Abstract
Background
Changes in circulating plasma amino acids (pAA) may be associated with potential anti-seizure effects of ketogenic
dietary treatment (KDT). We aim to analyze alterations in specific pAA concentrations (alanine (ala), glycine (gly),
glutamine (gln), isoleucine (ile), leucine (leu), valine (val), lysine (lys)) and to investigate the effect of KDT on their
respective changes.
Case Study/Methods
Routinely collected data from 53 patients before and after onset of KDT between 01/01/2010 and 12/31/2020 were
grouped in ten three-month follow-up intervals and analyzed retrospectively. Generalized estimating equations (GEE)
were used to investigate the effect of KDT on changes in pAA concentrations. Metabolites were determined by ion
exchange chromatography with post-column derivatization with ninhydrine (Biochrom 30+, Laborservice Onken,
Germany). β-hydroxybutyric acid was measured via enzymatic essays (Wako Chemicals, Germany).
Results
We report specific alterations in pAA in 53 patients (26/49% male, 27/51% female) undergoing KDT (10/19% classical KDT,
43/81% modified Atkins diet) due to structural anomaly (n=6/11%), metabolic disease (n=7/13%), genetic disease
(n=11/21%), epileptic syndrome (n=26/49%) and unclear primary diagnosis (n=3/6%). KDT led to ketosis, and tests of
model effects from GEE show a significant effect of KDT on ile, leu, val, lys, gly, ala and gln (p<0.001) over the subsequent
follow-up measurements. Effects of pairwise comparison were mainly significant for ile, leu, val, ala and gln.
Discussion/Conclusion
In our cohort, a significant effect of KDT on specific pAA was shown. These metabolic adaptions may be linked to
stabilizing mechanisms of action in patients with refractory epilepsy and inherited metabolic disease. As the mechanistic
effects of KDT are not yet fully understood, further investigations in prospective randomized trials comprising complete
metabolomic assessment in addition to classical clinical endpoints, coupled with causal mediation analysis are needed.
Changes in circulating plasma amino acids (pAA) may be associated with potential anti-seizure effects of ketogenic
dietary treatment (KDT). We aim to analyze alterations in specific pAA concentrations (alanine (ala), glycine (gly),
glutamine (gln), isoleucine (ile), leucine (leu), valine (val), lysine (lys)) and to investigate the effect of KDT on their
respective changes.
Case Study/Methods
Routinely collected data from 53 patients before and after onset of KDT between 01/01/2010 and 12/31/2020 were
grouped in ten three-month follow-up intervals and analyzed retrospectively. Generalized estimating equations (GEE)
were used to investigate the effect of KDT on changes in pAA concentrations. Metabolites were determined by ion
exchange chromatography with post-column derivatization with ninhydrine (Biochrom 30+, Laborservice Onken,
Germany). β-hydroxybutyric acid was measured via enzymatic essays (Wako Chemicals, Germany).
Results
We report specific alterations in pAA in 53 patients (26/49% male, 27/51% female) undergoing KDT (10/19% classical KDT,
43/81% modified Atkins diet) due to structural anomaly (n=6/11%), metabolic disease (n=7/13%), genetic disease
(n=11/21%), epileptic syndrome (n=26/49%) and unclear primary diagnosis (n=3/6%). KDT led to ketosis, and tests of
model effects from GEE show a significant effect of KDT on ile, leu, val, lys, gly, ala and gln (p<0.001) over the subsequent
follow-up measurements. Effects of pairwise comparison were mainly significant for ile, leu, val, ala and gln.
Discussion/Conclusion
In our cohort, a significant effect of KDT on specific pAA was shown. These metabolic adaptions may be linked to
stabilizing mechanisms of action in patients with refractory epilepsy and inherited metabolic disease. As the mechanistic
effects of KDT are not yet fully understood, further investigations in prospective randomized trials comprising complete
metabolomic assessment in addition to classical clinical endpoints, coupled with causal mediation analysis are needed.
Originalsprache | Englisch |
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Seiten | 130-130 |
Seitenumfang | 1 |
Publikationsstatus | Veröffentlicht - 20 Aug. 2024 |
Veranstaltung | Annual Symposium of the Society for the Study of Inborn Errors of Metabolism 2024 - Porto, Porto, Portugal Dauer: 3 Sept. 2024 → 6 Sept. 2024 https://www.ssiem2024.org/ |
Konferenz
Konferenz | Annual Symposium of the Society for the Study of Inborn Errors of Metabolism 2024 |
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Kurztitel | SSIEM |
Land/Gebiet | Portugal |
Stadt | Porto |
Zeitraum | 3/09/24 → 6/09/24 |
Internetadresse |
Research Field
- Exploration of Digital Health