Search

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.

Background and Aims This survey of centres caring for patients on home parenteral nutrition (HPN) was conducted to assess the impact of the COVID‐19 crisis on the management of these patients in terms of provision of care, monitoring, regular follow‐up and any changes to service infrastructure. Methods A survey was devised and publically published on the REDCap database management system with individual centres responding to a public link. Results 78 adult and paediatric centres worldwide contributed to the survey, representing at least 3500 patients’ experience. Centres (n/78 unless otherwise specified, %) reported infrastructure maintenance for PN bag deliveries to patients (60, 76.92%) or delivery of ancillary items (dressing packs, gloves, bungs) (57, 73.08%) home delivery and HPN administration (65, 83.33%) but homecare nurse shortages (25, 32.05%). Follow up of routine HPN patients changed to either all telemed or mixed with emergency clinic review (70, 89.74%). In 26 centres (33.33%), new discharges on HPN for benign conditions were reduced or stopped. Based on clinical history the centres reported psychological distress for patients (52, 66.67%) with anxiety, worry, concern and apprehension reported most frequently (37/52, 71.15%) but also fear (10/52, 19.23%), depression (5/52, 9.62%) and issues related to isolation/confinement (12/52, 23.08%). Conclusions The COVID‐19 pandemic was reported by clinicians to have had a far reaching adverse impact on patients on home parenteral nutrition, especially their safety in terms of provision of PPE, PN bags, available nursing staff and psychological well‐being. Healthcare systems responded to the challenge presented with new ways of working. This article is protected by copyright. All rights reserved

An appropriate patient description is mandatory in clinical practice and as criteria for patient inclusion in research protocols. The classification of adult patients with an SBS is based on anatomical, evolutional, pathophysiological, and clinical categories and could be considered as the “SBS-ID” to report patient characteristics in clinical practice and in research studies.

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/m2), 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: 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.

Contains fulltext : 229351.pdf (Publisher’s version ) (Open Access) BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) and enteric dysmotility (ED) are small intestinal motility disorders defined by radiological and manometric criteria. In the absence of consensus guidelines, we surveyed opinions on the diagnosis and management of CIPO and ED among experts from different countries. METHODS: A survey questionnaire was circulated electronically to members of the European society for Clinical Nutrition and Metabolism, European Society of Neurogastroenterology and Motility, and United European Gastroenterology. Only responses from participants completing all required components were included. KEY RESULTS: Of 154 participants, 93% agreed that CIPO and ED should be classified separately. Overall, 73% reported an increasing incidence of CIPO and ED, with hypermobile Ehlers-Danlos Syndrome the group with the largest increase in referrals (37%), particularly in the UK (P 50% cases) and full thickness biopsies were reported to seldom influence medical treatment, nutritional management, and prognosis. Respondents reported that very few treatments are useful for most patients, with bacterial overgrowth treatment, prucalopride, and psychological therapies felt to be the most useful. While only 23% of clinicians felt that parenteral nutrition (PN) improves gastrointestinal symptoms in >50% of cases, 68% reported PN dependency at 5 years in the majority of cases. CONCLUSIONS AND INFERENCES: These data highlight the difficulties with diagnosing and managing CIPO and ED and underscore the urgent need for international, multidisciplinary, clinical practice guidelines.

Summary The management of patients with chronic intestinal failure requiring home parenteral nutrition has been and will continue to be impaired during the SARS-CoV-2 pandemic. Multidisciplinary intestinal failure teams may have to adapt their clinical approaches to home care, outpatient care as well as hospital admission and discharge in order to keep this vulnerable group of patients as safe and well as possible during the unprecedented challenges that countries are facing during the pandemic. Equally, it is important that expert advice from intestinal failure teams is available when home parenteral nutrition (HPN)-dependent patients require admission with SARS-CoV-2 infection. The Home Artificial Nutrition & Chronic Intestinal Failure Special Interest Group of the European Society for Clinical Nutrition and Metabolism (ESPEN) has developed a position paper to outline areas for intestinal failure teams to consider when managing patients with chronic intestinal failure during the SARS-CoV-2 pandemic.

Background and aims: The prevalence of malnutrition is over 70% in advanced cancer patients and impacts negatively on survival and quality of life. Artificial nutrition can be integrated into a home palliative care program. This observational study aims to describe the criteria for identifying the cancer patients that could benefit from home artificial nutrition (HAN) and to evaluate its impact on survival and performance status. Methods: The selection criteria for patient's eligibility to HAN were: Karnofsky Performance Status (KPS) ≥40, life expectancy ≥6 weeks, inadequate caloric intake ± malnutrition, suitable psycho-physical conditions and informed consent. The access route for nutritional therapy (home parenteral nutrition, HPN; home enteral nutrition, HEN) was chosen according to the ESPEN Guidelines. The parameters considered were: primary site of the tumor; oral food intake; nutritional status; stage of cachexia; fluid, energy and protein supplied by HAN; survival. Results: From 1990 to 2019, 43,474 cancer patients were assisted at home in Bologna (Italy). HAN started in 969 patients (2.2% of total patients, 571 men and 398 women, mean age 65.7 ± 12.7 years): HPN in 629 patients (64.9%), with gastrointestinal obstruction as the main indication; HEN in 340 patients (35.1%), with dysphagia as the main indication. Considering the 890 deceased patients, the mean survival after the start of HAN was 18.3 weeks and 649 patients (72.9%) survived more than 6 weeks. The mean survival was higher in HEN (22.1 weeks) compared to HPN patients (16.1 weeks) (p < .001). After one month, KPS was unchanged in 649 (67.0%), increased in 232 (23.9%) and decreased in 88 patients (9.1%). The mean KPS increased in patients starting HAN in pre-cachexia and cachexia (p < .001). Cachexia and refractory cachexia at the entry were associated with a reduced survival [odds ratio: 1.5 and 2.3 respectively, p < .001 for both condition] respect to pre-cachexia. Conclusions: The selection criteria allow the identification of the patient who can take advantage of HAN. HAN can be effective in avoiding death from malnutrition in 73% of patients, and in maintaining or improving the KPS at one month in 90% of cases. The benefits provided by HAN on survival and performance status depend on the cachexia degree at the entry.

