Title
The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria
Author
Hoeksma, M.
van Rijn, M.
Verkerk, P.H.
Bosch, A.M.
Mulder, M.F.
de Klerk, J.B.C.
de Koning, T.J.
Rubio-Gozalbo, E.
de Vries, M.
Sauer, P.J.J.
van Spronsen, F.J.
TNO Preventie en Gezondheid
Publication year
2005
Abstract
In a previous study, Dutch children with phenylketonuria (PKU) were found to be slightly shorter than their healthy counterparts. In the literature, it has been hypothesized that a higher protein intake is necessary to optimize growth in PKU patients. The study aimed to investigate whether protein intake (total, natural and protein substitute) in this group might be an explanatory factor for the observed growth. Growth of height and head circumference and dietary data on protein intake (total, natural and protein substitute) from 174 Dutch PKU patients born between 1974 and 1996 were analysed retrospectively for the patients' first 3 years of life. Analyses were corrected for energy intake during the first year of life and for the clinical severity of the deficiency of phenylalanine hydroxylase by means of plasma phenylalanine concentration at birth. Neither protein nor energy intake correlated with height growth. A positive, statistically significant relation between head circumference growth and natural protein and total protein intake was found, but not with the intake of the protein substitute or energy. Therefore, this study suggests that improvement of the protein substitute rather than an increase of total protein intake may be important in optimizing head circumference growth in PKU patients. © SSIEM and Springer 2005. Chemicals / CAS: phenylalanine 4 monooxygenase, 9029-73-6; phenylalanine, 3617-44-5, 63-91-2; protein, 67254-75-5; Dietary Proteins; Phenylalanine, 63-91-2; Proteins
Subject
Phenylalanine 4 monooxygenase
Amino acid blood level
Caloric intake
Child growth
Controlled study
Correlation function
Data analysis
Dietary intake
Disease severity
Head circumference
Major clinical study
Preschool child
Protein intake
Retrospective study
Statistical significance
Body Height
Cephalometry
Child, Preschool
Dietary Proteins
Energy Intake
Growth
Head
Humans
Infant
Infant, Newborn
Models, Statistical
Netherlands
Nutritional Requirements
Phenylalanine
Phenylketonurias
Proteins
Regression Analysis
Retrospective Studies
Time Factors
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DOI
https://doi.org/10.1007/s10545-005-0122-x
TNO identifier
238853
ISSN
0141-8955
Source
Journal of Inherited Metabolic Disease, 28 (6), 845-854
Document type
article