Impact of supplementation with L-citrulline/arginine after liver transplantation in individuals with Urea Cycle Disorders

Medientyp: E-Artikel

Titel: Impact of supplementation with L-citrulline/arginine after liver transplantation in individuals with Urea Cycle Disorders

Beteiligte: Posset, Roland [Verfasser:in]; Garbade, Sven [Verfasser:in]; Gleich, Florian [Verfasser:in]; Nagamani, Sandesh C. S. [Verfasser:in]; Gropman, Andrea L. [Verfasser:in]; Epp, Friederike [Verfasser:in]; Ramdhouni, Nesrine [Verfasser:in]; Druck, Ann-Catrin [Verfasser:in]; Hoffmann, Georg F. [Verfasser:in]; Kölker, Stefan [Verfasser:in]; Zielonka, Matthias [Verfasser:in]; Schulze, Andreas [Verfasser:in]; García-Cazorla, Angeles [Verfasser:in]; Ficicioglu, Can [Verfasser:in]; Harding, Cary O. [Verfasser:in]; Lam, Christina [Verfasser:in]; Coughlin, Curtis R. [Verfasser:in]; Le Mons, Cynthia [Verfasser:in]; Wong, Derek [Verfasser:in]; Dobbelaere, Dries [Verfasser:in]; Diaz, George A. [Verfasser:in]; Berry, Gerard T. [Verfasser:in]; Enns, Gregory M. [Verfasser:in]; Wilkening, Greta [Verfasser:in]; Lawrence Merritt, J. [Verfasser:in]; Seminara, Jennifer [Verfasser:in]; Konczal, Laura [Verfasser:in]; Burrage, Lindsay C. [Verfasser:in]; Breilyn, Margo [Verfasser:in]; Lindner, Martin [Verfasser:in]; Baumgartner, Matthias R. [Verfasser:in]; Mew, Nicholas Ah. [Verfasser:in]; Gallagher, Renata C. [Verfasser:in]; McCandless, Shawn E. [Verfasser:in]; Berry, Susan A. [Verfasser:in]; Stricker, Tamar [Verfasser:in]

Erschienen: March 2024

Sprache: Englisch

DOI: 10.1016/j.ymgme.2023.108112

Identifikator:

Schlagwörter: -IMD ; L-citrulline/arginine supplementation ; Liver transplantation ; UCDC ; Urea cycle disorders

Entstehung:

Anmerkungen: Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) consortia study group: Andreas Schulze, Angeles García-Cazorla, Can Ficicioglu, Cary O. Harding, Christina Lam, Curtis R. Coughlin, Cynthia Le Mons, Derek Wong, Dries Dobbelaere, George A. Diaz, Gerard T. Berry, Gregory M. Enns, Greta Wilkening, J. Lawrence Merritt, Jennifer Seminara, Laura Konczal, Lindsay C. Burrage, Margo Breilyn, Martin Lindner, Matthias R. Baumgartner, Nicholas Ah. Mew, Renata C. Gallagher, Shawn E. McCandless, Susan A. Berry, Tamar Stricker

Beschreibung: 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.

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