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Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria: 6 Months Follow-Up
In phenylketonuria (PKU), a previous intervention study assessing the patients ability to tolerate fruits and vegetables containing phenylalanine 76–100 mg/100 g without limit or measurement, found that an extra 50 mg/day phenylalanine, but not 100 mg/day, was tolerated from these fruits and vegetab...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10347155/ https://www.ncbi.nlm.nih.gov/pubmed/37447372 http://dx.doi.org/10.3390/nu15133046 |
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author | Pinto, Alex Daly, Anne Rocha, Júlio César Ashmore, Catherine Evans, Sharon Jackson, Richard Hickson, Mary MacDonald, Anita |
author_facet | Pinto, Alex Daly, Anne Rocha, Júlio César Ashmore, Catherine Evans, Sharon Jackson, Richard Hickson, Mary MacDonald, Anita |
author_sort | Pinto, Alex |
collection | PubMed |
description | In phenylketonuria (PKU), a previous intervention study assessing the patients ability to tolerate fruits and vegetables containing phenylalanine 76–100 mg/100 g without limit or measurement, found that an extra 50 mg/day phenylalanine, but not 100 mg/day, was tolerated from these fruits and vegetables. In a further 6-month extension study, we examined the effect of the ‘free’ use of this group of fruits and vegetables on blood phenylalanine control. For 6 months, the patients ate fruits and vegetables containing phenylalanine 76–100 mg/100 g without limit or measurement. Three-day diet diaries and the patients’ weights were collected monthly. Blood phenylalanine spots were collected weekly aiming for blood phenylalanine levels <360 μmol/L. Retrospective blood phenylalanine was collected 6 months pre-trial. All 16 patients (69% females) from the intervention study took part in the extension study. Most of the patients (n = 14/16) had classical PKU with a median age of 10.5 years (range: 6–13). There was no statistically significant difference in the median blood phenylalanine pre-study (270, range: 50–760 μmol/L) compared to the 6-month extension study (250, range: 20–750 μmol/L) (p= 0.4867). The patients had a median of 21 and 22 bloodspots, pre- and post-trial, respectively. In the extension study, the patients had an actual mean intake of 11 g/day (4–37) natural protein and 65 g/day (60–80) protein equivalent from a protein substitute. The mean phenylalanine intake was 563 mg/day (200–1850) with only 19 mg/day (0–146) phenylalanine from fruits and vegetables containing phenylalanine 76–100 mg/100 g. The weight z-scores remained unchanged (1.52 vs. 1.60, p = 0.4715). There was no adverse impact on blood phenylalanine control when fruits and vegetables containing phenylalanine 76–100 mg/100 g were eaten without limit or measurement. However, the fruits and vegetable portion sizes eaten were small (60 g/week). Further longitudinal work is necessary to examine the ‘free’ use of fruits and vegetables containing phenylalanine 76–100 mg/100 g on metabolic control in patients with PKU. |
format | Online Article Text |
id | pubmed-10347155 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-103471552023-07-15 Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria: 6 Months Follow-Up Pinto, Alex Daly, Anne Rocha, Júlio César Ashmore, Catherine Evans, Sharon Jackson, Richard Hickson, Mary MacDonald, Anita Nutrients Article In phenylketonuria (PKU), a previous intervention study assessing the patients ability to tolerate fruits and vegetables containing phenylalanine 76–100 mg/100 g without limit or measurement, found that an extra 50 mg/day phenylalanine, but not 100 mg/day, was tolerated from these fruits and vegetables. In a further 6-month extension study, we examined the effect of the ‘free’ use of this group of fruits and vegetables on blood phenylalanine control. For 6 months, the patients ate fruits and vegetables containing phenylalanine 76–100 mg/100 g without limit or measurement. Three-day diet diaries and the patients’ weights were collected monthly. Blood phenylalanine spots were collected weekly aiming for blood phenylalanine levels <360 μmol/L. Retrospective blood phenylalanine was collected 6 months pre-trial. All 16 patients (69% females) from the intervention study took part in the extension study. Most of the patients (n = 14/16) had classical PKU with a median age of 10.5 years (range: 6–13). There was no statistically significant difference in the median blood phenylalanine pre-study (270, range: 50–760 μmol/L) compared to the 6-month extension study (250, range: 20–750 μmol/L) (p= 0.4867). The patients had a median of 21 and 22 bloodspots, pre- and post-trial, respectively. In the extension study, the patients had an actual mean intake of 11 g/day (4–37) natural protein and 65 g/day (60–80) protein equivalent from a protein substitute. The mean phenylalanine intake was 563 mg/day (200–1850) with only 19 mg/day (0–146) phenylalanine from fruits and vegetables containing phenylalanine 76–100 mg/100 g. The weight z-scores remained unchanged (1.52 vs. 1.60, p = 0.4715). There was no adverse impact on blood phenylalanine control when fruits and vegetables containing phenylalanine 76–100 mg/100 g were eaten without limit or measurement. However, the fruits and vegetable portion sizes eaten were small (60 g/week). Further longitudinal work is necessary to examine the ‘free’ use of fruits and vegetables containing phenylalanine 76–100 mg/100 g on metabolic control in patients with PKU. MDPI 2023-07-06 /pmc/articles/PMC10347155/ /pubmed/37447372 http://dx.doi.org/10.3390/nu15133046 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 Pinto, Alex Daly, Anne Rocha, Júlio César Ashmore, Catherine Evans, Sharon Jackson, Richard Hickson, Mary MacDonald, Anita Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria: 6 Months Follow-Up |
title | Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria: 6 Months Follow-Up |
title_full | Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria: 6 Months Follow-Up |
title_fullStr | Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria: 6 Months Follow-Up |
title_full_unstemmed | Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria: 6 Months Follow-Up |
title_short | Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria: 6 Months Follow-Up |
title_sort | preliminary data on free use of fruits and vegetables containing phenylalanine 76–100 mg/100 g of food in 16 children with phenylketonuria: 6 months follow-up |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10347155/ https://www.ncbi.nlm.nih.gov/pubmed/37447372 http://dx.doi.org/10.3390/nu15133046 |
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