In 2019 the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and the Italian Society of Artificial Nutrition and Metabolism (SINPE) created a joint panel of experts with the aim of preparing an official statement on transition in Chronic Intestinal Failure (CIF). The transition from pediatric to adult care has a key role in managing all chronic diseases and in optimizing the compliance to care. Thus SIGENP and SINPE, in light of the growing number of patients with IF who need long-term Parenteral Nutrition (PN) and multidisciplinary rehabilitation programs throughout adulthood, shared a common protocol to provide an accurate and timely process of transition from pediatric to adult centers for CIF. The main objectives of the transition process for CIF can be summarized as the so-called "acronym of the 5 M": 1)Motivate independent choices which are characteristics of the adult world; 2)Move towards adult goals (e.g. self-management of his pathology and sexual issues); 3)Maintain the habitual mode of care; 4) Minimize the difficulties involved in the transition process and 5)Modulate the length of the transition so as to fully share with the adult's team the children's peculiarities.

Background:In multiple clinical studies, teduglutide reduced parenteral support (PS) with a consistent safety profile in adults with short bowel syndrome–associated intestinal failure (SBS–IF). The objective of this study was to assess adverse events (AEs) from a pooled data set.Methods:Safety data from four prospective clinical trials of teduglutide in patients with SBS–IF were assimilated. AEs were evaluated in patient groups based on treatment received in each study and in populations stratified to create distinct subgroups based on aetiology, bowel anatomy and baseline PS volume requirements.Results:Safety data are reported for up to 2.5 years, totalling 222 person-years exposure to teduglutide. In most patients, AEs were reported as mild or moderate in severity in all patient groups and occurred at comparable rates between patients who received teduglutide or placebo. Several common gastrointestinal AEs, including abdominal pain, nausea and abdominal distension, were reported more frequently earlier in the course of treatment, with their frequency declining over time. Fewer gastrointestinal AEs were reported in patients with vascular causes of SBS–IF and patients with most of their colon-in-continuity than in other patient subgroups. Across the patient stratification subgroups, the predominant treatment-emergent AEs for which patients receiving teduglutide had a significantly increased relative risk were abdominal distension and gastrointestinal stoma complication compared with patients receiving placebo.Conclusions:Teduglutide had a safety profile consistent with prior adult data and no new safety concerns were identified. The most frequently reported AEs were gastrointestinal in origin, consistent with the underlying disease condition and intestinotrophic actions of teduglutide.Clinical Trial Registry information:NCT00081458/EudraCT, 2004-000438-35; NCT00798967/EudraCT, 2008-006193-15; NCT00172185/EudraCT, 2004-000439-27; NCT00930644/EudraCT, 2009-011679-65

BACKGROUND AND PURPOSE: Mitochondrial neurogastrointestinal encephalopathy is a rare disorder due to recessive mutations in the thymidine phosphorylase gene, encoding thymidine phosphorylase protein required for mitochondrial DNA replication. Clinical manifestations include gastrointestinal dysmotility and diffuse asymptomatic leukoencephalopathy. This study aimed to elucidate the mechanisms underlying brain leukoencephalopathy in patients with mitochondrial neurogastrointestinal encephalopathy by correlating multimodal neuroradiologic features to postmortem pathology. MATERIALS AND METHODS: Seven patients underwent brain MR imaging, including single-voxel proton MR spectroscopy and diffusion imaging. Absolute concentrations of metabolites calculated by acquiring unsuppressed water spectra at multiple TEs, along with diffusion metrics based on the tensor model, were compared with those of healthy controls using unpaired t tests in multiple white matters regions. Brain postmortem histologic, immunohistochemical, and molecular analyses were performed in 1 patient. RESULTS: All patients showed bilateral and nearly symmetric cerebral white matter hyperintensities on T2-weighted images, extending to the cerebellar white matter and brain stem in 4. White matter, N -acetylaspartate, creatine, and choline concentrations were significantly reduced compared with those in controls, with a prominent increase in the radial water diffusivity component. At postmortem examination, severe fibrosis of brain vessel smooth muscle was evident, along with mitochondrial DNA replication depletion in brain and vascular smooth-muscle and endothelial cells, without neuronal loss, myelin damage, or gliosis. Prominent periependymal cytochrome C oxidase deficiency was also observed. CONCLUSIONS: Vascular functional and histologic alterations account for leukoencephalopathy in mitochondrial neurogastrointestinal encephalopathy. Thymidine toxicity and mitochondrial DNA replication depletion may induce microangiopathy and blood-brain-barrier dysfunction, leading to increased water content in the white matter. Periependymal cytochrome C oxidase deficiency could explain prominent periventricular impairment.

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.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.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 was50% 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.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.

