Table 3.
Levels of phenylacetate in plasma, accumulated phenylacetylglutamine in urine (PAGNU), PAGNU adjusted to creatinine and percentage of PA eliminated in urine in patients treated with ornithine phenylacetate and placebo.
| PAP
µmol/l Mean (SD) |
PAGNU accumulated mmol Mean (SD) |
PAGN/CreatinineU
mmol/mol Mean (SD) |
% PA eliminated in urine**
Molar ratio*** |
|
|---|---|---|---|---|
| Placebo | OP | Placebo | OP | Placebo | OP | OP | |
| Baseline | 3.5 (7.7) | 2.3 (3.4) | |||
| 12h | 2.2 (3.3) | 89 (52.1)* | 1.7 (1.7) | 13.5 (9.6)* | 325 (287) | 2338 (904)* | 59 |
| 24h | 1.3 (2.3) | 125.4 (91.9)* | 3.1 (2.8) | 33.5 (17.2)* | 329 (367) | 3497 (1084)* | 73 |
| 36h | 0.8 (1) | 181.2 (168.4)* | 3.8 (3.4) | 50.6 (24.1)* | 163 (220) | 3776 (2723)* | 74 |
| 48h | 1.1 (1.8) | 149.2 (96.2)* | 4.9 (4.3) | 76.6 (32.4)* | 178 (287) | 3513 (1591)* | 84 |
| 72h | 0.6 (0.8) | 140.7 (130.9)* | 6 (4.8) | 120 (51)* | 125 (172) | 3571 (952)* | 91 |
| 96h | 0.9 (1.54) | 140 (112.2)* | 8.3 (7.9) | 160 (67.6)* | 191 (328) | 3764 (1560)* | 92 |
| 120h | 1.1 (1.8) | 110.5 (131.4)* | 9.5 (9.8) | 190 (85)* | 178 (232) | 3177 (1400)* | 87 |
| 144h | 0.74 (1.1) | 0.93 (0.9) | 10.6 (11.2) | 200 (90)* | 130 (175) | 284 (672)* | – |
OP, ornithyne phenylactetate; PA, phenylacetate; PAP, phenylacetate in plasma; PAGNU, accumulated phenylacetylglutamine in urine; CreatinineU, creatinine in urine; SD, standard deviation.
*
p < 0.05, OP group compared with placebo.
**
In OP group only.
***
Molar ratios were calculated by dividing PAGNU accumulated (mmol) by OP administered (mmol), considering that 1 mol of PAGN results from 1 mol of PA (or OP).