PMID: 8942417Dec 1, 1996Paper

Large daily fluctuations in plasma tyrosine in treated patients with phenylketonuria

The American Journal of Clinical Nutrition
F J van SpronsenH S Heymans

Abstract

In patients with phenylketonuria (PKU), extra tyrosine supplementation is advocated in addition to tyrosine-enriched amino acid mixtures. PKU patients have low fasting plasma tyrosine concentrations, but little is known about tyrosine fluctuations during the day. Plasma tyrosine concentrations were studied in 12 PKU patients in response to a test without breakfast and to three tests with different tyrosine contents in breakfast and lunch: 0%/30%, 25%/30%, 50%/10%, and 75%/10% tests, reflecting the protein consumption at breakfast and lunch, respectively. Prolonged fasting resulted in a small decrease in the already low overnight fasting plasma tyrosine concentrations. Breakfast and lunch with 25% and 30% of the daily tyrosine intake resulted in both lower than normal and higher than normal tyrosine concentrations. The 50%/10% and 75%/10% tests resulted in excessively high plasma tyrosine concentrations in most patients. Therefore, both lower than normal and higher than normal postprandial plasma tyrosine concentrations were found in treated PKU patients, even if the daily tyrosine intake was distributed evenly. When there was a large fractional tyrosine intake from one meal, very high plasma tyrosine concentrations were found. ...Continue Reading

Citations

Oct 28, 1999·The American Journal of Clinical Nutrition·R ArtuchJ Campistol
Jan 30, 2020·Hormones : International Journal of Endocrinology and Metabolism·Csaba SumanszkiPeter Reismann
Apr 5, 2020·Orphanet Journal of Rare Diseases·Kimber van VlietM Rebecca Heiner-Fokkema
Apr 15, 2003·European Journal of Pediatrics·Margreet van RijnFrancjan J van Spronsen
Oct 14, 2017·Orphanet Journal of Rare Diseases·A M J van WegbergF J van Spronsen
Oct 5, 2018·Nutrition Research Reviews·Anita MacDonaldFrancjan J van Spronsen
Apr 16, 1999·Journal of the American College of Nutrition·P B AcostaM Korson
Oct 26, 2018·Nutritional Neuroscience·Penelope D Manta-VogliKleopatra H Schulpis
Mar 10, 2007·Journal of Inherited Metabolic Disease·M GiovanniniE Riva
Feb 7, 2001·The American Journal of Clinical Nutrition·F J van SpronsenP G Smit
Jun 7, 2012·Journal of Inherited Metabolic Disease·Raphaël TeissierUNKNOWN AFDPHE (Association Française pour le Dépistage et la Prévention des Handicaps de l’Enfant)
Feb 9, 2000·American Journal of Physiology. Endocrinology and Metabolism·R BrossP B Pencharz
Aug 7, 2001·Journal of Inherited Metabolic Disease·R ArtuchJ Campistol
Feb 14, 2021·Journal of Pediatric Endocrinology & Metabolism : JPEM·Francesco PortaMarco Spada
Mar 27, 2021·Molecular Genetics and Metabolism·Amj van WegbergF J van Spronsen
Jun 13, 2021·Molecular Genetics and Metabolism·Krista ViauFran Rohr

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