Rationale: Patients with SARS-CoV-2 infection (COVID-19) are potentially at risk of malnutrition because of reduced food intake and catabolism-related inflammation, reduced mobility due to hospitalization, older age and comorbidities. The prevalence and the causes of malnutrition as well as the provided nutrition care were evaluated in all the COVID-19 adults hospitalized in a 3rd level hospital in Italy. Method(s): A one-day audit was carried out recording: age, measured or estimated body weight (BW) and height, BMI (Kg/m2), 30-day weight loss (WL), comorbidities, serum albumin and C-reactive protein (CRP;nv =30, 13% (higher in ICU,p=0.011);WL>=5%, 52% (higher in ICU and RU,p 5: 36% (higher in ICU and lower in RU,p =3, 76% (greater in ICU and RU,p 0.05), respectively. Conclusion(s): Malnutrition risk was present in three-fourth of patients and malnutrition in about one-third. The frequency increased according to the severity of the COVID-19 clinical feature. A very low dietary intake and a very high inflammatory state were present in one-third. Most of the patients were on oral diet alone. Protein intake was appropriate, whereas energy intake was at the lower limit of the recommended amount. Disclosure of Interest: None declaredCopyright © 2020

Background Vaccine hesitancy, the delay in acceptance or refusal of vaccination despite its availability, represents a threat to the diffusion and lethality of infectious diseases. COVID-19 vaccination has been recommended by national and international organizations in patients with Inflammatory Bowel Diseases (IBD). The aim of the study was to evaluate COVID-19 vaccine willingness and hesitancy in a population of Italian patients with IBD, and their determinants. Methods In February 2021 an online questionnaire focused on COVID-19 vaccine was proposed to a representative sample of Italian IBD patients. Socio-demographic characteristics, IBD features, lifestyle, perception of COVID-19, general attitude towards vaccinations and to COVID-19 vaccines were investigated. Patients were divided into willing, hesitant and refusive towards COVID-19 vaccine. The hesitation reasons were investigated. The associations between baseline characteristics and willingness (determinants) were evaluated by calculating crude and adjusted Odds Ratio (AdjOR) with 95% confidence intervals (CI). Results The baseline characteristics are shown below. 1252 surveys were collected. 1005 (80.3%), 222 (18.1%) and 33 (2.63 %) patients were defined as willing, hesitant and refusive, respectively. Concerns for vaccine adverse effects constituted the main reason for refusal (73.4%) (Figure). 49.1% of hesitant patients reported that presence of IBD exerted an influence on their answer. Among the patients willing to be vaccinated whenever possible, 78.2% (786) thought that their IBD represented a valid motivation to access vaccination with priority. Willingness to COVID-19 vaccine was significantly associated with adherence to previous vaccinations (AdjOR 17.6, 95% CI 11.4–27.2), male gender (1.68 95% CI 1.16–2.43), graduation degree (1.48, 95% CI 1.03–2.13), perceived higher risk of COVID-19 due to IBD (1.47, 95% CI 1.05–2.08), alcohol intake (1.69, 95% CI 1.16–2.45). Conversely, the hesitancy was significantly associated with use of complementary and alternative medicines (0.58, 95% CI 0.36–0.92). Notably, 54.5% of patients were under the impression to have a higher risk of COVID-19 and 31.8% a more severe course due to their IBD. Conclusion Most IBD patients would accept COVID-19 vaccines, though one out of five is hesitant to date, principally due to fear of adverse events. The knowledge of determinants and reasons for COVID-19 vaccination acceptance could be a key element in developing targeted communication strategies to address vaccine hesitancy.

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

Introduction Severe gastrointestinal dysmotility (GID) can be sub-classified into Chronic Intestinal Pseudo-obstruction (CIPO) and Enteric Dysmotility (ED) subtypes. We surveyed current opinions on the diagnosis and management of GID amongst experts from different countries. Methods An survey questionnaire developed by the European society for Clinical Nutrition and Metabolism (ESPEN) was circulated electronically to members of ESPEN, European Society of Neurogastroenterology and Motility, and United European Gastroenterology. Only participants that completed all required components were included in the analysis. Results Of 154 included participants, 82% were European, the majority were attending clinicians/professors (85%), based at either national/regional referral centres and/or academic institutions (87%). Almost all (93%) agreed that CIPO and ED should be classed separately. Most (73%), reported increased incidence of GID, with 69% reporting an increase in ED. GID associated with hypermobile Ehlers-Danlos Syndrome was the group with the largest increase in referrals (37%), however this trend was driven by observations from UK participants only (P 5 years according to 16%; ED: by >5 years according to 19%). Moreover, by the time of diagnosis, >10% of patients have had inappropriate operations according to 82% of clinicians. Small Bowel Manometry, a test mandated to diagnose ED, is surprisingly never used by 44%, and is only used in >50% cases by 21%. Full thickness biopsies are usually requested from planned/previous resections (33%) or when the diagnosis is unclear (43%), but seldom change medical treatment (12%), nutritional management (16%) and prognosis (25%). Very few treatments are useful for >50% of patients, with antibiotics for SIBO, prucalopride, and psychology felt to be the most useful. Parenteral Nutrition (PN) rarely leads to improvement in symptoms (28%), and is associated with PN dependency at 5-years according to the majority (56%). Conclusion These data highlight the difficulties with diagnosing and managing GID, even in ‘expert’ hands, and inform the urgent need for international, multidisciplinary, clinical practice guidelines.

