PMID: 7539309Dec 1, 1994Paper

The phenylalanine response curve in relation to growth and mental development in the first year of life

Acta Paediatrica. Supplement
L W van der SchotR C Sengers

Abstract

Thirty-three children with classical phenylketonuria were treated from 9 to 21 days of age onward. The standard for phenylalanine concentration was 0.2-0.5 mmol/l. During the first years of life phenylalanine concentrations were measured once every two to six weeks. For each child these values were graphically plotted in a so-called phenylalanine response curve. Growth and mental development indices were assessed at about one and two years of age. Measurements for weight, height and head circumference were normal at one month of age, showed a significant decline at the age of one year but were restored for normal values at the age of two. No associations were found with phenylalanine parameters. Mental development indices at one and two years were not different from normal. Significant negative correlations were found between mental development indices at one year and phenylalanine values above 0.5 mmol/l. Changes in mental development indices between the first and second years of life are significantly related to the phenylalanine levels during the second year of life.

References

Dec 1, 1991·Biochemical Medicine and Metabolic Biology·F A Hommes
Jan 1, 1990·European Journal of Pediatrics·U MichelU Batzler
Jan 1, 1989·Journal of Inherited Metabolic Disease·C R Scriver
Jan 1, 1987·European Journal of Pediatrics·R KochK Fishler

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Citations

Jul 14, 2007·Journal of Inherited Metabolic Disease·M HuemerS Stöckler-Ipsiroglu
Mar 10, 2007·Journal of Inherited Metabolic Disease·M GiovanniniE Riva
Dec 1, 1994·Acta Paediatrica. Supplement·I Smith
Oct 25, 2011·Molecular Genetics and Metabolism·M DemirkolJ Walter
Sep 6, 2019·Nutrients·Fatma IlgazAnita MacDonald
Jul 23, 2003·Journal of Inherited Metabolic Disease·D DobbelaereJ P Farriaux
Sep 10, 2021·Pediatric Research·Geertje B LiemburgFrancjan J van Spronsen

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