Long-term comparative effectiveness of pegvaliase versus medical nutrition therapy with and without sapropterin in adults with phenylketonuria.

Phenylketonuria is characterized by intellectual disability and behavioral, psychiatric, and movement disorders resulting from phenylalanine (Phe) accumulation. Standard-of-care treatment involves a Phe-restricted diet plus medical nutrition therapy (MNT), with or without sapropterin dihydrochloride, to reduce blood Phe levels. Pegvaliase is an injectable enzyme substitution treatment approved for adult patients with blood Phe >600 µmol/L despite ongoing management. A previous comparative effectiveness analysis using data from the Phase 3 PRISM trials of pegvaliase (NCT01819727 and NCT01889862) and the Phenylketonuria Demographics, Outcomes and Safety Registry (PKUDOS; NCT00778206) suggested that pegvaliase was more effective at lowering mean blood Phe levels than sapropterin + MNT or MNT alone at 1 and 2 years of treatment. The current work augments and complements the previous analysis by including additional follow-up from the completed studies, robust methods reflecting careful consideration of issues with the distribution of Phe, and alternative methods for adjustment that are important for control of potential confounding in comparative effectiveness. Median blood Phe levels were lower, and median intact protein intakes were higher, in the pegvaliase group (n = 183) than in the sapropterin + MNT (n = 82) and MNT (n = 67) groups at Years 1, 2, and 3. In the pegvaliase group, median blood Phe levels decreased from baseline (1244 µmol/L) to Year 1 (535 µmol/L), Year 2 (142 µmol/L), and Year 3 (167 µmol/L). In the sapropterin + MNT group, median blood Phe levels decreased from baseline (900 µmol/L) to Year 1 (588 µmol/L) and Year 2 (592 µmol/L), and increased at Year 3 (660 µmol/L). In the MNT group, median blood Phe levels decreased slightly from baseline (984 µmol/L) to Year 1 (939 µmol/L) and Year 2 (941 µmol/L), and exceeded baseline levels at Year 3 (1157 µmol/L). The model-estimated proportions of participants achieving blood Phe ≤600 µmol/L were 41%, 100%, and 100% in the pegvaliase group at Years 1, 2, and 3, respectively, compared with 55%, 58%, and 38% in the sapropterin + MNT group and 5%, 16%, and 0% in the MNT group. The estimated proportions of participants achieving more stringent blood Phe targets of ≤360 µmol/L and ≤120 µmol/L were also higher in the pegvaliase group than in the other groups at Years 2 and 3. Overall, our results indicate that, compared with standard therapy, pegvaliase induces a substantial, progressive, and sustained decrease in blood Phe levels - to a much greater extent than sapropterin + MNT or MNT alone - which is expected to improve long-term outcomes in patients with phenylketonuria.

Feasibility and Acceptability of Home-Delivered Medically Tailored Meals for Treatment of Diabetes in Pregnancy.

BACKGROUND: Diabetes complicates ≤7% of pregnancies in the United States. Although medical nutrition therapy is the mainstay of diabetes treatment, many barriers exist to the successful implementation of dietary modifications. Home-delivered medically tailored meals (MTMs) are promising to overcome such barriers. OBJECTIVE: The objective of this study was to evaluate the feasibility and acceptability of home-delivered MTM in pregnant patients with diabetes. METHODS: We performed a prospective cohort study of home-delivered MTM for pregnant patients with diabetes using a mixed-methods approach. Participants <35 wk of gestation at the time of enrollment received weekly home delivery of diabetes-specific meals. Qualitative semistructured interviews were conducted to gain insight into participants' experience. Diabetes self-efficacy was assessed pre- and postintervention using the Diabetes Self-Efficacy Scale and 2-Item Diabetes Distress Screening Scale. The difference in mean scores was compared using t-tests with P value of <0.05 considered significant. Feasibility and acceptability were evaluated through participants' attitude toward MTM in qualitative interviews and indirectly evaluated through diabetes self-efficacy surveys. RESULTS: Twenty pregnant people with diabetes who received home-delivered MTM during pregnancy were interviewed postpartum. Participants found this program convenient for various reasons, including reduced time for grocery shopping and preparing meals. Participants were satisfied with meals, citing a positive impact on diabetes management, accessibility of healthy foods, reduced stress with meal planning, and greater perceived control of blood glucose. Most participants shared meals with their families or received specific meals for their dependents, which was positively received. Reduced financial and mental stress was also widely reported. Diabetes self-efficacy was significantly improved postintervention with MTM. CONCLUSION: Home-delivered MTM is feasible and acceptable in pregnant patients with diabetes and may improve diabetes self-efficacy. Individual experiences offered insight into various barriers overcome by using this service. Home-delivered MTM may help ensure an accessible, healthy diet for pregnant patients with diabetes.