Item does not contain fulltext BACKGROUND: Hepatic disturbances in the context of intestinal failure and parenteral nutrition (PN) are frequently encountered and carry a significant burden of morbidity and sometimes mortality. The term intestinal failure-associated liver disease (IFALD) refers to liver injury due to intestinal failure and associated PN, in the absence of another evident cause of liver disease, encompassing a spectrum of conditions from deranged liver enzymes, steatosis/ steatohepatitis, cholestasis as well as progressive fibrosis, cirrhosis and end-stage liver disease. AIMS: To present an up to date perspective on the diagnosis/definition, aetiologies and subsequent management of IFALD and to explore future consideration for the condition, including pharmacological therapies RESULTS: In adults using long-term PN for benign chronic intestinal failure, 1%-4% of all deaths are attributed to IFALD. The aetiology of IFALD is multifactorial and can be broadly divided into nutritional factors (eg lipid emulsion type) and patient-related factors (eg remaining bowel anatomy). Given its multifaceted aetiology, the management of IFALD requires clinicians to investigate a number of factors simultaneously. Patients with progressive liver disease should be considered for combined liver-intestine transplantation, although multivisceral grafts have a worse prognosis. However, there is no established non-invasive method to identify progressive IFALD such that liver biopsy, where appropriate, remains the gold standard. CONCLUSION: A widely accepted definition of IFALD would aid in diagnosis, monitoring and subsequent management. Management can be complex with a number of factors to consider. In the future, dedicated pharmacological interventions may become more prominent in the management of IFALD.

Intestinal failure-associated liver disease (IFALD) is characterized by either liver steatosis or cholestasis and may develop in patients on long-term home parenteral nutrition for chronic intestinal failure. The pathogenesis of IFALD is multifactorial and includes gastrointestinal disease-related, parenteral nutrition-related, and systemic-related factors. Alteration of bile acid enterohepatic circulation, gut microbiome, and intestinal permeability, seem to be the main mechanisms. Patients forced to a total oral fasting regimen are at greater risk. Parenteral nutrition overfeeding and/or of soybean-based lipid emulsion may be contributing factors. Prevention and treatment are based on avoiding and promptly treating all the risk factors.

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.” SBS is the main cause of chronic intestinal failure, 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. The prevalence of SBS has been estimated to range from 1.5 to 13 cases/million inhabitants. The classification of SBS is based on anatomical (length and anatomy of the residual bowel), evolutional (early, adaptation, and maintenance stages), pathophysiological (with or without a colon in continuity), and clinical (type and volume of the IVS) categories. The described classification could be considered as the “SBS-ID” to be used to report patient characteristics either in clinical practice or in research studies.

The European Society for Clinical Nutrition and Metabolism defined intestinal failure (IF) as "the reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth". IF is classified as type 1-acute, type 2-prolonged acute and type 3-chronic IF. A short bowel syndrome (SBS) due to the intestinal malabsorption associated with a functional small intestine length of less than 200 cm is the most frequent mechanism of IF. SBS is a difficult and multifaced disease. Complications due to SBS itself and to treatments, such as long term home parenteral nutrition, can adversely affect the patient outcome. The care of SBS requires complex technologies and multidisciplinary and multiprofessional activity and expertise. Patient outcome is strongly dependent on care and support from an expert specialist team. This paper focuses on the aspects of the pathophysiology and on the complications of SBS, which are most relevant in the clinical practice, such as intestinal failure associated liver disease, renal failure, biliary and renal stones, dehydration and electrolyte depletion, magnesium deficiency and d-lactic acidosis.

Summary Background & aims Catheter-related central vein thrombosis (CRVT) is a severe complication of home parenteral nutrition (HPN) that may be clinically manifest or subclinical. The aims of the study were to prospectively investigate the incidence of CRVT in patients on HPN with benign disease and determine the influence of different variables on this complication. Methods A prospective, multicentre, observational study in the Home Artificial Nutrition-Chronic Intestinal Failure ESPEN group was performed. Patients with benign disease starting HPN or already on HPN after the insertion of a new catheter, were recruited and followed up with Color Doppler Duplex Sonography (CDDS) evaluations at baseline, 1 week, 3, 6 and 12 months after catheter insertion. Fisher's exact test was used to calculate the association of different variables (related to the patient, type of catheter, vascular access, insertion method, catheter care and anticoagulant treatment) with CRVT events. Results Sixty-two patients (31 males, 31 females) aged 50 ± 19 (19–83) years were included and followed for a median 363 days, with an Inter Quartile Range of 180–365 days, and a total of 16,186 catheter-days. Six patients had previous CRVT and 16 had history of thromboembolic disease (pulmonary and mesenteric). Forty one patients were receiving anticoagulant treatment. Fifty two patients had tunneled catheters and 10 implanted ports. Two patients had symptomatic thrombosis at 3 and 12 months of follow-up (2 and 3 weeks after normal routine CDDS evaluation). The incidence of CRVT was 0.045/catheter/year. CRVT was not significantly associated with any of the variables analyzed. Conclusions The incidence of CRVT in patients on HPN for benign disease followed by CDDS is low in the first year of catheterization. We did not observe any case of asymptomatic CRVT. Based on our data, CDDS seems to have low effectiveness as a screening tool for CRVT in asymptomatic patients on HPN with benign disease.

Objectives: The aim of this study was to evaluate the safety and efficacy of home parenteral nutrition (HPN) service in patients with benign chronic intestinal failure (CIF). Methods: This was a 10-y retrospective, non-interventional, multicenter study conducted with adult and pediatric patients with CIF who received HPN service. We analyzed data prospectively collected from a dedicated register by HPN nurses. Results: From January 2002 to December 2011 a total of 794 patients (49.7% male, median age 1 y for children and 57 y for adults) were included in the analysis. Over the 10-y period, 723 central venous catheter (CVC) complications occurred, of which 394 were infectious (54.5%), 297 were mechanical (41.1%), and 32 (3.3%) were defined as CVC-related thrombosis. The complication rate was higher in children (1.11 per patient) than in adults (0.70 per patient). During the observation period, the rates of both infectious and mechanical complications showed a global declining trend and ∼75% of patients had neither infectious nor mechanical CVC complications. HPN efficacy was evaluated in 301 patients with a minimum follow-up of 36 mo. Body mass index and Karnofsky score showed that the median growth significantly increased (P < 0.001) over baseline for adults and pediatric patients in the 0 to 2 age range. Conclusions: The use of a structured register has proved to be a key strategy for monitoring the outcomes of long-term treatment, improving time efficiency, and preventing potential malpractice. To our knowledge, this is largest survey ever documented; the results were consistent despite the heterogeneity of the centers because of duly applied standard rules and protocols.

