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Background: Home parenteral nutrition (HPN) can be associated with increased liver enzymes, catheter-related bloodstream infections (CRBSI), and hospitalizations. Mixed oil (MO) versus soybean oil (SO) lipid emulsion reduces risks in hospitalized patients, but there are no randomized double-blinded controlled trials in HPN. Therefore, the primary objective was to test the study's feasibility such as recruitment and retention in the HPN population and the secondary objective was to assess changes in liver enzymes between MO and SO as well as other clinical and biochemical outcomes. Methods: This 13-month prospective double-blind crossover randomized pilot trial took place in Toronto, Canada. Participants were HPN patients who were a part of the HPN program at Toronto General Hospital. We recruited patients from the HPN program. HPN patients receiving SO were randomized to either MO or SO, and the study duration was 6 months in each arm (MO or SO) with a 1-month washout period resuming SO. As this is a crossover trial design, the patient is his/her own control. The main outcome measures were descriptions of study feasibility, namely the study recruitment and retention. We also collected biochemical parameters, CRSBI, hospitalization rate, antibiotic use, and mortality. Demographic, nutritional, clinical, and laboratory data were collected at baseline, 3 and 6 months of each arm. The primary analysis population was defined as the per-protocol population who completed the trial including all lipid measurements. Results: A total of 65 HPN patients were assessed, and 60 met the inclusion criteria for the study. Thirty-five percent (21/60) were randomized using a computer-generated random number sequence generator: 10 participants were randomized to receive SO first while 11 were randomized to receive MO first. At 13 months, 3/10 who received SO first completed the study, whereas 9/11 who received MO first completed the study. This did not meet our a priori criteria for success in recruitment and retention. Between types of lipid emulsions, there were no significant differences in changes in liver enzymes or biochemical and clinical outcomes, despite significant changes in plasma free fatty acid composition reflecting MO or SO. Conclusions: Overall, this pilot trial demonstrated that the use of a prospective double-blind, crossover, randomized trial design was not feasible to conduct in the HPN population because of difficulties in recruiting and retaining patients. In addition, there was no significant impact of MO versus SO lipid emulsion on liver enzymes or most parameters. The lack of significance may be attributed to low sample size from low recruitment and high drop-out rate, short study duration (6 months/arm), and complex care. In a future definitive trial, a multicenter study of longer duration and a larger sample size is recommended, and drop-outs may be reduced by using a parallel study design. Trial registration: ClinicalTrials.gov, NCT02796833. Registered on 13 June 2016—retrospectively registered. [ABSTRACT FROM AUTHOR]

Intestinal failure is defined as the inability to absorb the minimum of macro and micronutrients, minerals and vitamins due to a reduction in gut function. In a subpopulation of patients with a dysfunctional gastrointestinal system, treatment with total or supplemental parenteral nutrition is required. The golden standard for the determination of energy expenditure is indirect calorimetry. This method enables an individualized nutritional treatment based on measurements instead of equations or body weight calculations. The possible use and advantages of this technology in a home PN setting need critical evaluation. For this narrative review, a bibliographic search is performed in PubMed and Web of Science using the following terms: 'indirect calorimetry', 'home parenteral nutrition', 'intestinal failure', 'parenteral nutrition', 'resting energy expenditure', 'energy expenditure' and 'science implementation'. The use of IC is widely embedded in the hospital setting but more research is necessary to investigate the role of IC in a home setting and especially in IF patients. It is important that scientific output is generated in order to improve patients' outcome and develop nutritional care paths. [ABSTRACT FROM AUTHOR]

This guideline will inform physicians, nurses, dieticians, pharmacists, caregivers and other home parenteral nutrition (HPN) providers, as well as healthcare administrators and policy makers, about appropriate and safe HPN provision. This guideline will also inform patients requiring HPN. The guideline is based on previous published guidelines and provides an update of current evidence and expert opinion; it consists of 71 recommendations that address the indications for HPN, central venous access device (CVAD) and infusion pump, infusion catheter and CVAD site care, nutritional admixtures, program monitoring and management. Meta-analyses, systematic reviews and single clinical trials based on clinical questions were searched according to the PICO format. The evidence was evaluated and used to develop clinical recommendations implementing Scottish Intercollegiate Guidelines Network methodology. The guideline was commissioned and financially supported by ESPEN and members of the guideline group were selected by ESPEN. [ABSTRACT FROM AUTHOR]

Background: Most data on anemia in children with intestinal failure (IF) have been obtained from studies in which the data were collected at a single point in time. We aimed to identify the frequency of anemia and factors associated with hemoglobin levels in children with IF during their course of home parenteral nutrition. Methods: We performed a longitudinal cohort study of patients with IF followed up at a pediatric intestinal rehabilitation center. Outcome variables were hemoglobin levels and prevalence of anemia during the follow‐up period. The exposure variables were age, duration of parenteral nutrition, chronic disease, and serum concentrations of C‐reactive protein, iron, copper, selenium, vitamins A, D, B12, and folic acid. Results: Twenty‐five children with a median time of receiving parenteral nutrition of 40.7 months were included. A median (and interquartile range) of 40.7 (25.2–58) hemoglobin measurements were performed per patient. Mean (SD) hemoglobin was 10.7 (1.8) g/dL at baseline and 11.6 (0.9) g/dL in the last observation (paired t test, P = 0.07); 32% of patients had mean hemoglobin values below the lower limit for age. In a multivariable predictive model, having C‐reactive protein >1 mg/dL was associated with a decrease of 0.57 g/dL in hemoglobin (95% CI, −0.90 to −0.24, P = 0.01), and an increase of 1 mg/L in vitamin A concentration was associated with the increase of 0.93 g/dL in Hb level (95% CI, 0.24–1.61; P = 0.008). Conclusion: Anemia affects almost one‐third of children with IF and its frequency decreases during the follow‐up period. Hemoglobin levels are associated with inflammatory response and serum micronutrient concentrations. [ABSTRACT FROM AUTHOR]

Intestinal failure (IF) is characterized by a critical reduction in functional gut mass below the minimum needed for optimal growth in children. It requires parenteral nutrition (PN) and home-PN (HPN), which is challenging in terms of meeting nutritional needs according to age, growth velocity, clinical situation, and rapid changes in fluid and electrolyte requirements. Due to these complex requirements, age-adapted multi-chamber bags (MCBs) are important additions to the nutrition armamentarium. The launch of composite fish oil (FO)-containing intravenous lipid emulsions (ILEs) heralded the development of MCBs containing these ILEs in combination with a crystalline amino acid solution adapted for pediatric use. The safety and efficacy of lipid and amino acid components in this context have been widely documented in numerous published studies. This narrative manuscript includes a review of the articles published in PudMed, Embase, and Google Scholar up to June 2023 for the age groups of term infants to children and adolescents. Preterm infants with their highly specific demands are not included. It aims to offer an overview of the clinical experience regarding the use of a composite FO-based ILE and a developed specific amino acid solution.

