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Table 2 Biochemical data (mean ± SD, range, and number of patients) detected in plasma of HHH syndrome patients at diagnosis

From: The hyperornithinemia–hyperammonemia-homocitrullinuria syndrome

Metabolites HHH patients Reference value
Ammonia 215 ± 279 (18–2300) n = 93 <55
Ornithine 575 ± 265 (216–1915) n = 96 30-110
Citrulline 26 ± 14 (1–57) n = 21 10-52
Glutamine 1149 ± 592 (447–3688) n = 43 333-809
Arginine 66 ± 26 (27–126) n = 22 20-112
Lysine 178 ± 177 (35–858) n = 26 80-257
  1. Values are expressed as μmol/L.