Protein substitute for children and adults with phenylketonuria
نویسندگان
چکیده
منابع مشابه
Are tablets a practical source of protein substitute in phenylketonuria?
BACKGROUND A phenylalanine-free amino acid based protein substitute is necessary to provide the major source of protein in phenylketonuria (PKU). Protein substitutes in PKU are usually given as drinks. These are unpalatable and compliance is often poor. Tablets containing a suitable mixture of phenylalanine-free amino acids (Aminogran Food Supplement, UCB) are now available. AIMS To compare t...
متن کاملCaring for children with phenylketonuria.
OBJECTIVE To provide an overview of the diagnosis and management of phenylketonuria (PKU) in childhood with an emphasis on aspects relevant to family physicians providing ongoing care. SOURCES OF INFORMATION The author's experience as the clinic physician in a regional pediatric PKU clinic is supplemented with references providing evidence for key points. MAIN MESSAGE While metabolic clinic...
متن کاملNeurological deterioration in young adults with phenylketonuria.
7 patients with phenylketonuria who developed neurological disability in adolescence or early adult life are described. 4 had been diagnosed by routine neonatal screening and started a low phenylalanine diet in infancy. 3 were diagnosed in early childhood because of developmental delay, and then started dietary treatment. Dietary control deteriorated in later years and was withdrawn in mid to l...
متن کاملProblem-Solving Skills in Children with Early Treated Phenylketonuria
Objectives: This study aimed to investigate the level of problem-solving skills in 1-4 year-old children with early treated phenylketonuria (ETPKU). Methods:This analytic, cross-sectional study was conducted on 70 1-4 year-old children referred to phenylketonuria (PKU) clinics that had been diagnosed on screening at birth and had been treated. The measurements were a demographic questionnair...
متن کاملNet protein utilization determined by rat bioassay of a protein hydrolysate and a diet for children with phenylketonuria.
In a previous study (Kindt et al. 1983, 1984) it was assumed that a protein hydrolysate, devoid of phenylalanine, together with intact protein as given to children with phenylketonuria (PKU), was equivalent to egg or milk protein. One group of children was given this 'PKU protein' in amounts corresponding to the Joint FAO/WHO ad hoc Expert Committee (1973) recommendations. The results indicated...
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ژورنال
عنوان ژورنال: Cochrane Database of Systematic Reviews
سال: 2015
ISSN: 1465-1858
DOI: 10.1002/14651858.cd004731.pub4