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2018, Number S1

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Acta Pediatr Mex 2018; 39 (S1)

Which aspects should be considered when starting with the Phenylketonuria diet?

López-Mejía L, Vergara-Vázquez M, Guillén-López S

Language: Spanish
References: 29
Page: 66-74
PDF size: 331.14 Kb.


ABSTRACT

Classical phenylketonuria (PKU) is a genetic disease of the phenylalanine (PHE) metabolism, which is hindered by the deficiency of an enzyme called phenylalanine hydroxylase, as a result of this blockage, PHE levels accumulate in the blood and other tissues, causing irreversible damage to the central nervous system. The goal of nutritional support is to provide a specific amount of PHE and at the same time, meeting protein requirements for patients to grow and avoid nutritional deficiencies associated with the elimination of high protein foods, in addition to maintain a proper mental functioning. Nutritional treatment requires the use of a formula without PHE, to provide proteins, vitamins and minerals. In patients with PKU is necessary to offer breastfeeding, however it should not be an exclusive feeding, it needs to be combined with a free PHE formula.


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