-PCR, we assessed the mtDNA copy number in each layer of full-thickness ileal samples of a naive MNGIE cohort vs. controls and in a patient pre- and post-TP replacement. The treatment led to a significant recovery of gut tissue mtDNA amount, thus showing its efficacy. Our results indicate that a timely TP replacement is needed to maximize therapeutic success before irreversible degenerative tissue changes occur in MNGIE.

Background & Aims: Malnutrition is a well-recognized risk factor for major surgery-related complications, but the impact of preoperative nutritional therapy is still debated due to a lack of high-level evidence. The study aims to evaluate the role of preoperative malnutrition in the postoperative course of patients who underwent pancreatic resection., Methods: This is a retrospective study involving 488 patients who underwent pancreatic resection. An entropy balance was applied to 134 patients at risk for moderate or severe malnutrition (M/S-MAL) to obtain a cohort equal to 354 patients, with the null or low risk of malnutrition (N/L-MAL). The reweighting scheme was made in two steps. In the 1st reweighting, the two cohorts were homogenized for confounding factors not modifiable. In the 2nd reweighting, the two cohorts were matched for modifiable factors by preoperative dietary support. The entropy balance was evaluated with the d-value. The postoperative results were reported as mean differences (MD) or odds ratio (OR) with a confidence interval at 95% (95 CI)., Results: The M/S-MAL included patients with lower values of BMI (d < 0.750), hemoglobin (d = 0.671), serum albumin (d = 0.554), total protein (d = 0.381). The M/S-MAL patients were more frequent ECOG 1-2 (d = 0.418), with jaundice (d = 0.445) or back pain (d = 0.366). The pancreaticoduodenectomy (d = 0.440) and vascular resection (d = 0.620) in the M/S-MAL group were performed more frequently. The pancreatic remnant was more often hard (d = 0.527), and the Wirsung duct dilated (d = 0.459) in the N/L-MAL group. The rate of pancreatic ductal adenocarcinoma was higher in M/S-MAL (d = 0.399). After 1st weighting, M/S-MAL patients have a high comprehensive complication index (CCI) (MD = 5.5; 0.3 to 10.7), were more frequently discharged not at home (OR 2.3; 1.1 to 5.4) with a prolonged mean hospital stay (MD 6.1.1; 0.1 to 12.1, days), After 2nd weighting, the two groups have similar postoperative results., Conclusion: The correction of malnutrition could play an independent role in reducing the severity of complication, length of stay, and type of discharge in patients who underwent pancreatic resection., Competing Interests: Conflict of interest All authors disclosed any actual or potential conflict of interest, including any financial, personal, or other relationships with other people or organisations within that could inappropriately influence (bias) this work. The study was not preregistered., (Copyright © 2022 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Background: Trace elements and vitamins, named together micronutrients (MNs), are essential for human metabolism. Recent research has shown the importance of MNs in common pathologies, with significant deficiencies impacting the outcome., Objective: This guideline aims to provide information for daily clinical nutrition practice regarding assessment of MN status, monitoring, and prescription. It proposes a consensus terminology, since many words are used imprecisely, resulting in confusion. This is particularly true for the words "deficiency", "repletion", "complement", and "supplement"., Methods: The expert group attempted to apply the 2015 standard operating procedures (SOP) for ESPEN which focuses on disease. However, this approach could not be applied due to the multiple diseases requiring clinical nutrition resulting in one text for each MN, rather than for diseases. An extensive search of the literature was conducted in the databases Medline, PubMed, Cochrane, Google Scholar, and CINAHL. The search focused on physiological data, historical evidence (published before PubMed release in 1996), and observational and/or randomized trials. For each MN, the main functions, optimal analytical methods, impact of inflammation, potential toxicity, and provision during enteral or parenteral nutrition were addressed. The SOP wording was applied for strength of recommendations., Results: There was a limited number of interventional trials, preventing meta-analysis and leading to a low level of evidence. The recommendations underwent a consensus process, which resulted in a percentage of agreement (%): strong consensus required of >90% of votes. Altogether the guideline proposes sets of recommendations for 26 MNs, resulting in 170 single recommendations. Critical MNs were identified with deficiencies being present in numerous acute and chronic diseases. Monitoring and management strategies are proposed., Conclusion: This guideline should enable addressing suboptimal and deficient status of a bundle of MNs in at-risk diseases. In particular, it offers practical advice on MN provision and monitoring during nutritional support., Competing Interests: Conflict of Interest The expert members of the working group were accredited by the ESPEN Guidelines Group, the ESPEN Education and Clinical Practice Committee, and the ESPEN executive. All expert members have declared their individual conflicts of interest according to the rules of the International Committee of Medical Journal Editors (ICMJE). If potential conflicts were indicated, they were reviewed by the ESPEN guideline officers and, in cases of doubts, by the ESPEN executive. None of the expert panel had to be excluded from the working group or from co-authorship because of serious conflicts. The conflict-of-interest forms are stored at the ESPEN guideline office and can be reviewed by ESPEN members with legitimate interest upon request to the ESPEN executive., (Copyright © 2022 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd.. All rights reserved.)

Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1−4 and 14−18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores < −2. One-third had %IVSE/REE > 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children.

Objectives: This work aimed to report the demographic and clinical characteristics of two new cases with non-paraneoplastic anti-Hu-associated gut motility impairment, and perform a thorough revision covering anti-Hu-associated paraneoplastic (PGID) and non-paraneoplastic (nPGID) gastrointestinal dysmotility., Background: Several case series have clearly established a relationship between certain type of cancers, the development of circulating anti-Hu antibodies, and the concomitant usually severe gastrointestinal dysmotility; in contrast, a few studies focused on anti-Hu-associated nPGID., Methods: We searched for studies regarding anti-Hu-associated gastrointestinal manifestations and extracted data concerning clinical characteristics of patients, including specific demographic, oncological, neurological, gastrointestinal, histological, and treatment response features., Results: Forty-nine articles with a total of 59 cases of anti-Hu-associated gastrointestinal dysmotility were analyzed. The patients' age at symptom onset significantly differed between PGID and nPGID (median 61 vs 31 years, p < 0.001). Most cancers (95%) in PGID were detected within 24 months from the beginning of gastrointestinal symptoms. The impairment of gastrointestinal motility was generalized (i.e., involving the whole gut) in 59.3% of patients, with no significant differences between PGID vs nPGID group. nPGID patients showed a better response to immunomodulatory/immunosuppressive treatment and a longer life expectancy., Conclusions: Anti-Hu-associated gastrointestinal dysmotility covers a wide clinical spectrum. Patients with otherwise unexplained gastrointestinal dysmotility, especially when associated with other neurological symptoms, should be tested for anti-Hu antibodies regardless age of onset and disease duration. Compared to PGID, nPGID occurs in younger patients with a long duration of disease., (© 2021. Springer-Verlag GmbH Germany, part of Springer Nature.)

This ESPEN practical guideline will inform physicians, nurses, dieticians, pharmacists, caregivers and other home enteral nutrition (HEN) providers in a concise way about the indications and contraindications for HEN, as well as its implementation and monitoring. This guideline will also inform interested patients requiring HEN. Home parenteral nutrition is not included but will be addressed in a separate ESPEN guideline. The guideline is based on the ESPEN scientific guideline published before, which consists of 61 recommendations that have been reproduced and renumbered, along with the associated commentaries that have been shorted compared to the scientific guideline. Evidence grades and consensus levels are indicated. The guideline was commissioned and financially supported by ESPEN and the members of the guideline group were selected by ESPEN., Competing Interests: Conflict of interest The expert members of the working group were accredited by the ESPEN Guidelines Group, the ESPEN Education and Clinical Practice Committee, and the ESPEN executive. All expert members have declared their individual conflicts of interest according to the rules of the International Committee of Medical Journal Editors (ICMJE). If potential conflicts were indicated, they were reviewed by the ESPEN guideline officers and, in cases of doubts, by the ESPEN executive. None of the expert panel had to be excluded from the working group or from co-authorship because of serious conflicts. The conflict of interest forms are stored at the ESPEN guideline office and can be reviewed with legitimate interest upon request to the ESPEN executive., (Copyright © 2021 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd.. All rights reserved.)

