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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. 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.

Contains fulltext : 290886.pdf (Publisher’s version ) (Closed access) 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.

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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.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).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.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.

SummaryBackground & aimsHome 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.MethodsThis 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.ResultsThe 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.ConclusionsPriorities identified will assist researchers to focus on research questions important to patients, family members and healthcare professionals.

Introduction. The glucagon-like peptide-1 analogue liraglutide 3.0 mg is an out-of-pocket medication approved for weight management in obesity. We aimed to investigate the relationship between psychiatric symptoms (i.e., depression, anxiety, binge eating) and adherence to therapy. Methods. A clinical audit was carried out on 54 adults with obesity treated with liraglutide 3.0 mg. We retrospectively analyzed the relation between (1) psychiatric symptoms evaluated through the State-Trait Anxiety Inventory (STAI-Y1), the Beck Depression Inventory (BDI), the Binge Eating Scale (BES); and (2) adherence to therapy by assessing the maximum dosage (MD) and treatment duration (TD). Results. In the whole cohort, the average weight loss was 4.43% (± SD = 5.5). We found a negative correlation between anxiety symptoms (STAI-Y1 score) and MD (r=-,276), between depression symptoms (BDI score) and TD (r=-,276), and between a high probability of binge eating (BES score > 17) and TD (r=-,275). Linear regression analysis demonstrated that STAI-Y1 score predicted MD [R2 = .076, p = .044], BDI score predicted TD [R2 = .076, p = .044], and significant binge eating predicted TD [R2 = .076, p = .044]. Despite the lower adherence, the presence of psychiatric symptoms did not lead to a reduction in drug effectiveness on weight loss. Conclusion. Psychiatric symptoms can predict reduced adherence to liraglutide 3.0 mg therapy in real life. However, this does not appear to jeopardize its effect on weight loss. These findings suggest that persons with obesity and impaired mental health can also benefit from treatment. Level of evidence. Level V, descriptive studies.

Background and aimsTo 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).MethodsPeriod 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.ResultsSixty-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.ConclusionsIn 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. Background and aimsTo 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).MethodsPeriod 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.ResultsSixty-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.ConclusionsIn 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.

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.

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.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.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.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.

The coronavirus disease 2019 (COVID-19) pandemic has not finished yet, and the most promising option towards its ending is widespread vaccination. Because patients with inflammatory bowel diseases (IBDs), namely Crohn’s disease (CD) and ulcerative colitis (UC), often require immune-modifying treatment, which might increase the risk of opportunistic infection,1 their vaccination history for several infectious diseases is routinely checked, and when inadequate, vaccination is performed at diagnosis or ideally before immune suppressive treatment is started.1 However, IBD patients were not found to be at an increased risk of developing COVID-19 or of experiencing a more severe disease course.2

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.

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. 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. 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. 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

BACKGROUND & AIMS: 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 (AD) in this cohort have not yet been described.METHODS: The study involved prospective data collection from patients attending HPN clinic at a national intestinal failure referral centre. Patients requiring HPN as a result of active malignancy were excluded. Patients were divided in two groups according to AD usage; demographic, anthropometric, socioeconomic characteristics and 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%. 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 = 0.039) with the proportion of patients needing high volume IVS (≥3000ml/day) being three-times higher in the AD group (20.0% vs. 6.1%, P = 0.006). The average energy IVS infusion per day was similar between the two groups. Hypnotic use (25.7% vs. 5.1%, P

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.

IntroductionPregnancy 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.MethodAn 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.ResultsSeventy 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.ConclusionThis 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.

Summary 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.

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. ispartof: Clinical Nutrition vol:41 issue:6 ispartof: location:England status: Published online

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.

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 line 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.

Background and aimNo marker to categorise the severity of chronic intestinal failure (CIF) has been developed. A 1-year international survey was carried out to investigate whether the European Society for Clinical Nutrition and Metabolism clinical classification of CIF, based on the type and volume of the intravenous supplementation (IVS), could be an indicator of CIF severity.MethodsAt baseline, participating home parenteral nutrition (HPN) centres enrolled all adults with ongoing CIF due to non-malignant disease; demographic data, body mass index, CIF mechanism, underlying disease, HPN duration and IVS category were recorded for each patient. The type of 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 categorised as 3 L/day. The severity of CIF was determined by patient outcome (still on HPN, weaned from HPN, deceased) and the occurrence of major HPN/CIF-related complications: intestinal failure-associated liver disease (IFALD), catheter-related venous thrombosis and catheter-related bloodstream infection (CRBSI).ResultsFifty-one HPN centres included 2194 patients. The analysis showed that both IVS type and volume were independently associated with the odds of weaning from HPN (significantly higher for PN 1 L/day), patients’ death (lower for FE, p=0.079), presence of IFALD cholestasis/liver failure and occurrence of CRBSI (significantly higher for PN 2–3 and PN >3 L/day).ConclusionsThe type and volume of IVS required by patients with CIF could be indicators to categorise the severity of CIF in both clinical practice and research protocols.

