Table 2.
Age distribution of the cohort, abbreviations as in Table 1
| Disease | Mean age in years (range) | Pediatric patients (< 16 years) | Adult patients (≥ 16 years) |
|---|---|---|---|
| OTCD (all) | 22.2 (0.1–66.5) | 47 (39%) | 74 (61%) |
| OTCD (females) | 24.5 (2.2–66.5) | 28 (34%) | 55 (66%) |
| Cohort except OTCD | 13.4 (0.7–47.0) | 56 (64%) | 31 (36%) |
| Cohort except female OTCD | 14.5 (0.1–47.0) | 75 (60%) | 50 (40%) |
| ASLD | 16.8 (1.5–35.8) | 14 (45%) | 17 (55%) |
| ASSD | 9.0 (0.7–26.8) | 35 (81%) | 8 (19%) |
| ARG1D | 23.5 (4.2–47.0) | 4 (50%) | 4 (50%) |
| HHH syndrome | 4.7 (3.0–6.3) | 2 (100%) | 0 |
| CPS1D | 15.5 (8.1–22.9) | 1 (50%) | 1 (50%) |
| NAGSD | 27.2 | 0 | 1 (100%) |
| Total | 18.5 (0.1–66.5) | 103 (50%) | 105 (50%) |