Background & Aims: The preoperative use of carbohydrate loading (CHO) is recommended in patients undergoing abdominal surgery, even if the advantages remain debatable. The aim was to evaluate the CHO benefits in patients undergoing abdominal surgery., Methods: A systematic search of randomized clinical trials was made. A frequentist random-effects network meta-analysis was carried out, reporting the surface under the cumulative ranking (SUCRA). The primary endpoint regarded the morbidity rate. The secondary endpoints were aspiration/regurgitation rates, the length of stay (LOS), the rate of postoperative nausea and vomiting (PONV), the changes (Δ) in insulin sensitivity or resistance, and the postoperative C- reactive protein (CRP) values., Results: CHO loading and water administration had a similar probability of being the approach with a lower morbidity rate (SUCRA = 62.4% and 64.7%). CHO and clear water also had a similar chance of avoiding the PONV (SUCRA of 80.8% and 77%). The aspiration regurgitation rate was not relevant in non-fasting patients (0.06%). CHO administration was associated with the shorter hospitalization (SUCRA 86.9%), with the best metabolic profile (SUCRA values for insulin resistance and sensitivity were 81.1% and 76%). CHO enriched was the best approach for postoperative CRP values. Preoperative fasting was the worst approach for morbidity, PONV, insulin resistance and sensitivity, and CRP (SUCRA values of 32.1%, 21.7%, 10.2%, 3.2%, and 2.0%)., Conclusion: Both preoperative CHO loading and clear water use were superior to the fasting about morbidity. CHO drinks use could provide specific advantages, reduce the PONV rate, and improve carbohydrate homeostasis, inflammatory pathway, and hospitalization., Competing Interests: Conflict of Interest All authors disclosed any actual or potential conflict of interest, including any financial, personal, or other relationships with other people or organisations within that could inappropriately influence (bias) this work. The study was not preregistered., (Copyright © 2021 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Introduction: Advanced cancer (AC) is increasingly an indication for home parenteral nutrition (HPN) but an area with possible variation in practice between geographical locations. The aims of this study are to explore the views and experiences of international multi-disciplinary teams to determine opinions and practices., Methods: An online questionnaire was developed with members of the Home Artificial Nutrition and Chronic Intestinal Failure interest group of the European Society for Clinical Nutrition and Metabolism (ESPEN) and distributed to colleagues involved in managing patients with AC on HPN., Results: A total of 220 responses were included from 5 continents including 36 countries, with 90% of all responses from Europe. Predicted survival was a key factor influencing the decision to commence HPN for most respondents 152/220 (75%), with the majority of participants reporting that patients should have a predicted survival of ≥3 months if considered for HPN (≥3 months: n = 124, 56% vs. <3 months: n = 47, 21%, p < 0.001). However, most respondents were not confident about predicting overall survival in more than 50% of cases (confident n = 40, 23% vs not confident n = 135, 77%, p < 0.001). Barriers to utilising HPN in AC included colleagues' objections (n = 91, 46%), lack of local expertise (n = 55, 28%) and funding restrictions (n = 34, 17%)., Conclusions: Significant consensus was observed regarding AC as indication for HPN, while areas of variation exist. Survival prognostication is often used as an indication for commencing HPN in people with AC, although the majority of respondents were not confident in prognosticating, suggesting better clinical prognostication tools will be of assistance. Further studies are also required to better understand the obstacles faced by clinical teams to commencing HPN that may explain variations in clinical practice between countries, as well as adressing variation in funding., Competing Interests: Declaration of competing interest None., (Copyright © 2021. Published by Elsevier Ltd.)

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.)

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

Epidemiological evidence has confirmed the potential causal relationship between specific dietary factors and non-communicable diseases. However, currently nutrition was shown to be insufficiently integrated into medical education, regardless of the country. Without an adequate nutrition education, it is reasonable to assume that future physicians, as well as other health care professionals, will be not able to provide the highest quality care to patients in preventing and treating non-communicable diseases. Furthermore, the insufficient availability of physicians with specializations in nutrition has posed the basis for the development of non-medical careers in the field of nutrition. The present document was drafting by the Italian College of Academic Nutritionists, MED-49 (ICAN-49), with the aim to provide an overview on the nutritional competency standards covered by several health care professionals (Physicians Clinical Nutrition Specialists, Clinical Dietitians, Professional Clinical Nutrition Specialists, etc) for the prevention of diseases and/or support of pharmacological therapies. The aim of the ICAN 49 is to suggest a major shift in practice opportunities and roles for many nutritionists, especially for the management of the metabolic diseases, and promote a paradigm change: a clinical and educational leadership role for Physician Clinical Nutrition Specialists in the hospital setting., Competing Interests: Declaration of competing interest The authors have no competing financial interests in relation to the work described., (Copyright © 2021 The Italian Diabetes Society, the Italian Society for the Study of Atherosclerosis, the Italian Society of Human Nutrition and the Department of Clinical Medicine and Surgery, Federico II University. Published by Elsevier B.V. All rights reserved.)

