Table 4.
Medication given during the initial crisis
| CPS1D | OTCD | ASSD | All patients | |
|---|---|---|---|---|
| Number of patients | 12 | 23 | 27 | 63 |
| Medication during initial crisis | ||||
| unclear | – | 5 (21.7 %) | 7 (25.9 %) | 12 (19.0 %) |
| yes | 12 (100 %) | 18 (78.3 %) | 20 (74.1 %) | 51 (81.0 %) |
| L-arginine | 8 (66.7 %) | 15 (65.2 %) | 20 (74.1 %) | 43 (68.3 %) |
| Benzoic acid | 1 (8.3 %) | – | – | 1 (1.6 %) |
| Carglumic acid | 1 (8.3 %) | 3 (13.0 %) | 2 (7.4 %) | 6 (9.5 %) |
| L-citrulline | 1 (8.3 %) | 2 (8.7 %) | – | 3 (4.8 %) |
| Levocarnitine | 3 (25.0 %) | 7 (30.4 %) | 8 (29.6 %) | 18 (28.6 %) |
| Sodium benzoate | 7 (58.3 %) | 15 (65.2 %) | 18 (66.7 %) | 41 (65.1 %) |
| Sodium phenylacetate & sodium benzoate | – | 2 (8.7 %) | 2 (7.4 %) | 4 (6.3 %) |
| Sodium phenylbutyrate | 1 (8.3 %) | 4 (17.4 %) | 7 (25.9 %) | 12 (19.0 %) |
Unclear means patients have not received medication or data were not entered