Association between chronic intestinal failure etiology and eGFR trajectory in adults receiving home parenteral nutrition: A retrospective longitudinal cohort study.

BACKGROUND: Patients with chronic intestinal failure (CIF) are at increased risk of developing renal impairment. The aim of this study was to evaluate the occurrence of chronic kidney disease (CKD) in patients dependent on home parenteral nutrition (HPN) and assess risk factors for renal impairment, including patients with all mechanisms of CIF. METHODS: This was a cohort study of patients initiated on HPN between March 1, 2015, and March 1, 2020, at a national UK IF Reference Centre. Patients were followed from their first discharge with HPN until HPN cessation or the end of follow-up on December 31, 2021. RESULTS: There were 357 patients included in the analysis. Median follow-up time was 4.7 years. At baseline, >40% of patients had renal impairment, with 15.4% fulfilling the criteria for CKD. Mean estimated glomerular filtration rate (eGFR) decreased significantly during the first year after initiation of HPN from 93.32 ml/min/1.73 m2 to 86.30 ml/min/1.73 m2 at the first year of follow-up (P = 0.002), with sequential stabilization of renal function. Increased age at HPN initiation and renal impairment at baseline were associated with decreased eGFR. By the end of follow-up, 6.7% patients developed renal calculi and 26.1% fulfilled the criteria for CKD. CONCLUSION: This is the largest study of renal function in patients receiving long-term HPN. After the first year following HPN initiation, the rate of decline in eGFR was similar to that expected in the general population. These findings should reassure patients and clinicians that close monitoring of renal function can lead to good outcomes.

Protein-based oral rehydration solutions for patients with an ileostomy: A randomised, double-blinded crossover study.

BACKGROUND & AIM: Patients with an ileostomy are at increased risk of dehydration and sodium depletion. Treatments recommended may include oral rehydration solutions (ORS). We aimed to investigate if protein type or protein hydrolysation affects absorption from iso-osmolar ORS in patients with an ileostomy. METHODS: This was a randomised, double-blinded, active comparator-controlled 3 × 3 crossover intervention study. We developed three protein-based ORS with whey protein isolate, caseinate or whey protein hydrolysate. The solutions contained 40-48 g protein/L, 34-45 mmol sodium/L and had an osmolality of 248-270 mOsm/kg. The patients ingested 500 mL/d. The study consisted of three 4-week periods with a >2-week washout between each intervention. The primary outcome was wet-weight ileostomy output. Ileostomy output and urine were collected for a 24-h period before and after each intervention. Additionally, blood sampling, dietary records, muscle-strength tests, bioimpedance analyses, questionnaires and psychometric tests were conducted. RESULTS: We included 14 patients, of whom 13 completed at least one intervention. Ten patients completed all three interventions. Wet-weight ileostomy output did not change following either of the three interventions and did not differ between interventions (p = 0.38). A cluster of statistically significant improvements related to absorption was observed following the intake of whey protein isolate ORS, including decreased faecal losses of energy (-365 kJ/d, 95% confidence interval (CI), -643 to -87, p = 0.012), potassium (-7.8 mmol/L, 95%CI, -12.0 to -3.6, p = 0.001), magnesium (-4.0 mmol/L, 95%CI, -7.4 to -0.7, p = 0.020), improved plasma aldosterone (-4674 pmol/L 95%CI, -8536 to -812, p = 0.019), estimated glomerular filtration rate (eGFR) (2.8 mL/min/1.73 m2, 95%CI, 0.3 to 5.4, p = 0.03) and CO2 (1.7 mmol/L 95%CI, 0.1 to 3.3, p = 0.04). CONCLUSION: Ingestion of 500 mL/d of iso-osmolar solutions containing either whey protein isolate, caseinate or whey protein hydrolysate for four weeks resulted in unchanged and comparable ileostomy outputs in patients with an ileostomy. Following whey protein isolate ORS, we observed discrete improvements in a series of absorption proxies in both faeces and blood, indicating increased absorption. The protein-based ORS were safe and well-tolerated. Treatments should be tailored to each patient, and future studies are warranted to explore treatment-effect heterogeneity and whether different compositions or doses of ORS can improve absorption and nutritional status in patients with an ileostomy. GOV STUDY IDENTIFIER: NCT04141826.

A look inside the gut as a clue to nutritional status.

