The Czech Home Parenteral Nutrition Registry REDNUP: Comprehensive Analysis of Adult Patients' Data.

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.

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

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

High-protein home parenteral nutrition in malnourished oncology patients: a systematic literature review.

INTRODUCTION: Up to 83% of oncology patients are affected by cancer-related malnutrition, depending on tumour location and patient age. Parenteral nutrition can be used to manage malnutrition, but there is no clear consensus as to the optimal protein dosage. The objective of this systematic literature review (SLR) was to identify studies on malnourished oncology patients receiving home parenteral nutrition (HPN) where protein or amino acid delivery was reported in g/kg bodyweight/day, and to compare outcomes between patients receiving low (< 1 g/kg bodyweight/day), standard (1-1.5 g/kg/day), and high-protein doses (> 1.5 g/kg/day). METHODS: Literature searches were performed on 5th October 2021 in Embase, MEDLINE, and five Cochrane Library and Centre for Reviews and Dissemination databases. Searches were complemented by hand-searching of conference proceedings, a clinical trial registry, and bibliographic reference lists of included studies and relevant SLRs/meta-analyses. RESULTS: Nineteen publications were included; sixteen investigated standard protein, two reported low protein, and one included both, but none assessed high-protein doses. Only one randomised controlled trial (RCT) was identified; all other studies were observational studies. The only study to compare two protein doses reported significantly greater weight gain in patients receiving 1.15 g/kg/day than those receiving 0.77 g/kg/day. CONCLUSION: At present, there is insufficient evidence to determine the optimal protein dosage for malnourished oncology patients receiving HPN. Data from non-HPN studies and critically ill patients indicate that high-protein interventions are associated with increased overall survival and quality of life; further studies are needed to establish whether the same applies in malnourished oncology patients.

Parenteral nutrition at home/long-term parenteral nutrition.

PURPOSE: Some diseases require that patients receive parenteral nutrition (PN) over a prolonged time period. Long-term administration of PN can further complicate an already complex therapy, posing additional risk of potential complications. This article is based on presentations and discussions held at the International Safety and Quality of PN Summit, providing insights into aspects of home PN (HPN) and examples of good HPN practice. SUMMARY: One critical step in the HPN process is when patients transition from a hospital to a home setting, and vice versa. Generally, electronic PN ordering is not feasible in an HPN setting, leading to potential difficulties in communication and coordination. HPN requires that patients (or their home caregivers) administer PN, and thus their education and competency are crucial. Likewise, the choice of PN formulation is of great importance. For example, using more modern intravenous lipid emulsions containing medium-chain triglycerides, olive oil, and/or fish oil can provide benefits in terms of liver function during long-term HPN. Internationally, there are wide variations in delivery of HPN, with compounded PN dominating in some countries while others make greater use of market-authorized multichamber bags (MCBs). Patient-related factors, institutional considerations, and the availability of different MCB formulations, are also contributing factors guiding formulation and delivery system preferences. CONCLUSION: Education and communication remain key components of a successful HPN process. The information shared here may help to motivate efforts to improve HPN processes and to consider the often-differing perspectives of patients and their healthcare professionals.

High Dose Fish Oil Added to Various Lipid Emulsions Normalizes Superoxide Dismutase 1 Activity in Home Parenteral Nutrition Patients.

(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.

Funding and resource availability for home parenteral nutrition in Australia: A national cross-sectional survey.

