A new, low‐volume protein substitute for teenagers and adults with phenylketonuria

Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low‐volume (amino acids 72 g/100 g), low‐carbohydrat...

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Bibliographic Details
Published in:Journal of inherited metabolic disease 2004-01, Vol.27 (2), p.127-135
Main Authors: MacDonald, A., Lilburn, M., Cochrane, B., Davies, P., Daly, A., Asplin, D., Hall, S. K., Cousins, A., Chakrapani, A., Robinson, P., Lee, P.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Body Height
Body Weight
Child
Dietary Supplements
Electrolytes - blood
Feeding. Feeding behavior
Female
Fundamental and applied biological sciences. Psychology
Genetics of eukaryotes. Biological and molecular evolution
Hemoglobins
Humans
Male
Molecular and cellular biology
Phenylalanine - blood
Phenylketonurias - blood
Phenylketonurias - diet therapy
Phenylketonurias - drug therapy
Prospective Studies
Proteins - administration & dosage
Trace Elements - blood
Vertebrates: anatomy and physiology, studies on body, several organs or systems
Vitamins - blood
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Summary:Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low‐volume (amino acids 72 g/100 g), low‐carbohydrate, phenylalanine‐free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty‐three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8–37 years) took the substitute for 8weeks. A 3‐day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin‐puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140–180) daily to 100 g (range 100–125) daily (p
ISSN:0141-8955
1573-2665
DOI:10.1023/B:BOLI.0000028784.11080.9b