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

BACKGROUND & AIMS: In 2016, ESPEN published the guideline for Chronic Intestinal Failure (CIF) in adults. An updated version of ESPEN guidelines on CIF due to benign disease in adults was devised in order to incorporate new evidence since the publication of the previous ESPEN guidelines. METHODS: The grading system of the Scottish Intercollegiate Guidelines Network (SIGN) was used to grade the literature. Recommendations were graded according to the levels of evidence available as A (strong), B (conditional), 0 (weak) and Good practice points (GPP). The recommendations of the 2016 guideline (graded using the GRADE system) which were still valid, because no studies supporting an update were retrieved, were reworded and re-graded accordingly. RESULTS: The recommendations of the 2016 guideline were reviewed, particularly focusing on definitions, and new chapters were included to devise recommendations on IF centers, chronic enterocutaneous fistulas, costs of IF, caring for CIF patients during pregnancy, transition of patients from pediatric to adult centers. The new guideline consist of 149 recommendations and 16 statements which were voted for consensus by ESPEN members, online in July 2022 and at conference during the annual Congress in September 2022. The Grade of recommendation is GPP for 96 (64.4%) of the recommendations, 0 for 29 (19.5%), B for 19 (12.7%), and A for only five (3.4%). The grade of consensus is "strong consensus" for 148 (99.3%) and "consensus" for one (0.7%) recommendation. The grade of consensus for the statements is "strong consensus" for 14 (87.5%) and "consensus" for two (12.5%). CONCLUSIONS: It is confirmed that CIF management requires complex technologies, multidisciplinary and multiprofessional activity, and expertise to care for the underlying gastrointestinal disease and to provide HPN support. Most of the recommendations were graded as GPP, but almost all received a strong consensus.

Bridging-to-Surgery in Patients with Type 2 Intestinal Failure.

AIM: Type 2 intestinal failure (IF) is characterized by the need for longer-term parenteral nutrition (PN). During this so-called bridging-to-surgery period, morbidity and mortality rates are high. This study aimed to evaluate to what extent a multidisciplinary IF team is capable to safely guide patients towards reconstructive surgery. METHODS: A consecutive series of patients with type 2 IF followed up by a specialized IF team between January 1st, 2011, and March 1st, 2016, was analyzed. Data on their first outpatient clinic visit (T1) and their last visit before reconstructive surgery (T2) was collected. The primary outcome was a combined endpoint of a patient being able to recover at home, have (partial) oral intake, and a normal albumin level (> 35 g/L) before surgery. RESULTS: Ninety-three patients were included. The median number of previous abdominal procedures was 4. At T2 (last visit prior to reconstructive surgery), significantly more patients met the combined primary endpoint compared with T1 (first IF team consultation) (66.7% vs. 28.0% (p < 0.0001), respectively); 86% had home PN. During "bridging-to-surgery," acute hospitalization rate was 40.9% and acute surgery was 4.3%. Postoperatively, 44.1% experienced a major complication, 5.4% had a fistula, and in-hospital mortality was 6.5%. Of the cohort, 86% regained enteral autonomy, and when excluding in-hospital mortality and incomplete follow-up, this was 94.1%. An albumin level < 35 g/L at T2 and weight loss of > 10% at T2 compared with preadmission weight were significant risk factors for major complications. CONCLUSION: Bridging-to-surgery of type 2 IF patients under the guidance of an IF team resulted in the majority of patients being managed at home, having oral intake, and restored albumin levels prior to reconstructive surgery compared with their first IF consultation.

Body Composition Using Air Displacement Plethysmography in Children With Intestinal Failure Receiving Long-Term Home Parenteral Nutrition.

