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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 ÎĽmol/L.