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The role of long‐term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long‐term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro‐intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut–brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro‐gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify – and hopefully reduce the potential for harm associated with – the use of long‐term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life‐threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time‐limited period to achieve nutritional safety, while the wider multi‐disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition. [ABSTRACT FROM AUTHOR]

The role of long-term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long-term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro-intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut–brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro-gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify - and hopefully reduce the potential for harm associated with – the use of long-term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life-threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time-limited period to achieve nutritional safety, while the wider multi-disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition. [ABSTRACT FROM AUTHOR]

Introduction: Chronic intestinal failure patients (CIF) require a central venous access device (CVAD) to administer parenteral nutrition. Most serious complication related to a CVAD is a central line-associated bloodstream infection (CLABSI). The golden standard to diagnose a CLABSI are blood cultures, however, they may require 1–5 days before getting a result. Droplet digital polymerase chain reaction (ddPCR) for the detection of pathogen 16S/28S rRNA is a novel culture-independent molecular technique that has been developed to enhance and expedite infection diagnostics within two and a half hours. In this study, we prospectively compared ddPCR with blood cultures to detect pathogens in whole blood. Methods: We included adult CIF patients with a clinical suspicion of CLABSI in this prospective single-blinded clinical study. Blood cultures were routinely collected and subsequently two central samples from the CVAD and two peripheral samples from a peripheral venous access point. Primary outcome was the sensitivity and specificity of ddPCR. Results: In total, 75 patients with 126 suspected CLABSI episodes were included, with 80 blood samples from the CVAD and 114 from peripheral veins. The central ddPCR samples showed a sensitivity of 91% (95%CI 77–98), and specificity of 96% (95%CI 85–99). Peripheral ddPCR samples had a sensitivity of 63% (95%CI 46–77) and specificity of 99% (95%CI 93–100). Conclusion: ddPCR showed a high sensitivity and specificity relative to blood cultures and enables rapid pathogen detection and characterization. Clinical studies should explore if integrated ddPCR and blood culture outcomes enables a more rapid pathogen guided CLABSI treatment and enhancing patient outcomes.

The exact microbiome composition and function of patients with Short Bowel Syndrome (SBS) and Chronic Intestinal Failure (CIF) are still unknown. Patients with type I SBS-CIF (end-jejunostomy/ileostomy) are little represented in available studies. The aim of this study is to evaluate the microbiome characteristics of adult type 1 SBS-CIF patients according to their clinical features. Fecal microbiota was studied by amplicon-based sequencing and volatile organic compounds (VOCs) were assessed by solid-phase microextraction and gas chromatography–mass spectrometry. A total of 44 adult type 1 SBS-CIF patients were enrolled. At the family level, Lactobacillaceae (38% of the relative frequency) and Streptococcaceae (24%) were predominant; at the genus level, Streptococcus (38% of the relative frequency) and Lactobacillus (24%) were the dominant amplicon sequence variants (ASVs). Patients with increased stomal output showed higher ASVs for Lactobacillus (Rho = +0.38; p = 0.010), which was confirmed after adjusting for small bowel length (OR = 1.04; 95% CI 1.01–1.07, p = 0.023). Hyperphagia was associated with higher concentrations of short-chain fatty acid (SCFA) esters, such as butanoic acid ethyl ester (p = 0.005) and hexanoic acid ethyl ester (p = 0.004). Dietary fiber intake was directly correlated with most VOCs. Hyperphagia was associated with dietary fiber, after adjusting for small bowel length (OR = 1.35; 95% CI 1.01–1.81; p = 0.040). In type 1 SBS-CIF patients, a greater frequency of Lactobacilli was associated with increased stomal outputs, while increased fiber intake and concentrations of SCFA esters were associated with hyperphagia. These results might have implications for clinical practice. [ABSTRACT FROM AUTHOR]

Children with chronic intestinal failure (IF) require a long-term central venous catheter (CVC) for provision of parenteral nutrition. Vascular, mechanical and infectious complications such as central line-associated bloodstream infections (CLABSI) may lead to progressive loss of venous access sites. Handling and management of CVCs therefore play an important role. Our vascular rehabilitation concept (VRC) is a core component of our intestinal rehabilitation program (IRP) and consists of an education program, optimization of skin care, catheter fixation and lock solution, and the use of hybrid technique for catheter placement. Aim of this study is to analyse the effectiveness of our VRC on CLABSI rates and need for CVC replacements. Retrospective analysis of all children treated in our IRP that were followed up between 2018 and 2023. A total of 117 children with chronic IF could be included for analysis of 248864 catheter days (CD). 91 patients were referred from other hospitals (127117 CD before and 89359 CD after entry into our IRP). Children receiving primary care at our IRP (32388 CD) showed a significantly lower CLABSI and line replacement rate than patients referred from external centers (p < 0.001). After entering our IRP, CLABSI rates and need for CVC replacements per 1000 CD decreased significantly in referred patients: 1.19 to 0.26 and 1.77 to 0.59, respectively (p < 0.001). Management of paediatric chronic IF patients by an IRP with a vascular rehabilitation concept significantly lowers the rate of CLABSI episodes and the need for catheter replacements. [ABSTRACT FROM AUTHOR]

Introduction: Home parenteral nutrition (HPN) is the primary treatment modality for patients with chronic intestinal failure, one of the least common organ failures. This article provides a retrospective analysis of the data collected on HPN patients in the Czech Republic over the past 30 years. Methods: National registry data were collected using a standardised online form based on the OASIS registry (Oley – A.S.P.E.N. Information System) across all centres providing HPN in the Czech Republic. Data collected prospectively from adult patients in the HPN program were analysed in the following categories: epidemiology, demographics, underlying syndrome, diagnosis, complications, and teduglutide therapy prevalence. Results: The registry identified a total of 1,838 adult patient records, reflecting almost 1.5 million individual catheter days. The prevalence of HPN has risen considerably over the last few decades, currently reaching 5.5 per 100,000 population. The majority of patients have short bowel syndrome and GI obstruction, with cancer being the most prevalent underlying disease. Catheter-related bloodstream infections have been the most prevalent acute complication. However, the incidence in 2022 was only 0.15 per 1,000 catheter days. The study also observed an increase in the prevalence of patients on palliative HPN over the last decade. Conclusion: This study presents a thorough analysis of data from the Czech REgistr Domaci NUtricni Podpory (REDNUP) registry. It shows an increasing prevalence of HPN, namely, in the palliative patient group. The sharing of national data can improve understanding of this rare condition and facilitate the development of international guidelines. [ABSTRACT FROM AUTHOR]