Home parenteral nutrition (HPN) is increasingly prescribed for patients with advanced cancer. This therapy improves free-fat mass, quality of life and survival, but it is not free from complications, especially catheter-related bloodstream infections (CRBSIs). The use of commercial multichamber bags in HPN has not been extensively explored in oncologic patients and their association with complications is not well known. In this prospective cohort study, we included 130 patients with advanced cancer and HPN. We compared the effects of individual compounded bags (n = 87) vs. commercial multichamber bags (n = 43) on complications. There were no differences in any complication, including thrombosis (p > 0.05). There were 0.28 episodes of CRBSI per 1000 catheter days in the individual compounded bag group and 0.21 in the multichamber bag group (p > 0.05). A total of 34 patients were weaned off HPN, 22 with individual bags and 12 with multichamber bags (p = 0.749). Regarding survival when on HPN, the group with individual bags showed a median of 98 days (95% CI of 49–147), whereas those with multichamber bags showed a median of 88 days (95% CI of 43–133 (p = 0.913)). In conclusion, commercial multichamber bags for HPN in patients with advanced cancer are non-inferior when compared to individual compounded bags in terms of complications.

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]

Introduction: Patients with chronic intestinal failure (IF) have a low degree of physical activity, decreased muscle mass, and decreased muscle strength, leading to a high risk of sarcopenia. We aimed to test the prevalence of sarcopenia by the use of SARC‐F and EWGSOP and to investigate the association between the two at baseline and after 12 weeks of an exercise intervention. Methods: Thirty‐one patients with chronic IF completed 12 weeks of three weekly home‐based individualized exercise sessions. Body composition was measured by bioimpedance analysis and physical function by handgrip strength (HGS) and timed up‐and‐go (TUG). Sarcopenia was assessed by SARC‐F and EWGSOP. Multiple regression analysis was used to test for the association between the two tools. Results: The prevalence of sarcopenia measured by EWGSOP was 59%. This prevalence did not change after the intervention. At baseline, 38.8% of patients were screened as at risk for sarcopenia by SARC‐F. This decreased to 29.0% after the intervention (P < 0.001). A statistically significant increase was achieved in muscle mass (P = 0.017) and muscle mass index (P = 0.016). Furthermore, both TUG (P = 0.033) and HGS (P = 0.019) improved. Conclusions: Sarcopenia is prevalent in patients with chronic IF. EWGSOP finds more patients to be at risk of sarcopenia than SARC‐F but was not sufficiently sensitive to measure changes induced by the physical intervention. The significant change in SARC‐F may illustrate that patients, themselves, find an improvement in self‐perceived health. [ABSTRACT FROM AUTHOR]

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

OBJECTIVE: The goal of our research was to determine the impact of clinical nutrition in the form of home parenteral nutrition (HPN) in patients with nutritional disorders, most often caused by diseases of the digestive tract, with the risk of developing malnutrition. PATIENTS AND METHODS: We retrospectively evaluated 39 patients from the Gastroenterology Clinic and the Home Parenteral Nutrition Center of the University Hospital Bratislava, whose nutritional status was evaluated based on the determination of the body mass index (BMI), the completed nutritional risk screening (NRS) questionnaire and the determination of performance status. Subsequently, after fulfilling the criteria for HPN, the initiation of parenteral nutrition (PN) followed, implemented in a domestic environment for the following two years as HPN. During this period, we did a monthly check-up of the objective condition and laboratory parameters of the enrolled patients, which were the basis for adjusting the nutritional treatment. We also evaluated the occurrence of infectious and thrombotic complications clinically and on the basis of laboratory parameters focused on culture and hemocoagulation examination. After two years, we performed control exit examinations, which we compared with the entrance examinations and statistically evaluated the success of the treatment. We evaluated the obtained data using standard statistical methods. RESULTS: During HPN, there was a statistically significant elevation of the individual monitored values ​​(BMI, absolute lymphocytes count, cholesterol, cholinesterase, total proteins, albumins), which clearly proves correctly indicated and managed HPN. We recorded vein thrombosis in v. subclavia and v. jugularis in 6 (15 %) patients. Subsequent catheter extraction was necessary after unsuccessful catheter insertion. In 13 (33 %) patients, tunneled catheter replacement was required due to infection. The mortality rate in our group was 8 % (3 patients). These were female patients aged 39, 42, and 66 years. The cause of death in all of these patients was the underlying diagnosis (oncohematological disease, systemic connective tissue disease, and repeated resections of the digestive tract for inflammatory GIT disease with the development of severe malnutrition). We recorded a positive effect of applied HPN in all three patients until death. We did not register any factors that would have a relevant influence on the success of administered HPN. CONCLUSION: Based on our results, we can conclude that the patients included in the HPN were correctly indicated, and all of them, based on the monitored parameters (regardless of gender, age, initial diagnosis, or BMI value), benefited from the applied treatment, which was correctly chosen based on their individual needs. Our results clearly document the irreplaceable role of HPN in the management of patients with nutritional intake disorders leading to the development of malnutrition. [ABSTRACT FROM AUTHOR]

Background: The emerging field of chrononutrition investigates the effects of the timing of nutritional intake on human physiology and disease pathology. It remains largely unknown when patients receiving home nutrition support routinely administer home parenteral nutrition (HPN) and/or home enteral nutrition (HEN). Methods: The present descriptive study included data collected from a patient‐oriented survey designed to assess the timing of infusions and sleep habits of patients receiving HPN and HEN in the United States. Results: A total of 100 patients were included. Patients had a mean age of 44.1 years and 81% were female. Among 73 patients supported with HPN and 27 patients supported with HEN, 86% and 44% reported overnight infusions, respectively. The median start and end times of overnight infusions were 2100 (interquartile range [IQR] = 1900–2200) and 0800 (IQR = 0700–1000), respectively, for HPN and 2000 (IQR = 1845–2137) and 0845 (IQR = 0723–1000), respectively, for HEN. Overnight infusions started 2.0 h (IQR = 1.1–3.0) and 2.0 h (IQR = 0.6–3.3) before bedtime for HPN and HEN, respectively, and stopped 12.9 min (IQR = −21.3 to 29.1) and 30.0 min (IQR = −17.1 to 79.3) after wake time for HPN and HEN, respectively. Sleep disruption because of nutrition support or urination was most common among patients receiving infusions overnight compared with those receiving infusions continuously or during the daytime. Conclusions: Our survey study focusing on a novel and medically relevant dimension of nutrition found that most HPN‐dependent and HEN‐dependent patients receive infusions overnight while asleep. Our findings suggest that overnight infusions coinciding with sleep may result in sleep and circadian disruption. [ABSTRACT FROM AUTHOR]

