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The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria

Hoeksma, M. ; van Rijn, M. ; Verkerk, P. H. ; Bosch, A. M. ; Mulder, M. F. ; de Klerk, J. B.C. ; de Koning, T. J. LU ; Rubio-Gozalbo, E. ; de Vries, M. and Sauer, P. J.J. , et al. (2005) In Journal of Inherited Metabolic Disease 28(6). p.845-854
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... (More)

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.

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publishing date
type
Contribution to journal
publication status
published
subject
in
Journal of Inherited Metabolic Disease
volume
28
issue
6
pages
10 pages
publisher
Springer
external identifiers
  • pmid:16435176
  • scopus:31644433344
ISSN
0141-8955
DOI
10.1007/s10545-005-0122-x
language
English
LU publication?
no
id
0e822c99-8f27-42b7-9c4f-e6fc3a8ac253
date added to LUP
2020-03-03 19:04:25
date last changed
2020-04-07 05:44:18
@article{0e822c99-8f27-42b7-9c4f-e6fc3a8ac253,
  abstract     = {<p>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.</p>},
  author       = {Hoeksma, M. and van Rijn, M. and Verkerk, P. H. and Bosch, A. M. and Mulder, M. F. and de Klerk, J. B.C. and de Koning, T. J. and Rubio-Gozalbo, E. and de Vries, M. and Sauer, P. J.J. and van Spronsen, F. J.},
  issn         = {0141-8955},
  language     = {eng},
  month        = {12},
  number       = {6},
  pages        = {845--854},
  publisher    = {Springer},
  series       = {Journal of Inherited Metabolic Disease},
  title        = {The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria},
  url          = {http://dx.doi.org/10.1007/s10545-005-0122-x},
  doi          = {10.1007/s10545-005-0122-x},
  volume       = {28},
  year         = {2005},
}