This guideline will inform physicians, nurses, dieticians, pharmacists, caregivers and other home enteral nutrition (HEN) providers about the indications and contraindications for HEN, and its implementation and monitoring. Home parenteral nutrition is not included but will be addressed in a separate ESPEN guideline. This guideline will also inform interested patients requiring HEN. The guideline is based on current evidence and expert opinion and consists of 61 recommendations that address the indications for HEN, relevant access devices and their use, the products recommended, the monitoring and criteria for termination of HEN, and the structural requirements needed to perform HEN. We searched for meta-analyses, systematic reviews and single clinical trials based on clinical questions according to the PICO format. The evidence was evaluated and used to develop clinical recommendations implementing the SIGN method. The guideline was commissioned and financially supported by ESPEN and the members of the guideline group were selected by ESPEN.

Background & aims: Intestinal failure (IF) is defined from a requirement or intravenous supplementation due to failing capacity to absorb nutrients and fluids. Acute IF is an acute, potentially reversible form of IF. We aimed to identify the prevalence, underlying causes and outcomes of acute IF.Methods: This point-of-prevalence study included all adult patients hospitalized in acute care hospitals and receiving parenteral nutrition (PN) on a study day. The reason for PN and the mechanism of IF (if present) were documented by local investigators and reviewed by an expert panel.Results: Twenty-three hospitals (19 university, 4 regional) with a total capacity of 16,356 acute care beds and 1237 intensive care unit (ICU) beds participated in this study. On the study day, 338 patients received PN (21 patients/1000 acute care beds) and 206 (13/1000) were categorized as acute IF. The categorization of reason for PN was revised in 64 cases (18.9% of total) in consensus between the expert panel and investigators. Hospital mortality of all study patients was 21.5%; the median hospital stay was 36 days. Patients with acute IF had a hospital mortality of 20.5% and median hospital stay of 38 days (P > 0.05 for both outcomes). Disordered gut motility (e.g. ileus) was the most common mechanism of acute IF, and 71.5% of patients with acute IF had undergone abdominal surgery. Duration of PN of >= 42 days was identified as being the best cut-off predicting hospital mortality within 90 days. PN >= 42 days, age, sepsis and ICU admission were independently associated with 90-day hospital mortality.Conclusions: Around 2% of adult patients in acute care hospitals received PN, 60% of them due to acute IF. High 90-day hospital mortality and long hospital stay were observed in patients receiving PN, whereas presence of acute IF did not additionally influence these outcomes. Duration of PN was associated with increased 90-day hospital mortality. (C) 2019 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.

Summary 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.

Chronic intestinal pseudo-obstruction (CIPO) is a severe form of intestinal dysmotility disorder, characterized by the impairment of gastrointestinal propulsion of the gut content in the absence of fixed occluding lesions. CIPO is a rare disease that can develop in both children and adults. CIPO is classified as primary/idiopathic, when no underlying disorder is demonstrated, or secondary, when related to systemic diseases. Diagnosis relies on the finding of chronic/recurrent obstructive type symptoms with radiological features of dilated intestine with air/fluid levels without any lumen occluding lesion. Therapy is based on nutrition, pharmacologic and surgical intervention and requires a multidisciplinary approach.

Contains fulltext : 244566.pdf (Publisher’s version ) (Closed access) 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 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.

Contains fulltext : 205187.pdf (Publisher’s version ) (Closed access) BACKGROUND & AIMS: Home parenteral nutrition-quality of life (HPN-QOL((c))) is a self-assessment tool for the measurement of QOL in patients on HPN. The aims of this study were: to re-assess the basic psychometric properties of the HPN-QOL((c)) in a multinational sample of adult patients; to provide a description of QOL dimensions by short and long HPN treatment duration; to explore clinical factors potentially associated to QOL scores. METHODS: Patients (n = 699) from 14 countries completed the HPN-QOL((c)). The questionnaires were analysed to evaluate data completeness, convergent/discriminant validity and internal-consistency reliability. The association of overall QOL and HPN treatment duration as well as other clinical factors were investigated using multivariable linear regression models. RESULTS: The analysis of the multitrait-scaling and internal consistency indicates a good fit with the questionnaire structure for most items. Item discriminant validity correlation was satisfactory and psychometric evaluation of the HPN-QOL((c)) in the different English, French and Italian language patient sub-groups confirmed psychometric equivalence of the three questionnaire versions. The results of the multivariable linear regression showed that QOL scores were significantly associated with HPN duration (better in long-term), underlying disease (better in Crohn's disease and mesenteric ischaemia) and living status (worse in living alone) and, after adjusting for the other factors, with the number of days of HPN infusion per week. CONCLUSIONS: The HPN-QOL((c)), is a valid tool for measurement of QOL in patients on HPN, to be used in the clinical practice as well as in research.

Objective: The aim of this study was to evaluate kidney function outcome in adults on home parenteral nutrition (HPN) for chronic intestinal failure using the newly recommended equations for estimated glomerular filtration rate (eGFR) assessment in clinical practice. Methods: This was an observational study with 72 patients. Clinical and biochemical parameters were collected at initiation of HPN (retrospective baseline [BL]), at inclusion in the study (cross-sectional [CS]), and at the end of a 30-mo prospective follow-up (Fup). The eGFR (mL/min/1.73 m2 body surface) was calculated by the Chronic Kidney Disease Epidemiology Collaboration creatinine and categorized as normal, mildly decreased (MDKF), and chronic kidney disease (CKD) when ≥90, 60 to 89, and

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.

Summary 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.

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.

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