Introduction: Home parenteral nutrition (HPN) and/or home parenteral hydration (HPH) are the gold-standard treatment for patients with long-term intestinal failure (IF). The authors aimed to assess the impact of HPN/HPH on nutritional status and survival of long-term IF patients, as well as HPN/HPH-related complications. Methods: This was a retrospective study including IF patients under HPN/HPH followed in a single large tertiary Portuguese hospital. The data collected included demographics, underlying conditions, anatomical characteristics, type and duration of parenteral support, IF functional, pathophysiological, and clinical classifications, body mass index (BMI) at the beginning and end of follow-up, complications/hospitalizations, current patient status (deceased, alive with HPN/HPH, and alive without HPN/HPH), and cause of death. Survival after HPN/HPH beginning, until death or August 2021, was recorded in months. Results: Overall 13 patients were included (53.9% female, mean age 63.46 years), and 84.6% of patients presented type III IF and 15.4% type II. Short bowel syndrome caused 76.9% of IF. Nine patients received HPN and 4 HPH. Eight patients (61.5%) were underweight at the beginning of HPN/HPH. At the end of follow-up, 4 patients were alive without HPN/HPH, 4 maintained HPN/HPH, and 5 died. All patients improved their BMI (mean initial BMI 18.9 vs. 23.5 at the end, p < 0.001). Eight patients (61.5%) were hospitalized due to catheter-related complications, mainly infectious (mean hospitalization episodes 2.25, mean hospital stay of 24.5 days). No deaths were related to HPN/HPH. Conclusion: HPN/HPH significantly improved IF patients’ BMI. HPN/HPH-related hospitalizations were common, however causing no deaths, reinforcing that HPN/HPH is an adequate and safe therapy for long-term IF patients.

Patients on long-term home parenteral nutrition (HPN) occasionally develop glomerulonephritis due to chronic central venous catheter (CVC)-related infection. Most previously reported cases were membranoproliferative glomerulonephritis (MPGN). This is a case report of a 16-year-old girl receiving HPN for short bowel syndrome. After 11 years on HPN, she developed acute kidney injury with macroscopic hematuria, nephrotic-range proteinuria, and a reduced glomerular filtration rate (GFR). Initially, MPGN associated with chronic bacteremia was suspected with the assumption that the condition would be treated with antibiotics and CVC replacement. However, her kidney biopsy revealed antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis (AAG). This was consistent with the fact that the patient tested positive for proteinase 3-ANCA. Immunosuppressive therapy with methylprednisolone pulses (followed by oral prednisone) and rituximab led to remission. Her GFR and protein excretion returned to normal. Chronic bacteremia as a complication of long-term HPN may cause various types of glomerulonephritis including, rarely, AAG requiring immunosuppressive therapy.

Long term outcomes have been reported in home parenteral nutrition (HPN)-dependent patients with type 3 intestinal failure (IF), but there are limited survival data standardised to the general population that would help provide a meaningful prognosis for patients and clinicians. The primary aim of this study was therefore to investigate the survival of HPN-dependent patients and to evaluate the specific impact of type 3 IF on their life expectancy standardised to that of the general population. This was a cohort study of adult patients initiated on HPN between 1978 and 2018 at a national UK IF reference centre and followed up until death or censoring date of 31st December 2020. The standardised mortality ratio (SMR) was calculated as observed deaths divided by expected deaths using UK Office for National Statistics database. Excess Life Years Lost (LYL) were calculated separately for each sex as the differences in average life expectancy between patients with type 3 IF and the general population. Survival data were evaluated using cox regression models adjusting for confounding. In total, 1046 patients were identified, with a total observation time of 7344.1 patient-years. Patients with malignancy (n = 206) were excluded from the survival analysis. Of the remaining 840 patients, 398 were alive by the end of follow-up. The probability of survival was 91.8% at 1 year, 69.3% at 5 years, 54.3% at 10 years, 29.8% at 20 years and 16.7% at 30 years. Patients who did not achieve nutritional autonomy had an increased likelihood of death compared to patients who ceased HPN. In total, 40 (9.0%) deaths were HPN or IF-related, while underlying disease leading to IF accounted for 98 (22.2%) deaths. There were 270 (61.1%) deaths not related to IF, with the majority of these patients dying from infections unrelated to HPN. Overall mortality rates were higher among patients with a diagnosis of type 3 IF compared with the general UK population with a SMR of 7.48 (95% CI 6.80 to 8.21) and an excess mortality rate of 54.0 per 1000 person-years. All mechanisms of IF were associated with excess mortality, with SMR ranging from 6.82 (95% CI 5.98 to 7.72) for short bowel syndrome to 15.51 (95% CI 11.73 to 20.03) for dysmotility. On average, the excess LYL was 17.45 years for males and 17.39 years for females compared with the general population of the same age. This the largest single-centre series reporting survival outcomes in patients with type 3 IF over more than a four-decade period and the first to report LYL in this patient cohort. Type 3 IF was associated with more than seven-fold higher mortality rates than for the general UK population and shorter life expectancies of more than 17 years. Survival, however, was better in those able to achieve nutritional autonomy. Since the majority of deaths were due to non-HPN or non-IF causes, there is clearly a need now to further explore these causes of death in order to improve our understanding of excessive mortality in type 3 IF and develop ways to prevent it. [ABSTRACT FROM AUTHOR]

Qualitative research is a scientific method that systematically examines a phenomenon with the purpose of understanding and describing human experiences, exploring meanings and patterns, and illuminating the patient's lived experience. The Rhoads Research Lecture will highlight the power of patient voice and its importance to clinicians and researchers in addressing key clinical needs that are most relevant to patients receiving nutrition support. The subjective experience of patients who are dependent on home parenteral nutrition (HPN) will be shared, including how patients view HPN, define their quality of life (QOL), and describe the meaning of food in the context of being intravenously fed. As a result of these exploratory studies, the HPN patient-reported outcome questionnaire (HPN-PROQ) was developed and validated. Incorporating the HPN-PROQ in practice empowers patients to identify and communicate QOL and HPN therapy goals and clinicians to delve deeper in the provision of holistic and empathetic care. [ABSTRACT FROM AUTHOR]

