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Nutrient Status among Latvian Children with Phenylketonuria

(1) Introduction: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism. The main treatment for PKU is to manage nutrition, thereby restricting phenylalanine intake. Part of patient management is analyzing eating habits to substitute missing nutrients and limit the...

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Autores principales: Lubina, Olga, Gailite, Linda, Borodulina, Julija, Auzenbaha, Madara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10297135/
https://www.ncbi.nlm.nih.gov/pubmed/37371168
http://dx.doi.org/10.3390/children10060936
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author Lubina, Olga
Gailite, Linda
Borodulina, Julija
Auzenbaha, Madara
author_facet Lubina, Olga
Gailite, Linda
Borodulina, Julija
Auzenbaha, Madara
author_sort Lubina, Olga
collection PubMed
description (1) Introduction: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism. The main treatment for PKU is to manage nutrition, thereby restricting phenylalanine intake. Part of patient management is analyzing eating habits to substitute missing nutrients and limit the overdose of nutrients. This is mainly done by analyzing food diaries. This is the first review of Latvian PKU patients eating habits performed by analyzing 72-h food diaries (FD). (2) Materials and Methods: This study included individuals between the ages of 18 and 31 years, PKU patients and 31 age- and sex-matched control groups. All respondents kept 72-h food diaries (FD) and underwent testing for zinc, selenium and ferritin levels in the blood. Food diary data were analyzed by Nutritics software to calculate the theoretical intake of nutrients, and these values were compared with the Ministry of Health of the Republic of Latvia’s recommended values. (3) Results: A lack of motivation and diet therapy compliance in PKU patients was observed during this research. A total of 32% of PKU patients refused to fill out their FD or filled it out incorrectly. The analysis of nutrient intake was observed, and there was a statistically significant difference between PKU patients in the 1–3 age group and the control group in fat intake. Fat intake in PKU patients was below MRHL recommendations. The intake of iron was found to be surplus in all PKU patients in the age group of 1–3, 91% of PKU patients in the age group of 4–6 years, 63% in the age group of 7–12 and 71% in the 13–18 year age group. Although there were no instances in the PKU patients nor the control group who had ferritin levels above the normal range. Selenium intake was surplus in 80% of PKU patients in the 1–3 age group, 91% in PKU patients in the 4–6 age group, 88% in the 7–12 age group and 86% in the 13–18 age group. None of the patients had Se levels in the blood above the normal range. Zn intake was surplus in 100% of PKU patients in the 1–3 age group, 82% in PKU patients in the 4–6 age group, 88% in the 7–12 age group and 57% in the 13–18 age group, and no PKU patients had high Zn levels. None of the control group participants had levels below the normal range of Zn and Se while 11% of PKU patients in the 13–18 age group had inadequate levels of Se, although Se intake based on their FD was optimal. (4) Conclusions: Regular PKU patient nutritional status evaluation is important to define and prevent possible nutrient deficiency, and further investigation should be continued to find out the mechanism of nutrient absorption in PKU patients. To prevent macronutrient deficiency such as fat and micronutrient deficiency in PKU patients, one could use supplements or try an improved nutrient content of Phe-free formula in the future.
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spelling pubmed-102971352023-06-28 Nutrient Status among Latvian Children with Phenylketonuria Lubina, Olga Gailite, Linda Borodulina, Julija Auzenbaha, Madara Children (Basel) Article (1) Introduction: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism. The main treatment for PKU is to manage nutrition, thereby restricting phenylalanine intake. Part of patient management is analyzing eating habits to substitute missing nutrients and limit the overdose of nutrients. This is mainly done by analyzing food diaries. This is the first review of Latvian PKU patients eating habits performed by analyzing 72-h food diaries (FD). (2) Materials and Methods: This study included individuals between the ages of 18 and 31 years, PKU patients and 31 age- and sex-matched control groups. All respondents kept 72-h food diaries (FD) and underwent testing for zinc, selenium and ferritin levels in the blood. Food diary data were analyzed by Nutritics software to calculate the theoretical intake of nutrients, and these values were compared with the Ministry of Health of the Republic of Latvia’s recommended values. (3) Results: A lack of motivation and diet therapy compliance in PKU patients was observed during this research. A total of 32% of PKU patients refused to fill out their FD or filled it out incorrectly. The analysis of nutrient intake was observed, and there was a statistically significant difference between PKU patients in the 1–3 age group and the control group in fat intake. Fat intake in PKU patients was below MRHL recommendations. The intake of iron was found to be surplus in all PKU patients in the age group of 1–3, 91% of PKU patients in the age group of 4–6 years, 63% in the age group of 7–12 and 71% in the 13–18 year age group. Although there were no instances in the PKU patients nor the control group who had ferritin levels above the normal range. Selenium intake was surplus in 80% of PKU patients in the 1–3 age group, 91% in PKU patients in the 4–6 age group, 88% in the 7–12 age group and 86% in the 13–18 age group. None of the patients had Se levels in the blood above the normal range. Zn intake was surplus in 100% of PKU patients in the 1–3 age group, 82% in PKU patients in the 4–6 age group, 88% in the 7–12 age group and 57% in the 13–18 age group, and no PKU patients had high Zn levels. None of the control group participants had levels below the normal range of Zn and Se while 11% of PKU patients in the 13–18 age group had inadequate levels of Se, although Se intake based on their FD was optimal. (4) Conclusions: Regular PKU patient nutritional status evaluation is important to define and prevent possible nutrient deficiency, and further investigation should be continued to find out the mechanism of nutrient absorption in PKU patients. To prevent macronutrient deficiency such as fat and micronutrient deficiency in PKU patients, one could use supplements or try an improved nutrient content of Phe-free formula in the future. MDPI 2023-05-26 /pmc/articles/PMC10297135/ /pubmed/37371168 http://dx.doi.org/10.3390/children10060936 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
Lubina, Olga
Gailite, Linda
Borodulina, Julija
Auzenbaha, Madara
Nutrient Status among Latvian Children with Phenylketonuria
title Nutrient Status among Latvian Children with Phenylketonuria
title_full Nutrient Status among Latvian Children with Phenylketonuria
title_fullStr Nutrient Status among Latvian Children with Phenylketonuria
title_full_unstemmed Nutrient Status among Latvian Children with Phenylketonuria
title_short Nutrient Status among Latvian Children with Phenylketonuria
title_sort nutrient status among latvian children with phenylketonuria
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10297135/
https://www.ncbi.nlm.nih.gov/pubmed/37371168
http://dx.doi.org/10.3390/children10060936
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