BACKGROUND: Home parenteral nutrition (HPN) is a life-saving therapy required for the management of type III intestinal failure, one of the rarest organ failures. It requires a multidisciplinary approach to manage the complexity of the underlying medical, surgical, and nutrition issues, but the current levels of healthcare funding in Australia are unknown. This study aimed to quantify the caseload, staffing, and capacity of existing HPN centers nationally. METHODS: This was a cross-sectional survey inviting centers known to provide HPN care. The survey was designed to capture metrics related to the national framework for the delivery of HPN. These centered on staffing levels, patient load, capacity to audit key outcomes, and service challenges. RESULTS: A total of 24 (89%) of 27 invited centers responded to the survey. There were 17 (71%) adult centers and 7 (29%) pediatric centers. Adult centers managed a median of 12 (interquartile range [IQR]: 6-25) patients vs 16 (IQR: 9-17) in pediatric centers. Several centers did not have dedicated funding for core team members. The total funded clinician time each week per patient was 7 min (IQR: 0-12 min) in adult centers and 14 min (IQR: 10-21 min) in pediatric centers. Fewer than half of centers reported having sufficient resources to regularly audit key metrics. CONCLUSION: The availability of dedicated expertise to manage the highly complex needs of people living with type III intestinal failure is lacking in Australia. Current funding of HPN services falls well short of being sufficient to meet the requirements outlined in the national quality framework.

Insights from home parenteral nutrition infusion data.

OBJECTIVE: Patients with chronic intestinal failure use home parenteral nutrition infusion support. Non-compliance of home parenteral nutrition treatment is well documented, especially if clinical resources are remote. Objective delivery data from Infusion Pump reports have the potential to support treatment progress and planning. The aim of this study was to report the efficacy and accuracy of the Eitan Insights digital health platform for home parenteral nutrition use (a platform providing data-driven insights from the pump-recorded data). METHODS: A prospective, single-center observational study of 20 patients treated with home parenteral nutrition ≥3 d/wk was conducted over 2022. The patients recorded the pre- and postinfusion home parenteral nutrition bag weight, duration of infusion, and alarms. We compared manual records to the pump data. Repeated measures analysis of variance was used for statistical analysis. RESULTS: A total of 45 data sets were collected, with no adverse events noted. In multiple comparisons between patient factors and descriptive statistics, there was no significant difference between manually recorded and pump-recorded data for volume infused (mean values of manual versus pump were 1707 ± 362 mL and 1708 ± 405 mL; P = 0.939) and infusion duration (mean values of manual versus pump iwere 9h 43 min ± 2.48 SD versus 9h 45 min ± 2.41 SD; P = 0.858). CONCLUSION: The data collected by the digital platform accurately reflect patients' infusion data. This connected device has the potential to allow clinicians to be more informed and assess treatment trends and proactive resource planning through the Infusion Pump data insights.

Assessment of clinical outcome after sudden alteration of home parenteral nutrition program from customised to commercially premixed admixtures. Experience with short bowel syndrome treatment.

BACKGROUND & AIMS: Disruptions in the home parenteral nutrition (HPN) process may lead to failure to achieve the intended treatment purposes. This study aimed to evaluate the mid-term clinical outcome in a group of patients with short bowel syndrome (SBS) after a sudden change in the type of home parenteral nutrition programs from customized to commercially premixed admixtures. METHODS: The study conducted in 2020 identified 51 patients with SBS: 27 (53%) women and 24 (47%) men. The SBS were classified as SBS with end jejunostomy 23 (45%) patients, with jejuno-colon anastomosis 23 (45%) patients, with jejuno-ileo anastomosis and total colon in continuity 5 (10%) patients. The following therapeutic program-related data were analysed: changes in nutritional status, body mass index (BMI), Controlling Nutritional Status (CONUT) score, and biochemical assessment. For statistical analysis, the Wilcoxon rank-sum and signed-rank paired tests with continuity corrections were used to compare the results. A p-value of <0.001 was considered statistically significant. RESULTS: There was no statistically significant difference between the analysed groups in total energy, amino acid concentrations, and intravenous volume supplementation. BMI and CONUT assessments of nutritional status and selected biochemical parameters were stable during the study period. CONCLUSIONS: The study demonstrated that a sudden change in the HPN therapy program from parenteral admixtures, tailored to meet individual patients' needs, to commercially premixed admixtures had no significant impact on the mid-term clinical condition of patients with SBS.

