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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: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.

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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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