PURPOSE OF REVIEW: Malabsorption and malnutrition are common gastrointestinal manifestations clinicians face, requiring diagnostic workup for effective diagnosis and management of the underlying cause. This review discusses recent advances in diagnostic approaches to malabsorption and maldigestion of macronutrients - lipids, proteins, and carbohydrates. We highlight underrecognized causes, available testing modalities, and ongoing diagnostic unmet needs. RECENT FINDINGS: Innovations in the diagnostic landscape are enhancing our understanding of malabsorption syndromes. Stool collection and handling is uncomfortable and commonly avoided. The objective quantification of stool lipids, bile acids, and gut enzymes is therefore underused in the diagnosis and management of common disorders such as exocrine pancreatic insufficiency, bile acid diarrhea, protein-losing enteropathy, and more. We review the recent advancements in spot quantification of stool fat and bile acid content, endoscopic imaging techniques such as endocytoscopy, confocal laser endomicroscopy, and optical coherence tomography and the future place in clinical practice. SUMMARY: Malabsorption and maldigestion represent significant challenges in clinical nutrition and gastroenterology. Through the integration of advanced diagnostic techniques, clinicians will be better equipped to tailor therapy and monitor treatment response, ultimately improving patient health outcomes. This review underscores the critical role of innovative diagnostic tools in accurately detecting and effectively managing gastrointestinal disorders linked to nutritional status.

Patients' and caregivers' perspective on challenges and outcomes with tube feeding: Analysis of home enteral nutrition survey data.

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.

A scoping review of parenteral requirements (macronutrients, fluid, electrolytes and micronutrients) in adults with chronic intestinal failure receiving home parenteral nutrition.

INTRODUCTION: Home parenteral nutrition (HPN) prescriptions should be individualised in adults with chronic intestinal failure (IF). The aims of the review were to explore HPN requirements and available guidelines and to determine whether adults (≥ 18 years) receive recommended parenteral nutrient doses. METHODS: Online databases searches identified empirical evidence (excluding case-reports), reviews and guidelines (Published 2006-2024 in English language). Additional reference lists were hand-searched. Older studies, cited in national guidelines were highlighted to map evidence source. Two reviewers screened 1660 articles independently, with 98 full articles assessed and 78 articles included (of which 35 were clinical studies). Citation tracking identified 12 older studies. RESULTS: A lack of evidence was found assessing parenteral macronutrient (amounts and ratios to meet energy needs), fluid and electrolyte requirements. For micronutrients, 20 case series reported serum levels as biomarkers of adequacy (36 individual micronutrient levels reported). Studies reported levels below (27 out of 33) and above (24 out of 26) reference ranges for single micronutrients, with associated factors explored in 11 studies. Guidelines stated recommended parenteral dosages. Twenty-four studies reported variable proportions of participants receiving HPN dosages outside of guideline recommendations. When associated factors were assessed, two studies showed nutrient variation with type of HPN administered (multichamber or individually compounded bags). Five studies considered pathophysiological IF classification, with patients with short bowel more likely to require individualised HPN and more fluid and sodium. CONCLUSIONS: This review highlights substantial evidence gaps in our understanding of the parenteral nutritional requirements of adult receiving HPN. The conclusions drawn were limited by temporal bias, small samples sizes, and poor reporting of confounders and dose. Optimal HPN nutrient dose still need to be determined to aid clinical decision-making and further research should explore characteristics influencing HPN prescribing to refine dosing recommendations.

Outcomes for central venous catheter repair in patients receiving long-term home parenteral support: A descriptive cohort study.

BACKGROUND: Central venous catheter (CVC) complications are frequently reported in patients receiving home parenteral support (HPS). Compromised CVC integrity or breakage is one such issue. Repairing such breakages can potentially avoid costly and risky catheter replacements. METHODS: We completed a retrospective descriptive cohort study using a prospectively maintained data set, in a national UK intestinal failure reference center. Repair success, CVC longevity, and catheter-related bloodstream infection (CRBSI) rates after repair were the primary outcome measures. RESULTS: A total of 763 patients received HPS. There were 137 CVC repairs: 115 (84%) tunneled CVCs and 22 peripherally inserted central catheters (PICCs) attempted in 72 patients. Of the 137 attempts at CVC repair, 120 (88%) were deemed to be successful, allowing a median duration of subsequent CVC use of 336 days following repair (range 3-1696 days), which equates to 99,602 catheter days of HPS infusion. Three patients had a CRBSI within 90 days of repair, and patients required admission to the hospital for refeeding on 14 occasions following repair, such that hospitalization was avoided in 103/120 (86%) occasions following successful CVC repair. There was no increase in the recorded rate of CRBSIs in patients undergoing CVC repair compared with the CRBSI rates of all HPS-dependent patients under our care during the study period (0.03 vs 0.344/1000 catheter days, respectively). CONCLUSION: This is the largest single-center experience to demonstrate that CVCs, including PICCs, used for the administration of HPS can be safely repaired, prolonging CVC longevity without leading to an increased risk of CRBSI.

