title: 'Ready to drink' protein substitute is easier is for people with phenylketonuria.
abstract: In phenylketonuria (PKU), compliance with taking protein substitute is an issue in teenage and older patients. A 'ready to drink' protein substitute may overcome many of the practical issues associated with its administration.
  
  
  OBJECTIVE
  To investigate the efficacy of a liquid protein substitute in a 6-week, three-part, randomized, crossover, controlled study.
  
  
  METHODS
  27 subjects (15 female; 12 male) with PKU with a median age of 30 years (range 8-49 years) were recruited. One subject withdrew from the study. Their median daily dose of protein equivalent was 60 g (range 45-75 g). In parts 1 and 2, subjects were randomized to either a liquid or a powder protein substitute with the same nutritional composition per unit (each 130 ml liquid pouch or 25 g powder sachet contained 15 g protein equivalent). In part 3, subjects chose liquid, powder or a combination of both. Weekly blood phenylalanine (Phe) concentrations were estimated, and during weeks 2, 4 and 6 subjects completed a daily questionnaire on administration issues.
  
  
  RESULTS
  All but one of 26 subjects chose the liquid in part 3 as either their sole (69%, n = 18) or partial source (28%, n = 7) of protein substitute. Blood Phe concentrations were significantly better on the liquid (p = 0.03). With the liquid protein substitute, subjects were less self-consciousness (p = 0.003) and found it easier to take away from home (p = 0.001). Overall, the liquid was easier (p < 0.0001), more convenient (p = 0.002) and resulted in less wastage of protein substitute (p = 0.001).
  
  
  CONCLUSIONS
  Liquid protein substitute was popular and efficacious, reduced self-consciousness and overall improved compliance of teenagers and adults with PKU.
authors.affiliation: Birmingham Children's Hospital, Steelhouse Lane, Birmingham, BH4 6NH, UK. anita.macdonald@bch.nhs.uk
authors.forename: A
authors.initials: A
authors.lastname: MacDonald
authors.forename: M
authors.initials: M
authors.lastname: Lilburn
authors.forename: P
authors.initials: P
authors.lastname: Davies
authors.forename: S
authors.initials: S
authors.lastname: Evans
authors.forename: A
authors.initials: A
authors.lastname: Daly
authors.forename: S K
authors.initials: SK
authors.lastname: Hall
authors.forename: C
authors.initials: C
authors.lastname: Hendriksz
authors.forename: A
authors.initials: A
authors.lastname: Chakrapani
authors.forename: P
authors.initials: P
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D001628:Beverages; D002648:Child; D018592:Cross-Over Studies; D004032:Diet; D019587:Dietary Supplements; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010649:Phenylalanine / Q000097:blood* / Q000737:chemistry; D010661:Phenylketonurias / Q000628:therapy*; D011506:Proteins / Q000737:chemistry; D014443:Tyrosine / Q000097:blood* / Q000737:chemistry
chemical_list: D011506:Proteins; D014443:Tyrosine; D010649:Phenylalanine