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Analysis of body composition and nutritional status in Brazilian phenylketonuria patients
BACKGROUND: Phenylketonuria (PKU) is characterized by phenylalanine (Phe) accumulation to toxic levels due to the low activity of phenylalanine-hydroxylase. PKU patients must follow a Phe-restricted diet, which may put them in risk of nutritional disturbances. Therefore, we aimed to characterize bod...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4789337/ https://www.ncbi.nlm.nih.gov/pubmed/27014574 http://dx.doi.org/10.1016/j.ymgmr.2015.12.003 |
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author | Mazzola, Priscila Nicolao Nalin, Tatiele Castro, Kamila van Rijn, Margreet Derks, Terry G.J. Perry, Ingrid D.S. Mainieri, Alberto Scofano Schwartz, Ida Vanessa D. |
author_facet | Mazzola, Priscila Nicolao Nalin, Tatiele Castro, Kamila van Rijn, Margreet Derks, Terry G.J. Perry, Ingrid D.S. Mainieri, Alberto Scofano Schwartz, Ida Vanessa D. |
author_sort | Mazzola, Priscila Nicolao |
collection | PubMed |
description | BACKGROUND: Phenylketonuria (PKU) is characterized by phenylalanine (Phe) accumulation to toxic levels due to the low activity of phenylalanine-hydroxylase. PKU patients must follow a Phe-restricted diet, which may put them in risk of nutritional disturbances. Therefore, we aimed to characterize body composition parameters and nutritional status in Brazilian PKU patients also considering their metabolic control. METHODS: Twenty-seven treated PKU patients older than 5 years, and 27 age- and gender-matched controls, were analyzed for anthropometric features and body composition by bioelectrical impedance (BIA). Patients' metabolic control was assessed by historical Phe levels. RESULTS: There was no effect of PKU type, time of diagnosis, or metabolic control for any analyzed parameter. About 75% of patients and controls were eutrophic, according to their BMI values. There were no difference between groups regarding body composition and other BIA-derived parameters. CONCLUSIONS: Brazilian PKU patients do not show differences in body composition and nutritional status in comparison with controls, regardless metabolic control. Although similar to controls, PKU patients may be in risk of disturbed nutritional and metabolic markers as seen for the general population. |
format | Online Article Text |
id | pubmed-4789337 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-47893372016-03-24 Analysis of body composition and nutritional status in Brazilian phenylketonuria patients Mazzola, Priscila Nicolao Nalin, Tatiele Castro, Kamila van Rijn, Margreet Derks, Terry G.J. Perry, Ingrid D.S. Mainieri, Alberto Scofano Schwartz, Ida Vanessa D. Mol Genet Metab Rep Research Paper BACKGROUND: Phenylketonuria (PKU) is characterized by phenylalanine (Phe) accumulation to toxic levels due to the low activity of phenylalanine-hydroxylase. PKU patients must follow a Phe-restricted diet, which may put them in risk of nutritional disturbances. Therefore, we aimed to characterize body composition parameters and nutritional status in Brazilian PKU patients also considering their metabolic control. METHODS: Twenty-seven treated PKU patients older than 5 years, and 27 age- and gender-matched controls, were analyzed for anthropometric features and body composition by bioelectrical impedance (BIA). Patients' metabolic control was assessed by historical Phe levels. RESULTS: There was no effect of PKU type, time of diagnosis, or metabolic control for any analyzed parameter. About 75% of patients and controls were eutrophic, according to their BMI values. There were no difference between groups regarding body composition and other BIA-derived parameters. CONCLUSIONS: Brazilian PKU patients do not show differences in body composition and nutritional status in comparison with controls, regardless metabolic control. Although similar to controls, PKU patients may be in risk of disturbed nutritional and metabolic markers as seen for the general population. Elsevier 2016-01-09 /pmc/articles/PMC4789337/ /pubmed/27014574 http://dx.doi.org/10.1016/j.ymgmr.2015.12.003 Text en © 2015 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Research Paper Mazzola, Priscila Nicolao Nalin, Tatiele Castro, Kamila van Rijn, Margreet Derks, Terry G.J. Perry, Ingrid D.S. Mainieri, Alberto Scofano Schwartz, Ida Vanessa D. Analysis of body composition and nutritional status in Brazilian phenylketonuria patients |
title | Analysis of body composition and nutritional status in Brazilian phenylketonuria patients |
title_full | Analysis of body composition and nutritional status in Brazilian phenylketonuria patients |
title_fullStr | Analysis of body composition and nutritional status in Brazilian phenylketonuria patients |
title_full_unstemmed | Analysis of body composition and nutritional status in Brazilian phenylketonuria patients |
title_short | Analysis of body composition and nutritional status in Brazilian phenylketonuria patients |
title_sort | analysis of body composition and nutritional status in brazilian phenylketonuria patients |
topic | Research Paper |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4789337/ https://www.ncbi.nlm.nih.gov/pubmed/27014574 http://dx.doi.org/10.1016/j.ymgmr.2015.12.003 |
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