BACKGROUND & AIMS: Intestinal failure (IF) is 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 is required to maintain health and/or growth". Functionally, it may be classified as type I acute intestinal failure (AIF), type II prolonged AIF and type III chronic intestinal failure (CIF) The ESPEN Workshop on IF was held in Bologna, Italy, on 15-16 October 2017 and the aims of this document were to highlight the current state of the art and future directions for research in IF.METHODS: This paper represents the opinion of experts in the field, based on current evidence. It is not a formal review, but encompasses the current evidence, with emphasis on epidemiology, classification, diagnosis and management.RESULTS: IF is the rarest form of organ failure and can result from a variety of conditions that affect gastrointestinal anatomy and function adversely. Assessment, diagnosis, and short and long-term management involves a multidisciplinary team with diverse expertise in the field that aims to reduce complications, increase life expectancy and improve quality of life in patients.CONCLUSIONS: Both AIF and CIF are relatively rare conditions and most of the published work presents evidence from small, single-centre studies. Much remains to be investigated to improve the diagnosis and management of IF and future studies should rely on multidisciplinary, multicentre and multinational collaborations that gather data from large cohorts of patients. Emphasis should also be placed on partnership with patients, carers and government agencies in order to improve the quality of research that focuses on patient-centred outcomes that will help to improve both outcomes and quality of life in patients with this devastating condition.

Background Clinical and psychosocial outcomes of a multimodal surgical approach for chronic intestinal pseudo-obstruction were analyzed in 24 patients who were followed over a 2- to 12-year period in a single center after surgery or intestinal/multivisceral transplant (CTx). Methods The main reasons for surgery were sub-occlusion in surgery and parenteral nutrition–related irreversible complications with chronic intestinal failure in CTx. Results At the end of follow-up (February 2015), 45.5% of CTx patients were alive: after transplantation, improvement in intestinal function was observed including a tendency toward recovery of oral diet (81.8%) with reduced parenteral nutrition support (36.4%) in the face of significant mortality rates and financial costs (mean, 202.000 euros), frequent hospitalization (mean, 8.8/re-admissions/patient), as well as limited effects on pain or physical wellness. Conclusions Through psychological tests, transplant recipients perceived a significant improvement of mental health and emotional state, showing that emotional factors were more affected than were functional/cognitive impairment and social interaction.

Item does not contain fulltext We recommend that intestinal failure associated liver disease (IFALD) should be diagnosed by the presence of abnormal liver function tests and/or evidence of radiological and/or histological liver abnormalities occurring in an individual with IF, in the absence of another primary parenchymal liver pathology (e.g. viral or autoimmune hepatitis), other hepatotoxic factors (e.g. alcohol/medication) or biliary obstruction. The presence or absence of sepsis should be noted, along with the duration of PN administration. Abnormal liver histology is not mandatory for a diagnosis of IFALD and the decision to perform a liver biopsy should be made on a case-by-case basis, but should be particularly considered in those with a persistent abnormal conjugated bilirubin in the absence of intra or extra-hepatic cholestasis on radiological imaging and/or persistent or worsening hyperbilirubinaemia despite resolution of any underlying sepsis and/or any clinical or radiological features of chronic liver disease. Nutritional approaches aimed at minimising PN overfeeding and optimising oral/enteral nutrition should be instituted to prevent and/or manage IFALD. We further recommend that the lipid administered is limited to less than 1 g/kg/day, and the prescribed omega-6/omega-3 PUFA ratio is reduced wherever possible. For patients with any evidence of progressive hepatic fibrosis or overt liver failure, combined intestinal and liver transplantation should be considered.

BACKGROUND & AIMS: The aim of the study was to evaluate the applicability of the ESPEN 16-category clinical classification of chronic intestinal failure, based on patients' intravenous supplementation (IVS) requirements for energy and fluids, and to evaluate factors associated with those requirements.METHODS: ESPEN members were invited to participate through ESPEN Council representatives. Participating centers enrolled adult patients requiring home parenteral nutrition for chronic intestinal failure on March 1st 2015. The following patient data were recorded though a structured database: sex, age, body weight and height, intestinal failure mechanism, underlying disease, IVS volume and energy need.RESULTS: Sixty-five centers from 22 countries enrolled 2919 patients with benign disease. One half of the patients were distributed in 3 categories of the ESPEN clinical classification. 9% of patients required only fluid and electrolyte supplementation. IVS requirement varied considerably according to the pathophysiological mechanism of intestinal failure. Notably, IVS volume requirement represented loss of intestinal function better than IVS energy requirement. A simplified 8 category classification of chronic intestinal failure was devised, based on two types of IVS (either fluid and electrolyte alone or parenteral nutrition admixture containing energy) and four categories of volume.CONCLUSIONS: Patients' IVS requirements varied widely, supporting the need for a tool to homogenize patient categorization. This study has devised a novel, simplified eight category IVS classification for chronic intestinal failure that will prove useful in both the clinical and research setting when applied together with the underlying pathophysiological mechanism of the patient's intestinal failure.