Background: Maturity Onset Diabetes of the Young (MODY) is a monogenic, autosomal, dominant disease that results in beta-cells dysfunction with consequent hyperglycaemia. It represents a rare form of diabetes (1-2% of all the cases). Sulphonylureas (SUs) represent the first-line treatment for this form of diabetes mellitus. NEUROD1 is expressed by the nervous and the pancreatic tissues, and it is necessary for the proper development of beta cells. A neurogenic differentiation factor 1 (NEUROD1) gene mutation causes beta-cells dysfunction, inadequate insulin secretion, and hyperglycaemia (MODY 6)., Case Presentation: We have documented a new missense mutation (p.Met114Leu c.340A > C) of the NEUROD1 gene, pathogenetic for diabetes mellitus, in a 48 years-old man affected by diabetes since the age of 25 and treated with insulin basal-bolus therapy. Unfortunately, an attempt to replace rapid insulin with dapagliflozin has failed. However, after the genetic diagnosis of MODY6 and treatment with SUs, he was otherwise able to suspend rapid insulin and close glucose monitoring. Interestingly, our patient had an early onset dilated cardiomyopathy, though no data about cardiac diseases in patients with MODY 6 are available., Conclusions: Diagnostic criteria for MODY can overlap with other kinds of diabetes and most cases of genetic diabetes are still misdiagnosed as diabetes type 1 or 2. We encourage to suspect this disease in patients with a strong family history of diabetes, normal BMI, early-onset, and no autoimmunity. The appropriate therapy simplifies disease management and improves the quality of the patient's life., (© 2021. The Author(s).)

Objectives: Malnutrition negatively affects the quality of life, survival, and clinical outcome of patients with cancer. Home artificial nutrition (HAN) is an appropriate nutritional therapy to prevent death from cachexia and to improve quality of life, and it can be integrated into a home palliative care program. The choice to start home enteral nutrition (HEN) or home parenteral nutrition (HPN) is based on patient-specific indications and contraindications. The aim of this observational study was to analyze the changes that occurred in the criteria for choosing the access route to artificial nutrition during 30 y of activity of a nutritional service team (NST) in a palliative home care setting, as well as to compare indications, clinical nutritional outcomes, and complications between HEN and HPN., Methods: The following parameters were analyzed and compared for HEN and HPN: tumor site and metastases; nutritional status (body mass index, weight loss in the past 6 mo); basal energy expenditure and oral food intake; Karnofsky performance status; access routes to HEN (feeding tubes) and HPN (central venous catheters); water and protein-calorie support; and survival and complications of HAN., Results: From 1990 to 2020, HAN was started in 1014 patients with cancer (592 men, 422 women; 65.6 ± 12.7 y of age); HPN was started in 666 patients (66%); and HEN was started in 348 patients (34%). At the end of the study, 921 patients had died, 77 had suspended HAN for oral refeeding and 16 were in the progress of HAN. The oral caloric intake was <50% basal energy expenditure in all patients: 721 (71.1%) were unable to eat at all (HEN 270, HPN 451), whereas in 293 patients (28.9%), artificial nutrition was supplementary to oral intake. From 2010 to 2020, the number of central venous catheters for HPN, especially peripherally inserted central catheters, doubled compared with that in the previous 20 y, with a decrease of 71.6% in feeding tubes for HEN. At the beginning, patients on HEN and HPN had comparable nutrition and performance status, and there was no difference in nutritional outcome after 1 mo of HAN. In 215 patients who started supplemental parenteral nutrition to oral feeding, total protein-calorie intake allowed a significant increase in body mass index and Karnofsky performance status. The duration of HEN was longer than that of HPN but was similar to that of supplemental parenteral nutrition., Conclusions: Over 30 y of nutritional service team activity, the choice of central venous catheters as an access route to HAN increased progressively and significantly due to personalized patient decision-making choices. Nutritional efficacy was comparable between HEN and HPN. In patients who maintained food oral intake, supplemental parenteral nutrition improved weight, performance status, and survival better than other types of HAN., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background and Aims: The case-mix of patients with intestinal failure due to short bowel syndrome (SBS-IF) can differ among centres and may also be affected by the timeframe of data collection. Therefore, the ESPEN international multicenter cross-sectional survey was analyzed to compare the characteristics of SBS-IF cohorts collected within the same timeframe in different countries., Methods: The study included 1880 adult SBS-IF patients collected in 2015 by 65 centres from 22 countries. The demographic, nutritional, SBS type (end jejunostomy, SBS-J; jejuno-colic anastomosis, SBS-JC; jejunoileal anastomosis with an intact colon and ileocecal valve, SBS-JIC), underlying disease and intravenous supplementation (IVS) characteristics were analyzed. IVS was classified as fluid and electrolyte alone (FE) or parenteral nutrition admixture (PN). The mean daily IVS volume, calculated on a weekly basis, was categorized as <1, 1-2, 2-3 and >3 L/day., Results: In the entire group: 60.7% were females and SBS-J comprised 60% of cases, while mesenteric ischaemia (MI) and Crohn' disease (CD) were the main underlying diseases. IVS dependency was longer than 3 years in around 50% of cases; IVS was infused ≥5 days/week in 75% and FE in 10% of cases. Within the SBS-IF cohort: CD was twice and thrice more frequent in SBS-J than SBS-JC and SBS-JIC, respectively, while MI was more frequent in SBS-JC and SBS-JIC. Within countries: SBS-J represented 75% or more of patients in UK and Denmark and 50-60% in the other countries, except Poland where SBS-JC prevailed. CD was the main underlying disease in UK, USA, Denmark and The Netherlands, while MI prevailed in France, Italy and Poland., Conclusions: SBS-IF type is primarily determined by the underlying disease, with significant variation between countries. These novel data will be useful for planning and managing both clinical activity and research studies on SBS., Competing Interests: Declaration of competing interest None declared., (Copyright © 2021 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

