Table 2.

Ammonia, glutamine and citrulline concentrations (in μmol/L) in blood samples from patients with CPS I, OTC and ASS deficiencies

	CPS I <6 years n = 3	CPS I >6 years n = 6	OTC <6 years n = 50	OTC >6 years n = 29	ASS <6 years n = 8	ASS >6 years n = 0
Ammonia	Median value = 86; range = 37–231; IQR = 62–159	Median value = 131; range = 17–171; IQR = 67–154	Median value = 172; range = 23–479; IQR = 109–241	Median value = 167; range = 38–389; IQR = 93–243	Median value = 209; range = 48–330; IQR = 175–286	No samples
Glutamine	Median value = 780; range = 662–933; IQR = 721–857	Median value = 846; range= 607–920.9; IQR = 710–897	Median value = 1061; range = 688–1658; IQR = 919–1,250	Median value = 1095; range = 723–2,015; IQR = 997–1,250	Median value= 917; range = 320–1,299; IQR = 553–1,050	No samples
Citrulline	Median value = 7; range= 4–9; IQR = 6–8	Median value = 7; range= 4–263; IQR = 5–167	Median value = 26; range=7–497; IQR = 14–66	Median value = 32; range= 5–578; IQR = 13–69	Median value = 3,297; range = 1,330–15,433; IQR = 2,300–4,090	No samples

IQR Interquartile range