| Title |
Large neutral amino acids in the treatment of PKU: from theory to practice
|
|---|---|
| Published in |
Journal of Inherited Metabolic Disease, October 2010
|
| DOI | 10.1007/s10545-010-9216-1 |
| Pubmed ID | |
| Authors |
Francjan J. van Spronsen, Martijn J. de Groot, Marieke Hoeksma, Dirk‐Jan Reijngoud, Margreet van Rijn |
| Abstract |
Notwithstanding the success of the traditional dietary phenylalanine restriction treatment in phenylketonuria (PKU), the use of large neutral amino acid (LNAA) supplementation rather than phenylalanine restriction has been suggested. This treatment modality deserves attention as it might improve cognitive outcome and quality of life in patients with PKU. Following various theories about the pathogenesis of cognitive dysfunction in PKU, LNAA supplementation may have multiple treatment targets: a specific reduction in brain phenylalanine concentrations, a reduction in blood (and consequently brain) phenylalanine concentrations, an increase in brain neurotransmitter concentrations, and an increase in brain essential amino acid concentrations. These treatment targets imply different treatment regimes. This review summarizes the treatment targets and the treatment regimens of LNAA supplementation and discusses the differences in LNAA intake between the classical dietary phenylalanine-restricted diet and several LNAA treatment forms. |
Mendeley readers
The data shown below were compiled from readership statistics for 112 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Netherlands | 1 | <1% |
| Unknown | 111 | 99% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Bachelor | 17 | 15% |
| Researcher | 15 | 13% |
| Student > Ph. D. Student | 11 | 10% |
| Student > Master | 11 | 10% |
| Student > Postgraduate | 10 | 9% |
| Other | 21 | 19% |
| Unknown | 27 | 24% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 20 | 18% |
| Biochemistry, Genetics and Molecular Biology | 17 | 15% |
| Agricultural and Biological Sciences | 16 | 14% |
| Nursing and Health Professions | 10 | 9% |
| Chemistry | 6 | 5% |
| Other | 15 | 13% |
| Unknown | 28 | 25% |
Attention Score in Context
This research output has an Altmetric Attention Score of 12. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 18 July 2023.
All research outputs
#2,532,934
of 23,306,612 outputs
Outputs from Journal of Inherited Metabolic Disease
#111
of 1,880 outputs
Outputs of similar age
#10,164
of 100,599 outputs
Outputs of similar age from Journal of Inherited Metabolic Disease
#4
of 17 outputs
Altmetric has tracked 23,306,612 research outputs across all sources so far. Compared to these this one has done well and is in the 88th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 1,880 research outputs from this source. They receive a mean Attention Score of 4.7. This one has done particularly well, scoring higher than 94% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 100,599 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 89% of its contemporaries.
We're also able to compare this research output to 17 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 76% of its contemporaries.