Background and aims: Home parenteral nutrition (HPN) is a life-saving treatment for patients affected by chronic intestinal failure (CIF). Both this clinical condition and its therapy require radical lifestyle modifications, affecting life quality and psychological balance in patients as well as family members. Patient psychological burden has rarely been taken into consideration, not to mention that of caregivers. This study aims to evaluate the levels of anxiety in CIF patients on HPN, and their caregivers, consequently determining their impact on the psychological and physical aspects. Methods: After a brief introductory interview, adult patients on HPN for CIF and their caregivers were asked to fill in the HAMA-A questionnaire. Results: Fifty patients and their respective caregivers were enrolled. Mean HAMA-A scores were similar in patients and caregivers and testified the presence of a mild to severe impact of CIF and HPN in both groups, with a significantly higher impact on female patients and caregivers. After adjusting age, education level, duration of CIF and HPN dependence, and degree of kinship, no differences were revealed in the scores. Conclusions: The study confirms that CIF patients on HPN and their caregivers have a significant anxiety burden independently from the duration of the disease, therefore needing appropriate support. [ABSTRACT FROM AUTHOR]

Le « proche aidant » est celui qui apporte son aide à une personne en perte d'autonomie pour une partie ou la totalité des actes de la vie quotidienne de manière régulière et fréquente et, à titre non professionnel. La nutrition artificielle à domicile est une situation de soin où la place des aidants peut être cruciale. Le fardeau de l'aidant, concept développé initialement pour la gériatrie, a été décrit et analysé à la fois chez les aidants des patients en nutrition entérale et, plus récemment chez ceux qui accompagnent les patients en nutrition parentérale. Nous proposons dans cette revue un état des lieux de la littérature et une mise en perspective éthique, sur ce sujet de société difficile. Non-professional caregivers provide assistance to patients losing their autonomy for some or all of the actions of daily life on a regular and frequent basis. Home artificial nutrition is a healthcare situation where the role of non-professional caregivers can be crucial. Non-professional caregiver burden, a concept initially developed for geriatrics, has been described and analyzed both among non-professional caregivers of enteral nutrition patients and, more recently, among those who support parenteral nutrition patients. In this article, we offer a literature review and an ethical perspective on this difficult social subject. [ABSTRACT FROM AUTHOR]

(1) Objectives: Intestinal failure in home parenteral nutrition patients (HPNPs) results in oxidative stress and liver damage. This study investigated how a high dose of fish oil (FO) added to various lipid emulsions influences antioxidant status and liver function markers in HPNPs. (2) Methods: Twelve HPNPs receiving Smoflipid for at least 3 months were given FO (Omegaven) for a further 4 weeks. Then, the patients were randomized to subsequently receive Lipoplus and ClinOleic for 6 weeks or vice versa plus 4 weeks of Omegaven after each cycle in a crossover design. Twelve age- and sex-matched healthy controls (HCs) were included. (3) Results: Superoxide dismutase (SOD1) activity and oxidized-low-density lipoprotein concentration were higher in all baseline HPN regimens compared to HCs. The Omegaven lowered SOD1 compared to baseline regimens and thus normalized it toward HCs. Lower paraoxonase 1 activity and fibroblast growth factor 19 (FGF19) concentration and, on the converse, higher alkaline phosphatase activity and cholesten concentration were observed in all baseline regimens compared to HCs. A close correlation was observed between FGF19 and SOD1 in baseline regimens. (4) Conclusions: An escalated dose of FO normalized SOD1 activity in HPNPs toward that of HCs. Bile acid metabolism was altered in HPNPs without signs of significant cholestasis and not affected by Omegaven. [ABSTRACT FROM AUTHOR]

Introduction: The human intestinal tract reacts to extensive resection with spontaneous intestinal adaptation. We analyzed whether gene expression analyses or intestinal permeability (IP) testing could provide biomarkers to describe regulation mechanisms in the intestinal barrier in short bowel syndrome (SBS) patients during adaptive response or treatment with the glucagon-like peptide-2 analog teduglutide. Methods: Relevant regions of the GLP-2 receptor gene were sequenced. Gene expression analyses and immunohistochemistry were performed from mucosal biopsies. IP was assessed using a carbohydrate oral ingestion test. Results: The study includes 59 SBS patients and 19 controls. Increases in gene expression with teduglutide were received for sucrase-isomaltase, sodium/glucose cotransporter 1, and calcium/calmodulin serine protein kinase. Mannitol recovery was decreased in SBS but elevated with teduglutide (Δ 40%), showed a positive correlation with remnant small bowel and an inverse correlation with parenteral support. Conclusions: Biomarkers predicting clinical and functional features in human SBS are very limited. Altered specific gene expression was shown for genes involved in nutrient transport but not for genes controlling tight junctions. However, mannitol recovery proved useful in describing the absorptive capacity of the gut during adaptation and treatment with teduglutide. [ABSTRACT FROM AUTHOR]

