title: A new, low-volume protein substitute for teenagers and adults with phenylketonuria.
abstract: 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 8 weeks. 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 <0.001). Median change in energy intake decreased by a median of 10% (95% CI 2.0 to 18.0) when compared to intake on original protein substitute. On PKU Express, the intakes of all nutrients exceeded the dietary reference values but none was excessively high. Blood phenylalanine decreased by a mean of 37 micromol/L (95% CI-27 to 102) during the trial. Body mass index decreased in 40% of subjects. Changes in blood phenylalanine or body mass index were not statistically significant. Most of the nutritional, haematological and biochemical indices stayed within normal reference ranges for the analytes studied. Sixteen (70%) of the subjects had low plasma selenium at the start, but only 13 (57%) at the study end. Plasma vitamin B12 was high in 8 subjects at the start of the study and 9 at the end. Twenty-one subjects (96%) stated that the product was convenient and easy to prepare. However, 7 (32%) described the smell and 9 (46%) the texture as the same as or worse than those of previous protein substitutes. Because of the use of the premeasured sachets, some subjects were able to prepare their own protein substitute for the first time. PKU Express is a safe, efficacious, protein substitute that significantly reduces the daily volume of prescribed protein substitute.
authors.affiliation: The Children's Hospital, Dietetic Department, Steelhouse Lane, Birmingham, UK.
authors.forename: A
authors.initials: A
authors.lastname: MacDonald
authors.forename: M
authors.initials: M
authors.lastname: Lilburn
authors.forename: B
authors.initials: B
authors.lastname: Cochrane
authors.forename: P
authors.initials: P
authors.lastname: Davies
authors.forename: A
authors.initials: A
authors.lastname: Daly
authors.forename: D
authors.initials: D
authors.lastname: Asplin
authors.forename: S K
authors.initials: SK
authors.lastname: Hall
authors.forename: A
authors.initials: A
authors.lastname: Cousins
authors.forename: A
authors.initials: A
authors.lastname: Chakrapani
authors.forename: P
authors.initials: P
authors.lastname: Robinson
authors.forename: P
authors.initials: P
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D001827:Body Height; D001835:Body Weight; D002648:Child; D019587:Dietary Supplements; D004573:Electrolytes / Q000097:blood; D005260:Female; D006454:Hemoglobins; D006801:Humans; D008297:Male; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000097:blood / Q000178:diet therapy* / Q000188:drug therapy*; D011446:Prospective Studies; D011506:Proteins / Q000008:administration & dosage*; D014131:Trace Elements / Q000097:blood; D014815:Vitamins / Q000097:blood
chemical_list: D004573:Electrolytes; D006454:Hemoglobins; D011506:Proteins; D014131:Trace Elements; D014815:Vitamins; D010649:Phenylalanine