Factors that impact on the quality of life of intestinal failure patients treated with home parenteral nutrition: protocol for a multicentre, longitudinal observational study.

BACKGROUND: Home parenteral nutrition (HPN) refers to the intravenous administration of macronutrients, micronutrients and fluid. The aims of treatment are to increase survival and improve quality of life (QoL). However, patients struggle with physiological symptoms, time-consuming invasive therapy and an increased occurrence of depression and social isolation. Our aim is to understand how HPN impacts the QoL of patients, and the contribution played by the complications of treatment, for example, liver disease. METHODS AND ANALYSIS: A multicentre, longitudinal, observational study will be conducted using routinely collected clinical data. Participants will also be asked to complete three QoL questionnaires (EuroQol-5 Dimensions, Short Form 36 and HPN-QoL) at baseline and 12 months. The primary outcome is mean change in QoL scores over 12 months. Secondary outcomes include how factors including liver function, gut microbiota, number of infusions of PN per week, nutritional composition of PN and nutritional status impact on QoL scores. ETHICS AND DISSEMINATION: Ethical approval was obtained from HRA and Health and Care Research Wales Research Ethics Committee (21/SC/0316). The study was eligible for portfolio adoption, Central Portfolio Management System ID 50506. Results will be disseminated through peer-reviewed scientific journals and presented at national and international meetings.

Ready-to-Use Multichamber Bags in Home Parenteral Nutrition for Patients with Advanced Cancer: A Single-Center Prospective Study.

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.

Epidemiology of home enteral and parenteral nutrition in adults: Comprehensive national data.

BACKGROUND AND AIMS: Although HEN and HPN are important parts of home healthcare, lack of information about the number of patients concerned is surprising. This educational paper presents a review of literature concerning published national data on epidemiology of both HEN and HPN, for different countries. It compares these publications with an exhaustive retrospective observational study on our national adult population for the year 2019. METHODS: Our presented retrospective observational national study was carried out on patients aged 20 and over, divided by sex and into three age groups: 20-39, 40-59, 60 years old and over. Open data from the National Health Insurance obtained the number of patients who started a HEN or HPN during 2019 (incidence), as well as those followed in HEN or HPN during this year (prevalence). Results are compared with a literature review compiling all available publications presenting national data on epidemiology of both HEN and HPN for a same country. RESULTS: This work covered an entire national population of 50.881 948 adults. The incidence and prevalence of HEN were respectively 37.4/100 000 inhab/year and 74.0/100 000 inhab, with significant gender and age differences. The incidence and overall prevalence of HPN were respectively 22.0/100 000 inhab/year and 25.3/100 000 inhab. The prevalence of HPN during over 12 weeks was 6.2/100 000 inhab. The HEN/HPN ratio was 3:1. A total of 37 657 and 12 859 adults respectively required HEN and HPN among 50 881 948 people aged 20 and over. Published papers to compare, aggregating national data for both HEN and HPN, are only few. They do not have exhaustive data, they are often carried out on limited populations and often indiscriminately affect adults and children. CONCLUSIONS: Epidemiology of HEN and HPN remains poorly described in the literature. We present here the first results to accurately assess the incidence and prevalence of HAN on a complete national adult population and for a same year. The completeness of our data may explain these results of incidence and prevalence significantly higher than almost all available information in the existing literature, but as close as possible to real data.

Avoiding the use of long-term parenteral support in patients without intestinal failure: A position paper from the European Society of Clinical Nutrition & Metabolism, the European Society of Neurogastroenterology and Motility and the Rome Foundation for Disorders of Gut-Brain Interaction.

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.

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

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

Important issues in proposing autonomy training in home parenteral nutrition for short bowel syndrome patients: a qualitative insight from the patients' perspectives.

