TY - JOUR AU - Molema, Femke AU - Gleich, Florian AU - Burgard, Peter AU - van-der-Ploeg, Ans T AU - Summar, Marshall L AU - Chapman, Kimberly A AU - Baric, Ivo AU - Lund, Allan M AU - Kölker, Stefan AU - Williams, Monique PY - 2019 DO - 10.1002/jimd.12066 UR - http://hdl.handle.net/10668/13535 T2 - Journal of inherited metabolic disease AB - Organic acidurias (OAD) and urea-cycle disorders (UCD) are rare inherited disorders affecting amino acid and protein metabolism. As dietary practice varies widely, we assessed their long-term prescribed dietary treatment against published guideline... LA - en PB - Wiley KW - L-citrulline and L-arginine KW - amino acid mixtures KW - branched-chain amino acids KW - dietary and supplemental treatment KW - organic acidurias KW - urea-cycle disorders KW - Adolescent KW - Adult KW - Amino Acid Metabolism, Inborn Errors KW - Amino Acids KW - Child KW - Child, Preschool KW - Cross-Sectional Studies KW - Dietary Supplements KW - Europe KW - Feasibility Studies KW - Female KW - Humans KW - Hyperammonemia KW - Infant KW - Male KW - Ornithine KW - Propionic Acidemia KW - Registries KW - Retrospective Studies KW - Treatment Outcome KW - Urea Cycle Disorders, Inborn KW - Young Adult TI - Evaluation of dietary treatment and amino acid supplementation in organic acidurias and urea-cycle disorders: On the basis of information from a European multicenter registry. TY - research article VL - 42 ER -