Assuntos
Proteínas de Ligação a DNA/genética , Diabetes Mellitus Tipo 2/genética , Nefropatias/genética , Rim/anormalidades , Fatores de Transcrição/genética , Adulto , Criança , Feminino , Fator 1-beta Nuclear de Hepatócito , Humanos , Itália , Nefropatias/cirurgia , Falência Renal Crônica/genética , Transplante de Rim , Masculino , LinhagemRESUMO
BACKGROUND: To assess the acceptability and nutritional adequacy of a new phenylalanine-free amino acid mixture (Phenylade-Dietetic Metabolic Food). METHODS: Twenty PKU patients (aged 2.6-10 years) diagnosed and followed by our Department. The children were given this product for a minimum of 4 and a maximum of 12 months. The clinical control has been carried out before treatment and then every 3 months. The biochemical parameters included: quantitative plasma amino acids, hematologic measurements of nutritional adequacy folic acid and vitamin B12. RESULTS: All patients (except one) found this new product to be an acceptable alternatives as to taste and convenience to the previous low phenylalanine protein substitutes. CONCLUSIONS: Normal growth was maintained.