OBJECTIVES: The standard treatment for short bowel syndrome is home parenteral nutrition. Patients' strict adherence to protocols is essential to decrease the risk of complications such as infection or catheter thrombosis. Patient training can even result in complete autonomy in daily care. However, some patients cannot or do not want too much responsibility. However, doctors often encourage them to acquire these skills. Based on qualitative investigations with patients, we wanted to document issues of importance concerning perceptions of autonomy in daily care. METHODS: Semistructured interviews were conducted with 13 adult patients treated by home parenteral nutrition using a maximum variation sampling strategy. We proceeded to a thematic analysis following an inductive approach. RESULTS: After achieving clinical management of symptoms, a good quality of life is within the realm of possibility for short bowel syndrome patients with home parenteral nutrition. In this context, achieving autonomy in home parenteral nutrition could be a lever to sustain patients' quality of life by providing better life control. However, counterintuitively, not all patients aim at reducing constraints by reaching autonomy in home parenteral nutrition. First, they appreciate the social contact with the nurses, which is particularly true among patients who live alone. Second, they can feel safer with the nurse's visits. Regaining freedom was the main motivation for patients in the training program and the main benefit for those who were already autonomous. CONCLUSIONS: Medical teams should consider patients' health locus of control (internal or external) for disease management to support them concerning the choice of autonomy in daily care for parenteral nutrition.

Transition from pediatric to adult care in patients with chronic intestinal failure on home parenteral nutrition: How to do it right?

BACKGROUND: Life expectancy of children with chronic intestinal failure (CIF) on home parenteral nutrition has greatly improved. Children are now able to grow into adulthood which requires transfer from pediatric to adult health care. A guideline for structured transition is lacking and the demand for a more standardized care for this patient group is necessary. Therefore, we investigated the perceptions of health care professionals from various disciplines working in this specific field, concerning effective interventions regarding transition to adult health care. AIM: To create a standardized protocol which provides practical guidance for health care professionals in order to bridge the gap between pediatric and adult health care and to facilitate successful transition of children with chronic intestinal failure. METHODS: A survey consisting of 20 interventions for transition was sent out to members of the Intestinal Failure working group of European Reference Network for Rare Inherited Congenital (gastrointestinal and digestive) Anomalies (ERNICA) and the Network of Intestinal Failure and Intestinal Transplant in Europe (NITE) group - European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) healthcare professionals in 48 medical centers in various countries. Next to 20 interventions, an open-ended question to fill in any other suggestion with respect to most effective intervention was included. Interventions scoring higher than 80% by the participants were included in the protocol. Interventions scoring between 50% and 80% and other own suggestions were discussed during a consensus meeting and included when consensus, defined as unanimous agreement, was reached. Interventions scoring as effective by < 50% of participants were excluded directly. RESULTS: A total of 80 healthcare professionals from 33 medical centers (participation rate 69%) participated. The protocol consisted of modifiable components expected to be targets of interventions. The most important key outcomes of the survey were: 1) assessment of patient's transition readiness and provision of knowledge to the patient by the pediatric team, 2) involvement of parents in the transition process, and 3) collaboration between the pediatric and adult chronic intestinal failure team. In addition it is advised that the transition process should start 1-2 years before transfer. A nurse specialist working in both services should form a bridge. All interventions must be tailor-made and based on the maturity of the patient. CONCLUSION: This study provides a protocol describing transition of children with chronic intestinal failure from pediatric to adult care. This international protocol will serve as practical guidance for pediatric chronic intestinal failure which will provide a more structured, optimal transition process. It is advised to use this protocol as a formal checklist that can be placed in the patient's chart to review and track the transition process by CIF team members. Future research investigating transition readiness of CIF patients is needed.

Non-interventional, 5-year retrospective data of home parenteral nutrition in patients with benign chronic intestinal failure: Analysis of an Italian nurse registry (SERECARE II).

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.

Body composition of adults with chronic intestinal failure receiving home parenteral nutrition: A descriptive cohort study.

