Fig. 3
From: Management of late onset urea cycle disorders—a remaining challenge for the intensivist?
Distribution of different anomalies in the urea cycle. CPS1D: Carbamyl phosphate synthetase 1 deficiency, OTCD: Ornithine transcarbamylase deficiency, HHH: Hyperornithinemia, hyperammonemia, homocitrullinuria (HHH) syndrome; ORNT1D mitochondrial ornithine transporter 1 deficiency, CITRIN: Citrullinemia type II (mitochondrial aspartate/glutamate carrier deficiency