Background: This practical guideline is based on the ESPEN Guidelines on Chronic Intestinal Failure in Adults., Methodology: ESPEN guidelines have been shortened and transformed into flow charts for easier use in clinical practice. The practical guideline is dedicated to all professionals including physicians, dieticians, nutritionists, and nurses working with patients with chronic intestinal failure., Results: This practical guideline consists of 112 recommendations with short commentaries for the management and treatment of benign chronic intestinal failure, including home parenteral nutrition and its complications, intestinal rehabilitation, and intestinal transplantation., Conclusion: This practical guideline gives guidance to health care providers involved in the management of patients with chronic intestinal failure., Competing Interests: Conflicts of interest The authors declare no conflict of interest., (Copyright © 2021 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd.. All rights reserved.)

Background and Aims: The glucagon-like peptide-2 (GLP-2) analogue, teduglutide, allows to reduce the intravenous supplementation (IVS) dependency of patients with short bowel syndrome and intestinal failure (SBS-IF). The rate of candidacy of SBS-IF patients for the treatment is unknown. The candidacy for teduglutide treatment of our patient cohort was investigated by a systematic analysis., Methods: The indications, contraindications, special warnings and precautions for use of teduglutide, listed in the drug monographs and in the phase-III trial protocol were adopted to categorize the patients as non-candidates (NC), potential candidates (PC) or straight candidates (SC) for the treatment. All the SBS-IF adult patients who were cured at our centre were assessed according to their clinical status on January 1st, 2020., Results: Seventy-nine patients were evaluated: 34.2% were NC due to risk of digestive malignancy, recent history of any other cancer, or listing for intestinal transplantation; 30.4% were PC, because of other premalignant conditions, risk of intestinal obstruction, entero-cutaneous fistulas, or severe co-morbidities; 35.4% were SC. The SC group showed the lowest requirement of IVS: the lowest number of days of infusion per week (p = 0.0054), the lowest amount of energy (p = 0.0110) and volume (p = 0.0136)., Conclusions: This systematic analysis allowed a pragmatic categorization of the candidacy of patients with SBS-IF for GLP-2 analogue treatment. The SC group appeared to have the highest probability of a successful response to the treatment. A systematic analysis of SBS-IF patient candidate for GLP-2 analogue therapy would allow a homogeneous patient selection and facilitate the worldwide comparison of the results of clinical practice and research., Competing Interests: Conflict of interest LP received grant for participation in advisory board from Baxter, Takeda, and educational grant from Fresenius-Kabi and Seda. ASS, FMV, AM, CP and MG have no conflict of interest to declare., (Copyright © 2021 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive disease caused by thymidine phosphorylase (TP) enzyme defect. As gastrointestinal changes do not revert in patients undergone TP replacement therapy, one can postulate that other unexplored mechanisms contribute to MNGIE pathophysiology. Hence, we focused on the local TP angiogenic potential that has never been considered in MNGIE. In this study, we investigated the enteric submucosal microvasculature and the effect of hypoxia on fibrosis and enteric neurons density in jejunal full-thickness biopsies collected from patients with MNGIE. Orcein staining was used to count blood vessels based on their size. Fibrosis was assessed using the Sirius Red and Fast Green method. Hypoxia and neoangiogenesis were determined via hypoxia-inducible-factor-1α (HIF-1α) and vascular endothelial cell growth factor (VEGF) protein expression, respectively. Neuron-specific enolase was used to label enteric neurons. Compared with controls, patients with MNGIE showed a decreased area of vascular tissue, but a twofold increase of submucosal vessels/mm 2 with increased small size and decreased medium and large size vessels. VEGF positive vessels, fibrosis index, and HIF-1α protein expression were increased, whereas there was a diminished thickness of the longitudinal muscle layer with an increased interganglionic distance and