Long-term efficacy of 'ready-to-drink' protein substitute in phenylketonuria

Journal of Human Nutrition and Dietetics

Volumn 22, Issue 5, 2009, Pages 422-427

  Gokmen Ozel, H ^a   Macdonald, A ^a   Daly, A ^a   Hall, K ^b   Ryder, L ^c   Chakrapani, A ^b  

^a BIRMINGHAM CHILDREN S HOSPITAL   (United Kingdom)

^b BIRMINGHAM CHILDREN S HOSPITAL NHS FOUNDATION TRUST   (United Kingdom)

^c SELLY OAK HOSPITAL   (United Kingdom)

Author keywords

Liquid protein substitutes; Phenylalanine; Phenylketonuria

Indexed keywords

ALBUMIN; BIOCHEMICAL MARKER; CALCIUM; CYANOCOBALAMIN; FERRITIN; HEMOGLOBIN; PHENYLALANINE; SELENIUM; ZINC;

ADOLESCENT; ADULT; ANTHROPOMETRY; ARTICLE; CALCIUM BLOOD LEVEL; CHILD; CLINICAL ARTICLE; CLINICAL EFFECTIVENESS; CLINICAL EVALUATION; DIET SUPPLEMENTATION; FEMALE; HEMATOCRIT; HEMOGLOBIN BLOOD LEVEL; HUMAN; MALE; NUTRIENT CONCENTRATION; PATIENT COMPLIANCE; PHENYLKETONURIA; PROTEIN BLOOD LEVEL; RETROSPECTIVE STUDY; SCHOOL CHILD; SUBSTITUTION THERAPY; THERAPY EFFECT; VITAMIN BLOOD LEVEL; ZINC BLOOD LEVEL;

ADOLESCENT; ADULT; BIOLOGICAL MARKERS; CALCIUM; CHILD; DIETARY PROTEINS; FEMALE; FOOD, FORMULATED; HEMATOCRIT; HEMOGLOBINS; HUMANS; MALE; MIDDLE AGED; PHENYLALANINE; PHENYLKETONURIAS; RETROSPECTIVE STUDIES; SELENIUM; SERUM ALBUMIN; TREATMENT OUTCOME; VITAMIN B 12; YOUNG ADULT;

EID: 70149121924     PISSN: 09523871     EISSN: 1365277X     Source Type: Journal    
DOI: 10.1111/j.1365-277X.2009.00998.x     Document Type: Article

References (13)

1. Darling, G., Mathias, P., O'Regan, M. & Naughten, E. (1992) Serum selenium levels in individuals on PKU diets. J. Inherit. Metab. Dis. 15, 769-773.
2. MacDonald, A. (2000) Diet and compliance in phenylketonuria. Eur. J. Pediatr. 159(Suppl. 2), S136-S141.
3. MacDonald, A., Daly, A., Davies, P., Asplin, D., Hall, S.K., Rylance, G. & Chakrapani, A. (2004a) Protein substitutes for PKU: What's new? J. Inherit. Metab. Dis. 27, 363-371.
4. MacDonald, A., Lilburn, A., Cochrane, B., Davies, P., Daly, A., Asplin, D., Hall, S.K., Cousins, A., Chakrapani, A., Robinson, P. & Lee, P. (2004b) A new, low-volume protein substitute for teenagers and adults with phenylketonuria. J. Inherit. Metab. Dis. 27, 127-135.
5. MacDonald, A., Lilburn, M., Davies, P., Evans, S., Daly, A., Hall, S.K., Hendriksz, C., Chakrapani, A. & Lee, P. (2006) 'Ready to drink' protein substitute is easier is for people with phenylketonuria. J. Inherit. Metab. Dis. 29, 526-531.
6. MacDonald, A., Lee, P., Davies, P., Daly, A., Lilburn, M., Gokmen Ozel, H., Preece, M.A., Hendriksz, C. & Chakrapani, A. (2008) Long-term compliance with a novel vitamin and mineral supplement in older people with PKU. J. Inherit. Metab. Dis. 31, 718-723.
7. Medical Research Council (MRC) Working Party on Phenylketonuria (1993) Recommendations on the dietary management of phenylketonuria. Arch. Dis. Child. 68, 426-427.
8. Meli, C. & Bianca, S. (2002) Dietary control of phenylketonuria (correspondence). Lancet 360, 2075-2076.
9. Mundy, H., Lilburn, M., Cousins, A. & Lee, P. (2002) Dietary control of phenylketonuria [correspondence]. Lancet 360, 2076.
10. Prince, A.P., McMurry, M.P. & Buist, N.R.M. (1997) Treatment products and approaches for phenylketonuria: Improved palatability and flexibility demonstrate safety, efficacy and acceptance in US clinical trials. J. Inherit. Metab. Dis. 20, 486-498.
11. Strain, J.J. & Cashman, K.D. (2002) Minerals and trace elements. In Introduction to Human Nutrition. eds M.J. Gibney, H.H. Vorster & F.J. Kok, pp. 177-224. Oxford: Blackwell Publishing.
12. Walter, J.H. & White, F.J. (2004) Blood phenylalanine control in adolescents with phenylketonuria. Int. J. Adolesc. Med. Health 16, 41-45.
13. Walter, J.H., White, F.J., Hall, S.K., MacDonald, A., Rylance, G., Boneh, A., Francis, D.E., Shortland, G.J., Schmidth, M. & Vail, A. (2002) How practical recommendations for dietary control in phenylketonuria? Lancet 360, 55-57.