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Nutrient Intake and Nutritional Status in Adult Patients with Inherited Metabolic Diseases Treated with Low-Protein Diets: A Review on Urea Cycle Disorders and Branched Chain Organic Acidemias.

ABSTRACT: Low-protein diets (LPDs) are the main treatment for urea cycle disorders (UCDs) and organic acidemias (OAs). In most cases, LPDs start in childhood and must be continued into adulthood. The improved life expectancy of patients with UCDs and OAs raises the question of their consequences on nutritional status in adult subjects. As this topic has so far received little attention, we conducted a review of scientific studies that investigated the nutrient intake and nutritional status in adult patients with UCDs and branched chain organic acidemias (BCOAs) on LPD. METHODS:The literature search was conducted in PubMed/MEDLINE, Scopus, EMBASE and Google Scholar from 1 January 2000 to 31 May 2020, focusing on nutrient intake and nutritional status in UCD and OA adult patients. RESULTS:Despite protein restriction is recommended as the main treatment for UCDs and OAs, in these patients, protein intake ranges widely, with many patients who do not reach safety levels. When evaluated, micronutrient intake resulted below recommended values in some patients. Lean body mass resulted in most cases lower than normal range while fat body mass (FM) was often found normal or higher than the controls or reference values. Protein intake correlated inversely with FM both in adult and pediatric UCD patients. CONCLUSIONS:The clinical management of adult patients with UCDs and BCOAs should include an accurate assessment of the nutritional status and body composition. However, as little data is still available on this topic, further studies are needed to better clarify the effects of LPDs on nutritional status in adult UCD and BCOA patients.

SUBMITTER: Francini-Pesenti F

PROVIDER: S-EPMC7693747 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature

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Nutrient Intake and Nutritional Status in Adult Patients with Inherited Metabolic Diseases Treated with Low-Protein Diets: A Review on Urea Cycle Disorders and Branched Chain Organic Acidemias.

Francini-Pesenti Francesco F Gugelmo Giorgia G Lenzini Livia L Vitturi Nicola N

Nutrients 20201029 11

Low-protein diets (LPDs) are the main treatment for urea cycle disorders (UCDs) and organic acidemias (OAs). In most cases, LPDs start in childhood and must be continued into adulthood. The improved life expectancy of patients with UCDs and OAs raises the question of their consequences on nutritional status in adult subjects. As this topic has so far received little attention, we conducted a review of scientific studies that investigated the nutrient intake and nutritional status in adult patien ...[more]

PMID: 33138136

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Project description:Low-protein diets (LPDs) are the mainstream treatment for inborn errors of intermediary protein metabolism (IEIPM), but dietary management differs worldwide. Most studies have investigated pediatric populations and their goals such as growth and metabolic balance, showing a tendency toward increasing overweight and obesity. Only a few studies have examined nutritional status and dietary intake of adult IEIPM patients on LPDs. We assessed nutritional parameters (dietary intake using a 7-day food diary record, body composition by bioimpedance analysis, and biochemical serum values) in a group of 18 adult patients with urea cycle disorders (UCDs) and branched chain organic acidemia (BCOA). Mean total protein intake was 0.61 ± 0.2 g/kg/day (73.5% of WHO Safe Levels) and mean natural protein (PN) intake was 0.54 ± 0.2 g/kg/day; 33.3% of patients consumed amino acid (AA) supplements. A totally of 39% of individuals presented a body mass index (BMI) > 25 kg/m2 and patients on AA supplements had a mean BMI indicative of overweight. All patients reported low physical activity levels. Total energy intake was 24.2 ± 5 kcal/kg/day, representing 72.1% of mean total energy expenditure estimated by predictive formulas. The protein energy ratio (P:E) was, on average, 2.22 g/100 kcal/day. Plasmatic levels of albumin, amino acids, and lipid profiles exhibited normal ranges. Phase angle (PA) was, on average, 6.0° ± 0.9°. Fat mass percentage (FM%) was 22% ± 9% in men and 36% ± 4% in women. FM% was inversely and significantly related to total and natural protein intake. Data from IEIPM adults on LPDs confirmed the pediatric trend of increasing overweight and obesity despite a low energy intake. A low protein intake may contribute to an increased fat mass. Nutritional parameters and a healthy lifestyle should be routinely assessed in order to optimize nutritional status and possibly reduce risk of cardiovascular degenerative diseases in adult UCD and BCOA patients on LPDs.

Project description:BackgroundAccess to high-quality dietary intake data is central to many nutrition, epidemiology, economic, environmental, and policy applications. When data on individual nutrient intakes are available, they have not been consistently disaggregated by sex and age groups, and their parameters and full distributions are often not publicly available.ObjectivesWe sought to derive usual intake distributions for as many nutrients and population subgroups as possible, use these distributions to estimate nutrient intake inadequacy, compare these distributions and evaluate the implications of their shapes on the estimation of inadequacy, and make these distributions publicly available.MethodsWe compiled dietary data sets from 31 geographically diverse countries, modeled usual intake distributions for 32 micronutrients and 21 macronutrients, and disaggregated these distributions by sex and age groups. We compared the variability and skewness of the distributions and evaluated their similarity across countries, sex, and age groups. We estimated intake inadequacy for 16 nutrients based on a harmonized set of nutrient requirements and bioavailability estimates. Last, we created an R package-nutriR-to make these distributions freely available for users to apply in their own analyses.ResultsUsual intake distributions were rarely symmetric and differed widely in variability and skewness across nutrients and countries. Vitamin intake distributions were more variable and skewed and exhibited less similarity among countries than other nutrients. Inadequate intakes were high and geographically concentrated, as well as generally higher for females than males. We found that the shape of usual intake distributions strongly affects estimates of the prevalence of inadequate intakes.ConclusionsThe shape of nutrient intake distributions differs based on nutrient and subgroup and strongly influences estimates of nutrient intake inadequacy. This research represents an important contribution to the availability and application of dietary intake data for diverse subpopulations around the world.

Project description:BackgroundBoth fresh and processed foods are available in the modern food environment where taste can signal presence of nutrients. However, whether these taste-nutrient relationships are maintained across different degrees of food processing is not well understood, and less is known about the relative contribution of different taste qualities to population energy intakes.ObjectivesTo investigate the association between perceived intensity of 6 taste modalities and a food's nutrient content in the context of food processing and to further examine the relative contribution of different taste clusters to total energy intakes, stratified by weight status.MethodsDiet and lifestyle data from the Singapore Multi-Ethnic Cohort Phase 2 (N = 7011; aged 21-75 y) were collected through interviewer-administrated questionnaires. Taste and nutrient profiles for each of the 269 Singaporean foods were derived using a published taste database and food composition table. Each food was then categorized into the NOVA food-processing classification (unprocessed, processed, ultra-processed) to compare the strength of taste-nutrient relationships. Multivariable-adjusted models were used to examine associations between relative consumption of foods from different taste clusters and processing categories, energy intake, and BMI (in kg/m2) within a population cohort.ResultsSweet taste and mono- and disaccharide content of foods were significantly associated across all processing categories, although this association was weaker among ultra-processed foods (UPFs) (r = 0.42) than among unprocessed foods (r = 0.72). In contrast, associations between fat sensation and fat content (r = 0.74), as well as salt taste and sodium content (r = 0.84), were stronger for UPFs. Individuals who had higher energy intakes or were overweight (BMI >23) derived significantly greater percentage of energy from processed foods rather than UPFs, and this energy was higher from "savory-fatty" and lower from "neutral" tasting foods than those with lower energy intakes and normal weight (all P < 0.001). Eighty percent of individuals' dietary energy was from both "savory-fatty" and "neutral" foods, independent of differences in total energy intake and weight status.ConclusionsTaste-nutrient relationships are maintained across different degrees of food processing. Greater consumption of foods that have a high "savory-fatty" taste was associated with increased energy intakes and overweight in the Asian population.

Project description:In Brazilian universities, the university restaurant (UR) is essential in supporting students to complete their courses, as the UR offers free or low-cost food. In this sense, this research aimed to evaluate public policy effectiveness in offering food to low-income students attending the UR of the University of Brasília. This cross-sectional study compared low-income students (participating in the Student Assistance Program-Group 1) and students that did not participate in the Program (Group 2). Researchers assessed food consumption through direct observation of students while serving their plates at UR (in all meals consumed at UR) and completed food consumption with diet recalls for the meals outside the UR. In total, three complete days, including one weekend day, were evaluated for each student. Researchers also evaluated the participants' body mass composition and body fat percentage. The results of the comparisons between the evaluated groups showed that the groups presented similar intakes. Only sodium intake was significantly different for males, being higher for Group 1. The median sodium consumption among females and males in group 1 was 55% and 119%, respectively, above the upper limit (UL). In Group 2, sodium intake levels reached consumption percentages above UL by 36% for females and 79% for males. The prevalence of inadequate sodium consumption was 100% for both genders and groups. Extra salt was added to dishes by 19.7% of the students. For females, only fiber ingestion was statistically different, with higher intake for Group 1. The other evaluated parameters showed similarities among groups for each gender. The statistical analysis revealed a significant difference in the consumption of calories, fibers, sodium, iron, and calcium for the students who had three meals at the UR in the two weekdays. There was a statistical difference in nutrients for those who had three meals in the UR, reinforcing the importance of the UR's meals. The current food and nutrition policy at the UR proved to be extremely important in university students' lives and in maintaining healthy nutritional aspects. However, changes in sodium use, more calcium intake, and less cholesterol consumption should receive attention to better balance dietary elements of the food offered. Dish preparation should be carefully followed to ensure the quality of the food for university students.

Dataset Information

Nutrient Intake and Nutritional Status in Adult Patients with Inherited Metabolic Diseases Treated with Low-Protein Diets: A Review on Urea Cycle Disorders and Branched Chain Organic Acidemias.

Publications

Nutrient Intake and Nutritional Status in Adult Patients with Inherited Metabolic Diseases Treated with Low-Protein Diets: A Review on Urea Cycle Disorders and Branched Chain Organic Acidemias.

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