The relationships between sarcopenia, frailty, bioelectrical impedance analysis, and anthropometry in patients with type two intestinal failure.

INTRODUCTION: SARC-F (Strength, Assistance in walking, Rise from a chair, Climb stairs and Falls) is a validated screening questionnaire for rapidly assessing sarcopenia in older adults with a variety of diseases while the Rockwood clinical frailty scale (RCFS) is a commonly used tool for assessing frailty in older adults. However, there are no data regarding the use of these tools in patients with type two intestinal failure (T2IF). We aimed to compare SARC-F and RCFS to bioelectrical impedance analysis (BIA) and anthropometry in patients with T2IF. METHODS: In this cross-sectional study, patients with T2IF admitted to a national U.K. reference centre had SARC-F, RCFS, BIA and anthropometry performed. A SARC-F score ≥4 indicated potential sarcopenia while a RCFS ≥5 indicated frailty. Statistical analyses were conducted to assess correlation, concordance and differences between measurements. RESULTS: The study comprised 76 patients, of whom 59.2 % were female, with a mean age of 56.5 years ± 16. Seventy percent of patients had short bowel syndrome as an underlying cause of IF. Thirty eight percent of patients had a SARC-F ≥4 and 20 % had a RCFS ≥5. SARC-F was correlated with RCFS (rs 0.43, p < 0.001), phase angle (PA) (rs -0.55, p < 0.001) and hand grip strength (HGS) (rs -0.34, p 0.004). RCFS was correlated with HGS (rs -0.31, p 0.009). Significant differences emerged between PA (p 0.002) and HGS (p 0.003) values in patients with SARC-F scores ≥4 and < 4 and with HGS for patients with RCFS ≥5 and < 5 (p 0.01). Patients with SARC-F scores ≥4 had longer lengths of stays than those with scores <4 (p 0.002). Compared to PA and HGS, SARC-F had sensitivities of 39 % and 37 % and specificities of 92 % and 73 % respectively. CONCLUSION: We demonstrated SARC-F is correlated with PA and HGS with comparable sensitivity and specificity to its original use in older individuals. There is a role for SARC-F as a means of rapidly assessing muscle quality and function in hospitalised patients with IF with RCFS providing some useful information about muscle function.

Emerging uses of glucagon-like peptide 1 (GLP-1) receptor agonists following ileal resection: literature review and case examples.

Following ileal resection, the combination of severe bile acid (BA) malabsorption, rapid small bowel transit and unrestricted upper gastrointestinal (GI) secretion results in severe diarrhoea that can prove refractory to pharmacological therapies. While established therapies, including BA sequestrants and antidiarrhoeal drugs seek to ameliorate symptoms, they do not target the underlying pathophysiological mechanisms in this patient group. Their use can also be limited by both intolerance and adverse effects. The novel use of glucagon-like peptide-1 (GLP-1) receptor agonists (RAs) in these patients may allow restoration of the physiological negative feedback mechanisms lost in ileal resection and reduce diarrhoea by prolonging small bowel transit time, limiting upper GI secretions and perhaps by inhibiting hepatic BA synthesis. While recent evidence supports the use of GLP-1 RAs as a safe and effective therapy for bile acid diarrhoea (BAD), it remains uncertain whether those with severe BAD and subsequent short bowel syndrome secondary to extensive ileal resection will benefit. Here, we present three cases of severe diarrhoea secondary to extensive ileal resection in which the use of the GLP-1 RA, liraglutide, was well tolerated and resulted in an objective improvement in diarrhoeal symptoms. We further provide a narrative review of the emerging evidence base supporting the use of GLP therapies in this challenging condition.

A multi-national survey of experience and attitudes towards managing catheter related blood stream infections for home parenteral nutrition.

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.

Artificial intelligence & clinical nutrition: What the future might have in store.

