Table 2.
Characteristics of initial hyperammonemic episodes in all patients
| CPS1D | OTCD | ASSD | All patients | |
|---|---|---|---|---|
| Number of patients | 12 | 23 | 27 | 63 |
| First hyperammonemic crisis ≥ 150 μmol/L | N = 20a | N = 60a | ||
| No | – | 3 (15.0 %) | 6 (22.2 %) | 9 (15.0 %) |
| Yes | 12 (100.0 %) | 17 (85.0 %) | 21 (77.8 %) | 51 (85.0 %) |
| Peak concentration [μmol/L] | ||||
| Minimum | 203 | 385 | 161 | 161 |
| Maximum | 2627 | 4050 | 2690 | 4050 |
| Mean | 1257 | 1875 | 1084 | 1378 |
| SD | 929 | 1105 | 682 | 950 |
| 150 – < 250 | 1 (8.3 %) | – | 1 (4.8 %) | 2 (3.9 %) |
| 250 – < 500 | 2 (16.7 %) | 1 (5.9 %) | 3 (14.3 %) | 6 (11.8 %) |
| ≥ 500 | 9 (75.0 %) | 16 (94.1 %) | 17 (81.0 %) | 43 (84.3 %) |
| Duration of crisis | ||||
| 1 day | – | 3 (17.6 %) | 3 (14.3 %) | 6 (11.8 %) |
| 2–7 days | 8 (66.7 %) | 9 (52.9 %) | 16 (76.2 %) | 34 (66.7 %) |
| 8–14 days | 3 (25.0 %) | 4 (23.5 %) | 2 (9.5 %) | 9 (17.6 %) |
| 15–19 days | 1 (8.3 %) | 1 (5.9 %) | – | 2 (3.9 %) |
Peak concentration of ammonia is the highest level of plasma ammonia during the initial crisis. SD Standard deviation
athree missing values