BACKGROUND: Chronic intestinal failure (CIF) refers to the long-lasting reduction of gut function below the minimum necessary to absorb macronutrients, water, and/or electrolytes. Patients with CIF likely develop various forms of malnutrition and dehydration, yet studies that focus primarily on body composition are lacking. Therefore, this study aimed to evaluate the body composition of adult patients with CIF. METHODS: This retrospective descriptive cohort study was performed at the Radboud University Medical Center, a tertiary referral center for CIF treatment in the form of home parenteral nutrition. We collected available bioelectrical impedance analysis (BIA) data from routine care between 2019 and 2023. The primary outcome was body composition, which was evaluated by assessing body mass index (BMI), fat-free mass index (FFMI), and fat percentage (fat%). RESULTS: Overall, 147 adult patients with CIF were included with a median (interquartile range) age of 58 (25-68) years; 69% were female. The mean (SD) BMI was 22.1 (4.3) kg/m2, FFMI was 14.2 (1.9) kg/m2 in females and 17.0 (2.0) kg/m2 in males, and fat% was 33.7% (6.8%) in females and 24.6% (6.4%) in males. 63% had an FFMI below references, and 48% had a high fat%. CONCLUSION: This study found that most adult patients with CIF have an unfavorable body composition characterized by a high fat% and low FFMI despite having a normal mean BMI. These results highlight the necessity for in-depth nutrition assessment, including BIA measurement. Moreover, future studies should focus on exercise interventions to increase FFMI and improve body composition and function.

A case report of lactobacillus bacteremia in a patient on chronic parenteral nutrition.

BACKGROUND AND AIMS: Short bowel syndrome is a malabsorption disorder typically caused by the physical loss of a portion of the intestine, whereafter the body is unable to adequately absorb nutrients, fluids, and electrolytes. Many patients with short bowel syndrome are reliant on home parenteral nutrition through a tunneled or peripherally inserted central catheter to ensure sufficient hydration and nutrition. Central venous catheters are a nidus for bacteria, and patients are at risk for infections associated with high levels of morbidity and mortality. Lactobacillus is a ubiquitous microorganism that most frequently colonizes mucosal surfaces such as the gastrointestinal tract. Lactobacillus bacteremia is rare, with limited occurrence in current medical literature. METHODS: Our patient is a 60-year-old female with a past medical history significant for multiple abdominal surgeries resulting in short bowel syndrome, with subsequent dependence on home parenteral nutrition via peripherally inserted central catheter. She had type III chronic intestinal failure, category D2, and stage 1 moderate malnutrition. She was originally admitted to the hospital for a presumed pulmonary embolism and was found to have a deep vein thrombosis in the setting of her peripherally inserted central catheter. On admission her abdominal exam was unremarkable, she denied abdominal pain, and her only gastrointestinal complaint was chronic stable diarrhea. During the hospitalization she developed severe left lower quadrant abdominal pain and noted decreased frequency of her bowel movements. A computed tomography scan of her abdomen revealed chronic stable intestinal distension and was concerning for obstruction. Clinically she remained without symptoms of acute obstruction or ileus. During the admission she became febrile, with blood cultures from her peripherally inserted central catheter and peripheral IV growing out gram negative rods determined to be lactobacillus bacteremia. The infectious disease team recommended removal of her peripherally inserted central catheter given their concern for a line infection. RESULTS: The patient was treated with broad-spectrum antibiotics, did well clinically, and was ultimately discharged following reinsertion of her peripherally inserted central catheter after negative repeat blood cultures. Though she initially did well in the outpatient setting, she ultimately passed away ten months later after re-presenting to the hospital in septic shock, secondary to bowel ischemia and suspected fungemia of her peripherally inserted central catheter. CONCLUSIONS: In this case report, we describe an unusual case of a patient with short bowel syndrome on chronic parenteral nutrition who developed catheter-associated lactobacillus bacteremia - the first reported case in an adult patient on parenteral nutrition.

[The registers of pediatric patients with home artificial nutrition. The Spanish experience]. / Los registros de pacientes pediátricos con nutrición artificial en el domicilio. La experiencia española.

