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Metabolic control and body composition of children and adolescents with phenylketonuria / Controle metabólico e composição corporal de crianças e adolescentes com diagnóstico neonatal de fenilcetonúria
Nogueira, Zeni Drubi; Boa-Sorte, Ney; Leite, Maria Efigênia de Queiroz; Toralles, Maria Betânia Pereira; Amorim, Tatiana.
  • Nogueira, Zeni Drubi; Universidade Federal da Bahia. Salvador. BR
  • Boa-Sorte, Ney; Universidade do Estado da Bahia. Salvador. BR
  • Leite, Maria Efigênia de Queiroz; Universidade Federal da Bahia. Salvador. BR
  • Toralles, Maria Betânia Pereira; Universidade Federal da Bahia. Salvador. BR
  • Amorim, Tatiana; Association of Parents and Friends of Intelectually Disabled Individuals. BR
Rev. Paul. Pediatr. (Ed. Port., Online) ; 39: e2020095, 2021. tab, graf
Article in English, Portuguese | LILACS | ID: biblio-1155476
ABSTRACT
ABSTRACT

Objective:

To characterize metabolic control and verify whether it has any relation with socioeconomic, demographic, and body composition variables in children and adolescents with phenylketonuria (PKU) diagnosed in the neonatal period.

Methods:

This cohort study collected retrospective data of 53 phenylketonuric children and adolescents. Data on family income, housing, and mother's age and schooling level were collected, and anthropometric measures of body composition and distribution were taken. All dosages of phenylalanine (Phe) from the last five years (2015-2019) were evaluated and classified regarding their adequacy (cutoffs 0-12 years 2-6 mg/dL; 12-19 years 2-10 mg/dL). Adequate metabolic control was considered if ≥7%) of the dosages were within desired ranges.

Results:

The mean (±standard deviation) age in the last year was 10.1±4.6 years. Most of them were under 12 years old (33/53; 62.3%) and had the classic form of the disease (39/53; 73.6%). Better metabolic control was observed among adolescents (68.4 versus 51.4%; p=0.019). Overweight was found in 9/53 (17%) and higher serum Phe levels (p<0.001) were found in this group of patients. Metabolic control with 70% or more Phe level adequacy decreased along with the arm muscle area (AMA) (ptendency=0.042), being 70.0% among those with low reserve (low AMA), and 18.5% among those with excessive reserve (high AMA).

Conclusions:

Adequate metabolic control was observed in most patients. The findings suggest that, in this sample, the levels of phenylalanine may be related to changes in body composition.
RESUMO
RESUMO

Objetivo:

Caracterizar o controle metabólico e verificar se existe relação entre ele, variáveis socioeconômicas, demográficas e composição corporal de crianças e adolescentes com fenilcetonúria (FNC) diagnosticada no período neonatal.

Métodos:

Coorte com coleta retrospectiva de dados de 53 crianças e adolescentes fenilcetonúricos. Foram coletados dados de renda familiar, moradia, idade e escolaridade materna e realizaram-se medidas antropométricas de composição e distribuição corporal. Todas as dosagens de fenilalanina (Fal) dos últimos cinco anos (2015-2019) foram avaliadas e classificadas quanto à adequação (cortes 0-12 anos 2-6 mg/dL; 12-19 anos 2-10 mg/dL). A proporção de dosagens adequadas ≥70% foi considerada como controle metabólico adequado.

Resultados:

A média (±desvio padrão) de idade, no último ano, foi de 10,1±4,6 anos. A maioria tinha menos de 12 anos (33/53; 62,3%) e apresentava a forma clássica da doença (39/53; 73,6%). Observou-se melhor controle metabólico entre os adolescentes (68,4 vs. 51,4%; p=0,019). Excesso de peso foi encontrado em 9/53 (17%) e maiores níveis séricos de Fal foram descritos nesse grupo (p<0,001). O percentual de controle metabólico com 70% ou mais de adequação dos níveis de Fal foi decrescente de acordo com a área muscular do braço (AMB; ptendência=0,042), sendo de 70% entre os de baixa reserva (AMB reduzida) e de 18,5% entre os com excesso (AMB elevada).

Conclusões:

Observou-se controle metabólico adequado na maioria dos avaliados e os achados sugerem que, nesta amostra, os níveis de fenilalanina podem estar relacionados com alterações da composição corporal.
Subject(s)


Full text: Available Index: LILACS (Americas) Main subject: Phenylalanine / Phenylketonurias / Body Composition / Metabolism, Inborn Errors Type of study: Diagnostic study / Etiology study / Incidence study / Observational study / Risk factors Limits: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male / Infant, Newborn Language: English / Portuguese Journal: Rev. Paul. Pediatr. (Ed. Port., Online) Journal subject: Medicina / Patologia Year: 2021 Type: Article Affiliation country: Brazil Institution/Affiliation country: Association of Parents and Friends of Intelectually Disabled Individuals/BR / Universidade Federal da Bahia/BR / Universidade do Estado da Bahia/BR

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Full text: Available Index: LILACS (Americas) Main subject: Phenylalanine / Phenylketonurias / Body Composition / Metabolism, Inborn Errors Type of study: Diagnostic study / Etiology study / Incidence study / Observational study / Risk factors Limits: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male / Infant, Newborn Language: English / Portuguese Journal: Rev. Paul. Pediatr. (Ed. Port., Online) Journal subject: Medicina / Patologia Year: 2021 Type: Article Affiliation country: Brazil Institution/Affiliation country: Association of Parents and Friends of Intelectually Disabled Individuals/BR / Universidade Federal da Bahia/BR / Universidade do Estado da Bahia/BR