1995
DOI: 10.1007/bf00711746
|View full text |Cite
|
Sign up to set email alerts
|

Nutritional value of essential amino acids in the treatment of adults with phenylketonuria

Abstract: ConclusionThe present short‐term N‐balance study in adult PKU patients receiving a mixture of LNAA establishes a limited retention of nitrogen. Whether lysine could be the limiting amino acid remains to be determined by new short‐term N‐balance studies, with lysine added to the LNAA mixture. Therefore, special caution should be taken in the long‐term use in PKU patients of LNAA without any added lysine.

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
2
2
1

Citation Types

0
12
0
1

Year Published

1999
1999
2016
2016

Publication Types

Select...
7

Relationship

0
7

Authors

Journals

citations
Cited by 16 publications
(13 citation statements)
references
References 3 publications
0
12
0
1
Order By: Relevance
“…Referring to animal studies that proved lysine deficiency in hyperphenylalaninemic rats, Huether et al (44) noted that additional lysine supplementation should be considered in PKU treatment. Unfortunately, further data highlighting cerebral Phe concentrations or evaluations of brain function were not reported by Dotrement et al (43). Before use in the present study, identical LNAA supplementation was studied in three other adult control subjects with normal Phe plasma levels.…”
Section: Figurementioning
confidence: 83%
See 1 more Smart Citation
“…Referring to animal studies that proved lysine deficiency in hyperphenylalaninemic rats, Huether et al (44) noted that additional lysine supplementation should be considered in PKU treatment. Unfortunately, further data highlighting cerebral Phe concentrations or evaluations of brain function were not reported by Dotrement et al (43). Before use in the present study, identical LNAA supplementation was studied in three other adult control subjects with normal Phe plasma levels.…”
Section: Figurementioning
confidence: 83%
“…In addition to a Phe-restricted diet, Dotremont et al (43) used a supplement of 0.8 g/kg body weight LNAA mixture. This was well tolerated without adverse effects.…”
Section: Figurementioning
confidence: 99%
“…The first study of LNAA supplementation in the treatment of PKU was conducted using a formula of LNAA without lysine, such as PreKUnil (Dotremont et al 1995). Four patients were treated for one month using a formula with 0.8 g/kg LNAA and a low-protein diet, 0.6 g/kg.…”
Section: Discussionmentioning
confidence: 99%
“…The first study of LNAA supplementation in the treatment of PKU was conducted using formulas of LNAAs without lysine, such as PreKUnil (Dotremont et al 1995). Four patients were treated for one month using a formula with 0.8 g/kg LNAAs and a low-protein diet, 0.6 g/kg.…”
Section: Discussionmentioning
confidence: 99%