Nature of the evidence base and approaches to guide nutrition interventions for individuals: a position paper from the Academy of Nutrition Sciences.

This Position Paper from the Academy of Nutrition Sciences is the third in a series which describe the nature of the scientific evidence and frameworks that underpin nutrition recommendations for health. This paper focuses on evidence which guides the application of dietary recommendations for individuals. In some situations, modified nutrient intake becomes essential to prevent deficiency, optimise development and health, or manage symptoms and disease progression. Disease and its treatment can also affect taste, appetite and ability to access and prepare foods, with associated financial impacts. Therefore, the practice of nutrition and dietetics must integrate and apply the sciences of food, nutrition, biology, physiology, behaviour, management, communication and society to achieve and maintain human health. Thus, there is huge complexity in delivering evidence-based nutrition interventions to individuals. This paper examines available frameworks for appraising the quality and certainty of nutrition research evidence, the development nutrition practice guidelines to support evidence implementation in practice and the influence of other sources of nutrition information and misinformation. The paper also considers major challenges in applying research evidence to an individual and suggests consensus recommendations to begin to address these challenges in the future. Our recommendations target three groups; those who deliver nutrition interventions to individuals, those funding, commissioning or undertaking research aimed at delivering evidence-based nutrition practice, and those disseminating nutritional information to individuals.

The kidney: the critical organ system for guiding nutrition therapy in the ICU-patient?

Most randomized controlled studies on nutrition in intensive care patients did not yield conclusive results or were neutral or negative concerning the primary endpoints but also in most secondary endpoints. However, there is a consistent observation that in several of these studies there was a negative effect of the nutrition intervention on the kidneys in one of the study arms. During the early phase and in unstable periods during further course of disease an inadequate clinical nutrition can damage the kidneys, can elicit or aggravate acute kidney injury and/ or increase requirements of renal replacement therapy (RRT). This relates to total energy intake, glucose intake/hyperglycemia and protein/ amino acid intake at various stages of renal dysfunction. The kidney could present a critical organ system for guiding nutrition therapy, a close monitoring of kidney function should be observed and nutrition therapy may need to be adapted accordingly. The long-held dogma of performing full nutrition and accept an otherwise not necessary RRT is definitely to be refuted.

Nutritional therapy for reducing disability and improving activities of daily living in people after stroke.