Central line-associated bloodstream infections (CLABSIs) are the main complication in children with home parenteral nutrition (HPN) and some patients develop recurrent CLABSIs (REC-CLABSIs), defined as two or more infections within six months. Our aims were to assess the incidence and to characterize the risk factors of REC-CLABSIs in children with HPN. We characterized 79 HPN children from 2014 to 2019 and calculated the incidence of CLABSIs. To minimize the risk of bias related to the exposure time of the septic risk, we paired the patients according to their central venous catheter (CVC) dwell time. After analyzing the whole cohort, a univariate and multivariate unconditional logistic regression was performed on the paired cohort. We included 75 (94.9%) children with a mean age of 7.11 years. The rate of septicemia was 1.55/1000 CVC days, mainly with Staphyloccocus sp. The patients with recurrent CLABSIs (REC group) represented 25% of the cohort, with an incidence of 2.99/1000 CVC days. In the whole cohort, a higher risk of recurrent infections was significantly associated with a longer CVC dwell time (OR = 1.04, IC 95% [1.01–1.06], p = 0.004), and with care located in rehabilitation care facilities (RCF) compared to home (OR = 6, IC 95% [1.5–26.6], p = 0.012). When children were paired according to their CVC dwell time, only in univariate analysis did the care in RCF remain significant (OR = 6.27, IC 95% [1.21–32.5], p = 0.03). Recurrent CLABSIs incidence was 2.99/1000 CVC days. Our study suggests that preventive measures should be implemented especially in RCFs to reduce the proportion of children with recurrent infections. A multicenter study is needed to confirm our results in a larger cohort with several RCFs. [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: Patients supported with home parenteral nutrition (HPN) often report poor sleep; however, limited research has been conducted to objectively measure sleep patterns of HPN‐dependent patients. Methods: We aimed to characterize the sleep patterns of patients receiving HPN through 7‐day actigraphy in a home‐based observational study. Sleep measures of clinical importance were derived from actigraphy, including sleep duration, sleep efficiency, sleep onset latency, and wake after sleep onset. Participants also completed validated sleep surveys. Results: Twenty participants completed all study procedures (mean [SD]: age = 51.6 [13.9] years, body mass index = 21.4 [4.6], and 80% female). The population median (IQR) for sleep duration, sleep efficiency, sleep onset latency, and wake after sleep onset was 6.9 (1.1) h, 83.3% (7.8%), 11.8 (7.1) min, and 57.2 (39.9) min, respectively, and 55%, 60%, 35%, and 100% of participants did not meet the recommendations for these measures from the National Sleep Foundation. Sixty‐five percent of participants reported napping at least once during the 7‐day period. Based on the Insomnia Severity Index, 70% of participants were classified as having subthreshold or more severe insomnia. Based on the Pittsburgh Sleep Quality Index, 85% were classified as having significant sleep disturbance. Conclusion: Most HPN‐dependent patients likely have disrupted sleep largely driven by difficulty maintaining sleep. The extent to which HPN contributed to poor sleep cannot be elucidated from this observational study. Addressing known factors that contribute to sleep disruption and considering sleep interventions may improve the overall quality of life of patients receiving HPN. [ABSTRACT FROM AUTHOR]

Background: The administration of home parenteral nutrition improves quality of life for patients with intestinal failure, thus fostering their will to actively participate to social activities. Nevertheless, sports participation can be risky for patients with a central venous catheter (CVC). Despite literature thoroughly proving the positive impact of sports on motor-psychosocial development, no consistent evidence assessing its role on central-line complications is available. This study aimed to report the European centers' approach to children with intestinal failure on home parenteral nutrition and interested in playing sports, further assessing complications and how to prevent them., Materials and Methods: A questionnaire focusing on children with intestinal failure regarding physical activity was circulated to 20 centers. Questions assessed the centers' policy for CVC management, the sports-related recommendations for patients on home parenteral nutrition and complication rates., Results: Sixteen (80%) centers filled in the questionnaire. Twelve centers reported not to have a standardized formal protocol for catheter care during sports. All centers encouraged patients to perform mild/moderate exercise, whereas high-contact sports were allowed by one center only. Specific dressings were suggested to protect the vascular access device, especially for water sports. Only one sports-related complication (rupture) was reported., Conclusion: This survey emphasizes that sports should not be restricted in patients with intestinal failure and represents a blueprint for sports-related recommendations for these patients. Prospective studies assessing complication rates are advisable to ensure an improved access to sports for these patients., (© 2024 The Author(s). Nutrition in Clinical Practice published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Background & Aims: Home Parenteral Nutrition (HPN) prescriptions should be tailored to a patient's nutritional requirements and comprise of individually compounded regimens (IC-HPN) or standard licensed multi-chamber bags (MCB). There is a paucity of research exploring factors associated with admixture type. This study aimed to evaluate the nutrient composition of adult IC-HPN prescriptions and variance in nutrient dosing, make comparisons with the range of MCB available and dosing recommendations stated in international guidelines., Methods: This cross-sectional observational study analysed anonymised prescription data for adults under the care of a single home care company in England, commencing IC-HPN after January 2021., Results: There were 155 patient prescriptions (245 HPN bags) included, with body weight available in 82 (52.9 %) cases. Data were reported for each HPN bag, and summarised as an average daily amount, considering the number of different HPN bags and their daily frequency per patient prescription. A vast range of nutrients were prescribed, with variance in dosing highest for lipid, phosphate, and calcium; with coefficient of variance of 106 %, 74 % and 67 %, respectively. One-hundred-thirty-four (54.7 %) bags contained lipid, resulting in variable ratios of lipid to aqueous bags provided weekly. Negligible amounts of at least one electrolyte were prescribed in 93 (38.0 %) bags, of which 52 (21.2 %) contained ≤2 mmol calcium. Compared with MCB (65 bags), IC-HPN bags contained higher amounts of all nutrients, apart from nitrogen and phosphate. Mean sodium intake differed substantially between types of bags (IC-HPN 187.5, SD 100.1 mmol versus MCB 49.1, SD 31.7 mmol, p < 0.001). A substantial number of patients received HPN dosages beyond guideline recommendations, with higher amounts of sodium and fluid provided in 41 (50.0 %) cases; conversely, lower than recommended dosages were seen in 55 (67.1 %) for potassium and 61 (74.4 %) for calcium., Conclusions: This study has demonstrated vast variance in IC-HPN parenteral nutrient dosing, with minimal or an omission of at least one nutrient seen. Further research is needed to explore factors that influence usage of IC-HPN, clinical reasons related to lipid dosing and around the variation in doses prescribed outside of guideline recommendations., Competing Interests: Declaration of competing interest The authors declare no conflict of interests., (Copyright © 2024 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

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.)

