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Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet

There are concerns about muscle and bone health in patients with Phenylketonuria (PKU). Our aim was to compare muscle mass, function, and bone health among young adults with PKU who maintained or suspended dietary treatment. Methods: Three groups were considered—PKU-1: 10 patients who used a protein...

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Autores principales: Rojas-Agurto, Eugenia, Leal-Witt, María Jesús, Arias, Carolina, Cabello, Juan Francisco, Bunout, Daniel, Cornejo, Verónica
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10346392/
https://www.ncbi.nlm.nih.gov/pubmed/37447265
http://dx.doi.org/10.3390/nu15132939
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author Rojas-Agurto, Eugenia
Leal-Witt, María Jesús
Arias, Carolina
Cabello, Juan Francisco
Bunout, Daniel
Cornejo, Verónica
author_facet Rojas-Agurto, Eugenia
Leal-Witt, María Jesús
Arias, Carolina
Cabello, Juan Francisco
Bunout, Daniel
Cornejo, Verónica
author_sort Rojas-Agurto, Eugenia
collection PubMed
description There are concerns about muscle and bone health in patients with Phenylketonuria (PKU). Our aim was to compare muscle mass, function, and bone health among young adults with PKU who maintained or suspended dietary treatment. Methods: Three groups were considered—PKU-1: 10 patients who used a protein substitute (PS) without phenylalanine (Phe); PKU-2: 14 patients who used the PS without Phe until eighteen years old and then practiced mostly a vegan diet; and 24 matched healthy controls. A 24 h recall survey, blood parameters, body composition and bone mineral density through DEXA, rectus femoris thickness by ultrasound, hand grip strength, submaximal exercise test, and walking speed were assessed. Results: PKU-1 patients had lower hand grip strength than their matched controls, but no other differences. Compared to controls, the PKU-2 group had lower fat-free mass (p = 0.01), less spine and femoral bone mineral density (p = 0.04 and p < 0.01, respectively), and peak workload on the incremental test (p = 0.03). When comparing PKU groups, blood Phe levels were significantly lower in the PKU-1 group (p = 0.02). Conclusions: Among PKU patients, abandoning the dietary treatment and maintaining high blood Phe concentrations could be deleterious for muscles and bones. However, we cannot discard other causes of bone and muscle damage in these patients.
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spelling pubmed-103463922023-07-15 Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet Rojas-Agurto, Eugenia Leal-Witt, María Jesús Arias, Carolina Cabello, Juan Francisco Bunout, Daniel Cornejo, Verónica Nutrients Article There are concerns about muscle and bone health in patients with Phenylketonuria (PKU). Our aim was to compare muscle mass, function, and bone health among young adults with PKU who maintained or suspended dietary treatment. Methods: Three groups were considered—PKU-1: 10 patients who used a protein substitute (PS) without phenylalanine (Phe); PKU-2: 14 patients who used the PS without Phe until eighteen years old and then practiced mostly a vegan diet; and 24 matched healthy controls. A 24 h recall survey, blood parameters, body composition and bone mineral density through DEXA, rectus femoris thickness by ultrasound, hand grip strength, submaximal exercise test, and walking speed were assessed. Results: PKU-1 patients had lower hand grip strength than their matched controls, but no other differences. Compared to controls, the PKU-2 group had lower fat-free mass (p = 0.01), less spine and femoral bone mineral density (p = 0.04 and p < 0.01, respectively), and peak workload on the incremental test (p = 0.03). When comparing PKU groups, blood Phe levels were significantly lower in the PKU-1 group (p = 0.02). Conclusions: Among PKU patients, abandoning the dietary treatment and maintaining high blood Phe concentrations could be deleterious for muscles and bones. However, we cannot discard other causes of bone and muscle damage in these patients. MDPI 2023-06-28 /pmc/articles/PMC10346392/ /pubmed/37447265 http://dx.doi.org/10.3390/nu15132939 Text en © 2023 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
Rojas-Agurto, Eugenia
Leal-Witt, María Jesús
Arias, Carolina
Cabello, Juan Francisco
Bunout, Daniel
Cornejo, Verónica
Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet
title Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet
title_full Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet
title_fullStr Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet
title_full_unstemmed Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet
title_short Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet
title_sort muscle and bone health in young chilean adults with phenylketonuria and different degrees of compliance with the phenylalanine restricted diet
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10346392/
https://www.ncbi.nlm.nih.gov/pubmed/37447265
http://dx.doi.org/10.3390/nu15132939
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