Table 1.
All children | OTCD (females) | OTCD (males) | ASLD | ASS1D | Rare UCDs1 | |
---|---|---|---|---|---|---|
PedsQL Parent Report | ||||||
n | 190 | 74 | 32 | 35 | 32 | 17 |
Age in years Median (IQR) | 5.8 (3.0-8.9) | 5.9 (3.3-9.3) | 6.5 (3.3-9.3) | 5.0 (2.6-8.4) | 6.1 (3.4-8.5) | 4.6 (3.3-7.1) |
PedsQL Child Report | ||||||
n | 79 | 41 | 12 | 12 | 11 | 3 |
Age in years Median (IQR) | 8.6 (6.9-10.5) | 9.2 (7.2-11.0) | 9.0 (6.6-9.3) | 8.0 (7.4-10.1) | 7.8 (6.6-8.6) | 5.9 (5.8-6.5) |
All adults | OTCD (females)1 | OTCD (males) | ASLD | ASS1 | Rare UCDs | |
SF-36v2 | ||||||
n | 203 | 141 | 25 | 13 | 11 | 13 |
Age in years Median (IQR) | 32.9 (24.9-42.0) | 35.8 (28.5-42.8) | 30.0 (21.8-34.7) | 26.8 (23.1-38.5 | 23.9 (21.7-26.4) | 31.4 (20.2-51.2) |
Since the group sample sizes for individuals with NAGSD, CPS1D, ARG1D, HHH, and CTLN2 were too low to conduct statistical comparisons, they were grouped as “Rare UCDs”