Home parenteral nutrition (HPN) is a complex therapy, which requires dedicated facilities and expertise. However, the management and provision of HPN differs significantly between countries and between HPN centers within countries. These differences lead to heterogeneity in the quality of care received by patients, with variable impact on the appropriateness, safety, and effectiveness of HPN, and resultant variability in the quality of life that a patient may expect. The European Society for Clinical Nutrition and Metabolism (ESPEN) have published guidelines on the appropriate and safe provision of HPN, with an associated practical version describing a short and precise way to implement the guidelines' recommendations in clinical practice. This educational paper suggests means of implementation of evidence supported HPN guidelines, using "operational recommendations" applitngto healthcare professionals, administrators and stakeholders, with the ultimate aim of enhancing equity of patient access to an appropriate and safe HPN program of care., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Infusions of home parenteral nutrition (HPN) are often cycled at night coinciding with sleep episodes. Adult consumers of HPN are known to experience poor sleep attributed to frequent awakenings and long durations of wakefulness after falling asleep. Consequently, most consumers do not meet recommendations for sleep duration and quality or daytime napping. The primary underlying pathophysiology resulting in sleep problems is nocturia; however, other factors also exist, including disruptions caused by medical equipment (ie, pump alarms), comorbid conditions, dysglycemia, and medication use. Early guidance on sleep is imperative because of the central role of sleep in physical health and wellbeing, including mitigating complications, such as infection risk, gastrointestinal problems, pain sensitivity, and fatigue. Clinicians should routinely inquire about the sleep of their patients and address factors known to perturb sleep. Nonpharmacologic opportunities to mitigate sleep problems include education on healthy sleep practices (ie, sleep hygiene); changes in infusion schedules, volumes, rates, and equipment; and, possibly, behavioral interventions, which have yet to be examined in this population. Addressing comorbid conditions, such as mood disorders, and nutrition deficiencies may also help. Pharmacologic interventions and technological advancement in HPN delivery are also needed. Research on sleep in this population is considered a priority, yet it remains limited at this time., (© 2024 The Author(s). Nutrition in Clinical Practice published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare disorder commonly diagnosed in later disease stages when it prominently manifests as malnutrition. We report on a female patient diagnosed with MNGIE at the age of 36. She was severely malnourished due to loss of resorptive surface after several surgical procedures, gastrointestinal dysmotility, and small intestinal bacterial overgrowth. Therefore, early and aggressive total parenteral nutrition was introduced. Although no reports have shown that nutritional support can modify the clinical outcome, this case suggests that adequate nutritional support, particularly parenteral nutrition, supervised by an experienced nutritional team, may prolong the lifespan of patients with MNGIE.

Introduction: Maintaining adequate nutritional status can be a challenge for patients with small bowel neuroendocrine tumours (NETs). Surgical resection could result in short bowel syndrome (SBS), whilst without surgical resection there is a considerable risk of ischemia or developing an inoperable malignant bowel obstruction (IMBO). SBS or IMBO are forms of intestinal failure (IF) which might require treatment with home parenteral nutrition (HPN). Limited data exist regarding the use of HPN in patients with small bowel neuroendocrine tumours, and it is not frequently considered as a possible treatment. Methods: A systematic review was performed regarding patients with small bowel NETs and IF to report on overall survival and HPN-related complications and create awareness for this treatment. Results: Five articles regarding patients with small bowel NETs or a subgroup of patients with NETs could be identified, mainly case series with major concerns regarding bias. The studies included 60 patients (range 1–41). The overall survival time varied between 0.5 and 154 months on HPN. However, 58% of patients were alive 1 year after commencing HPN. The reported catheter-related bloodstream infection rate was 0.64–2 per 1000 catheter days. Conclusion: This systematic review demonstrates the feasibility of the use of HPN in patients with NETs and IF in expert centres with a reasonable 1-year survival rate and low complication rate. Further research is necessary to compare patients with NETs and IF with and without HPN and the effect of HPN on their quality of life.

The development of intestinal failure-related complications in Finnish adults is unknown. This study aimed to investigate the incidence of catheter-related bloodstream infections (CRBSI), and the longitudinal changes in biochemical liver and kidney tests in a nationwide cohort. The search for Finnish adults with intestinal failure (IF) utilized a survey to Finnish health-care providers (n = 111) with the potential to provide long-term parenteral support (PS) for adult IF. Our nationwide, cross-sectional cohort included all IF patients aged ≥ 18 years who had received PS for ≥ 120 d in 2017. Data regarding CRBSI and biochemical liver and kidney tests were collected from patient records at the start of PS up to the latest available measurement in 2017. In the nationwide cohort of 52 patients, the CRBSI incidence was 1.35/1000 catheter days. Seventy-three percent of CRBSI in a long-term catheter led to catheter replacement. During a median PS duration of 27.5 (interquartile range [IQR] 11.3–57.3) months, a statistically significant median change occurred in estimated glomerular filtration rate (eGFR; −8.5 ml/min/1.73 m2, IQR −30–7, p =.005) and alkaline phosphatase (ALP; 26 U/l, IQR −11–95, p =.019). In a multiple regression model for eGFR at data collection, baseline eGFR and age were strong explanatory variables. Incidence of CRBSI, but not treatment strategies, in this nationwide adult IF population correspond well to those reported from specialized centers. Decreased kidney function and abnormal liver test results are frequent findings, and even more so over time, emphasizing the importance of regular monitoring. [ABSTRACT FROM AUTHOR]

Introduction. Facing with an increasing demand for transition to adult care management and guidelines, our Home Parenteral Nutrition (HPN) team designed an Adolescent Therapeutic Educational Program (ATEP) specifically intended for adolescents on long-term HPN. The aim of this study was to report on the first sessions of this program. Methods. The ATEP is designed in 3 sessions of 5 consecutive days, during school holidays over the year. The ATEP includes group sessions on catheter handling, disconnecting and connecting the PN and catheter dressing, dealing with unforeseen events such as fever or catheter injury, but also sessions with psychologist, social worker, sports teacher, fashion specialist, meeting with adults who received HPN since childhood. Specific course for the accompanying parents were also provided. Six months after the last session, a 3-day trip to the attraction park "le Futuroscope", Poitiers, France, was organized without any parental presence. Results. Along the three training courses, a total of 16 adolescents were enrolled in the ATEP. They were aged between 13 and 17 years (median 14 years IQR: 14-16.25). All were on long term HPN starting during the neonatal period except for five who started PN at a median age of 10-year-old (IQR: 1-10). At the time of the ATEP, their median PNDI was 105% (IQR: 95.5-120.8) while receiving a median number of 6 infusions per week (IQR: 5-7). Thirteen out of 16 received Taurolidine lock procedure. At the end of the three sessions, 11 adolescents could be considered as fully autonomous, 4 as partially autonomous and one failed to gain any autonomy. Course evaluation by adolescents or parents was good to excellent. Conclusion. Through the holistic and multi-professional approach of this training and the group cohesion, the adolescents were not only able to handle catheter care and PN connections but were able to understand and accept better their illness and project themselves into their own future. [ABSTRACT FROM AUTHOR]