Artificial Intelligence (AI) is a rapidly emerging technology in healthcare that has the potential to revolutionise clinical nutrition. AI can assist in analysing complex data, interpreting medical images, and providing personalised nutrition interventions for patients. Clinical nutrition is a critical aspect of patient care, and AI can help clinicians make more informed decisions regarding patients' nutritional requirements, disease prevention, and management. AI algorithms can analyse large datasets to identify novel associations between diet and disease outcomes, enabling clinicians to make evidence-based nutritional recommendations. AI-powered devices and applications can also assist in tracking dietary intake, providing feedback, and motivating patients towards healthier food choices. However, the adoption of AI in clinical nutrition raises several ethical and regulatory concerns, such as data privacy and bias. Further research is needed to assess the clinical effectiveness and safety of AI-powered nutrition interventions. In conclusion, AI has the potential to transform clinical nutrition, but its integration into clinical practice should be carefully monitored to ensure patient safety and benefit. This article discusses the current and future applications of AI in clinical nutrition and highlights its potential benefits.

ESPEN guideline on chronic intestinal failure in adults - Update 2023.

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.

Experience and opinions relating to pregnancy in patients with chronic intestinal failure: an international survey.

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.

Disease Recurrence and Long-term Outcomes Following the Development of Intestinal Failure in Crohn's Disease: Over 20 Years of Experience from a National Reference Centre.

BACKGROUND AND AIMS: Intestinal failure [IF] is a recognised complication of Crohn's disease [CD]. The aim of this study was to identify factors predicting the development and recurrence of CD in patients with IF [CD-IF], and their long-term outcomes. METHODS: This was a cohort study of adults with CD-IF admitted to a national UK IF reference centre between 2000 and 2021. Patients were followed from discharge with home parenteral nutrition [HPN] until death or February 28, 2021. RESULTS: In all, 124 patients were included; 47 [37.9%] changed disease location and 55 [44.4%] changed disease behaviour between CD and CD-IF diagnosis, with increased upper gastrointestinal involvement [4.0% vs 22.6% patients], p <0.001. Following IF diagnosis, 29/124 [23.4%] patients commenced CD prophylactic medical therapy; 18 [62.1%] had a history of stricturing or penetrating small bowel disease; and nine [31.0%] had ileocolonic phenotype brought back into continuity. The cumulative incidence of disease recurrence was 2.4% at 1 year, 16.3% at 5 years and 27.2% at 10 years; colon-in-continuity and prophylactic treatment were associated with an increased likelihood of disease recurrence. Catheter-related bloodstream infection [CRBSI] rate was 0.32 episodes/1000 catheter days, with no association between medical therapy and CRBSI rate. CONCLUSIONS: This is the largest series reporting disease behaviour and long-term outcomes in CD-IF and the first describing prophylactic therapy use. The incidence of disease recurrence was low. Immunosuppressive therapy appears to be safe in HPN-dependent patients with no increased risk of CRBSI. The management of CD-IF needs to be tailored to the patient's surgical disease history alongside disease phenotype.

Oxygen utilisation in patients on prolonged parenteral nutrition; a case-controlled study.

BACKGROUND: Parenteral nutrition (PN) deficient in mitochondrial substrates and thiamine may lead to acidosis. This, combined with fatigue seen in patients with intestinal failure (IF), may suggest suboptimal oxidative metabolism. We therefore studied oxygen utilisation in otherwise apparently well-nourished individuals with intestinal failure receiving long term PN. METHODS: This was a retrospective analysis conducted in a tertiary IF institution, from 2010 to 2019, comparing treadmill/bicycle cardiopulmonary exercise test (CPET) derived variables including peak oxygen consumption (VO2 peak), anaerobic threshold (AT) and ventilatory efficiency (minute ventilation (VE)/CO2 output (VCO2) of patients with IF (cases) to those without (controls), matched in a 1:2 ratio for age ( ± 3 years), gender, use of beta-blockers and physiology parameters of p-POSSUM score ( ± 5). All subjects were free of sepsis and metastatic malignancy. Mann-Whitney or Student's t-test for continuous and Fisher's exact or chi-squared test for categorical variables were used as appropriate. Data shown represent mean or median values. RESULTS: Participants (31 cases, 62 controls) were comparable in age (65.4 vs. 65.3, p = 0.98); p-POSSUM parameters (18.0 vs. 17.0, p = 0.45); gender (p = 1.00); smoking status (p = 0.52); use of beta-blockers (p = 1.00) and ≤10 mg/day of oral steroids (p = 0.34). Participants had been on PN for 11.0 (6.0-24.0) months and were adequately nourished (requirements 27.6 kcal/kg/day, replacement 23.5 kcal/kg/day). No differences were found between VO2 peak (15.2 vs. 14.6 ml/kg/min, p = 0.96), AT (10.4 vs. 11.0 ml/kg/min, p = 0.44) and VE/VCO2 (33.0 vs. 33.0, p = 0.96) of the examined groups. CONCLUSION: Patients with intestinal failure receiving PN who are apparently well-nourished also appear to have normal oxygen utilisation, suggesting alternative causes for fatigue. More studies will be required to determine whether CPET could reliably be used to assess perioperative risk in this group of patients.

