biostudies-literaturePosset RIntellectual and Developmental Disabilities Research CenterNational Center for Advancing Translational SciencesEunice Kennedy Shriver National Institute of Child Health and Human DevelopmentNICHD NIH HHSKettering FundNational Institute of Diabetes and Digestive and Kidney DiseasesNational Institutes of HealthEuropean Commission108112https://www.ebi.ac.uk/biostudies/studies/S-EPMC12812261biostudies-literatureUnknown141(3)<h4>Objective</h4>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.<h4>Methods</h4>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 coMolecular genetics and metabolismImpact of supplementation with L-citrulline/arginine after liver transplantation in individuals with Urea Cycle Disorders.PMC12812261U54HD090257P50 HD103555U54HD061221U54 HD090257U54 HD061221Harding COSchulze ASeminara JLam CCoughlin CRStricker TKolker SUrea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD) consortia study groupKonczal LBurrage LCGallagher RCBreilyn MGleich FHoffmann GFFicicioglu CWong DGarbade SFBerry SADruck ACPosset RRamdhouni NZielonka MMew NAGropman ALGarcia-Cazorla AMcCandless SEDobbelaere DLawrence Merritt JLindner MEpp FBaumgartner MRNagamani SCSLe Mons CWilkening GEnns GMBerry GTDiaz GAfalseImpact of supplementation with L-citrulline/arginine after liver transplantation in individuals with Urea Cycle Disorders.<h4>Objective</h4>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.<h4>Methods</h4>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 co2024-01-01T00:00:00Z2024 Mar2026-06-06T15:57:38.17Z2026-06-02T03:10:42.266ZS-EPMC128122613830153010.1016/j.ymgme.2023.108112