While the history of nutrition support dates to the ancient world, modern home parenteral and enteral nutrition (HPEN) has been available since the 1960s. Home enteral nutrition is primarily for patients in whom there is a reduction in oral intake below the amount needed to maintain nutrition or hydration (i.e., oral failure), whereas home parenteral nutrition is used for patients when oral-enteral nutrition is temporarily or permanently impossible or absorption insufficient to maintain nutrition or hydration (i.e., intestinal failure). The development of home delivery of these therapies has revolutionized the field of clinical nutrition. The use of HPEN appears to be increasing on a global scale, and because of this, it is important for healthcare providers to understand all that HPEN entails to provide safe, efficacious, and cost-effective support to the HPEN patient. In this article, we provide a comprehensive review of the indications, patient requirements, monitoring, complications, and overall process of managing these therapies at home. Whereas some of the information in this article may be applicable to the pediatric patient, the focus is on the adult population. [ABSTRACT FROM AUTHOR]

Intestinal failure is defined as the inability to absorb the minimum of macro and micronutrients, minerals and vitamins due to a reduction in gut function. In a subpopulation of patients with a dysfunctional gastrointestinal system, treatment with total or supplemental parenteral nutrition is required. The golden standard for the determination of energy expenditure is indirect calorimetry. This method enables an individualized nutritional treatment based on measurements instead of equations or body weight calculations. The possible use and advantages of this technology in a home PN setting need critical evaluation. For this narrative review, a bibliographic search is performed in PubMed and Web of Science using the following terms: ‘indirect calorimetry’, ‘home parenteral nutrition’, ‘intestinal failure’, ‘parenteral nutrition’, ‘resting energy expenditure’, ‘energy expenditure’ and ‘science implementation’. The use of IC is widely embedded in the hospital setting but more research is necessary to investigate the role of IC in a home setting and especially in IF patients. It is important that scientific output is generated in order to improve patients’ outcome and develop nutritional care paths.

Background: Appropriate nutritional support, including supplemental home parenteral nutrition (sHPN), may improve prognosis and quality of life (Qol) of malnourished cancer patients. We aimed to explore the cost-effectiveness of sHPN for incurable gastrointestinal cancer patients from the Chinese healthcare perspective. Method: Clinical data were extracted from a randomized controlled trial (NCT02066363). Patients were randomized into the sHPN group or the non-sHPN group (receiving best practice nutritional care). A Markov model was established with a 6-week cycle length. Costs were acquired from local hospitals, effect parameters included quality-adjusted life year (QALY), Qol, body mass index, fat-free mass (FFM), FFM index, handgrip strength, and a 6-min walking test. Sensitivity analyses were conducted with a willingness-to-pay (WTP) set at 3 per capita gross domestic product ($29,307/QALY). Results: When considering QALY as a utility, the incremental cost-effectiveness ratio (ICER) was $24,289.17, with an incremental cost of $2,051.18 and an incremental QALY of 0.0844 between the sHPN group and the non-sHPN group. Furthermore, we explored the cost-effectiveness of sHPN from multidimensions, where we analyzed various effect parameters at different visits; the results showed a superior benefit for patients in the sHPN group except for the handgrip parameter at visit 2. Sensitivity analysis demonstrated the influence of utilities in the sHPN group, but the sHPN group was still cost-effective with a WTP of $2,500/QALY. Conclusion: In China, sHPN was cost-effective for patients with incurable gastrointestinal cancer, which suggested further applications in clinical practice and provided references for clinical decisions and pricing. [ABSTRACT FROM AUTHOR]

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]

Patient Support Programs (PSPs) reinforce patients' care provided by health care professionals with the aim to improve adherence and patient empowerment. PSPs may include interventions such as home-based care, individualized medication counseling, support, training, and home delivery of medicines and/or devices. This study described these services and its impact on patient-reported outcomes and health care savings. We conducted an integrative literature review which was limited to publications from the last 10 years (2009-2019) and focused on diseases that require special support and/or parenteral administration. From 7040 total citations, we identified 64 home-based care services performed worldwide. Among the home-based care services, most were provided by nurses (n = 47/64; 73.4%) and addressed to cancer patients (n = 22/64; 34.4%); 23 out of 64 services (35.9%) incorporated telepharmacy. In general, home-based services and PSPs showed a positive impact on patients' adherence to medication, patient satisfaction, and health-related quality of life. In addition, 14 (21.9%) services reported economic results, most of which showed that home therapy led to substantial cost savings. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) and enterocutaneous or enteroatmospheric fistulas are common indications for home parenteral nutrition (HPN). However, there are few data describing factors influencing surgical decision-making or outcomes particularly following fistula development. We aimed to compare outcomes between patients with SBS and fistulas and explore surgical decision-making. HPN-dependent adults from 2001–2018 at a national reference centre were included in this study. HPN cessation was analysed using death as competing risk. In total, 465 patients (SBS (62%), fistula (38%)) were included, with median HPN dependency of 2.6 years. In total, 203 patients underwent reconstructive surgery; while frailty was the commonest reason for not undergoing surgery (49.2%), 22.7% declined surgery. Overall, 170 ceased HPN, with a probability of 13.8%, 34.1% and 38.3% at 1, 5 and 10 years, respectively. Patients undergoing surgery had higher nutritional autonomy rates (109.8 incidences/1000 patient years) compared to those not undergoing surgery (18.1 incidences/1000 patient years; p < 0.001). A total of 295 patients (63.4%) were predicted to cease HPN based on gastrointestinal anatomy but only 162/295 (54.9%) achieved this; those unable to do so were older with a higher comorbidity index. There were no differences in long-term nutritional and survival outcomes or surgical decisions between patients with SBS and fistulas, or between enterocutaneous and enteroatmospheric fistulas. This study represents one of the largest datasets describing the ability of HPN-dependent patients with SBS or fistulas to achieve nutritional autonomy. While reconstructive surgery facilitates HPN cessation, approximately one-fifth of patients declined surgery despite HPN dependency. These data will better inform patient expectation and help plan alternative therapies. [ABSTRACT FROM AUTHOR]

Aim of the study: Parenteral nutrition associated liver disease (PNALD) is a frequently reported complication of long-term parenteral nutrition. Early diagnosis and treatment of PNALD can help prevent end-stage liver disease. The aim of the study was to evaluate the activity of aminotransferases as a marker of liver dysfunction in patients receiving home parenteral nutrition under the care of a reference center. Material and methods: A comprehensive analysis of patients' medical records from a 9-year period (December 2012 - December 2021) was conducted and the following parameters were evaluated: parenteral nutrition mixture composition, total plasma bilirubin, activity of the liver enzymes alanine aminotransferase (ALT) and aspartate aminotransferase (AST), standardized time factor prothrombin (international normalized ratio [INR] factor) and serum albumin. The analysis covered 630,537 days of parenteral nutrition. The study included 251 patients (140 women and 111 men) included in the Home Parenteral Nutrition Program. Results: PNALD was diagnosed in 11 parenteral fed patients, which gives the frequency of 8.3%/9 years of treatment. Two deaths were classified as cause of death related to liver disease but not related to PNALD. None of the patients included in the analysis developed end-stage liver failure. Conclusions: The above analysis shows that individual selection of the composition of the mixture for intrave- nous nutrition significantly reduces the risk of PNALD and may prevent liver failure in this context. [ABSTRACT FROM AUTHOR]