BACKGROUND: Children with intestinal failure (IF) are at risk of growth failure, but little information about body composition is available. Our aim was to assess body composition using air displacement plethysmography (ADP) and relate it to clinical and growth parameters. METHODS: In this prospective descriptive observational 2-center cohort study, children aged 2-18 years receiving home parenteral nutrition (PN) for ≥6 months underwent ADP measurement. Fat mass index (FMI) and fat-free mass index (FFMI) standard deviation scores (SDSs) were calculated to normalize for small body size. RESULTS: Twenty-one out of 22 children, median age 7.4 years, underwent successful ADP measurement after a median PN duration of 5.5 years. They were significantly lighter (median weight for age SDS -0.71, P = 0.004) and shorter (median height for age SDS -1.55, P < 0.001) than the normal population mean; 52% were growing below target height range. They had low FFMI (median SDS -1.53, P < 0.001) and high FMI (median SDS 0.80, P = 0.002). Weight for height and body mass index (BMI) were significantly associated with FFMI and BMI with FMI, but children with the same weight and height showed different body composition. In 13 patients with 1-year follow-up, growth and body composition did not change significantly. CONCLUSION: Children with IF receiving long-term PN show lower FFM and higher FM than healthy children. Additionally, children with similar routine growth parameters showed different body composition. Further studies should evaluate the effect of a patient-tailored approach including physical activity and nutrition advice based on body composition.

An international study of the quality of life of adult patients treated with home parenteral nutrition.

BACKGROUND & AIMS: Home parenteral nutrition-quality of life (HPN-QOL©) is a self-assessment tool for the measurement of QOL in patients on HPN. The aims of this study were: to re-assess the basic psychometric properties of the HPN-QOL© in a multinational sample of adult patients; to provide a description of QOL dimensions by short and long HPN treatment duration; to explore clinical factors potentially associated to QOL scores. METHODS: Patients (n = 699) from 14 countries completed the HPN-QOL©. The questionnaires were analysed to evaluate data completeness, convergent/discriminant validity and internal-consistency reliability. The association of overall QOL and HPN treatment duration as well as other clinical factors were investigated using multivariable linear regression models. RESULTS: The analysis of the multitrait-scaling and internal consistency indicates a good fit with the questionnaire structure for most items. Item discriminant validity correlation was satisfactory and psychometric evaluation of the HPN-QOL© in the different English, French and Italian language patient sub-groups confirmed psychometric equivalence of the three questionnaire versions. The results of the multivariable linear regression showed that QOL scores were significantly associated with HPN duration (better in long-term), underlying disease (better in Crohn's disease and mesenteric ischaemia) and living status (worse in living alone) and, after adjusting for the other factors, with the number of days of HPN infusion per week. CONCLUSIONS: The HPN-QOL©, is a valid tool for measurement of QOL in patients on HPN, to be used in the clinical practice as well as in research.

Intestinal failure in adults: Recommendations from the ESPEN expert groups.

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

[Undernourished in hospital]. / Ondervoed in het ziekenhuis.

Patients with a positive undernutrition screening score stay in hospital 1.4 days longer, with more associated complications and costs. A paper elsewhere in this journal describes the implementation of a novel in-hospital meal service resulting in improved protein and energy intake. Patient satisfaction was maintained and to some extent improved. However, the paper shows the difficulty of improving food intake in hospitalized patients. This may be due to factors such as illness-induced anorexia, poor nurse and physician nutritional awareness, and lack of a clear financial incentive. Despite substantial improvement, only 24% of patients had an adequate protein intake. Optimal nutrition should be inextricably associated with care, education and research. Moreover, achieving optimal nutrition needs continuous involvement from nutritional assistants, dieticians, nurses and physicians as well as an optimal in-hospital meal service. The road to optimal nutrition in underfed patients is still long in a world where overfeeding lies at the base of many healthcare problems.

Nutrition and the circadian timing system.

