Metodología de atención de niños con fenilcetonuria (FC) y enfermedad de la orina con olor a miel de arce (EOMA) / Methodology of care for children with phenylketonuria and maple-syrup urine disease

ABSTRACT

Maple Syrup Urine Disease (MUSD) and Phenylketonuria (PKU), are two metabolic disease in which the nutritional management are essential. Nevertheless, in Costa Rica and the rest of Central America, the dietetical attention of the children with this illness aren't normalized. These work was developed to fill this necessity. The nutritional management was separate in three steps: 1-Initial diet is the feeding that the children must receive while the blood amino acid level falls, 2-Stabilization diet is the one where the requirement of limits amino acid are defined, 3-Follow up diet this is the diet that is prescribed for the rest of his life. This attention methodology, was very important to an adequate physical and neurological development of the children with PKU and MSUD.