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Nutritional and metabolic parameters of children and adolescents with phenylketonuria.
de Almeida, Barbara Nara de Freitas; Laufer, July Ane; Mezzomo, Thais Regina; Shimada, Natália Cristina; Furtado, Ivy Hulbert Falcão; Dias, Marcia Regina Messaggi Gomes; Pereira, Rosana Marques.
Afiliação
  • de Almeida BNF; Positivo University, Pedro Viriato Parigot de Souza St, 5300, Campo Comprido, Curitiba, Parana State, 81280-330, Brazil.
  • Laufer JA; Positivo University, Pedro Viriato Parigot de Souza St, 5300, Campo Comprido, Curitiba, Parana State, 81280-330, Brazil.
  • Mezzomo TR; Programa de Pós-Graduação em Saúde da Criança e do Adolescente, Federal University of Parana, 181 Carneiro St, Alto da Glória, Curitiba, Parana State, 80060-900, Brazil. Electronic address: thaismezzomo@yahoo.com.br.
  • Shimada NC; Ecumenical Foundation for the Protection of the Exceptional, 836 Prefeito Lothário Meissner Ave, Jardim Botânico, Curitiba, Parara, 80210-170, Brazil.
  • Furtado IHF; Ecumenical Foundation for the Protection of the Exceptional, 836 Prefeito Lothário Meissner Ave, Jardim Botânico, Curitiba, Parara, 80210-170, Brazil.
  • Dias MRMG; Department of Nutrition, Federal University of Parana, 632 Prefeito Lothário Meissner Ave, Jardim Botânico, Curitiba, Parara, 80210-170, Brazil.
  • Pereira RM; Programa de Pós-Graduação em Saúde da Criança e do Adolescente, Federal University of Parana, 181 Carneiro St, Alto da Glória, Curitiba, Parana State, 80060-900, Brazil.
Clin Nutr ESPEN ; 37: 44-49, 2020 06.
Article em En | MEDLINE | ID: mdl-32359754
BACKGROUND AND AIMS: Considering that phenylalanine-poor diets may be monotonous and compromise the development and nutritional status of children and adolescents with phenylketonuria, the aim of this study was to evaluate the anthropometric and biochemical characteristics of children and adolescents with this condition. METHODS: Retrospective study with anthropometric and biochemical data collection from patients with phenylketonuria in the age group 2-19.9 years. Nutritional status was classified according to the World Health Organization. Biochemical tests were compared to current recommendations. RESULTS: A total of 84 patients (71.8%) were eligible, with a median age of 10.7 years (2.4-19.9 years). There was predominance of adequate (n = 58, 69%) with presence of overweight and obesity in 24 (28.5%) patients. The biochemical tests revealed hyperphosphatemia in 46 (55%), hypertriglyceridemia in 27 (50%), vitamin B12 elevated in 34 (41.2%), selenium deficiency in 10 (13.7%), insufficient zinc in 7 (8.9%), low globulin in 21 (26.9%), low HDL in 35 (59.3%) and elevated phenylalanine level in 28 (34.5%) patients in the sample. Overweight and obesity were correlated with low HDL (p = 0.04) and lowest adequate frequency of LDL (p = 0.09). Higher phosphorus values were associated with lower body weight (r = -0.72) and age (r = -0.75), as well as vitamin B12 in the same parameters (r = -0.67 and r = -0, 68). A positive correlation of phenylalanine with body weight and age (r = 0.62 and r = 0.66) was observed. CONCLUSION: Most patients presented adequate according to anthropometric parameters and appropriate biochemical tests, except HDL, and moderate metabolic control of the disease. However, attention should be paid to the presence of overweight and need for biochemical monitoring of triglycerides, selenium, zinc, HDL, and phenylalanine.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Fenilcetonúrias Tipo de estudo: Diagnostic_studies / Observational_studies / Risk_factors_studies Limite: Adolescent / Adult / Child / Child, preschool / Humans Idioma: En Revista: Clin Nutr ESPEN Ano de publicação: 2020 Tipo de documento: Article País de afiliação: Brasil País de publicação: Reino Unido

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Fenilcetonúrias Tipo de estudo: Diagnostic_studies / Observational_studies / Risk_factors_studies Limite: Adolescent / Adult / Child / Child, preschool / Humans Idioma: En Revista: Clin Nutr ESPEN Ano de publicação: 2020 Tipo de documento: Article País de afiliação: Brasil País de publicação: Reino Unido