Language
English
Publication Date
3-1-2024
Journal
Molecular Genetics and Metabolism
DOI
10.1016/j.ymgme.2023.108112
PMID
38301530
Abstract
Objective: Liver transplantation (LTx) is an intervention when medical management is not sufficiently preventing individuals with urea cycle disorders (UCDs) from the occurrence of hyperammonemic events. Supplementation with L-citrulline/arginine is regularly performed prior to LTx to support ureagenesis and is often continued after the intervention. However, systematic studies assessing the impact of long-term L-citrulline/arginine supplementation in individuals who have undergone LTx is lacking to date.
Methods: Using longitudinal data collected systematically, a comparative analysis was carried out by studying the effects of long-term L-citrulline/arginine supplementation vs. no supplementation on health-related outcome parameters (i.e., anthropometric, neurological, and cognitive outcomes) in individuals with UCDs who have undergone LTx. Altogether, 52 individuals with male ornithine transcarbamylase deficiency, citrullinemia type 1 and argininosuccinic aciduria and a pre-transplant "severe" disease course who have undergone LTx were investigated by using recently established and validated genotype-specific in vitro enzyme activities.
Results: Long-term supplementation of individuals with L-citrulline/arginine who have undergone LTx (n = 16) does neither appear to alter anthropometric nor neurocognitive endpoints when compared to their severity-adjusted counterparts that were not supplemented (n = 36) after LTx with mean observation periods between four to five years. Moreover, supplementation with L-citrulline/arginine was not associated with an increase of disease-specific plasma arithmetic mean values for the respective amino acids when compared to the non-supplemented control cohort.
Conclusion: Although supplementation with L-citrulline/arginine is often continued after LTx, this pilot study does neither identify altered long-term anthropometric or neurocognitive health-related outcomes nor does it find an adequate biochemical response as reflected by the unaltered plasma arithmetic mean values for L-citrulline or L-arginine. Further prospective analyses in larger samples and even longer observation periods will provide more insight into the usefulness of long-term supplementation with L-citrulline/arginine for individuals with UCDs who have undergone LTx.
Keywords
Male, Humans, Citrulline, Arginine, Liver Transplantation, Pilot Projects, Urea Cycle Disorders, Inborn, Dietary Supplements, Urea, E-IMD, L-citrulline/arginine supplementation, Liver transplantation, UCDC, Urea cycle disorders
Published Open-Access
yes
Recommended Citation
Posset, Roland; Garbade, Sven F; Gleich, Florian; et al., "Impact of Supplementation With L-Citrulline/Arginine After Liver Transplantation in Individuals With Urea Cycle Disorders" (2024). Faculty and Staff Publications. 4386.
https://digitalcommons.library.tmc.edu/baylor_docs/4386