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Causes of UCD-related hyperammonemia
| Causes | Patients N = 61 (%) |
Episodes N = 95 (%) |
|---|---|---|
| OTCD | 20 (32.8 %) | 35 (36.8 %) |
| ASSD | 18 (29.5 %) | 30 (31.6 %) |
| CPSD | 15 (24.6 %) | 18 (18.9 %) |
| ASLD | 3 (4.9 %) | 4 (4.2 %) |
| ARGD | 1 (1.6 %) | 2 (2.1 %) |
| NAGSD | 1 (1.6 %) | 2 (2.1 %) |
| Others | 3 (4.9 %)* | 4 (4.2 %) |
*Suspected OTCD without a molecular basis N = 2, HHH syndrome N = 1
