Calcium metabolism in phenilketonuria

• Autor(a) principal: Ferreira MARTINS, Fabiana
• Data de Publicação: 2023
• Outros Autores: Barcellos MENDES, Andréa, de Souza CRUZ, Wanise Maria, Teles BOAVENTURA, Gilson
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Revista de Nutrição
• Texto Completo: https://periodicos.puc-campinas.edu.br/nutricao/article/view/9494
• topic: Phenylketonuria; Bone density; Calcium metabolism; Densidade óssea; Fenilcetonúria; Metabolismo de cálcio

Phenylketonuria is an inborn error of the metabolism of the amino acid phenylalanine. The treatment is essentially dietetic and involves a severe restriction in the consumption of foods containing aminoacid phenylalanine. Although the diet is complemented with supplements in order to meet the vitamin, mineral and essential amino acid requirements, nutritional deficiencies still occur. This is mainly due to restricting the consumption of protein sources, which results in low intake of several nutrients, including calcium. Calcium is strongly related to bone mineral formation. Recent studies have demonstrated that patients withphenylketonuria often present osteopenia and fractures, the greatest incidence being in children older than 8 years. Rapid growth, a calcium-deficient diet and elevated aminoacid phenylalanine plasma levels have been described as the principal factors responsible for inadequate bone formation. It has been shown that calcium-supplementation in healthy children had a positive effect on bone mass acquisition during prepuberty. Therefore, it is pertinent to understand the need of calcium-supplementation in phenylketonuria in order to promote full bone development.