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Tyrosine supplementation for phenylketonuria

  1. Diana Webster1,*,
  2. Joanne Wildgoose2

Editorial Group: Cochrane Cystic Fibrosis and Genetic Disorders Group

Published Online: 5 JUN 2013

Assessed as up-to-date: 1 MAY 2013

DOI: 10.1002/14651858.CD001507.pub3


How to Cite

Webster D, Wildgoose J. Tyrosine supplementation for phenylketonuria. Cochrane Database of Systematic Reviews 2013, Issue 6. Art. No.: CD001507. DOI: 10.1002/14651858.CD001507.pub3.

Author Information

  1. 1

    Bristol Royal Hospital for Children, Nutrition and Dietetic Department, Bristol, UK

  2. 2

    Bradford Royal Infirmary, Physio Corridor, Level 1, Bradford, UK

*Diana Webster, Nutrition and Dietetic Department, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, UK. Diana.Webster@ubht.nhs.uk.

Publication History

  1. Publication Status: New search for studies and content updated (no change to conclusions)
  2. Published Online: 5 JUN 2013

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References

References to studies included in this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to studies awaiting assessment
  4. Additional references
  5. References to other published versions of this review
Mazzocco 1992 {published data only}
  • Mazzocco MM, Yannicelli S, Nord AM, Van Doorninck W, Davidson-Mundt AJ, Greene CL. Cognition and tyrosine supplementation among school-aged children with phenylketonuria. American Journal of Diseases of Children 1992;146(11):1261-4.
Pietz 1995 {published data only}
  • Pietz J, Landwehr R, Kutscha A, Schmidt H, de Sonneville L, Trefz FK. Effect of high-dose tyrosine supplementation on brain function in adults with phenylketonuria. Journal of Pediatrics 1995;127(6):936-43.
Smith 1998 {published data only}
  • Smith ML, Hanley WB, Clarke JTR, Klim P, Schoonheyt W, Austin V, et al. Randomised controlled trial of tyrosine supplementation on neuropsychological performance in phenylketonuria. Archives of Disease in Childhood 1998;78(2):116-21.

References to studies excluded from this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to studies awaiting assessment
  4. Additional references
  5. References to other published versions of this review
Kalkanoglu 2005 {published data only}
  • Kalkanoglu HS, Ahring KK, Sertkaya D, Moller LB, Romstad A, Mikkelsen I, et al. Behavioural effects of phenylalanine-free amino acid tablet supplementation in intellectually disabled adults with untreated phenylketonuria. Acta Paediatrica 2005;94(9):1218-22.
Lou 1987 {published data only}
Lykkelund 1988 {published data only}
  • Lykkelund C, Nielsen JB, Lou HC, Rasmussen V, Gerdes AM, Christensen E, et al. Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine. European Journal of Pediatrics 1988;148(3):238-45.
MacDonald 2003 {published data only}
  • MacDonald A, Ferguson C, Rylance G, Morris AA, Asplin D, Hall SK, et al. Are tablets a practical source of protein substitute in phenylketonuria?. Archives of Disease in Childhood 2003;88(4):327-9.
Wasser 1992 {published data only}
  • Wasser S, Ettrich KU, Schmidt KD, Selle D, Theile H. Case studies of the effect of tyrosine administration in children with phenylketonuria on cognitive processes [Fallstudien zum einfluss von tyrosingaben bei phenylketonurischen kindern auf kognitive prozesse]. Klinische Padiatrie 1992;204(6):417-21.

References to studies awaiting assessment

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to studies awaiting assessment
  4. Additional references
  5. References to other published versions of this review
Lines 1997 {published data only}

Additional references

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to studies awaiting assessment
  4. Additional references
  5. References to other published versions of this review
Dixon 1994
  • Dixon M. Disorders of amino acid metabolism, organic acidaemias and uria cycle defects. In: Shaw V, Lawson M editor(s). Clinical Paediatric Dietetics. Oxford: Blackwell Science, 1994:177-209.
Elbourne 2002
  • Elbourne DR, Altman DG, Higgins JPT, Curtin F, Worthington HV, Vail A. Meta-analysis involving cross-over trials: methodological issues. International Journal of Epidemiology 2002;31(1):140-9.
Lou 1985
  • Lou HC, Guttler F, Lykkelund C, Bruhn P, Niederwieser A. Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents. European Journal of Pediatrics 1985;144(1):17-20.
MRC1 1993
  • Medical Research Council Working Party on Phenylketonuria. Phenylketonuria due to phenylalanine hydroxylase deficiency: an unfolding story. British Medical Journal 1993;306(6870):115-9.
MRC2 1993
  • Medical Research Council Working Party on Phenylketonuria. Recommendations on the dietary management of phenylketonuria. Archives of Disease in Childhood 1993;68(3):426-7.
Oldendorf 1973
  • Oldendorf WH. Saturation of blood brain barrier transport of amino acids in phenylketonuria. Archives of Neurology 1973;28(1):45-8.
Paine 1957
Schulz 1995
  • Schulz KF, Chalmers I, Hayes RJ, Altman DG. Empirical evidence of bias. Dimensions of methodological quality associated with estimates of treatment effects in controlled trials. Journal of the American Medical Association 1995;273(5):408-12.
Scriver 1995
  • Scriver CR, Kaufman S, Woo SLC. The hyperphenylalaninaemias. In: Scriver CR, Beaudet AL, Sly WS, Valle D editor(s). The Metabolic and Molecular Bases of Inherited Disease. 7th Edition. New York: McGraw-Hill, 1995:1015-76.

References to other published versions of this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to studies awaiting assessment
  4. Additional references
  5. References to other published versions of this review
Webster 2010