BACKGROUND: Stroke patients often face disabilities that significantly interfere with their daily lives. Poor nutritional status is a common issue amongst these patients, and malnutrition can severely impact their functional recovery post-stroke. Therefore, nutritional therapy is crucial in managing stroke outcomes. However, its effects on disability, activities of daily living (ADL), and other critical outcomes have not been fully explored. OBJECTIVES: To evaluate the effects of nutritional therapy on reducing disability and improving ADL in patients after stroke. SEARCH METHODS: We searched the trial registers of the Cochrane Stroke Group, CENTRAL, MEDLINE (from 1946), Embase (from 1974), CINAHL (from 1982), and AMED (from 1985) to 19 February 2024. We also searched trials and research registries (ClinicalTrials.gov, World Health Organization International Clinical Trials Registry Platform) and reference lists of articles. SELECTION CRITERIA: We included randomised controlled trials (RCTs) that compared nutritional therapy with placebo, usual care, or one type of nutritional therapy in people after stroke. Nutritional therapy was defined as the administration of supplemental nutrients, including energy, protein, amino acids, fatty acids, vitamins, and minerals, through oral, enteral, or parenteral methods. As a comparator, one type of nutritional therapy refers to all forms of nutritional therapies, excluding the specific nutritional therapy defined for use in the intervention group. DATA COLLECTION AND ANALYSIS: We used Cochrane's Screen4Me workflow to assess the initial search results. Two review authors independently screened references that met the inclusion criteria, extracted data, and assessed the risk of bias and the certainty of the evidence using the GRADE approach. We calculated the mean difference (MD) or standardised mean difference (SMD) for continuous data and the odds ratio (OR) for dichotomous data, with 95% confidence intervals (CIs). We assessed heterogeneity using the I2 statistic. The primary outcomes were disability and ADL. We also assessed gait, nutritional status, all-cause mortality, quality of life, hand and leg muscle strength, cognitive function, physical performance, stroke recurrence, swallowing function, neurological impairment, and the development of complications (adverse events) as secondary outcomes. MAIN RESULTS: We identified 52 eligible RCTs involving 11,926 participants. Thirty-six studies were conducted in the acute phase, 10 in the subacute phase, three in the acute and subacute phases, and three in the chronic phase. Twenty-three studies included patients with ischaemic stroke, three included patients with haemorrhagic stroke, three included patients with subarachnoid haemorrhage (SAH), and 23 included patients with ischaemic or haemorrhagic stroke including SAH. There were 25 types of nutritional supplements used as an intervention. The number of studies that assessed disability and ADL as outcomes were nine and 17, respectively. For the intervention using oral energy and protein supplements, which was a primary intervention in this review, six studies were included. The results for the seven outcomes focused on (disability, ADL, body weight change, all-cause mortality, gait speed, quality of life, and incidence of complications (adverse events)) were as follows: There was no evidence of a difference in reducing disability when 'good status' was defined as an mRS score of 0 to 2 (for 'good status': OR 0.97, 95% CI 0.86 to 1.10; 1 RCT, 4023 participants; low-certainty evidence). Oral energy and protein supplements may improve ADL as indicated by an increase in the FIM motor score, but the evidence is very uncertain (MD 8.74, 95% CI 5.93 to 11.54; 2 RCTs, 165 participants; very low-certainty evidence). Oral energy and protein supplements may increase body weight, but the evidence is very uncertain (MD 0.90, 95% CI 0.23 to 1.58; 3 RCTs, 205 participants; very low-certainty evidence). There was no evidence of a difference in reducing all-cause mortality (OR 0.57, 95% CI 0.14 to 2.28; 2 RCTs, 4065 participants; low-certainty evidence). For gait speed and quality of life, no study was identified. With regard to incidence of complications (adverse events), there was no evidence of a difference in the incidence of infections, including pneumonia, urinary tract infections, and septicaemia (OR 0.68, 95% CI 0.20 to 2.30; 1 RCT, 42 participants; very low-certainty evidence). The intervention was associated with an increased incidence of diarrhoea compared to usual care (OR 4.29, 95% CI 1.98 to 9.28; 1 RCT, 4023 participants; low-certainty evidence) and the occurrence of hyperglycaemia or hypoglycaemia (OR 15.6, 95% CI 4.84 to 50.23; 1 RCT, 4023 participants; low-certainty evidence). AUTHORS' CONCLUSIONS: We are uncertain about the effect of nutritional therapy, including oral energy and protein supplements and other supplements identified in this review, on reducing disability and improving ADL in people after stroke. Various nutritional interventions were assessed for the outcomes in the included studies, and almost all studies had small sample sizes. This led to challenges in conducting meta-analyses and reduced the precision of the evidence. Moreover, most of the studies had issues with the risk of bias, especially in terms of the absence of blinding and unclear information. Regarding adverse events, the intervention with oral energy and protein supplements was associated with a higher number of adverse events, such as diarrhoea, hyperglycaemia, and hypoglycaemia, compared to usual care. However, the quality of the evidence was low. Given the low certainty of most of the evidence in our review, further research is needed. Future research should focus on targeted nutritional interventions to reduce disability and improve ADL based on a theoretical rationale in people after stroke and there is a need for improved methodology and reporting.

Are dietitian recommendations followed? A descriptive study of paediatric hospitalised and ambulatory patients.

