| Isovaleric aciduria | Methylmalonic aciduria | Propionic aciduria | Neonates | Non-neonates | Total |
---|---|---|---|---|---|---|
N, patients | 4 | 21 | 16 | 28 | 13 | 41 |
Gender | Â | Â | Â | Â | Â | Â |
 Male | 2 | 9 | 11 | 14 | 8 | 22 |
 Female | 2 | 12 | 5 | 14 | 5 | 19 |
N, episodes | 4 | 25 | 19 | 29a | 19 | 48 |
Median age at episode, days | 7 (neonate) | 5 (neonate) | 6.5 (neonate) | 6 | 387 | 6.0 (neonate) |
387.0 (non-neonate) | ||||||
Median age at episode, months | 44.84 (non-neonate) | 2.53 (non-neonate) | 12.73 (non-neonate) | 0.20 | 12.73 | 0.2 (neonate) |
12.73 (non-neonate) | ||||||
Duration of episode, | ||||||
 Mean, days (SD) | 5.0 (1.8) | 7.8 (6.0) | 9.8 (10.5) | 6.6 (4.8) | 11.0 (10.8) | 8.4 (7.9) |
 Median, days | 5.0 | 7.0 | 6.0 | 6.0 | 7.0 | 6.0 |
 Range, days | 3–7 | 2–26 | 2–43 | 2–20 | 3–43 | 2–43 |
Concomitant treatmentb (%) | 0 (0.0) | 12 (48.0) | 9 (47.4) | 18 (62.1) | 3 (15.8) | 21 (43.8) |
Scavengers | 0 | 9 | 6 | 13 | 2 | 15 |
Haemodialysis/haemofiltration | 0 | 3 | 3 | 5 | 1 | 6 |
Baseline NH3, μmol/L | ||||||
 Mean (SD) | 666.8 (692.2) | 296.9 (206.2) | 355.0 (326.8) | 468.3 (365.3) | 171.3 (75.7) | 350.7 (321.3) |
 Median | 435.0 | 247.8 | 213.0 | 328.0 | 164.0 | 215.0 |
 Range | 164.0–1633.0 | 76.1–868.0 | 76.0–1200.0 | 96.0–1633.0 | 76.0–385.0 | 76.0–1633.0 |