caa a22 4500 475785851 CHVBK 20180406123658.0 cr unu---uuuuu 170329e20000901xx s 000 0 eng 10.1007/PL00014375 doi (NATIONALLICENCE)springer-10.1007/PL00014375 MacDonald Anita Dietetic Services, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK e-mail: anita.macdonald@bhamchildrens.wmids.nhs.uk Tel.: +44-121-3338020; Fax: +44-121-3338021, GB aut Diet and compliance in phenylketonuria [Elektronische Daten] [Anita MacDonald] In phenylketonuria, compliance and diet is a difficult issue; it is hard to quantify and is under-researched. It is influenced by many factors. Failure to consume prescribed quantity of protein substitute has been commonly reported and is probably affected by their acceptability, format and timing of administration. There are few reports documenting actual phenylalanine intake and blood phenylalanine control, but it is possible that the more rigorous systems for allocation of phenylalanine are associated with worse compliance. The dry, hard and insipid nature of many low protein products may lead to their under usage and consequent boredom and hunger. The diet becomes increasingly harder to maintain as children grow older and seek fewer constraints in their meals. Conclusion In phenylketonuria, encouraging adherence to diet requires continual education, reinforcement and support from the family and professionals within the support team. Springer-Verlag Berlin Heidelberg, 2000 Key words Compliance nationallicence Exchange system nationallicence Phenylalanine nationallicence Protein substitute nationallicence AbbreviationsPhe phenylalanine nationallicence PKU phenylketonuria nationallicence https://doi.org/10.1007/PL00014375 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/PL00014375 text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- MacDonald Anita Dietetic Services, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK e-mail: anita.macdonald@bhamchildrens.wmids.nhs.uk Tel.: +44-121-3338020; Fax: +44-121-3338021, GB aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer