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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MDPI AG
2023-06-01
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| Series: | Nutrients |
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| Online Access: | https://www.mdpi.com/2072-6643/15/13/2939 |
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| author | Eugenia Rojas-Agurto María Jesús Leal-Witt Carolina Arias Juan Francisco Cabello Daniel Bunout Verónica Cornejo |
| author_facet | Eugenia Rojas-Agurto María Jesús Leal-Witt Carolina Arias Juan Francisco Cabello Daniel Bunout Verónica Cornejo |
| author_sort | Eugenia Rojas-Agurto |
| collection | DOAJ |
| 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 (<i>p</i> = 0.01), less spine and femoral bone mineral density (<i>p</i> = 0.04 and <i>p</i> < 0.01, respectively), and peak workload on the incremental test (<i>p</i> = 0.03). When comparing PKU groups, blood Phe levels were significantly lower in the PKU-1 group (<i>p</i> = 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. |
| first_indexed | 2024-03-11T01:32:16Z |
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| last_indexed | 2024-03-11T01:32:16Z |
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| spelling | doaj.art-5de50192dd8c4fa8a057973bd140b9052023-11-18T17:14:42ZengMDPI AGNutrients2072-66432023-06-011513293910.3390/nu15132939Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted DietEugenia Rojas-Agurto0María Jesús Leal-Witt1Carolina Arias2Juan Francisco Cabello3Daniel Bunout4Verónica Cornejo5Instituto de Nutrición y Tecnología de Alimentos INTA, Universidad de Chile, Santiago 7830490, ChileInstituto de Nutrición y Tecnología de Alimentos INTA, Universidad de Chile, Santiago 7830490, ChileInstituto de Nutrición y Tecnología de Alimentos INTA, Universidad de Chile, Santiago 7830490, ChileInstituto de Nutrición y Tecnología de Alimentos INTA, Universidad de Chile, Santiago 7830490, ChileInstituto de Nutrición y Tecnología de Alimentos INTA, Universidad de Chile, Santiago 7830490, ChileInstituto de Nutrición y Tecnología de Alimentos INTA, Universidad de Chile, Santiago 7830490, ChileThere 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 (<i>p</i> = 0.01), less spine and femoral bone mineral density (<i>p</i> = 0.04 and <i>p</i> < 0.01, respectively), and peak workload on the incremental test (<i>p</i> = 0.03). When comparing PKU groups, blood Phe levels were significantly lower in the PKU-1 group (<i>p</i> = 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.https://www.mdpi.com/2072-6643/15/13/2939inborn error of metabolismphenylalaninemuscle massmuscle function |
| spellingShingle | Eugenia Rojas-Agurto María Jesús Leal-Witt Carolina Arias Juan Francisco Cabello Daniel Bunout Verónica Cornejo Muscle and Bone Health in Young Chilean Adults with Phenylketonuria and Different Degrees of Compliance with the Phenylalanine Restricted Diet Nutrients inborn error of metabolism phenylalanine muscle mass muscle function |
| 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 | inborn error of metabolism phenylalanine muscle mass muscle function |
| url | https://www.mdpi.com/2072-6643/15/13/2939 |
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