Background: Teduglutide is a synthetic glucagon‐like peptide‐2 analogue approved for the treatment of short bowel syndrome associated with chronic intestinal failure (SBS‐IF) in adult patients. Clinical trials have demonstrated its ability to reduce parenteral support (PS) requirement. This study aimed to describe the effect of 18‐month treatment with teduglutide, evaluating PS and factors associated with PS volume reduction of ≥20% from baseline and weaning. Two‐year clinical outcomes were also assessed. Methods: This descriptive cohort study collected data prospectively from adult patients with SBS‐IF treated with teduglutide and enrolled in a national registry. Data were collected every 6 months and included demographics, clinical, biochemical, PS regimen, and hospitalizations. Results: Thirty‐four patients were included. After 2 years, 74% (n = 25) had a PS volume reduction of ≥20% from baseline, and 26% (n = 9) achieved PS independency. PS volume reduction was significantly associated with longer PS duration, significantly lower basal PS energy intake, and absence of narcotics. PS weaning was significantly associated with fewer infusion days, lower PS volume, longer PS duration, and lower narcotics use at baseline. Alkaline phosphatase was significantly lower in weaned patients after 6 and 18 months of treatment. During the 2‐year study duration, patients who had PS volume reduction of ≥20% had significantly fewer yearly hospitalizations and hospital‐days. Conclusions: Teduglutide reduces PS volume and promotes weaning in adults with SBS‐IF. Lack of narcotics and longer PS duration were associated with PS volume reduction and weaning, and lower baseline PS volume and fewer infusion days were favorable in obtaining enteral autonomy. [ABSTRACT FROM AUTHOR]

Introduction: This study aimed to assess the clinical, economic, and humanistic impact of short-bowel syndrome/chronic intestinal failure (SBS/CIF) in Portugal. Methods: This is a retrospective multicenter cohort chart review study, with a cross-sectional component for quality-of-life (QoL) evaluation. Inclusion criteria comprised patients with SBS/CIF, aged ≥1 year, with stable parenteral nutrition (PN). Data collection included patient chart review over a 12-month period and patient/caregiver self-report and SF-36/PedsQL™ questionnaires. Main endpoints comprised clinical and PN characterization, healthcare resource use (HRU), direct costs, and patient QoL. Results: Thirty-one patients were included (11 adults and 20 children). Patients’ mean age (standard deviation [SD]) was 57.9 (14.3) years in adults and 7.5 (5.0) years in children, with a mean time since diagnosis of 10.2 (5.9) and 6.6 (4.2) years, respectively. PN was administered for a mean of 5.2 and 6.6 days/week in adults and children, respectively; home PN occurred in 81.8% of adults and 90.0% of children for a mean of 9.6 and 10.8 months/year, respectively. The mean annual number of hospitalizations was 1.9 and 2.0 which lasted for a mean of 34.0 and 29.4 days in adults and children, respectively. Twenty-one and forty hospitalization episodes were reported in adults and children, respectively, of which 71.4% and 85.0% were due to catheter-related complications. Mean annual direct costs per patient amounted to 47,857.53 EUR in adults and 74,734.50 EUR in children, with PN and hospitalizations as the main cost-drivers. QoL assessment showed a clinically significant impaired physical component in adults and a notable deterioration in the school functioning domain in children. Conclusion: In Portugal, SBS/CIF patient management is characterized by a substantial therapeutic burden and HRU, translating into high direct costs and a substantial impairment of the adults’ physical function and children’s school functioning.

Intestinal failure (IF) is a rare disease that requires ongoing intravenous supplementation to sustain growth and health. Advancements in parenteral nutrition (PN) and central venous access in the 1960s and 1970s transformed a life‐limiting disease to a chronic one in which patients are able to administer hydration, electrolytes, micronutrients, and macronutrients in the comfort of their home. However, despite ongoing advancements in the field of home PN (HPN), complications—whether related to central venous catheters or PN itself—remain common and can be associated with significant morbidity and mortality. Central venous access can be associated with thrombosis, central line–associated bloodstream infection, or damage and can result in loss of access over time. PN can be associated with IF‐associated liver disease or hyperglycemia. The key to preserving central venous access and quality of life and maintaining health for patients with chronic IF (CIF) is education focused on prevention and prompt management of CIF complications as they arise. This education typically takes place at the time of initiation of HPN, either in the hospital setting or in the patient's home. The present manuscript describes the historical progression of HPN, prevalence and characteristics of CIF, and an in‐depth discussion of the most common catheter‐related and PN‐related complications and their management, along with a discussion of our education and training process. [ABSTRACT FROM AUTHOR]

L'hépatopathie liée à la nutrition parentérale impacte négativement le pronostic des patients insuffisants intestinaux, par son évolution possible vers la cirrhose et l'insuffisance hépatique. Ses facteurs de risque sont multiples, mais la composition de la nutrition parentérale et l'insuffisance intestinale sous-jacente jouent probablement un rôle majeur. Cette complication est très certainement sous-diagnostiquée, et il n'existe pas à ce jour de définition consensuelle de cette pathologie. Actuellement, son diagnostic repose essentiellement sur un faisceau d'arguments biologiques, radiologiques et/ou histologiques, en absence de diagnostic différentiel. Son traitement est majoritairement fondé sur la prévention, en repérant les patients à risque, notamment les patients ayant un syndrome de grêle court ; en adaptant les apports glucido-lipidiques et la composition des émulsions lipidiques ; et en traitant précocement les complications infectieuses, qu'elles soient liées aux accès veineux ou à une translocation bactérienne. À l'avenir, des études supplémentaires sont nécessaires pour mieux comprendre les mécanismes physiopathologiques de cette complication, proposer une stratégie diagnostique, un suivi adapté et des axes thérapeutiques préventifs et curatifs innovants. Hepatopathy related to parenteral nutrition, because of its possible evolution towards cirrhosis and liver failure, has a negative impact on the prognosis of patients with intestinal failure. The pathogenesis of this complication is multifactorial, ranging from the composition of the parenteral nutrition to the length of the remaining small intestine, and to date there is no consensual definition. Its diagnosis is essentially based on a range of biological, radiological and/or histological arguments. Its treatment is based on prevention, identification of risk factors, adaptation of carbohydrate-lipid intake and the composition of lipid emulsions and the earliest possible management of any sepsis, related to venous access or bacterial translocation. In the future, additional studies are needed to better understand the pathophysiological mechanisms of this complication, to propose a diagnostic strategy, adapted follow-up and innovative preventive and curative therapies. [ABSTRACT FROM AUTHOR]