Life on earth has evolved under the daily rhythm of light and dark. Consequently, most creatures experience a daily rhythm in food availability. In this review, we first introduce the mammalian circadian timing system, consisting of a central clock in the suprachiasmatic nucleus (SCN) and peripheral clocks in various metabolic tissues including liver, pancreas, and intestine. We describe how peripheral clocks are synchronized by the SCN and metabolic signals. Second, we review the influence of the circadian timing system on food intake behavior, activity of the gastrointestinal system, and several aspects of glucose and lipid metabolism. Third, the circadian control of digestion and metabolism may have important implications for several aspects of food intake in humans. Therefore, we review the human literature on health aspects of meal timing, meal frequency, and breakfast consumption, and we describe the potential implications of the clock system for the timing of enteral tube feeding and parenteral nutrition. Finally, we explore the connection between type 2 diabetes and the circadian timing system. Although the past decade has provided exciting knowledge about the reciprocal relation between biological clocks and feeding/energy metabolism, future research is necessary to further elucidate this fascinating relationship in order to improve human health.

"MY PKU": increasing self-management in patients with phenylketonuria. A randomized controlled trial.

BACKGROUND: Phenylketonuria (PKU) is an autosomal recessive disorder of phenylalanine metabolism. The inability to convert phenylalanine (Phe) into tyrosine causes Phe to accumulate in the body. Adherence to a protein restricted diet, resulting in reduced Phe levels, is essential to prevent cognitive decline. Frequent evaluation of plasma Phe levels and, if necessary, adjustment of the diet are the mainstay of treatment. We aimed to assess whether increased self-management of PKU patients and/or their parents is feasible and safe, by providing direct online access to blood Phe values without immediate professional guidance. METHODS: Thirty-eight patients aged ≥ 1 year participated in a 10 month randomized controlled trial. Patients were randomized into a study group (1) or a control group (2). Group 2 continued the usual procedure: a phone call or e-mail by a dietician in case of a deviant Phe value. Group 1 was given a personal "My PKU" web page with a graph of their recent and previous Phe values, online general information about the dietary treatment and the Dutch PKU follow-up guidelines, and a message-box to contact their dietician if necessary. Phe values were provided on "My PKU" without advice. Outcome measures were: differences in mean Phe value, percentage of values above the recommended range and Phe sample frequency, between a 10-month pre-study period and the study period in each group, and between the groups in both periods. Furthermore we assessed satisfaction of patients and/or parents with the 'My PKU' procedure of online availability. RESULTS: There were no significant differences in mean Phe value, percentage of values above recommended range or in frequency of blood spot sampling for Phe determination between the pre-study period and the study period in each group, nor between the 2 groups during the periods. All patients and/or parents expressed a high level of satisfaction with the new way of disease management. CONCLUSIONS: Increased self-management in PKU by providing patients and/or parents their Phe values without advice is feasible and safe and is highly appreciated. TRIAL REGISTRATION: The trial was registered with The Netherlands National Trial Register (NTR #1171) before recruitment of patients.

How nutritional risk is assessed and managed in European hospitals: a survey of 21,007 patients findings from the 2007-2008 cross-sectional nutritionDay survey.

BACKGROUND & AIMS: Recognition and treatment of undernutrition in hospitalized patients are not often a priority in clinical practice. OBJECTIVES: We investigated how the nutritional risk of patients is determined and whether such assessment influences daily nutritional care across Europe and in Israeli hospitals. METHODS: 1217 units from 325 hospitals in 25 countries with 21,007 patients participated in a longitudinal survey "nutritionDay" 2007/2008 undertaken in Europe and Israel. Screening practice, the type of tools used and whether energy requirements and intake are assessed and monitored were surveyed using standardized questionnaires. RESULTS: Fifty-two percent (range 21-73%) of the units in the different regions reported a screening routine which was most often performed with locally developed methods and less often with national tools, the Nutrition Risk Screening-2002, or the Malnutrition Universal Screening Tool. Twenty-seven percent of the patients were subjectively classified as being "at nutritional risk", with substantial differences existing between regions. Independent factors influencing the classification of nutritional risk included age, BMI <18.5 kg/m(2), unintentional weight loss, reduced food intake in the previous week and on nutritionDay (for all parameters, p < 0.0001). The energy goal was defined as >=1500 kcal in 76% of the patients, but 43% of patients did not reach this goal. CONCLUSIONS: The process of nutrition risk assessment varied between units and countries. Additionally, energy goals were frequently not met. More effort is needed to implement current guidelines within daily clinical practice.