Nutritional and metabolic parameters of children and adolescents with phenylketonuria

Almeida, Barbara de Freitas de Nara; Laufer, July Ane; Mezzomo, Thais Regina; Shimada, Natália Cristina; Furtado, Ivy Hulbert Falcão; Dias, Marcia Regina Messaggi Gomes; Pereira, Rosana Marques

doi:10.1016/j.clnesp.2020.03.024

Cited by 12 publications

(16 citation statements)

References 22 publications

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“…In the present study, vitamin B12 and folic acid de ciency were negligible, with 45% and 35% of patients having high serum concentrations, respectively, similar to that seen in the Crujeiras et al (25), and Kose et al study (38). Similar to the present study, the results of Evans et al (39) and Almeida et al (33) studies showed that more than 90% of the subjects had adequate and normal ferritin levels.…”

Section: Discussionsupporting

confidence: 92%

“…According to their BMI z-score values, about 44% of patients in this study were overweight or obese, with females having higher BMI z-score values. In a study conducted by Almeida et al (33) in Brazil, the prevalence of obesity and overweight was 28.5%. Also, in the studies of Camatta et al (12) (33).…”

Section: Discussionmentioning

confidence: 94%

“…In a study conducted by Almeida et al (33) in Brazil, the prevalence of obesity and overweight was 28.5%. Also, in the studies of Camatta et al (12) (33).…”

Section: Discussionmentioning

confidence: 94%

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Growth and Nutritional Status of Phenylketonuric Children and Adolescents

Ahmadzadeh

Sohrab

Alaei

et al. 2022

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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 4 to 18. Biochemical markers such as blood vitamin B12, folic acid, iron, ferritin, calcium, 25-hydroxy vitamin D3, zinc, and plasma phenylalanine (phe) and tyrosine (Tyr) levels were evaluated, as well as demographics and anthropometric measurements. A 3-day dietary recall questionnaire was completed by all individuals.Results: Eighty percent (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) indices, 37.5% (n = 30) and 43.8% (n = 35) of people are obese or overweight, respectively. Biochemical testing revealed raised vitamin B12 levels and 25-hydroxy vitamin D3 deficiency in 28 (35%) of the patients, insufficient folic acid in 10 (12.5%), and elevated phenylalanine levels in children under 12 years old 45 (70.3%) and adolescents 10 (62.5%). A high phe intake (OR = 4.44, CI %95 = 1.27-15.57) is one risk factor for obesity and overweight.Conclusion: Patients with phenylketonuria had a high rate of overweight and obesity, according to anthropometric measurements. 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).

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 94%

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Growth and Nutritional Status of Phenylketonuric Children and Adolescents

Ahmadzadeh

Sohrab

Alaei

et al. 2022

Preprint

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“…Almeida et al, who supported the findings of this investigation, found that 88.1% of the sample was sufficient in terms of the H/A z-score [38].…”

Section: Discussionsupporting

confidence: 88%

“…According to their BMI z-score values, about 44% of patients in this study were overweight or obese, and females had higher BMI z-score values. In a study conducted by Almeida et al [38] in Brazil, the prevalence of obesity and overweight was 28.5%. Also, in the studies of Camatta et al [14] and Mazzola Data are presented as mean ± SD, n (%); PKU: phenylketonuria; SD: standard deviation; Phe: phenylalanine; Tyr: tyrosine a The value is calculated using Mann-Whitney Test b The value is calculated using Pearson Chi-Square Test et al [39], the prevalence of obesity and overweight was lower than our study (19.13% and 22%, respectively).…”

Section: Discussionmentioning

confidence: 94%

Growth and Nutritional Status of Phenylketonuric Children and Adolescents

et al. 2022

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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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