BACKGROUND: The catheter lock solutions 2% taurolidine and 0.9% saline are both used to prevent catheter-related bloodstream infections (CRBSIs) in home parenteral nutrition patients.AIMS: To compare the effectiveness and safety of taurolidine and saline.METHODS: This multicentre double-blinded trial randomly assigned home parenteral nutrition patients to use either 2% taurolidine or 0.9% saline for 1 year. Patients were stratified in a new catheter group and a pre-existing catheter group. Primary outcome was the rate of CRBSIs/1000 catheter days in the new catheter group and pre-existing catheter group, separately.RESULTS: We randomised 105 patients, of which 102 were analysed as modified intention-to-treat population. In the new catheter group, rates of CRBSIs/1000 catheter days were 0.29 and 1.49 in the taurolidine and saline arm respectively (relative risk, 0.20; 95% CI, 0.04-0.71; P = 0.009). In the pre-existing catheter group, rates of CRBSIs/1000 catheter days were 0.39 and 1.32 in the taurolidine and saline arm respectively (relative risk, 0.30; 95% CI, 0.03-1.82; P = 0.25). Excluding one outlier patient in the taurolidine arm, mean costs per patient were $1865 for taurolidine and $4454 for saline (P = 0.03). Drug-related adverse events were rare and generally mild.CONCLUSIONS: In the new catheter group, taurolidine showed a clear decrease in CRBSI rate. In the pre-existing catheter group, no superiority of taurolidine could be demonstrated, most likely due to underpowering. Overall, taurolidine reduced the risk for CRBSIs by more than four times. Given its favourable safety and cost profile, taurolidine locking should be considered as an additional strategy to prevent CRBSIs.TRIAL REGISTRATION: Clinicaltrials.gov, identifier: NCT01826526.

Truncating mutations in PRNP have been associated with heterogeneous phenotypes ranging from chronic diarrhea and neuropathy to dementia, either rapidly or slowly progressive. We identified novel PRNP stop-codon mutations (p.Y163X, p.Y169X) in two Italian kindreds. Disease typically presented in the third or fourth decade with progressive autonomic failure and diarrhea. Moreover, one proband (p.Y163X) developed late cognitive decline, whereas some of his relatives presented with isolated cognitive and psychiatric symptoms. Our results strengthen the link between PRNP truncating mutations and systemic abnormal PrP deposition and support a wider application of PRNP screening to include unsolved cases of familial autonomic neuropathy.

Intestinal failure associated liver disease (IFALD) is frequent problem encountered when managing patients receiving parenteral nutrition (PN). Its occurrence is often multifactorial and modification of these factors is vital for the management of such hepatic dysfunction. The use of novel lipid preparations can form part of this management strategy. We present a case whereby such modification of contributing factors, including lipid preparations, led to improvements in IFALD and reversal of hepatic fibrosis.

Contains fulltext : 193361.pdf (Publisher’s version ) (Closed access) BACKGROUND & AIM: Home parenteral nutrition (HPN) is the primary treatment for chronic intestinal failure (IF). Intestinal transplantation (ITx) is indicated when there is an increased risk of death due to HPN complications or to the underlying disease. Age, pathophysiologic conditions and underlying disease are known predictors of HPN dependency and overall survival. Although the cause of death on HPN is mostly related to underlying disease in these patients, the relationship between mortality and duration of HPN use remains unclear. The purpose of the present study is to describe factors associated with survival and HPN dependency as well as causes of death in adult patients requiring HPN for chronic intestinal failure during the first 5 years of treatment with HPN. METHODS: A multicenter international (European and USA) questionnaire-based retrospective follow-up of a cohort of 472 IF patients who started HPN was conducted between June and December 2000. Study endpoint was either end of 5-year follow-up, weaned-off HPN, ITx, or death on HPN. Data were analyzed for HPN dependence and overall survival using Kaplan-Meier models and log rank tests. RESULTS: The overall survival probability was 88%, 74% and 64% at 1, 3 and 5 years respectively. Survival was inversely related to age (p < .001) and higher in patients with Crohn's disease or chronic idiopathic pseudo-obstruction. A total of 169 (36.5%) patients were weaned-off HPN mainly (80%) within the first year and most frequently in patients with fistulae. Five of the 14 patients who underwent ITx died. By the end of the study, 104 (23%) of patients died on HPN; 65% of deaths occurred within the first 2.5 years of HPN. CONCLUSIONS: Younger ages at HPN initiation and underlying pathologies are significantly predictive of survival on HPN. Risk of death is greatest during the first 2 years of HPN.

Background & aims: Intestinal failure associated liver disease (IFALD) has been defined using numerous criteria; however the clinical relevance of these criteria has never been compared. We therefore aimed to evaluate the prevalence, incidence, evolution of IFALD diagnosed by different criteria and to assess any clinical features that may be associated with its occurrence. Methods: A cross sectional (CS) and retrospective study were carried out on adults on home parenteral nutrition (HPN) for chronic intestinal failure (CIF) managed at a single center. Inclusion criteria at CS: age ≥18 years, benign disease. Collected data included: patient demographics, CIF and HPN characteristics, episodes of central venous catheter related bloodstream infection (CRBSI). IFALD was diagnosed by 9 criteria based on liver function tests and liver ultrasound (US) imaging. IFALD diagnoses were categorized as steatosis (2 criteria), cholestasis (3 criteria) or fibrosis (2 criteria) and unclassified (2 criteria). Prevalence was assessed at CS and at starting HPN (baseline, BS). Evolution was assessed as change of IFALD between BS and CS. Incidence was calculated as patients who developed IFALD from BS to CS. Results: A total of 113 patients were included. At CS, IFALD prevalence range in each diagnostic categories was: cholestasis 5–15%; steatosis 17–43%; fibrosis 10–20%; unclassified 7–38%. A 28.5% of patients did not have IFALD according to any criteria. Two cholestasis criteria and one fibrosis criterion were significantly (P < 0.05) associated with a short bowel syndrome as the pathophysiological mechanism of CIF, HPN requirement and the number of CRBSI episodes. At BS, IFALD prevalence range was: cholestasis 13–40%; steatosis 27–90%; fibrosis 2–5%; unclassified 8–75%. The incidence range of IFALD was: cholestasis 0–7%; steatosis 0–39%; fibrosis 7–18%; unclassified 4–9%. IFALD steatosis diagnosed by US was the most frequent diagnosis at both CS prevalence and incidence assessments. Notably, IFALD criteria normalized in various percentages (2–70%), depending on the diagnostic categories, between BS and CS. Conclusions: This is the first study to systematically demonstrate that the frequency of IFALD varies greatly depending on diagnostic criteria used, confirming the need for a consensus definition to be used between different national and international IF units. IFALD can be present at HPN initiation but may resolve thereafter; further work is required to evaluate the factors associated with improvement.