reduced number of myenteric neurons. We demonstrated the occurrence of an angiopathy in the GI tract of patients with MNGIE. Neoangiogenetic changes, as detected by the abundance of small size vessels in the jejunal submucosa, along with hypoxia provide a morphological basis to explain neuromuscular alterations, vasculature breakdown, and ischemic abnormalities in MNGIE. NEW & NOTEWORTHY Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is characterized by a genetically driven defect of thymidine phosphorylase, a multitask enzyme playing a role also in angiogenesis. Indeed, major gastrointestinal bleedings are life-threatening complications of MNGIE. Thus, we focused on jejunal submucosal vasculature and showed intestinal microangiopathy as a novel feature occurring in this disease. Notably, vascular changes were associated with neuromuscular abnormalities, which may explain gut dysfunction and help to develop future therapeutic approaches in MNGIE.

Purpose of Review: To discuss the new guidelines on the indications for intestinal transplantation (ITx) devised in 2019 by the Intestinal Rehabilitation and Transplant Association., Recent Findings: Early referral of patients with intestinal failure to expert intestinal rehabilitation/transplant centre is strongly recommended. Listing for a life-saving transplantation is recommended for intestinal failure-associated liver disease (IFALD) evolving to liver failure, invasive intra-abdominal desmoids, acute diffuse intestinal infarction with hepatic failure, re-transplant, and children with loss of at least three of the four upper central venous access sites or with high morbidity intestinal failure. Developments in ITx made the probability of posttransplant survival equal to that on home parenteral nutrition (HPN) and the QoL after successful ITx better than on HPN. However, for patients who have not an actual increased risk of death on HPN, the matter of preemptive listing for ITx is still controversial. For these patients, a careful case-by-case decision is recommended., Summary: The new guidelines on ITx confirm the straight referral for ITx only for patients at actual risk of death on HPN. Improvements in ITx practice and results, advances in the severity classification of intestinal failure, monitoring of the evolution of IFALD, and measuring patients' QoL are required for an immediate progression in the treatment of intestinal failure., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background & Aims: There is intense research for drugs able to reduce disease progression in nonalcoholic fatty liver disease. We aimed to test the impact of novel antidiabetic drugs (dipeptidyl-peptidase-4 inhibitors - DPP-4Is, glucagon-like peptide-1 receptor agonists - GLP-1RAs, sodium-glucose cotransporter-2 inhibitors - SGLT-2Is) on non-invasive biomarkers of steatosis (fatty liver index, FLI) and fibrosis (Fibrosis-4 score, FIB-4) in patients with type 2 diabetes (T2D)., Methods: Clinical, anthropometric and biochemical parameters were retrospectively analysed in 637 consecutive T2D patients switched from metformin w/wo sulfonylureas and/or pioglitazone to DPP-4Is, GLP-1RAs and SGLT-2Is in a tertiary care setting. 165 patients maintained on original treatments served as controls. The effects on FLI and FIB-4 at 6- and 12-month follow-up were analysed by logistic regression after adjustment for baseline differences, computed by propensity scores, and additional adjustment for changes in glycosylated hemoglobin (HbA1c) and body mass index., Results: Body mass index, HbA1c and aminotrasferases significantly decreased following switching to GLP-1RAs and SGLT2-Is, compared with both controls and DPP-4Is, whereas only HbA1c was reduced on DPP-4Is. FLI and FIB-4 were reduced on GLP-1RA and SGLT-2I; logistic regression analysis confirmed a significant improvement of both biomarkers after adjustment for propensity score. The shift of FIB-4 values towards the category ruling out advanced fibrosis was maintained after additional adjustment for confounders. These effects were confirmed in a sensitivity analysis on effect size., Conclusions: Glucagon-like peptide-1 receptor agonists and SGLT-2Is improve biomarkers of steatosis and fibrosis, in keeping with beneficial effects on liver disease progression, and should be considered the treatment of choice in T2D., (© 2021 The Authors. Liver International published by John Wiley & Sons Ltd.)