Background: This study investigated the prevalence, characteristics, and management of patients with chronic intestinal failure (CIF) in the United States in 2012–2020, based on parenteral support (PS) prescription claims and healthcare utilization. Methods: Patients with CIF were identified from the Integrated DataVerse® claims database if they had at least two PS prescriptions within 6 months and a relevant diagnosis. Analysis included prevalence and characteristics of patients with CIF, their travel distance to receive PS prescriptions, and the distribution of PS providers and their prescribing history. Results: Up to 24,048 patients with CIF were identified, equivalent to 75 patients per million. CIF affected people of all ages, being more prevalent in women than in men. Many providers signed PS orders for small patient groups over short time periods, whereas few providers signed PS orders for large patient groups long term, indicating a lack of centralization. The distribution of PS providers suggested a disparity in healthcare coverage in rural vs urban areas, leading to patients traveling considerable distances to receive PS prescriptions. This may be exacerbated by a decline of providers with expertise in CIF and nutrition. Conclusions: Healthcare disparities for patients with CIF have likely been obscured by the lack of CIF‐specific diagnostic and procedure codes, obliging providers to code for their patients under other codes. Effective policy changes, including centralized care, revision of reimbursement models, and expansion of nutrition‐focused education in addition to the newly introduced International Classification of Diseases codes, are needed to provide the best care for patients. Clinical relevancy statement: Chronic intestinal failure (CIF) affects people of all ages and is more prevalent in women than in men. Healthcare coverage for patients with CIF is largely decentralized. Disparities are evident in rural compared with urban areas, leading to patients traveling considerable distances to receive parenteral support (PS) prescriptions, which may be exacerbated by a decline of providers with expertise in CIF and nutrition. These healthcare inequalities necessitate effective policy changes, including introduction of CIF‐specific diagnostic and procedure codes, such as the ongoing introduction of unique Classification of Diseases (ICD) codes for CIF and associated diseases, revision of reimbursement models and assurance of the continuity of providers with expertise in CIF and nutrition (eg, by expansion of nutrition‐focused education in medical schools). Importantly, improvements in healthcare centralization are required to secure management of patients by highly experienced multidisciplinary teams throughout the United States. [ABSTRACT FROM AUTHOR]

Background: Short bowel syndrome (SBS) is considered a low prevalence disease. In Argentina, no registries are available on chronic intestinal failure (CIF) and SBS. This project was designed as the first national registry to report adult patients with this disease. Methods: A prospective multicenter observational registry was created including adult patients with CIF/SBS from approved centers. Demographics, clinical characteristics, nutrition assessment, home parenteral nutrition (HPN) management, surgeries performed, medical treatment, overall survival, and freedom from HPN survival were analyzed. Results: Of the 61 enrolled patients, 56 with available follow‐up data were analyzed. At enrollment, the mean intestinal length was 59.5 ± 47.3 cm; the anatomy was type 1 (n = 41), type 2 (n = 10), and type 3 (n = 5). At the end of the interim analysis, anatomy changed to type 1 in 31, type 2 in 17, and type 3 in 8 patients. The overall mean time on HPN before enrollment was 33.5 ± 56.2 months. Autologous gastrointestinal reconstruction surgery was performed before enrollment on 21 patients, and afterward on 11. Nine patients (16.1%) were weaned off HPN with standard medical nutrition treatment; 12 patients received enterohormones, and 2 of them suspended HPN; one patient was considered a transplant candidate. In 23.7 ± 14.5 months, 11 of 56 patients discontinued HPN; Kaplan‐Meier freedom from HPN survival was 28.9%. The number of cases collected represented 19.6 new adult CIF/SBS patients per year. Conclusion: The RESTORE project allowed us to know the incidence, the current medical and surgical approach for this pathology, as well as its outcome and complications at dedicated centers. [ABSTRACT FROM AUTHOR]

Background: Home parenteral nutrition (HPN) is the recommended treatment in patients with chronic intestinal failure (CIF). HPN is associated with a worsening of patients' quality of life and has a dramatic impact on personal and familial daily life. Little is known about the informal caregiver burden of patients receiving HPN. Aim: To assess informal caregiver burden and the factors associated with moderate‐to‐severe caregiver burden in patients treated with HPN. Methods: Informal caregivers of consecutive patients treated with HPN in a French nutrition referral center were invited to participate in a survey between January 2021 and June 2021. They had to fill out an anonymous standardized self‐questionnaire evaluating burden by the Zarit Burden Interview (ZBI) and depression and anxiety symptoms by the Hospital Anxiety and Depression Scale (HADS) score since HPN introduction. Results: Among the 87 HPN patients having informal caregivers, 53 (61%) completed the questionnaire. Thirty (57%) informal caregivers were women. The caregiver's median age was 62 years (IQR, 21.0). Twelve (22%) expressed moderate to severe burden (ZBI score, 41–88). In a multivariate analysis, the caregiver's male gender (odds ratio [OR], 16.45; 95% CI, 2.30–238.75; P = 0.014) and the number of simultaneous infusions per day >1 (OR, 9.92; 95% CI, 1.35–121.60; P = 0.0038) were associated with a moderate to severe burden. Twenty caregivers reported anxious and depressive symptoms with an elevated HADS score. Conclusion: In this prospective survey, a minority of informal caregivers of patients with CIF being treated with HPN expressed moderate to severe burden. The caregiver's male gender was associated with a higher burden in patients with CIF. [ABSTRACT FROM AUTHOR]