A systematic bibliographic research concerning patients operated on for SBS was performed: inclusion criteria were adult age, reconnection surgery and SBS < 100 cm. Autologous gastrointestinal reconstruction represented an exclusion criteria. The outcomes of interest were the rate of total parenteral nutrition (TPN) independence and the length of follow-up (minimum 1 year) after surgery. We reviewed our experience from 2003 to 2013 with minimum 1-year follow-up, dealing with reconnection surgery in 13 adults affected by < 100 cm SBS after massive small bowel resection: autologous gastrointestinal reconstruction was not feasible. Three (out of 5168 screened papers) non randomized controlled trials with 116 adult patients were analysed showing weaning from TPN (40%, 50% and 90% respectively) after reconnection surgery without autologous gastrointestinal reconstruction. Among our 13 adults, mean age was 54.1 years (53.8 % ASA III): 69.2 % had a high stomal output (> 500 cc/day) and TPN dependence was 100%. We performed a jejuno-colonic anastomosis (SBS type II) in 53.8%, in 46.1% of cases without ileo-cecal valve, leaving a mean residual small bowel length of 75.7 cm. In-hospital mortality was 0%. After a minimum period of 1 year of intestinal rehabilitation, all our patients (100%) went back to oral intake and 69.2% were off TPN (9 patients). No one was listed for transplantation. A residual small bowel length of minimum 75 cm, even if reconnected to part of the colon, seems able to produce a TPN independence without autologous gastrointestinal reconstruction after a minimum period of 1 year of intestinal rehabilitation.

Background. A review was performed on entero-cutaneous fistula (ECF) repair and early recurrence, adding our twenty adult patients (65% had multiple fistulas). Methods. The search yielded 4.098 articles but only 15 were relevant: 1.217 patients underwent surgery. The interval time between fistula's diagnosis and operative repair was between 3 months and 1 year. A bowel resection with primary anastomosis was performed in 1.048 patients, 192 (18.3%) underwent a covering stoma: 856 patients (81.7%) had a fistula takedown in one procedure. Results. The patients had 14.3% recurrence and 13.1% mortality rate. In our experience 75% were surgically treated after a period equal or above one year from fistula occurrence: surgery was very demolitive (in 40% remnant small bowel was less than 100 cm). We performed a bowel resection with a hand-sewn anastomosis (95%) without temporary stoma. In-hospital mortality was 0% and at discharge all were back to oral intake with 0% early re-fistulisation. Conclusions. Literature supports our experience: ECF takedown could be safely performed after an adequate period of recovery from 3 months to one year from fistula occurrence. In our series primary repair (bowel resection plus reconnection surgery without temporary stoma) avoided an early recurrence without mortality.

Contains fulltext : 153557.pdf (Publisher’s version ) (Closed access) BACKGROUND AND AIMS: Patients receiving home parenteral nutrition (HPN) deserve a high-quality and patient-centered care. Patient-centered care can be delivered only if the patient's priorities and concerns are known. Therefore, the aim is to identify the top 3 most important outcome indicators according to patients' perspectives and the differences between several centers, HPN regimen, and HPN experience. METHODS: A questionnaire, based on previously developed outcome indicators, was translated into the mother tongue using forward-backward translation and distributed to adult HPN patients with benign disease in March 2013. To identify differences, a Kruskal-Wallis or Mann-Whitney test was performed with GraphPad Prism (significance level 2 years HPN) vs less experienced patients. For this outcome indicator, patients' view also differed significantly based on number of HPN days per week (.0103). CONCLUSION: A cohort of HPN patients identified incidence of CRI, survival, and QoL as the most important outcome indicators for their care; however, there were significant differences between the participating centers. For one outcome indicator (independence), there were significant differences based on experience and regimen.

An adult male underwent a bowel transplant for tufting enteropathy, receiving alemtuzumab, tacrolimus, and steroids as immunosuppressants. Five years later, he developed an autoimmune hemolytic anemia (AIHA), anti-IgG positive, with reduced reticulocyte count, leukopenia, and thrombocytopenia with antiplatelet antibodies. After an unsuccessful initial treatment with high dose steroids, reduction in tacrolimus dose, and intravenous immunoglobulin (IVIG), a bone marrow biopsy revealed absence of erythroid maturation with precursor hyperplasia. The patient was switched to sirolimus and received four doses of rituximab plus two courses of plasmapheresis, which decreased his transfusion requirements. After a febrile episode one month later, the AIHA relapsed with corresponding decreases in platelet and leukocyte count: cyclosporine A (CsA) was started with a second course of rituximab and IVIG without response, even though repeat bone marrow biopsy did not reveal morphology correlated to an acquired pure red cell aplasia (APRCA). Considering the similarity in his clinical and laboratory findings to APRCA, alemtuzumab was added (three doses over a week) with CsA followed by steroids. The patient was eventually discharged transfusion-independent, with increasing hemoglobin (Hb) levels and normal platelet and leukocyte count. One year later he is still disease-free with functioning graft.