BACKGROUND: The primary objective of this cross-sectional retrospective study was to describe the implementation of dietitian prescribed nutrition recommendations in malnourished paediatric patients in the hospital and ambulatory settings. We also aimed to investigate other characteristics that could be associated with differences in implementation. METHODS: Data were collected from 186 hospitalised and 565 ambulatory patients between February 2020 and January 2021. Data included age, hospital or ambulatory specialty departments, primary diagnosis, malnutrition status, hospital length of stay (LOS), and medical nutrition therapy recommendations. Implementation by the medical team in the hospital setting and adherence by the family in the outpatient setting were categorised as "Full", "Partial" or "None". "Partial" and "None" were combined for analysis. RESULTS: Dietitian prescribed recommendations were implemented in 79.6% of hospitalised patients. In the ambulatory population, 46.4% of patients were adherent with nutrition recommendations. Within the hospital, there was a significant difference in implementation of nutrition recommendations based on age (p = 0.047), hospital department (p = 0.002) and LOS (p = 0.04), whereas, in the ambulatory population, there were no significant differences in the rate of adherence among any of the studied characteristics. CONCLUSIONS: Dietitian recommendations are frequently implemented in the hospital, whereas adherence to such recommendations is poor in the outpatient population. Interventions to improve adherence to nutrition recommendations in the ambulatory setting are needed.

Evolution of nutritional management in children with cystic fibrosis - a narrative review.

Nutrition has played a central role in the management and outcomes of people with cystic fibrosis (pwCF) since the 1970s. Advances in therapies and practices in recent decades have led to a significant change in the patient landscape with dramatic improvements in life expectancy, as well as quality of life, bringing with it new issues. Historically, cystic fibrosis was a condition associated with childhood and malnutrition; however, changes in patient demographics, nutritional assessment and fundamental nutritional management have evolved, and it has become an increasingly prevalent adult disease with new nutritional challenges, including obesity. This paper aims to describe these changes and the impact and challenges they bring for those working in this field. Nutritional professionals will need to evolve, adapt and remain agile to the wider range of situations and support required for a new generation of pwCF. Specialised nutrition support will continue to be required, and it will be additionally important to improve and optimise quality of life and long-term health.

Health professional attitudes and perceptions of prehabilitation and nutrition before haematopoietic cell transplantation.

BACKGROUND: Nutritional prehabilitation may improve haematopoietic cell transplantation (HCT) outcomes, although little evidence exists. The present study aimed to understand healthcare professional (HCP) perceptions of prehabilitation and nutritional care pre-HCT in UK centres. METHODS: An anonymous online survey (developed and refined via content experts and piloting) was administered via email to multidisciplinary HCPs in 39 UK adult centres, between July 2021 and June 2022. Data are presented as proportions of responses. Routine provision denotes that care was provided >70% of time. RESULTS: Seventy-seven percent (n = 66) of HCPs, representing 61.5% (n = 24) of UK adult HCT centres, responded. All HCPs supported prehabilitation, proposing feasible implementation between induction chemotherapy (60.4%; n = 40) and first HCT clinic (83.3%; n = 55). Only 12.5% (n = 3) of centres had a dedicated prehabilitation service. Nutrition (87.9%; n = 58), emotional wellbeing (92.4%; n = 61) and exercise (81.8%; n = 54) were considered very important constituents. HCPs within half of the HCT centres (n = 12 centres) reported routine use of nutrition screening pre-HCT with a validated tool; 66.7% of HCPs (n = 36) reported using the malnutrition universal screening tool (MUST). Sixty-two percent (n = 41) of HCPs reported those at risk, received nutritional assessments, predominantly by dietitians (91.6%; n = 22) using the dietetic care process (58.3%; n = 14). Body mass index (BMI) was the most frequently reported body composition measure used by HCPs (70.2%, n = 33). Of 59 respondents, non-dietitians most routinely provided dietary advice pre-HCT (82.4%; n = 28 vs. 68%; n = 17, p = 0.2); including high-energy/protein/fat and neutropenic diet advice. Prophylactic enteral feeding pre-HCT was rare, indicated by low BMI and significant unintentional weight loss. Just under half (n = 25 of 59, 42.4%) HCPs reported exercise advice was given routinely pre-HCT. CONCLUSIONS: Nutrition and prehabilitation pre-HCT are considered important and deliverable by HCPs, but current provision in UK centres is limited and inconsistent.

Chinese Guidelines for Medical Nutrition Therapy for Patients with Diabetes (2022 Edition).

