Growth and Nutritional Status of Phenylketonuric Children and Adolescents

Abstract Background The goal of this study was to assess the anthropometric and biochemical parameters of children and adolescents with phenylketonuria (PKU). Methods The participants in this cross-sectional study ranged in age from four to 18 years old. Biochemical markers such as vitamin B12, foli...

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Main Authors: Mina Ahmadzadeh, Golbon Sohrab, Mohammadreza Alaei, Hassan Eini-Zinab, Behzad mohammadpour-Ahranjani, Samira Rastgoo, Zahra Namkhah
Format: Article
Language:English
Published: BMC 2022-11-01
Series:BMC Pediatrics
Subjects:
Online Access:https://doi.org/10.1186/s12887-022-03715-2
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author Mina Ahmadzadeh
Golbon Sohrab
Mohammadreza Alaei
Hassan Eini-Zinab
Behzad mohammadpour-Ahranjani
Samira Rastgoo
Zahra Namkhah
author_facet Mina Ahmadzadeh
Golbon Sohrab
Mohammadreza Alaei
Hassan Eini-Zinab
Behzad mohammadpour-Ahranjani
Samira Rastgoo
Zahra Namkhah
author_sort Mina Ahmadzadeh
collection DOAJ
description Abstract Background The goal of this study was to assess the anthropometric and biochemical parameters of children and adolescents with phenylketonuria (PKU). Methods The participants in this cross-sectional study ranged in age from four to 18 years old. Biochemical markers such as vitamin B12, folic acid, iron, ferritin, calcium, 25-hydroxy vitamin D3, zinc, plasma phenylalanine (Phe) and tyrosine (Tyr) levels in blood were evaluated, as well as demographics and anthropometric measurements. A three-day dietary recall questionnaire was completed by all individuals. Results 80% (64) of the 80 patients (42 females, 52.5%) had typical PKU. Consanguineous marriages were found in 57.5% (46) of the patients’ parents. According to the height for age index, 17.5% of the study group (n = 14) were short or very short. According to age-related weight and body mass index (BMI), 37.5% (n = 30) and 43.8% (n = 35) of people are obese or overweight, respectively. Biochemical tests revealed increased vitamin B12 levels and 25-hydroxy vitamin D3 deficiency in 35% (n = 28) of the patients, insufficient folic acid in 12.5% (n = 10), and elevated phenylalanine levels in 70.3% (n = 45) of children under 12 years old, and adolescents 62.5% (n = 10). A high Phe intake (OR = 4.44, CI %95 = 1.27–15.57) is a risk factor for obesity and overweight. Conclusion Patients with PKU had a high rate of overweight and obesity. PKU patients who are overweight or obese do not differ from normal-weight patients in terms of dietary intake or laboratory findings (except for serum iron levels). One-third of patients with phenylketonuria were vitamin D deficient and had a BMI/A index of overweight/obese. It is recommended to use special medical food to help solve energy and nutrient deficiencies.
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spelling doaj.art-c7633bc0a1f54d8b9f2a2cf2132c05622022-12-22T02:47:16ZengBMCBMC Pediatrics1471-24312022-11-012211710.1186/s12887-022-03715-2Growth and Nutritional Status of Phenylketonuric Children and AdolescentsMina Ahmadzadeh0Golbon Sohrab1Mohammadreza Alaei2Hassan Eini-Zinab3Behzad mohammadpour-Ahranjani4Samira Rastgoo5Zahra Namkhah6Department of Clinical Nutrition and Dietetics, Faculty of Nutrition Sciences and Food Technology, National Nutrition and Food Technology Research Institute, Shahid Beheshti University of Medical SciencesDepartment of Clinical Nutrition and Dietetics, Faculty of Nutrition Sciences and Food Technology, National Nutrition and Food Technology Research Institute, Shahid Beheshti University of Medical SciencesDepartment of Pediatric Endocrinology, Faculty of Medicine, Shahid Beheshti University of Medical SciencesDepartment of Community Nutrition, Faculty of Nutrition Sciences and Food Technology, National Nutrition and Food Technology Research institute, Shahid Beheshti University of Medical Sciences, Shahid Beheshti University of Medical SciencesDepartment of Pediatric Cardiology, Bahrami Hospital, Tehran University of Medical SciencesDepartment of Clinical Nutrition and Dietetics, Faculty of Nutrition Sciences and Food Technology, National Nutrition and Food Technology Research Institute, Shahid Beheshti University of Medical SciencesDepartment of Clinical Nutrition, School of Nutritional Sciences and Dietetics, Tehran University of Medical ScienceAbstract Background The goal of this study was to assess the anthropometric and biochemical parameters of children and adolescents with phenylketonuria (PKU). Methods The participants in this cross-sectional study ranged in age from four to 18 years old. Biochemical markers such as vitamin B12, folic acid, iron, ferritin, calcium, 25-hydroxy vitamin D3, zinc, plasma phenylalanine (Phe) and tyrosine (Tyr) levels in blood were evaluated, as well as demographics and anthropometric measurements. A three-day dietary recall questionnaire was completed by all individuals. Results 80% (64) of the 80 patients (42 females, 52.5%) had typical PKU. Consanguineous marriages were found in 57.5% (46) of the patients’ parents. According to the height for age index, 17.5% of the study group (n = 14) were short or very short. According to age-related weight and body mass index (BMI), 37.5% (n = 30) and 43.8% (n = 35) of people are obese or overweight, respectively. Biochemical tests revealed increased vitamin B12 levels and 25-hydroxy vitamin D3 deficiency in 35% (n = 28) of the patients, insufficient folic acid in 12.5% (n = 10), and elevated phenylalanine levels in 70.3% (n = 45) of children under 12 years old, and adolescents 62.5% (n = 10). A high Phe intake (OR = 4.44, CI %95 = 1.27–15.57) is a risk factor for obesity and overweight. Conclusion Patients with PKU had a high rate of overweight and obesity. PKU patients who are overweight or obese do not differ from normal-weight patients in terms of dietary intake or laboratory findings (except for serum iron levels). One-third of patients with phenylketonuria were vitamin D deficient and had a BMI/A index of overweight/obese. It is recommended to use special medical food to help solve energy and nutrient deficiencies.https://doi.org/10.1186/s12887-022-03715-2PhenylketonuriaPhenylalanineNutritional status
spellingShingle Mina Ahmadzadeh
Golbon Sohrab
Mohammadreza Alaei
Hassan Eini-Zinab
Behzad mohammadpour-Ahranjani
Samira Rastgoo
Zahra Namkhah
Growth and Nutritional Status of Phenylketonuric Children and Adolescents
BMC Pediatrics
Phenylketonuria
Phenylalanine
Nutritional status
title Growth and Nutritional Status of Phenylketonuric Children and Adolescents
title_full Growth and Nutritional Status of Phenylketonuric Children and Adolescents
title_fullStr Growth and Nutritional Status of Phenylketonuric Children and Adolescents
title_full_unstemmed Growth and Nutritional Status of Phenylketonuric Children and Adolescents
title_short Growth and Nutritional Status of Phenylketonuric Children and Adolescents
title_sort growth and nutritional status of phenylketonuric children and adolescents
topic Phenylketonuria
Phenylalanine
Nutritional status
url https://doi.org/10.1186/s12887-022-03715-2
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AT behzadmohammadpourahranjani growthandnutritionalstatusofphenylketonuricchildrenandadolescents
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