Factors affecting the variation in plasma phenylalanine in patients with phenylketonuria on diet

Archives of Disease in Childhood

Volumn 74, Issue 3 SUPPL., 1996, Pages 412-417

  MacDonald, A ^a,c   Rylance, G ^a   Hall, S K ^a   Asplin, D ^a   Booth, I W ^b  

^a BIRMINGHAM CHILDREN S HOSPITAL   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c Department of Dietetic Services ^*

Author keywords

Diet; Phenylketonuria; Protein substitute

Indexed keywords

PHENYLALANINE;

ADOLESCENT; AMINO ACID BLOOD LEVEL; ARTICLE; CALORIC INTAKE; CHILD; CLINICAL ARTICLE; DIET; FEMALE; FOOD INTAKE; HUMAN; MALE; PHENYLKETONURIA; PRIORITY JOURNAL; PROTEIN INTAKE; RISK FACTOR;

EID: 0030048431     PISSN: 00039888     EISSN: 14682044     Source Type: Journal    
DOI: 10.1136/adc.74.5.412     Document Type: Article

References (26)

1. Report of Medical Research Council Working Party on Phenylketonuria. Phenylketonuria due to phenylalanine hydroxylase deficiency: an unfolding story. BMJ 1993; 306: 115-9.
2. Smith I, Beasley M, Ades AE. Intelligence and quality of dietary treatment in phenylketonuria. Arch Dis Child 1990; 65: 472-8.
3. Welsh MC, Pennington BF, Ozonoff S, Rouse B, McCabe ERB. Neuropsychology of early treated phenylketonuria: specific executive function deficits. Child Dev 1990; 61: 1697-713.
4. Lou HC, Guttler G, Lykkelund C, Bruhn P, Niederweiser A. Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment of phenylketonuria in adolescents. Eur J Pediatr 1985; 144: 17-20.
5. Pennington BF, van B Doorninck WJ, McCabe LL, McCabe ER. Neurological deficits in early-treated phenylketonurics. Am J Ment Deficiency 1985; 89: 467-74.
6. de Sonnerville LMJ, Schmidt E, Michel U, Batzier U. Preliminary neuropsychological test results. Eur J Pediatr 1990; 149 (suppl 1): S39-44.
7. Smith I, Beasley ME, Wolff OH, Ades AE. Behavior disturbance in 8 year old children with early treated phenylketonuria. J Pediatr 1988; 112: 403-8.
8. Melnick CR, Michals KK, Matalon R. Linguistic development of children with phenylketonuria and normal intelligence. J Pediatr 1981; 98: 269-72.
9. Thompson AJ, Smith I, Brenton D, et al. Neurological deterioration in young adults with phenylketonuria. Lancet 1990; 336: 602-5.
10. Bick U, Fahrendorf G, Ludolph AC, Vassallo P, Weglage J, Ullrich K. Disturbed myelination in patients with treated hyperphenylalaninemia: evaluation with magnetic resonance imaging. Eur J Pediatr 1991; 150: 185-9.
11. Thompson AJ, Tillotson S, Smith I, Kendall B, Moore SG, Brenton DP. Brain MRI changes in phenylketonuria. Brain 1993; 116: 811-21.
12. Report of Medical Research Council Working Party on Phenylketonuria. Recommendations on the dietary management of phenylketonuria. Arch Dis Child 1993; 68: 426-7.
13. MacDonald A. Diet and phenylketonuria: time for change? J Hum Nutr Diet 1994; 7: 105-14.
14. Holland B, Welch AA, Unwin ID, Buss DH, Paul AA, Southgate DAT. McCance and Widdowson's 'The composition of foods', 5th ed. Cambridge: Royal Society of Chemistry and Ministry of Agriculture, Fisheries and Food, 1991.
15. Holland B, Unwin ID, Buss DH. Fruit and nuts. Cambridge: Royal Society of Chemistry and Ministry of Agriculture, Fisheries and Food, 1991.
16. Holland B, Unwin ID, Buss DH. Vegetables, herbs and spices. Cambridge: Royal Society of Chemistry and Ministry of Agriculture, Fisheries and Food, 1991.
17. Holland B, Unwin ID, Buss DH. Milk products and eggs. Cambridge: Royal Society of Chemistry and Ministry of Agriculture, Fisheries and Food, 1989.
18. Holland B, Unwin ID, Buss DH. Cereals and cereal products. Cambridge: Royal Society of Chemistry and Ministry of Agriculture, Fisheries and Food, 1988.
19. Department of Health. Dietary reference values for food, energy and nutrients for the Uniled Kingdom. Report on Health and Social Subjects No 41. London: HMSO, 1991.
20. Atherton ND, Green A. HPLC measurement of phenylalanine in plasma. Clin Chem 1988; 34: 2241-4.
21. Güttler F, Oleson ES, Wamberg E. Inverse diurnal variations of serum phenylalanine and tyrosine in phenylketonuric children on low-phenylalanine diet. In: Allan JD, Holt KS, Ireland JT, Politt KJ, eds. Enzymopenic anaemias, lysosomes and other papers. Proceedings of the Sixth Symposium of the Society for the Study of Inborn Errors of Metabolism. Edinburgh: Livingstone, 1969: 149-58.
22. Farquhar DL, Steven F, Westwood A. Preliminary report on inverse diurnal variation of phenylalanine: implications in maternal phenylketonuria. Hum Nutr Appl Nutr 1985; 39A: 224-6.
23. van Spronsen FJ, van Rijn M, van Dijk T, et al. Plasma phenylalanine and tyrosine responses to different nutritional conditions (fasting/postprandial) in patients with phenylketonuria: effect of sample timing. Pediatrics 1993; 92: 570-3.
24. Gropper SS, Gropper DM, Acosta PB. Plasma amino acid responses to ingestion of L-amino acids and whole protein. J Pediatr Gastroenterol Nutr 1993; 16: 143-50.
25. Hanley WB, Linsao L, Davidson W, Moes CAF. Malnutrition with early treatment of phenylketonuria. Pediatr Res 1970; 4: 318-27.
26. Smith BA, Waisman HA. Adequate phenylalanine intake for optimum growth and development in the treatment of phenylketonuria. Am J Clin Nutr 1971; 24: 423-31.