Medical nutrition therapy (MNT) is the foundation of the comprehensive treatment of patients with diabetes. In 2010, the Chinese Clinical Nutritionist Center of the Chinese Medical Doctor Association developed the first Chinese guideline on MNT for patients with diabetes, and it was updated in 2015. Since then, new evidence has emerged in the field of MNT and metabolic therapy in patients with diabetes. The Nutrition and Metabolic Management Branch of the China International Exchange and Promotive Association for Medical and Health Care organized a team of experts from related institutions, including the Clinical Nutrition Branch of the Chinese Nutrition Society, Chinese Diabetes Society, Chinese Society for Parenteral and Enteral Nutri-tion, and Chinese Clinical Nutritionist Center of the Chinese Medical Doctor Association. Their task was to develop the Chinese Guidelines of Medical Nutrition Therapy in Diabetes (2022 Edition) in accordance with the requirements of the Guidelines for the Formulation/Revision of Clinical Guidelines in China (2022 Edition) by combining the questions raised and evidence gathered in clinical practices in China, to guide and standardize the clinical MNT.

A practical approach to nutritional intervention for people with chronic kidney disease in Vietnam.

BACKGROUND AND OBJECTIVES: A comprehensive nutritional management is necessary for favourable outcomes in patients with chronic kidney disease (CKD). We aimed to assess the changes in nutritional status and disease progression with nutritional management where renal replacement therapy (RRT) was not in place. METHODS AND STUDY DESIGN: A quasi-experiment intervention was conducted on 70 CKD patients at stages 3-5 from July to December 2022. Participants were excluded if they underwent RRT, including dialy-sis (hemodialysis or peritoneal dialysis), or kidney transplantation. The nutritional regimen covered nutrition-al counseling, samples of the dietary menu, and supplement products. We evaluated nutritional status using Subjective Global Assessment (SGA) scale and sub-clinical blood test at T0 (hospital admission) and T1 (two weeks after the admission or 24 hours before the discharge). RESULTS: After the intervention, the number of patients classified as malnutrition or at risk of malnourished reduced significantly (65.7% to 54.3% and 25.7% and 5.7%, respectively). The serum concentration of urea, creatinine and parathyroid hormone decreased remarkably, especially in patients receiving nutritional management. In the intervention group, the dietary pattern provided increased intakes of calcium and iron at T1, while phosphorus, sodium and potassium decreased after follow-up. Nausea/vomiting, loss of appetite, tiredness and sleep disorders were improved in the intervention compared to the control group. CONCLUSIONS: Nutritional therapy enhanced the nutritional sta-tus, and quality of dietary and renal function in CKD patients without RRT. Applying nutrition education and treatment at an early stage can slow CKD progression, which should be applicable elsewhere in Vietnam.

Implementation of nutritional care in hospitals: A qualitative study of barriers and facilitators using implementation theory.

BACKGROUND: Disease-related malnutrition is prevalent among hospitalised patients, but not all patients achieve the needed nutritional care. At a Danish University Hospital, focus has been on implementing nutritional practices based on clinical guidelines, but there is continuously variation between the wards regarding the quality of nutritional care. AIM: The aim of this study was to identify the potential barriers and facilitators for implementation of the clinical guidelines for nutritional practices and to recommend suggestions for development of nutritional practices, using a theoretical implementation strategy. METHOD: The design was a qualitative interview study of employees at a Danish University Hospital, using a semi-structured interview guide. The participants were nurses, nurse's assistant, nurse nutrition expert, head nurse and dieticians. We recruited 11 employees, representing eight different wards. FINDINGS: The analysis identified six themes: (1) clear allocation of responsibilities and committed management enhances nutrition practices, (2) leadership support is essential, (3) physical settings and tools affect possibilities for action, (4) selection of equivalent staff is core, (5) teaching promotes the knowledge and skills and (6) a dietitian in the ward facilitates implementation of nutritional care. Barriers and facilitators among the themes were identified and has led to suggestions to strengthen nutritional care, based on implementation theory. CONCLUSION: Various factors were identified as having impact on the implementation of nutrition practices and different suggestions have emerged to accommodate those factors, as well as to apply an implementation strategy to facilitate change in practice.

[Nutrition counseling in medical practices-a cross-sectional study]. / Ernährungsberatung in ärztlichen Praxen verschiedener Fachrichtungen ­ eine Querschnittsstudie.