Background: Catheter‐related venous thrombosis (CRVT) is a severe complication of home parental nutrition. Although primary prevention of CRVT is crucial, there is no consensus on anticoagulant use to prevent this adversity. The aim was to compare CRVT risk in patients with chronic intestinal failure (CIF) in the presence or absence of anticoagulants, and to identify CRVT risk factors. Methods: This retrospective cohort study comprised adult patients with CIF with a central venous access device (CVAD) between 2010 and 2020 that were treated at our national CIF referral center. Analyses were performed at a CVAD level. Results: Overall, 1188 CVADs in 389 patients were included (540.800 CVAD days). Anticoagulants were used in 403 CVADs. In total, 137 CRVTs occurred in 98 patients, resulting in 0.25 CRVTs/1000 CVAD days (95% CI, 0.22–0.29). Anticoagulant use was associated with a decreased CRVT risk (odds ratio [OR] = 0.53; 95% CI, 0.31–0.89; P = 0.02). Left‐sided CVAD insertion (OR = 2.00; 95% CI, 1.36–2.94), a history of venous thrombosis (OR = 1.73; 95% CI, 1.05–2.84), and a shorter period postinsertion (OR = 0.78; 95% CI, 0.65–0.92) were independently associated with an increased CRVT risk. Conclusion: Anticoagulants decreased the CRVT risk. In addition, we identified left‐sided vein insertion, a history of venous thrombosis, and a shorter period post‐CVAD insertion as CRVT risk factors. Further prospective studies should provide guidance whether prophylactic anticoagulant use, especially in higher‐risk patients with a left‐sided CVAD or a history of venous thrombosis, is justified. [ABSTRACT FROM AUTHOR]

Background: Patients with chronic intestinal failure (CIF) may be predisposed to poor oral health outcomes. This study explored the self-reported oral health status, function, and psychological impacts of oral health of adult patients with CIF, their access to dental care, and how these compare with the broader population., Methods: All patients >18 years old receiving home intravenous therapies for CIF were invited to complete a self-reported questionnaire providing information on oral health status and access to oral health services. Collateral information was provided by treating clinicians. Descriptive data analysis was undertaken, including subgroup analysis of clinical characteristics, and was compared with the available population-level data., Results: Twenty-four patients participated. Short gut and dysmotility accounted for 88% of the etiologies of CIF. Respondents reported good preventative oral health behaviors (96%), accessing dental care within the last 12 months (75%), and limited barriers to receiving care. Dry mouth (96%), oral pain (59%), and temperature sensitivity (60%) were commonly reported across the cohort. Smoking history and reduced oral diet were associated with significantly worse self-reported oral health outcomes. Patients with CIF reported worse oral health outcomes despites better oral health access than the general population., Conclusion: Patients with CIF appear to be at risk of poor oral health outcomes, especially where smoking or reduced oral intake are concurrently involved. Clinicians involved in CIF care should be alert to the oral health needs of this population and consider oral and dental health as part of the multidisciplinary care required for optimal CIF care., (© 2024 American Society for Parenteral and Enteral Nutrition.)

Background: Children with intestinal failure (IF) are at risk of loss of vascular access because of catheter‐related venous thrombosis. Whether primary prophylactic anticoagulation is effective and safe in preventing catheter‐related thrombosis is largely unknown. Our aim was to assess the incidences of catheter‐related venous thrombosis and bleeding complications in children with IF receiving home parenteral nutrition (HPN) treated with primary prophylactic anticoagulation. Methods: All children, aged 0–18 years, treated with HPN at the Emma Children's Hospital/Amsterdam UMC were followed from January 2007 to July 2019. All patients were offered primary prophylactic anticoagulation from the start of HPN. The primary outcomes were catheter‐related venous thrombosis and bleeding on prophylactic anticoagulation. Results: In total, 55 (76%) of 74 patients received primary prophylactic anticoagulation. The median age at the start of prophylaxis was 8.4 (interquartile range [IQR], 5.0–55.7) months. Patients were followed for a median of 31.2 (IQR, 10.7–53.5) months, with a total of 65,463 catheter days. The incidence of catheter‐related thrombosis on prophylactic anticoagulation was 0.2 per 1000 catheter days. In total, the incidence of clinically relevant bleeding was 0.1 per 1000 catheter days. The median time to first event was 1268 (IQR, 149–2014) days for thrombosis and 389 (IQR, 227–2912) days for clinically relevant bleeding. Cumulative event‐free survival after 5 years was 78% for thrombosis. Conclusions: Our study shows a low rate of catheter‐related venous thrombosis and a slightly elevated rate of clinically relevant bleeding in children receiving HPN and primary prophylactic anticoagulation. [ABSTRACT FROM AUTHOR]

Although central venous catheter (CVC)-related thrombosis (CRT) is a severe complication of home parenteral nutrition (HPN), the amount and quality of data in the diagnosis and management of CRT remain low. We aimed to describe current practices regarding CVC management in French adult and pediatric HPN centers, with a focus on CVC obstruction and CRT. Current practices regarding CVC management in patients on HPN were collected by an online-based cross-sectional survey sent to expert physicians of French HPN centers. We compared these practices to published guidelines and searched for differences between pediatric and adult HPN centers' practices. Finally, we examined the heterogeneity of practices in both pediatric and adult HPN centers. The survey was completed by 34 centers, including 21 pediatric and 13 adult centers. We found a considerable heterogeneity, especially in the responses of pediatric centers. On some points, the centers' responses differed from the current guidelines. We also found significant differences between practices in adult and pediatric centers. We conclude that the management of CVC and CRT in patients on HPN is a serious and complex situation for which there is significant heterogeneity between HPN centers. These findings highlight the need for more well-designed clinical trials in this field. [ABSTRACT FROM AUTHOR]

