Fig. 4
From: Management of late onset urea cycle disorders—a remaining challenge for the intensivist?
diagnostic algorythm for late onset hyperammonemia. In case of hyperammonemia, the levels of citrulin and urinary orotic acid makes it possible to direct towards a specific analysis group leading to the final diagnosis. Unless indicated, with (U) to indicate urine. Ala: alanine, Arg: arginine, ARG1: arginase 1, 3-MGA: 3-methylglutaconic acid, NAGS: N-acetylglutamate synthase, CPS: carbamoylphosphate synthetase, Orn: ornithine, Pro: proline, P5CS: pyrroline-5-carboxyl synthetase, HHH: hyperornithinemia-hyperammonemia-homocitrullinuria, OAT: ornithine aminotransferase, OTC: ornithine transcarbamylase, Lys: lysine, LPI: lysinurix protein intolerance, ASA: arginosuccinic acid, ASS: arginosuccinate synthetase