COVID-19 infection in patients on long-term home parenteral nutrition for chronic intestinal failure.

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.

Sodium depletion and secondary hyperaldosteronism in outpatients with an ileostomy: a cross-sectional study.

OBJECTIVE: Patients with an ileostomy may experience postoperative electrolyte derangement and dehydration but are presumed to stabilise thereafter. We aimed to investigate the prevalence of sodium depletion in stable outpatients with an ileostomy and applied established methods to estimate their fluid status. METHODS: We invited 178 patients with an ileostomy through a region-wide Quality-of-Life-survey to undergo outpatient evaluation of their sodium and fluid status. The patients delivered urine and blood samples, had bioelectrical impedance analysis performed and answered a questionnaire regarding dietary habits. RESULTS: Out of 178 invitees, 49 patients with an ileostomy were included; 22 patients (45%, 95% CI, 31-59%) had unmeasurably low urinary sodium excretion (<20 mmol/L), indicative of chronic sodium depletion, and 26% (95% CI, 16-41%) had plasma aldosterone levels above the reference value. Patients with unmeasurably low urinary sodium excretion had low estimated glomerular filtration rates (median 76, IQR 63-89, mL/min/1.73m2) and low venous blood plasma CO2 (median 24, IQR 21-26, mmol/L), indicative of chronic renal impairment and metabolic acidosis. Bioelectrical impedance analysis, plasma osmolality, creatinine and sodium values were not informative in determining sodium status in this population. CONCLUSION: A high proportion of patients with an ileostomy may be chronically sodium depleted, indicated by absent urinary sodium excretion, secondary hyperaldosteronism and chronic renal impairment, despite normal standard biochemical tests. Sodium depletion may adversely affect longstanding renal function. Future studies should investigate methods to estimate and monitor fluid status and aim to develop treatments to improve sodium depletion and dehydration in patients with an ileostomy.IMPACT AND PRACTICE RELEVANCE STATEMENTSodium depletion in otherwise healthy persons with an ileostomy was identified in a few publications from the 1980s. The magnitude of the problem has not been demonstrated before. The present study quantifies the degree of sodium depletion and secondary hyperaldosteronism in this group, and the results may help guide clinicians to optimise treatment. Sodium depletion is easily assessed with a urine sample, and sequelae may possibly be avoided if sodium depletion is detected early and treated. This could ultimately help increase the quality of life in patients with an ileostomy.

Survey of healthcare professionals' experiences of care delivery in patients with chronic intestinal failure: ATLAS of Variance.

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.

Investigating the Relationship between Home Parenteral Support and Needs-Based Quality of Life in Patients with Chronic Intestinal Failure: A National Multi-Centre Longitudinal Cohort Study.

Home parenteral support (HPS) is an essential but potentially burdensome treatment that can affect quality of life (QoL). The aims of this longitudinal study were to understand whether any changes in HPS over time were associated with QoL. The Parenteral Nutrition Impact Questionnaire (PNIQ) was used, and data were collected on HPS prescribed at three time points. Data were analysed using multi-level mixed regression models presented as effect size and were adjusted for confounders. Study recruited 572 participants from 15 sites. Of these, 201 and 145 completed surveys at second and third time-points, respectively. PNIQ score was out of 20 with a higher score indicating poorer QoL. Any reduction in HPS infusions per week was associated with an improved PNIQ score of -1.10 (95% CI -2.17, -0.02) unadjusted and -1.34 (95% CI -2.45, -0.24) adjusted. Per day change to the number of infusions per week was associated with a change in the PNIQ score of 0.32 (95% CI -0.15, 0.80) unadjusted and 0.34 (95% CI -0.17, 0.85) adjusted. This is the largest national study to demonstrate improvements in QoL associated with HPS reduction over time using an HPS-specific and patient-centric tool, adding unique data for use of therapies in intestinal failure.

ESPEN practical guideline: Home parenteral nutrition.

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.

Patients, family members and healthcare professionals' top ten research priorities for adults receiving home parenteral nutrition for malignant or benign disease.

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.