Background: In short‐bowel syndrome (SBS) treated with parenteral nutrition (PN), multiple complications can occur. The etiology of kidney stones may be linked to the underlying disease thrombosis, surgical complications, complications of therapy for cancer, Crohn's disease, metabolic abnormalities resulting from morphological and functional changes in the gastrointestinal tract, and to treatment used. We analyzed all these parameters in a large cohort of patients receiving home PN (HPN), to define the incidence of stones and groups of patients particularly at risk of stone formation. One of the objectiveswas to develop a predictive model of urolithiasis. Methods: This observational retrospective study included 459 patients with SBS recieving HPN in a single center. Patient records were evaluated for demographics, SBS etiology, and underlying disease, anatomy of the gastrointestinal tract, intestinal failure classification, nutrition regimen, and presence of urolithiasis. Results: Kidney stones were diagnosed in 24% of patients. Nodifferences in incidence were noted between the various etiologic groups. The incidence in patients with a colon in continuity and those with an end stoma was similar. The length of residual small bowel did not play a role in stone formation. There were no differences between patients according to the severity of intestinal failure. In patients treated with PN and limited oral feeding, the risk of urolithiasis was twice as high as in patients receiving PN only. Conclusions: Patients developed urolithiasis with no relation to the SBS etiology. The risk of kidney stone formation was higher in patients recieving PN with oral feeding. [ABSTRACT FROM AUTHOR]

BackgroundAppropriate nutritional support, including supplemental home parenteral nutrition (sHPN), may improve prognosis and quality of life (Qol) of malnourished cancer patients. We aimed to explore the cost-effectiveness of sHPN for incurable gastrointestinal cancer patients from the Chinese healthcare perspective.MethodClinical data were extracted from a randomized controlled trial (NCT02066363). Patients were randomized into the sHPN group or the non-sHPN group (receiving best practice nutritional care). A Markov model was established with a 6-week cycle length. Costs were acquired from local hospitals, effect parameters included quality-adjusted life year (QALY), Qol, body mass index, fat-free mass (FFM), FFM index, handgrip strength, and a 6-min walking test. Sensitivity analyses were conducted with a willingness-to-pay (WTP) set at 3 per capita gross domestic product ($29,307/QALY).ResultsWhen considering QALY as a utility, the incremental cost-effectiveness ratio (ICER) was $24,289.17, with an incremental cost of $2,051.18 and an incremental QALY of 0.0844 between the sHPN group and the non-sHPN group. Furthermore, we explored the cost-effectiveness of sHPN from multidimensions, where we analyzed various effect parameters at different visits; the results showed a superior benefit for patients in the sHPN group except for the handgrip parameter at visit 2. Sensitivity analysis demonstrated the influence of utilities in the sHPN group, but the sHPN group was still cost-effective with a WTP of $2,500/QALY.ConclusionIn China, sHPN was cost-effective for patients with incurable gastrointestinal cancer, which suggested further applications in clinical practice and provided references for clinical decisions and pricing.

Background Composite lipid emulsions containing soybean oil (30%), medium-chain triglycerides (30%), olive oil (25%), and fish oil (15%) (SMOF) are now widely used. Objectives We aimed to evaluate the tolerance, the efficiency, and the erythrocyte fatty acid (FA) profile for children on long-term home parenteral nutrition (HPN) receiving a composite fish oil–based emulsion (FOLE). Methods At baseline, children (n  = 46) with severe intestinal failure highly dependent on parenteral nutrition (PN) for ≥1 y were included in the study when they had received the composite FOLE for >6 mo. Out of this baseline group, only 25 children remained highly PN-dependent (SMOF1, n  = 25) and could be assessed a second time, 2.4 y later (SMOF2, n  = 25). An independent control group ("weaned off PN" group; n  = 24) included children who had been weaned off PN for >2 y (median: 4 y). RBC-FA composition was established by GC-MS. Growth parameters, plasma citrulline, conjugated bilirubin, FA profiles, and the Holman ratio (20:3ω-9/20:4ω-6) were compared between groups. Results No difference for growth parameters, citrulline, and bilirubin was observed between the SMOF groups after 2.4 y (0.2 <  P  < 0.8). The weaned-off group did not differ from the SMOF groups for growth parameters (0.2 <  P  < 0.4) but citrulline was higher (P  < 0.0001) and conjugated bilirubin lower (P  < 0.01). The composite FOLE induced higher proportions of EPA (20:5n–3) (8.4% ± 2.9%) and DHA (22:6n–3) (11.7% ± 2.2%) than what was observed in weaned-off children (0.8% ± 0.4% and 6.6% ± 2.3%, respectively) but lower proportions of arachidonic acid (20:4n–6). However, the Holman ratio did not vary between groups (P  = 0.9), whereas the PUFA concentrations varied widely. Conclusions Long-term use of the composite FOLE was well tolerated in HPN-dependent children. The RBC-FA profile alterations were consistent with the ω-3 PUFA–enriched composition of this emulsion without evidence of essential FA deficiency. [ABSTRACT FROM AUTHOR]

Background: There is a demographic shift toward older patients receiving home parenteral nutrition (HPN), but data on clinical outcomes are limited. The objective of this study was to determine differences between older and younger HPN patients in regard to HPN indications, prescriptions, and outcomes over the first 2 years receiving HPN. Methods: This was a retrospective analysis of prospectively collected data from HPN adult patients entered in the Canadian HPN Registry. New HPN patients enrolled between 2003 and 2017 and receiving HPN for at least 2 years were selected. Data included demographics, PN prescriptions, catheter‐related bloodstream infections (CRBSIs) over the past year, survival, and quality of life based on Karnofsky Performance Status (KPS). Results: Four hundred two patients were included: 184 patients were ≥60 years old, and 219 patients were between 18 and 59 years old. There were no differences in the main indications for HPN, body mass index (BMI), and PN prescriptions at baseline. At 2 years, younger patients received more energy from PN than older patients (27.9 vs 19.6 kcal/kg; P <.001), but BMI remained comparable. There were fewer CRBSIs in the older group (20% vs 36%, P =.0023), but 78% of younger patients remained alive vs 69% in the older group (P =.0401). In those alive, the proportion of patients continuing to receive HPN was comparable and the proportion of patients with a KPS ≥60. Conclusions: Older HPN patients have similar clinical characteristics as younger patients but have fewer CRBSIs and higher 2‐year mortality. [ABSTRACT FROM AUTHOR]