Background and AimsChronic intestinal failure (CIF) therapy changed significantly in recent decades, and both survival and complication rates improved over time. International guidelines claim that early referral of long-term home parenteral nutrition (HPN) patients to an expert center with specific standards of care may positively affect long-term outcomes. Herein, we retrospectively analyse the long-term outcomes of a cohort of pediatric patients with CIF followed-up since our Pediatric Intestinal Failure Unit foundation, in 1989.MethodsData of the 120 children followed up at Pediatric Intestinal Failure Unit during the last 28 years were retrospectively collected. Patients' and HPN characteristics, as well as dependence, survival, and complication rates, were described.ResultsIncidence and prevalence of CIF increased during the study period particularly due to the increase of HPN for non-digestive disease (NDD) CIF (47.5% of the study sample). Catheter-related bloodstream infection (CRBSI) rate decreased over the study period: 0.33 episodes/1,000 catheters days before 2011 and 0.19 episodes/1,000 catheters days afterwards. Only 1 patient out of 12 died because of HPN complications. The survival rate of patients with PDD was 98.4% at 1 year from the beginning of HPN, 96.5% at 2 years, and 93.8% from the fifth year onwards. Concerning the dependence rate, 70.6% of patients were still on HPN 1 year after the start of HPN, 63.7% at 2 years, 52.4% at 5 years, and 40.8% from the 9th year onwards, with no significant difference according to the underlying intestinal pathology. The survival rate of NDD patients was 91.2% at 1 year from the beginning of HPN, 87.4% at 2 years, and 81.9% from the third year onwards. For what concerns the enteral autonomy, it was regained by 56.7% 1 year after the start of HPN, 74.5% at 2 years, and 95.0% in the 5th year.ConclusionsOur data confirmed the importance of appropriate standards of care and suggest that applying a specific set of standards and protocols may further improve patients' outcomes and survival. Indeed, both primary and non-digestive diseases HPN showed good outcomes.

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

Abstract Background Home parenteral nutrition (HPN) is a life-preserving therapy for patients with chronic intestinal failure (CIF) indicated for patients who cannot achieve their nutritional requirements by enteral intake. Intravenously administered lipid emulsions (ILEs) are an essential component of HPN, providing energy and essential fatty acids, but can become a risk factor for intestinal-failure-associated liver disease (IFALD). In HPN patients, major effort is taken in the prevention of IFALD. Novel ILEs containing a proportion of omega-3 polyunsaturated fatty acids (n-3 PUFA) could be of benefit, but the data on the use of n-3 PUFA in HPN patients are still limited. Methods/design The HOME study is a prospective, randomized, controlled, double-blind, multicenter, international clinical trial conducted in European hospitals that treat HPN patients. A total of 160 patients (80 per group) will be randomly assigned to receive the n-3 PUFA-enriched medium/long-chain triglyceride (MCT/LCT) ILE (Lipidem/Lipoplus® 200 mg/ml, B. Braun Melsungen AG) or the MCT/LCT ILE (Lipofundin® MCT/LCT/Medialipide® 20%, B. Braun Melsungen AG) for a projected period of 8 weeks. The primary endpoint is the combined change of liver function parameters (total bilirubin, aspartate transaminase and alanine transaminase) from baseline to final visit. Secondary objectives are the further evaluation of the safety and tolerability as well as the efficacy of the ILEs. Discussion Currently, there are only very few randomized controlled trials (RCTs) investigating the use of ILEs in HPN, and there are very few data at all on the use of n-3 PUFAs. The working hypothesis is that n-3 PUFA-enriched ILE is safe and well-tolerated especially with regard to liver function in patients requiring HPN. The expected outcome is to provide reliable data to support this thesis thanks to a considerable number of CIF patients, consequently to broaden the present evidence on the use of ILEs in HPN. Trial registration ClinicalTrials.gov, ID: NCT03282955. Registered on 14 September 2017.

Micronutrients (MN), i.e. trace elements and vitamins, are essential organic molecules, which are required in the diet in relatively small amounts in any form of nutrition (oral, enteral, parenteral). The probability of MN depletion or deficiencies should be considered in all chronic illnesses, especially in those that can interfere with intake, digestion, or intestinal absorption. Low socio-economic status and food deprivation are recognized as the most prevalent reasons for MN deficiencies world-wide. Elderly multimorbid patients with multimodal therapy, as well as patients with long-lasting menu restrictions, are at high risk for both disease related malnutrition as well as multiple MN deficiencies, needing careful specific follow-up. The importance of monitoring MN blood levels along with CRP is essential for optimal care. Drug interactions are also highlighted. In patients with chronic conditions depending on medical nutrition therapy, the provision of adequate dietary reference intakes (DRI) of MN doses and monitoring of their adequacy belongs to standard of care., Competing Interests: Declaration of competing interest None of the above authors declares any conflict., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Introduction: Short bowel syndrome with chronic intestinal failure (SBS/CIF) is the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balance due to a short bowel. Although SBS/CIF is rare, its clinical management is complex, challenging, expensive, and time-consuming., Aim: This study aimed to analyze a single center's experience with SBS/CIF in adult patients treated with home parenteral nutrition (HPN)., Materials and Methods: A retrospective single-center analysis of all 13 consecutive adult patients with SBS/CIF was included in an HPN program between January 1994 and August 2023., Results: Between 1992 and 2023, 13 patients were included in an HPN program. The primary underlying pathology was acute mesenteric ischemia. The median age of starting HPN was 44 years. Most were subjected to several surgeries of extensive intestinal resection with posterior intestinal reconstruction. Five of the 13 patients died while on HPN with a median duration of 42 months. The causes of death related to HPN were catheter sepsis, endocarditis with cardiac failure, or hepatic failure. One patient died due to underlying pathology: pelvic abscesses and bleeding related to radiotherapy. Eight patients remain alive, with a median time of HPN of 173 months. During the HPN support, the most frequent complications were venous catheter infection and venous territory thrombosis. None of the eight patients alive have hepatic failure. Two patients recently started teduglutide with good tolerance and need a reduction in HPN support. All eight patients have a satisfactory quality of life (parenteral support needs range between five and two nutrition bags per week).  Conclusion: Home parenteral nutrition remains the gold standard of SBS/CIF treatment, although teduglutide may reduce HPN needs and complications and provide a better quality of life. Despite the small number of patients, the results shown in this study are not inferior to those in large-volume centers. The existence of the commitment and interest of professionals involved in SBS/CIF at Centro Hospitalar Universitário de Santo António, Portugal, was a fundamental key to achieving those results. A multidisciplinary healthcare group for HPN support can be essential to ensuring these patients' survival and quality of life., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. National Committee for Data Protection and Ethics Committee of Unidade Local de Saúde de Santo António (ULSSA) issued approval (Ref. 2107.211 (181-DEFI/173-CES)). This study was conducted according to the gold standard of ethical recommendations, namely, the last update of the Helsinki Declaration and International Guidelines for Ethical Review of Epidemiological Studies. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Santos et al.)

