| BH4 responders (N = 19) | BH4 non-responders (N = 24) | p-value |
---|---|---|---|
Gender | 11 M/8 F | 9 M/15 F | 0.18 |
Age (years) at BH4 loading test (Mean ± SD) | 14.4 ± 4.5 | 13.6 ± 4.8 | 0.63 |
Phe§ at diagnosis (Mean ± SD) | 1148 ± 613 | 1612 ± 816 | 0.04 |
Phe tolerance* [Mean (range)] | 549 (235-1920) | 335 (220-630) | 0.02 |
Genotype One allele with PRA > 1% | 17 | 14 | 0.02 |
Two alleles with PRA > 1% | 13 | 5 | 0.001 |
Phe§ at loading test (T0) (Mean ± SD) | 615 ± 180 | 695 ± 228 | 0.2 |
Tyr§ at loading test (T0) (Mean ± SD) | 52.35 ± 8.5 | 48.12 ± 12.4 | 0.36 |
Phe/Tyr at loading test (T0) | 12.58 ± 4.5 | 16.9 ± 5.1 | 0.01 |