From: Clinical course of 63 patients with neonatal onset urea cycle disorders in the years 2001–2013
Parameter | CPS1D | OTCD | ASSD | All patients |
---|---|---|---|---|
Number of patients | 12 | 23 | 27 | 63 |
Type of hyperammonemic event | ||||
▪ no event ≥ 500 μmol/L | 3 | 6 | 10 | 19 |
▪ at least one event ≥ 500 μmol/L, patient alive | 5 | 7 | 13 | 26 |
individual number of events ≥ 500 μmol/L | ||||
1 | 3 | 6 | 10 | 20 |
2 | 1 | 1 | 2 | 4 |
3 | 1 | – | 1 | 2 |
▪ at least one event ≥ 500 μmol/L, patient died | 4 | 10 | 4 | 18 |
individual number of events ≥ 500 μmol/L | ||||
1 | 4 | 9 | 2 | 15 |
2 | – | 1 | – | 1 |
3 | – | – | 1 | 1 |
4 | – | – | 1 | 1 |