Introduction: Chronic intestinal failure patients (CIF) require a central venous access device (CVAD) to administer parenteral nutrition. Most serious complication related to a CVAD is a central line-associated bloodstream infection (CLABSI). The golden standard to diagnose a CLABSI are blood cultures, however, they may require 1-5 days before getting a result. Droplet digital polymerase chain reaction (ddPCR) for the detection of pathogen 16S/28S rRNA is a novel culture-independent molecular technique that has been developed to enhance and expedite infection diagnostics within two and a half hours. In this study, we prospectively compared ddPCR with blood cultures to detect pathogens in whole blood., Methods: We included adult CIF patients with a clinical suspicion of CLABSI in this prospective single-blinded clinical study. Blood cultures were routinely collected and subsequently two central samples from the CVAD and two peripheral samples from a peripheral venous access point. Primary outcome was the sensitivity and specificity of ddPCR., Results: In total, 75 patients with 126 suspected CLABSI episodes were included, with 80 blood samples from the CVAD and 114 from peripheral veins. The central ddPCR samples showed a sensitivity of 91% (95%CI 77-98), and specificity of 96% (95%CI 85-99). Peripheral ddPCR samples had a sensitivity of 63% (95%CI 46-77) and specificity of 99% (95%CI 93-100)., Conclusion: ddPCR showed a high sensitivity and specificity relative to blood cultures and enables rapid pathogen detection and characterization. Clinical studies should explore if integrated ddPCR and blood culture outcomes enables a more rapid pathogen guided CLABSI treatment and enhancing patient outcomes., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier B.V.)

L'insuffisance intestinale chronique (IIC) est une situation clinique rare dont la principale cause est le syndrome de grêle court (SGC). La pseudo-obstruction intestinale chronique (POIC) est également une cause d'IIC. La nutrition parentérale à domicile (NPAD) a permis d'augmenter la survie et la qualité de vie des patients ayant une insuffisance intestinale chronique. Plusieurs grossesses sous NPAD ont été décrites. L'objectif de cette revue est de faire la synthèse sur l'état des connaissances sur les grossesses en NPAD, et de faire le point sur les adaptations nécessaires des traitements et de la nutrition. En NPAD prolongée, les grossesses sont donc possibles, mais le taux de complications maternelles liées à la maladie sous-jacente et à la NP est élevé. Un suivi rapproché et une préparation à la grossesse doivent être réalisés afin de prévenir et traiter d'éventuelles carences et leurs effets délétères. Chronic intestinal failure is a rare disease and short bowel syndrome is its main etiology. Chronic intestinal pseudo obstruction (CIPO) is also a cause of IIC. Home parenteral nutrition (HPN) has improved survival and quality of life of patients with IIC. Some pregnancy during HPN have been reported. The aim of this review was to summarize the state of knowledge on pregnancy during HPN, and to resume the needed adaptations of treatments and PN. During long term HPN, pregnancy are possible but the complication rate, due to PN and to underlying disease, is high. A close follow-up end pregnancy preparation are necessary, in order to prevent and treat some deficiencies and their deleterious effects. [ABSTRACT FROM AUTHOR]

Home parenteral nutrition (HPN) is one of the most demanding medical therapies as it is the only option for patients for intestinal failure (IF). No unequivocal policy on how to start and progress with HPN has ever been presented. The IF Center at the Stanley Dudrick's Memorial Hospital in Skawina is one of the biggest centers in Poland, celebrating its twentieth birthday last year. It offered the unique chance to present how to create and grow the IF center, increasing the quality of care. The above became the aim of this study. A retrospective analysis of all medical records of HPN patients from the University Hospital and Stanley Dudrick's Memorial Hospital in Skawina. Patient profile, clinical course and treatment outcomes were assessed. The evolution of all aspects of HPN is presented. A brief historical perspective has been added to better illustrate the center's growth and transformation. 608 patients (363 female, 245 male, mean age 55.55 year) from all over Poland were treated between December 1999 and December 2019. The most frequent indication for HPN was mechanical obstruction (277, 45.7%), followed by short bowel syndrome (SBS, 208, 34.3%) and intestinal fistula (46, 7.59%). The most common primary disease was cancer (n = 267), followed by mesenteric ischemia (n = 104), and surgical complications (n = 62). 314 patients (51.8%) died. 73 (12.04%) were successfully weaned off PN. The catheter infection rate reached 0.39/1000 catheter days. the founding of the HPN center and its further development is possible provided that there is team of dedicated people, supported by hospital base. The real growth opportunity is guaranteed by the reimbursement. It is necessary to adapt to the changing circumstances. [ABSTRACT FROM AUTHOR]

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

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

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Chronic intestinal failure (IF) in children is a rare and heterogeneous disease requiring treatment with parenteral nutrition. A uniform definition for chronic IF and standardized outcome measures to compare therapeutic trials in these children are lacking. Therefore, the aim of this study is to systematically assess how definitions and outcome measures are defined in therapeutic trials of children with chronic IF. MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception till August 2018. No language restriction was used. A total of 1766 articles was found of which 70 studies fulfilled our inclusion criteria. 54 studies (76%) did not report any definition of IF. Of the 16 studies (23%) which reported a definition of IF, 7 different definitions were found. The two most frequently used definitions were: (1) the inability to absorb adequate nutrients to maintain body weight or normal growth and development (n = 5), and (2) the dependence upon parenteral nutrition to maintain minimal energy requirements for growth and development (n = 5). A total of 117 different outcomes were reported. The three most reported outcome measures were: mortality (n = 27), liver enzymes (alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, and gamma-glutamyl trans peptidase) (n = 27), and growth (n = 23). Quality of reporting was considered fair to poor in most studies. There is a lack of reported definitions in studies concerning pediatric IF. Heterogeneity exists in outcome reporting in research concerning pediatric chronic IF. Therefore, we recommend the development of a core outcome set. [ABSTRACT FROM AUTHOR]

