. Author manuscript; available in PMC: 2016 Jun 21.

Published in final edited form as: Mol Genet Metab. 2015 Nov 11;117(1):27–32. doi: 10.1016/j.ymgme.2015.11.005

Table 1.

Baseline Characteristics for Patients Enrolled in 12-Month Studies (N=100)^*

Gender: (%)
Male	33.0
Female	67.0
Race: (%)
White	81.0
Nonwhite	19.0
Age (years): mean (SD)	19.6 (15.9)
Age group: (%)
Pediatric: < 18 years	49.0
Adult: ≥ 18 years	51.0
Baseline Glutamine (μmol/L): (n=96)
Mean (SD)	740.1 (234.6)
Median (25^th, 75^th percentiles)	704.5 (586.5, 829.5)
Patients with baseline glutamine > 900 μmol/L: %	18.0
Baseline ammonia (μmol/L)
Mean (SD)	28.8 (19.9)
Median (25^th, 75^th percentiles)	28.9 (11.0, 37.5)
Patients with baseline ammonia: %
0–0.49 ULN	39.0
0.5–0.99 ULN	34.0
≥ 1.0 ULN	27.0

Number of HACs
Pre-study	54
During study	27
Patients with ≥1 HAC: %
Pre-study	30.0
During study	19.0

Baseline values for ammonia and glutamine represent the values at the time of enrollment into the glycerol phenylbutyrate clinical trials, at which time patients had been taking sodium phenylbutyrate for months to years. HAC: hyperammonemic crisis; ULN: upper limit of normal