Introduction: Background: the number of infants and children who receive artificial nutrition at home has been steadily increasing over the last decades, as better outcomes for children with chronic conditions have been achieved. In order to evaluate the need of resources to implement the technique it is necessary to know how many patients benefit from home artificial nutrition. This information can be estimated from the register of patients, when available. Methods: in this paper the characteristics of all registers were reviewed, especially those devoted to pediatric patients. Results: only two pediatric registers are active in 2023: the Canadian register and the Spanish one. NADYA register from the Spanish Society for Clinical Nutrition and Metabolism (SENPE) and the recent REPAFI, form the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition. The most valuable register from the British Society, BANS, stopped providing information in 2018. Conclusion: despite the fact of acknowledging the importance of having gathered information on the prevalence and incidence of home artificial nutrition, to fit resources to necessities, the number of active registers is quite short.

Introducción: Introducción: el número de pacientes pediátricos que reciben nutrición artificial en el domicilio (NAD) ha crecido en los países occidentales en la medida que ha mejorado el pronóstico de algunas enfermedades graves potencialmente letales. Con el fin de poder estimar la cantidad de recursos necesarios para implementar esta técnica, es necesario conocer el volumen real de pacientes que precisan NAD. Una manera aproximada de conocer estas necesidades puede establecerse a partir de los datos de los registros nacionales de pacientes. Métodos: se revisan las características de los registros de pacientes pediátricos con NAD, tanto los activos como los que no han continuado en el tiempo, con especial atención a los registros españoles. Resultados: solo dos registros de pacientes permanecen activos en 2023, el registro canadiense y los registros españoles de la Sociedad Española de Nutrición Clínica y Metabolismo (SENPE), a través del registro NADYA y el de la Sociedad Española de Gastroenterología Hepatología y Nutrición Pediátrica, registro REPAFI. El registro más completo de la Sociedad Británica, BANS, dejó de publicar resultados en 2018. Conclusiones: a pesar de reconocer la importancia de presentar datos actualizados de prevalencia e incidencia del uso de la NAD, con el fin de adecuar los recursos a las necesidades, el número de registros activos de pacientes pediátricos es excepcional.

Plasma metabolomics changes comparing daytime to overnight infusions of home parenteral nutrition in adult patients with short bowel syndrome: Secondary analysis of a clinical trial.

BACKGROUND: Home parenteral nutrition (HPN) is often cycled nocturnally and is expected to result in glucose intolerance and sleep disruption partly due to circadian misalignment. This study aimed to define the metabolic response when HPN is cycled during the daytime compared to overnight. METHODS: This secondary analysis leveraged samples from a clinical trial in adults with short bowel syndrome consuming HPN (ClinicalTrials.gov: NCT04743960). Enrolled patients received 1 week of HPN overnight followed by 1 week of HPN during the daytime. Fasting blood samples were collected following each study period and global metabolic profiles were examined from plasma samples. Differential metabolite abundance was determined from normalized and scaled data using adjusted Linear Models for MicroArray Data models followed by pathway enrichment analysis. RESULTS: Nine patients (mean age, 52.6 years; 78% female; mean BMI 20.7 kg/m2) provided samples. Among 622 identified metabolites, changes were observed in 36 metabolites at Punadj < 0.05 with higher abundance of fatty acids, long-chain and polyunsaturated fatty acids (Dihomo-gamma-linolenic acid, arachidonate (20:4n6), docosahexaenoate (DHA; 22:6n3)) and glycerolipids with daytime infusions. Enrichment analysis identified changes in pathways related to the biosynthesis of unsaturated fatty acids, d-arginine, and d-ornithine metabolism, and linoleic acid metabolism (Punadj<0.05). CONCLUSION: Daytime infusions of HPN may result in changes in circulating lipids and amino acid composing metabolic pathways previously implicated in circadian rhythms. As this is the first untargeted metabolomics study of HPN, larger studies are needed.