Background: Home parenteral nutrition (HPN) is a life-preserving therapy for patients with chronic intestinal failure (CIF) indicated for patients who cannot achieve their nutritional requirements by enteral intake. Intravenously administered lipid emulsions (ILEs) are an essential component of HPN, providing energy and essential fatty acids, but can become a risk factor for intestinal-failure-associated liver disease (IFALD). In HPN patients, major effort is taken in the prevention of IFALD. Novel ILEs containing a proportion of omega-3 polyunsaturated fatty acids (n-3 PUFA) could be of benefit, but the data on the use of n-3 PUFA in HPN patients are still limited.Methods/design: The HOME study is a prospective, randomized, controlled, double-blind, multicenter, international clinical trial conducted in European hospitals that treat HPN patients. A total of 160 patients (80 per group) will be randomly assigned to receive the n-3 PUFA-enriched medium/long-chain triglyceride (MCT/LCT) ILE (Lipidem/Lipoplus® 200 mg/ml, B. Braun Melsungen AG) or the MCT/LCT ILE (Lipofundin® MCT/LCT/Medialipide® 20%, B. Braun Melsungen AG) for a projected period of 8 weeks. The primary endpoint is the combined change of liver function parameters (total bilirubin, aspartate transaminase and alanine transaminase) from baseline to final visit. Secondary objectives are the further evaluation of the safety and tolerability as well as the efficacy of the ILEs.Discussion: Currently, there are only very few randomized controlled trials (RCTs) investigating the use of ILEs in HPN, and there are very few data at all on the use of n-3 PUFAs. The working hypothesis is that n-3 PUFA-enriched ILE is safe and well-tolerated especially with regard to liver function in patients requiring HPN. The expected outcome is to provide reliable data to support this thesis thanks to a considerable number of CIF patients, consequently to broaden the present evidence on the use of ILEs in HPN.Trial Registration: ClinicalTrials.gov, ID: NCT03282955. Registered on 14 September 2017. [ABSTRACT FROM AUTHOR]

Nutritional monitoring plays an important role in optimizing nutritional support in patients with chronic intestinal failure (CIF) receiving long-term supplementation. Unlike initial nutritional assessment, however, there are no recommended guidelines for establishing a nutritional monitoring index. This study is to evaluate the suitability of insulin-like growth factor-1 (IGF-1) as a nutritional monitoring factor in CIF patients. We retrospectively analyzed the correlation between serum nutritional indicators, including IGF-1 levels, and nutritional assessment, nutritional monitoring, and lean body mass in 197 patients with CIF. The mean age of the 197 enrolled patients was 47.22 ± 18.87 years old and; the mean BMI was 16.83 ± 3.31. The mean NRS-2002 score was 3.49 ± 0.83; and moreover, 76.3% of the patients were malnourished. The median length of hospital stay in hospital (LOS) was 18.5 days. IGF-1 was positively correlated with body mass index, hemoglobin, albumin, pre-albumin, retinol-binding protein (RBP), transferrin, serum creatinine (Scr) and cholesterol (p < 0.05 for all). Testing performed over 3 weeks post-admission showed that significantly different weekly changes were observed only in IGF-1, RBP, and Scr during the period of nutritional support (p < 0.05 for each). Multivariate linear regression analysis showed that IGF-1 and body mass index were independent factors influencing fat-free mass, skeletal muscle mass, and body protein mass (p < 0.05 for each). IGF-1 is suitable for monitoring short-term changes in the nutritional status in CIF patients. This may be attributed to its shorter half-life, greater sensitivity, and better correlation with lean body mass. ClinicalTrials.gov number, NCT03277014. [ABSTRACT FROM AUTHOR]

Home parenteral nutrition (HPN) provides life sustaining treatment for people with chronic intestinal failure. Individuals may require HPN for months or years and are dependent on regular intravenous infusions, usually 12–14 h overnight between 1 and 7 days each week. This regime can have adverse impact on the life of people dependent on the treatment. The aim of this study was to establish mean values for the Parenteral Nutrition Impact Questionnaire (PNIQ) and to determine the effect of disease, frequency of infusions per week and patient characteristics on quality of life of patients fed HPN. The PNIQ was distributed to patients across nine UK HPN clinics. Data were analysed using linear regression, with PNIQ score as the dependent variable and potential confounders as independent variables. Unadjusted and adjusted models are presented. Higher PNIQ scores reflect poorer quality of life. Completed questionnaires were received from 466 people dependent on HPN. Mean PNIQ score was 11.04 (SD 5.79). A higher PNIQ score (effect size 0.52, CI 0.184 to 0.853) was recorded in those dependent on a higher frequency of HPN infusions per week. Respondents with cancer had a similar mean PNIQ score to those with inflammatory bowel disease (mean 10.82, SD 6.00 versus 11.04, SD 5.91). Those with surgical complications reported a poorer QoL (effect size 3.03, CI 0.642 to 5.418) and those with severe gastro-intestinal dysmotility reported a better QoL (effect size −3.03, CI −5.593 to −0.468), compared to other disease states. This large cohort study of quality of life in chronic intestinal failure demonstrates that HPN impacts individuals differently depending on their underlying disease. Furthermore, since the number of HPN infusions required per week is inversely related to an individual's needs-based quality of life, therapies that reduce PN burden should lead to an improvement in QoL. • Study of quality of life in 466 individuals on Parenteral Nutrition infusions. • Parenteral Nutrition Impact Questionnaire gauges quality of life in those on infusions. • Patients requiring more frequent infusions per week reported poorer quality of life. • Parental nutrition impacts differently on people depending on underlying disease. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]