Demographic, clinical and laboratory characteristics
| Total | Supp. B6 vit. | No suppl. B6 vit. | P value | |
| n=37 | n=15 | n=22 | ||
| Sex M, n (%) | 22 (59.5) | 8 (53.3) | 14 (63.6) | |
| Age at inclusion* | 23.3±14.8 | 19.0±11.6 | 26.3±16.3 | |
| Age at diagnosis* | 9.7±5.2 | 10.0±4.4 | 9.5±5.8 | |
| <18 years, n (%) | 15 (40) | 8 (53) | 7 (32) | |
| Hepatic manifestations | 30 (81) | 11 (73) | 19 (86) | |
| Neurological manifestations | 7 (19%) | 4 (27%) | 3 (14%) | |
| Dose DP (mg)* | 948.6±369.2 | 830±334.8 | 1029.5±376.9 | |
| Dose (mg/kg)* | 17.4±5.3 | 16.3±4.4 | 18.1±5.8 | |
| Duration of treatment in months† | 59 (21.5–162.5) | 51 (22.3–78.1) | 78.2 (21.1–224.7) | |
| PLP level (nmol/L)† | 81.8 (61.9–117.4) | 137.2 (104.9–191.6) | 64.9 (52.6–83.4) | p<0.05 |
| PLP<35 (nmol/L), n (%) | 0 | 0 | 0 | |
| PLP˃110 (nmol/L), n (%) | 11 (30) | 11 (73) | 0 | p<0.05 |
| Hb (g/L)* | 14.1±1.5 | 14.0±1.5 | 14.1±1.6 | |
| WBC (G/L)* | 6.4±1.5 | 5.8±1.4 | 6.8±1.8 | |
| Platelets (G/L)* | 256.7±104.4 | 238.2±125.8 | 269.3±87.0 | |
| ALT (U/L) * | 46.9±33.8 | 41.5±32.8 | 50.6±34.7 | |
| Elevated ALT, n (%) | 11 (30) | 5 (33) | 6 (27) | |
| ASAT (U/L)* | 34.5±10.9 | 32.6±11.5 | 50.6±34.7 | |
| Elevated AST, n (%) | 10 (27) | 3 (20) | 7 (32) | |
| GGT (U/L)* | 36.9 (21.1) | 33.1±21.7 | 39.5±20.7 | |
| Elevated GGT, n (%) | 16.0 (43) | 6 (40) | 10.0 (45) | |
| Alkaline phosphatase (U/L)* | 158.0±119.4 | 175.2±120.8 | 147.4±120.2 | |
| Exchangeable copper (umol/L)* | 0.8±1.7 | 1.2±0.9 | 0.6±0.5 |
*Mean±SD.
†Median (IQR).
DP, D-penicillamine; PLP, pyridoxal phosphate.