caa a22 4500 467933405 CHVBK 20180406152949.0 cr unu---uuuuu 170328e20060801xx s 000 0 eng 10.1007/s10545-006-0234-y doi (NATIONALLICENCE)springer-10.1007/s10545-006-0234-y ‘Ready to drink' protein substitute is easier is for people with phenylketonuria [Elektronische Daten] [A. MacDonald, M. Lilburn M, P. Davies, S. Evans, A. Daly, S. Hall, C. Hendriksz, A. Chakrapani, P. Lee] Summary: 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). Conclusion Liquid protein substitute was popular and efficacious, reduced self-consciousness and overall improved compliance of teenagers and adults with PKU. SSIEM and Springer, 2006 MacDonald A. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut Lilburn M M. The Charles Dent Metabolic Unit, The National Hospital for Neurology and Neurosurgery, London, UK aut Davies P. Institute of Child Health, University of Birmingham, Birmingham, UK aut Evans S. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut Daly A. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut Hall S. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut Hendriksz C. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut Chakrapani A. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut Lee P. The Charles Dent Metabolic Unit, The National Hospital for Neurology and Neurosurgery, London, UK aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/4(2006-08-01), 526-531 0141-8955 29:4<526 2006 29 10545 https://doi.org/10.1007/s10545-006-0234-y text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-006-0234-y text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- MacDonald A. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut NATIONALLICENCE 700 1- Lilburn M M. The Charles Dent Metabolic Unit, The National Hospital for Neurology and Neurosurgery, London, UK aut NATIONALLICENCE 700 1- Davies P. Institute of Child Health, University of Birmingham, Birmingham, UK aut NATIONALLICENCE 700 1- Evans S. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut NATIONALLICENCE 700 1- Daly A. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut NATIONALLICENCE 700 1- Hall S. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut NATIONALLICENCE 700 1- Hendriksz C. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut NATIONALLICENCE 700 1- Chakrapani A. Birmingham Children's Hospital, Steelhouse Lane, BH4 6NH, Birmingham, UK aut NATIONALLICENCE 700 1- Lee P. The Charles Dent Metabolic Unit, The National Hospital for Neurology and Neurosurgery, London, UK aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/4(2006-08-01), 526-531 0141-8955 29:4<526 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer