METABOLIC CONTROL AND BODY COMPOSITION OF CHILDREN AND ADOLESCENTS WITH PHENYLKETONURIA

Autor: Zeni Drubi Nogueira, Ney Boa-Sorte, Maria Efigênia de Queiroz Leite, Maria Betânia Pereira Toralles, Tatiana Amorim
Jazyk: English<br />Spanish; Castilian<br />Portuguese
Rok vydání: 2021
Předmět:
Zdroj: Revista Paulista de Pediatria, Vol 39 (2021)
Druh dokumentu: article
ISSN: 1984-0462
DOI: 10.1590/1984-0462/2021/39/2020095
Popis: 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
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