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.)

Rationale: The prevalence of malnutrition and the provided nutritional therapy were evaluated in all the patients with SARS-CoV-2 infection (COVID-19) hospitalized in a 3rd level hospital in Italy., Methods: A one-day audit was carried out recording: age, measured or estimated body weight (BW) and height, body mass index (BMI, kg/m 2 ), 30-day weight loss (WL), comorbidities, serum albumin and C-reactive protein (CRP: nv < 0.5 mg/dL), hospital diet (HD) intake, oral nutritional supplements (ONS), enteral (EN) and parenteral nutrition (PN). Modified NRS-2002 tool and GLIM criteria were used for nutritional risk screening and for the diagnosis of malnutrition, respectively., Results: A total of 268 patients was evaluated; intermediate care units (IMCUs, 61%), sub-intensive care units (SICUs, 8%), intensive care units (ICUs, 17%) and rehabilitation units (RUs, 14%): BMI: <18.5, 9% (higher in RUs, p = 0.008) and ≥30, 13% (higher in ICUs, p = 0.012); WL ≥ 5%, 52% (higher in ICUs and RUs, p = 0.001); CRP >0.5: 78% (higher in ICUs and lower in RUs, p < 0.001); Nutritional risk and malnutrition were present in 77% (higher in ICUs and RUs, p < 0.001) and 50% (higher in ICUs, p = 0.0792) of the patients, respectively. HD intake ≤50%, 39% (higher in IMCUs and ICUs, p < 0.001); ONS, EN and PN were prescribed to 6%, 13% and 5%, respectively. Median energy and protein intake/kg BW were 25 kcal and 1.1 g (both lower in ICUs, p < 0.05) respectively., Conclusions: Most of the patients were at nutritional risk, and one-half of them was malnourished. The frequency of nutritional risk, malnutrition, disease/inflammation burden and decrease intake of HD differed among the intensity of care settings, where the patients were managed according to the severity of the disease. The patient energy and protein intake were at the lowest limit or below the recommended amounts, indicating the need for actions to improve the nutritional care practice., Competing Interests: Conflict of interest None declared., (Copyright © 2020 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)

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.)

We report the longest follow-up of clinical and biochemical features of two previously reported adult mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) patients treated with liver transplantation (LT), adding information on a third, recently transplanted, patient. All three patients overcame the early post-operative period and tolerated immunosuppressive therapy. Plasma nucleoside levels dramatically decreased, with evidence of clinical improvement of ambulation and neuropathy. Conversely, other features of MNGIE, as gastrointestinal dysmotility, low weight, ophthalmoparesis, and leukoencephalopathy were essentially unchanged. A similar picture characterized two patients treated with allogenic hematopoietic stem cell transplantation (AHSCT). In conclusion, LT promptly and stably normalizes nucleoside imbalance in MNGIE, stabilizing or improving some clinical parameters with marginal periprocedural mortality rate as compared to AHSCT. Nevertheless, restoring thymidine phosphorylase (TP) activity, achieved by both LT and AHSCT, does not allow a full clinical recovery, probably due to consolidated cellular damage and/or incomplete enzymatic tissue replacement.