Patients on long-term home parenteral nutrition (HPN) occasionally develop glomerulonephritis due to chronic central venous catheter (CVC)-related infection. Most previously reported cases were membranoproliferative glomerulonephritis (MPGN). This is a case report of a 16-year-old girl receiving HPN for short bowel syndrome. After 11 years on HPN, she developed acute kidney injury with macroscopic hematuria, nephrotic-range proteinuria, and a reduced glomerular filtration rate (GFR). Initially, MPGN associated with chronic bacteremia was suspected with the assumption that the condition would be treated with antibiotics and CVC replacement. However, her kidney biopsy revealed antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis (AAG). This was consistent with the fact that the patient tested positive for proteinase 3-ANCA. Immunosuppressive therapy with methylprednisolone pulses (followed by oral prednisone) and rituximab led to remission. Her GFR and protein excretion returned to normal. Chronic bacteremia as a complication of long-term HPN may cause various types of glomerulonephritis including, rarely, AAG requiring immunosuppressive therapy. [ABSTRACT FROM AUTHOR]

Home parenteral nutrition (HPN) is a life-sustaining therapy for individuals with intestinal failure in a community setting. It refers to the intravenous infusion of macronutrients, micronutrients, fluids and electrolytes. Routinely used HPN solutions contain different quantities of these components. Consequently, each HPN solution may have different impacts on metabolism, inflammation and oxidative stress. Long-term use of HPN can lead to a number of adverse health outcomes including the development of metabolic bone disease, intestinal failure associated liver disease and poor quality of life but whether, and how, the composition of HPN solutions contributes to these health sequelae is poorly understood. The aim of this study is to systematically review and evaluate the evidence for the differential effects of HPN solutions and to understand what features are associated with differences in clinical endpoints. A systematic literature search was conducted between September and December 2020, and updated in July 2021 using the MEDLINE (Ovid), EMBASE, Scopus, and Web of Science databases. Studies were selected according to the following criteria (a) adult participants (>18 years old) dependent on HPN; (b) randomised controlled trials, prospective cohort and cross-sectional study designs; (c) primary research comparing two or more HPN solutions and (d) published in English language. Data were extracted and study quality assessed using Cochrane Collaboration's tools: Risk of Bias for Randomised Controlled Trials (RCTs); Risk of Bias in Non-Randomised Studies of Interventions; and the Newcastle Ottawa Scale for cross-sectional studies. Of the 5148 articles identified, seven RCTs, two prospective cohort and one cross-sectional study were included with a total of 295 participants. Studies varied in terms of duration (one to 60 months) and sample size (n = 5 to 88). Ten studies compared lipid emulsions (LE) and one study also compared LE with lipid-free HPN. No studies were found that compared the amino acid, vitamin, trace element or electrolyte components of HPN. In general, LE were well tolerated with no significant adverse effects. LE containing olive +/or fish oil were associated with a lower ω-6:ω-3 fatty acid ratio, positive reductions in markers of liver function, and changes in blood and cell fatty acid profiles. Despite the increasing use of HPN, there is surprisingly little evidence available to guide the provision of macro and micronutrients in the adult population requiring this therapy. Although LE containing olive +/or fish oil show promise with regards to liver function and blood and cell fatty acid profiles, further studies are needed before drawing definitive conclusions on the clinical value of these emulsions. It is likely that one type of HPN solution alone cannot be uniformly applied to patient care, and each patient should be assessed on an individual basis. [ABSTRACT FROM AUTHOR]

Background: Renal dysfunction can complicate home parenteral nutrition (HPN). The aims were, in the context of pediatric HPN, to assess renal function using the measured glomerular filtration rate (mGFR), determine the most accurate formula(s) to estimate GFR, and identify possible underlying mechanisms of renal impairment.Methods: A retrospective study was performed in 2 centers. Patients receiving HPN and aged 2-16 years without medical history of nephropathy were included. GFR was measured using iohexol clearance. Estimated GFR (eGFR) was calculated using creatinine, cystatin C-based, and combined (eGFRcr+cyst ) Schwartz formulas.Results: A total of 36 patients (18 females) were included; they received HPN for 8 (2-16) years. The primary digestive disease was short-bowel syndrome for 16 (44%) patients, gastrointestinal motility disorder for 10 (28%), or congenital diarrhea for 10 (28%). The median (range) mGFR was 99 (33-136) ml/min/1.73 m2 ; 9 (25%) patients had mildly decreased mGFR (<90 and ≥60 ml/min/1.73 m2 ), and 2 (6%) had mildly to severely decreased mGFR (<60 ml/min/1.73 m2 ). The eGFRcr+cyst formula was the most accurate and precise to estimate GFR. A significant negative correlation between mGFR and PN duration was found for patients receiving PN for 6-7/7 days (P = .008). Activation of the renin-angiotensin system was identified in 15 of 36 (42%) patients.Conclusion: Renal dysfunction was frequent and correlated with the duration of PN only for patients with the most severe intestinal failure. The use of eGFRcr+cyst improves its detection in these patients. Chronic dehydration may be an underlying mechanism. [ABSTRACT FROM AUTHOR]

Background: Home parenteral nutrition (HPN) is a life‐saving therapy for patients with chronic intestinal failure but can be associated with a degree of psychological distress. The factors associated with the need for antidepressants (ADs) in this cohort have not yet been described. Methods: The study involved prospective data collection from patients attending an HPN clinic at a national intestinal failure referral center. Patients requiring HPN as a result of active malignancy were excluded. Patients were divided in 2 groups according to AD usage; demographic, anthropometric, socioeconomic characteristics, and intravenous supplementation (IVS) regimens were compared between groups. Results: A total of 184 patients were recruited between July 2018 and April 2019, with an overall prevalence of AD use of 41.7% (70/168 patients). Daily mean IVS volume was significantly higher among patients taking AD ("AD" group; 2125.48 ± 991.8 ml/day, "no‐AD" group; 1828.54 ± 847.0 ml/day, P =.039), with the proportion of patients needing high‐volume IVS (≥3000 ml/day) being 3 times higher in the AD group (20.0%(14/70 patients) vs 6.1% (6/98 patients), P =.006). The average energy IVS infusion per day was similar between the groups. Conclusion: This is the first study to demonstrate that AD use correlates with higher IVS volume rather than energy requirements in HPN patients, suggesting that high IVS volume requirements may be better associated with the patient's disease burden. Early and tailored mental health intervention may be beneficial in those with high IVS volume requirements. [ABSTRACT FROM AUTHOR]

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