INTRODUCTION: The purpose of this study was to assess the status of nutrition care and counseling in German medical practices, including physicians' nutrition interest and knowledge, existing obstacles, and possible ways of improving nutrition care. METHODS: After development and a two-stage pretest, questionnaires with 32 items were distributed to all resident doctors of general medicine, internal medicine, gynecology and obstetrics, orthopedics, and occupational medicine in Aachen (n = 360) in early spring 2022. RESULTS: The response rate was 29.2% and n = 105 participants were included in the analysis. During their consultation hours, 85.7% of the doctors conducted nutrition counseling (NC). While doctors estimated that 39.5% of their patients need NC, they counseled only 28.1%. General practitioners spent an average of 13 min on NC; all other specialties spent 9.5 min. Most participants felt well informed about healthy nutrition (95.2%). Nutrition was of high personal interest to 57.7% of the participants, and 47.6% considered it important for their work as a doctor. Barriers preventing better NC included the lack of nutrition education during medical studies (89.5%) and specialist training (79.6%), and the lack of time (75.2%). Most participants were willing to conduct more NC if they were paid better and had more time for NC during their working hours. DISCUSSION: Doctors consider NC a relevant part of their work, despite many barriers. To make better use of NC as a measure for treating and preventing common diseases, improvements like better nutrition education for doctors, better funding, and more time for NC are required.

Nutritional Care for Adults in Nursing Homes: A Scoping Review.

The need for improved nutrition in older adults requiring care has been acknowledged, but, to the best of our knowledge, there is a lack of systematic review and integration of nutritional care studies with older adults in nursing homes. This scoping review aimed to examine the scope and nature of nutritional care research for older adults in nursing homes and to identify research gaps, following the guidelines of the Joanna Briggs Institute. We found varied nutritional care for older adults living in nursing homes, including individualized sessions, such as nutrition counseling, the addition of foods and preparations for increased nutritional intake, and the maintenance of an eating environment, such as feeding assistance and calling. The nutritional care identified in this scoping review also included studies that have improved the nutritional status of older adults in nursing homes by implementing educational programs for care staff. For future research on effective nutritional care for older adults in nursing homes, we suggest evaluating both short- and long-term intervention effects with an adequate sample size.

[The development and innovation of clinical nutrition].

The strategy of nutrition therapy in the treatment of diseases is constantly optimized with the development and innovation of the concept and technology in the field of clinical nutrition, especially the rise of multi-disciplines such as imaging omics and artificial intelligence and the latest discoveries of clinical trials. Precise nutrition assessment, diversified products, multimodal nutrition therapy throughout the process and intelligent compliance management have efficiently improved the effect of nutrition therapy. The implementation of personalized and precise nutrition therapy still needs further investigation.

[Gastrointestinal symptoms and dietary intake of patients with irritable bowel syndrome following a low FODMAP diet].

INTRODUCTION: High FODMAP (fermentable oligo-, di, monosaccharides and polyols) foods have been linked with worsening symptoms of IBS patients. The aim was to compare gastrointestinal symptoms and dietary intake of patients with irritable bowel syndrome following a low FODMAP diet, with or without individual nutrition therapy. MATERIALS AND METHODS: A total of 54 patients that met Rome IV criteria for IBS were randomized into two groups, guided group (individual nutrition therapy, n=28) and self-management group (learned about low FODMAP diet online, n=26). Both groups followed low FODMAP diet for 4 weeks. Four-day food records were used to assess dietary intake. Symptoms were assessed by the IBS-severity scoring system (ISB-SSS). RESULTS: The number of subjects who did not complete the study was 13, thereof five in the nutrition therapy and eight in the self-management group, leaving 23 and 18 subjects available for analysis, respectively. Symptoms declined from baseline to endpoint in both groups, by 183±101 points on average in the group receiving nutrition therapy (p< 0.001) and 132±110 points in the self-management group (p< 0.001), with no difference between groups. At baseline, about 80% of meals in both groups contained food high in FODMAP's. The corresponding proportion was 9% and 36% in week 3 in the nutrition therapy and self-management group, respectively (p< 0.001). CONCLUSION: Both groups experienced relieve of symptoms, but compliance to the low FODMAP diet was better in the group receiving individual nutrition therapy compared with the group who only received instructions on how to learn about low FODMAP diet online.