Cargando…

Anthropometrics, Dietary Intake and Body Composition in Urea Cycle Disorders and Branched Chain Organic Acidemias: A Case Study of 18 Adults on Low-Protein Diets

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 increasin...

Descripción completa

Detalles Bibliográficos
Autores principales: Gugelmo, Giorgia, Lenzini, Livia, Francini-Pesenti, Francesco, Fasan, Ilaria, Spinella, Paolo, Valentini, Romina, Miraval, Angela, Avogaro, Angelo, Vitturi, Nicola
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8839969/
https://www.ncbi.nlm.nih.gov/pubmed/35276826
http://dx.doi.org/10.3390/nu14030467
_version_ 1784650502119620608
author Gugelmo, Giorgia
Lenzini, Livia
Francini-Pesenti, Francesco
Fasan, Ilaria
Spinella, Paolo
Valentini, Romina
Miraval, Angela
Avogaro, Angelo
Vitturi, Nicola
author_facet Gugelmo, Giorgia
Lenzini, Livia
Francini-Pesenti, Francesco
Fasan, Ilaria
Spinella, Paolo
Valentini, Romina
Miraval, Angela
Avogaro, Angelo
Vitturi, Nicola
author_sort Gugelmo, Giorgia
collection PubMed
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/m(2) 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.
format Online
Article
Text
id pubmed-8839969
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-88399692022-02-13 Anthropometrics, Dietary Intake and Body Composition in Urea Cycle Disorders and Branched Chain Organic Acidemias: A Case Study of 18 Adults on Low-Protein Diets Gugelmo, Giorgia Lenzini, Livia Francini-Pesenti, Francesco Fasan, Ilaria Spinella, Paolo Valentini, Romina Miraval, Angela Avogaro, Angelo Vitturi, Nicola Nutrients Article 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/m(2) 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. MDPI 2022-01-21 /pmc/articles/PMC8839969/ /pubmed/35276826 http://dx.doi.org/10.3390/nu14030467 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Gugelmo, Giorgia
Lenzini, Livia
Francini-Pesenti, Francesco
Fasan, Ilaria
Spinella, Paolo
Valentini, Romina
Miraval, Angela
Avogaro, Angelo
Vitturi, Nicola
Anthropometrics, Dietary Intake and Body Composition in Urea Cycle Disorders and Branched Chain Organic Acidemias: A Case Study of 18 Adults on Low-Protein Diets
title Anthropometrics, Dietary Intake and Body Composition in Urea Cycle Disorders and Branched Chain Organic Acidemias: A Case Study of 18 Adults on Low-Protein Diets
title_full Anthropometrics, Dietary Intake and Body Composition in Urea Cycle Disorders and Branched Chain Organic Acidemias: A Case Study of 18 Adults on Low-Protein Diets
title_fullStr Anthropometrics, Dietary Intake and Body Composition in Urea Cycle Disorders and Branched Chain Organic Acidemias: A Case Study of 18 Adults on Low-Protein Diets
title_full_unstemmed Anthropometrics, Dietary Intake and Body Composition in Urea Cycle Disorders and Branched Chain Organic Acidemias: A Case Study of 18 Adults on Low-Protein Diets
title_short Anthropometrics, Dietary Intake and Body Composition in Urea Cycle Disorders and Branched Chain Organic Acidemias: A Case Study of 18 Adults on Low-Protein Diets
title_sort anthropometrics, dietary intake and body composition in urea cycle disorders and branched chain organic acidemias: a case study of 18 adults on low-protein diets
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8839969/
https://www.ncbi.nlm.nih.gov/pubmed/35276826
http://dx.doi.org/10.3390/nu14030467
work_keys_str_mv AT gugelmogiorgia anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets
AT lenzinilivia anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets
AT francinipesentifrancesco anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets
AT fasanilaria anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets
AT spinellapaolo anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets
AT valentiniromina anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets
AT miravalangela anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets
AT avogaroangelo anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets
AT vitturinicola anthropometricsdietaryintakeandbodycompositioninureacycledisordersandbranchedchainorganicacidemiasacasestudyof18adultsonlowproteindiets