AIM To investigate the fatty acid-based functional lipidomics of patients on long-term home parenteral nutrition receiving different intravenous lipid emulsions. METHODS A cross-sectional comparative study was carried out on 3 groups of adults on home parenteral nutrition (HPN), receiving an HPN admixture containing an olive-soybean oil-based intravenous lipid emulsion (IVLE) (OO-IVLE; n = 15), a soybean- medium-chain triacylglycerol-olivefish oil-based IVLE (SMOF-IVLE; n = 8) or HPN without IVLE (No-IVLE; n = 8) and 42 healthy controls (HCs). The inclusion criteria were: duration of HPN ≥ 3 mo, current HPN admixtures ≥ 2 mo and HPN infusions ≥ 2/wk. Blood samples were drawn 4-6 h after the discontinuation of the overnight HPN infusion. The functional lipidomics panel included: the red blood cell (RBC) fatty acid (FA) profile, molecular biomarkers [membrane fluidity: saturated/monounsaturated FA ratio = saturated fatty acid (SFA)/monounsaturated fatty acid (MUFA) index; inflammatory risk: n-6/n-3 polyunsaturated fatty acid (PUFA) ratio = n-6/n-3 index; cardiovascular risk: sum of n-3 eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) = n-3 index; free radical stress: sum of FA trans isomers = %trans index] and FA pathway enzyme activity estimate (delta-9-desaturase = D9D; delta-6-desaturase = D6D; delta-5-desaturase = D5D; elongase = ELO). Statistics were carried out using nonparametric tests. The amount of each FA was calculated as a percentage of the total FA content (relative%). RESULTS In the OO-IVLE group, the percentage of oleic acid in the RBCs was positively correlated with the weekly load of OO-IVLE (r = 0.540, p = 0.043). In the SMOFIVLE cohort, the RBC membrane EPA and DHA were positively correlated with the daily amount of SMOFIVLE (r = 0.751, p = 0.044) and the number of HPN infusions per week (r = 0.753; p = 0.046), respectively. The SMOF-IVLE group showed the highest EPA and DHA and the lowest arachidonic acid percentages (p < 0.001). The RBC membrane linoleic acid content was lower, and oleic and vaccenic acids were higher in all the HPN groups in comparison to the HCs. Vaccenic acid was positively correlated with the weekly HPN load of glucose in both the OO-IVLE (r = 0.716; p = 0.007) and the SMOF-IVLE (r = 0.732; p = 0.053) groups. The estimated activity of D9D was higher in all the HPN groups than in the HCs (p < 0.001). The estimated activity of D5D was lower in the SMOF-IVLE group than in the HCs (p = 0.013). The SFA/MUFA ratio was lower in all the HPN groups than in the HCs (p < 0.001). The n-6/n-3 index was lower and the n-3 index was higher in the SMOF-IVLE group in comparison to the HCs and to the other HPN groups (p < 0.001). The %trans index did not differ among the four groups. CONCLUSION The FA profile of IVLEs significantly influenced the cell membrane functional lipidomics. The amount of glucose in the HPN may play a relevant role, mediated by the insulin regulation of the FA pathway enzyme activities.

To the author's regret, there is a mistake in Table 9 (recommended daily doses of trace elements for parenteral nutrition) regarding the conversion of the doses for copper and selenium to mcmols. Replace 4.7–9.6 by 4.7–7.9 mcmol (0.3–0.5 mg) for copper and 0.2–0.8 by 0.8–1.3 mcmol (60–100 mcg) for selenium, respectively. We apologise for any inconvenience caused.

Intestinal Failure Associated Liver Disease (IFALD) results from liver injury due to factors related to intestinal failure (IF) and/or to home parenteral nutrition (HPN), and no other evident cause. The epidemiology of IFALD depends on the criteria used for the diagnosis. The aim of the study was to assess the prevalence and the incidence of IFALD in the first 12 months of HPN in adult patients. 18 adult patients (13 Females; age 46±16 years; short bowel syndrome, 72%) who started HPN between Jan 2016 and December 2017 (T0) were enrolled in the study: 13 completed the 12-month follow-up (T12), 5 are ongoing. Biochemical liver function tests were collected at T0, 6-months (T6) and T12; liver ultrasound (US) was performed at T0 and T12. IFALD-criteria diagnosis: IFALD-Cholestasis: two of ɣglutamyltransferase, alkaline phosphatase and conjugated bilirubin ≥1.5 upper normal level for ≥ 6 months; IFALD-Steatosis: echogenicity at US; IFALD-Fibrosis: FIB-4 index. Statistic: descriptive analysis. Prevalence at T0, T6, T12, respectively: Cholestasis, 50%, 22%, 15%; Steatosis, 69%, 71%, 71%; Fibrosis, 16%, 27%, 0%. Incidence between T0 at T12: Cholestasis, 0%; Steatosis, 50%; Fibrosis, 0%. Our results indicate that IFALD can be present at the beginning of HPN, IFALD-cholestasis and IFALD-fibrosis may disappear during the first period of HPN, whereas IFALD-steatosis remains. This would suggest that at starting HPN, liver may be affected by the metabolic alterations of the acute IF, that subside when turning in chronic IF.