Table 4.
Relative frequency (%) of patients with different intake of TPA according to PKU severity.
| PKU Severity | TPA | Pediatrics (<12 Years) n = 18 |
Adolescents (12–18 Years) n = 32 |
Adults (>19 Years) (n = 49) |
Total Percentage of Patients |
|---|---|---|---|---|---|
| HPA (N = 24) |
TPA < 100% | 1/24 (4%) | 1/24 (4%) | 1/24 (4%) | 12% |
| TPA ≥ 100–120% | - | 1/24 (4%) | --- | 4% | |
| TPA ≥ 120–140% | - | --- | --- | ||
| TPA ≥ 140% | 5/24 (21%) | 11/24 (46%) | 4/24 (17%) | 84% | |
| MILD PKU (N = 48) |
TPA < 100% | -- | --- | 1/48 (2%) | 2% |
| TPA ≥ 100–120% | -- | --- | 3/48 (6%) | 6% | |
| TPA ≥ 120–140% | --- | 2/48 (4%) | 2/48 (4%) | 8% | |
| TPA ≥ 140% | 12/48 (25%) | 11/48 (23%) | 17/48 (36%) | 74% | |
| CLASSICAL PKU (N = 27) |
TPA < 100% | -- | --- | 2/27(7%) | 7% |
| TPA ≥ 100–120% | -- | --- | 1/27 (4%) | 4% | |
| TPA ≥ 120–140% | -- | 2/27(7%) | 5/27 (19%) | 26% | |
| TPA ≥ 140% | -- | 4/27 (15%) | 13/27 (48%) | 67% |
Abbreviations: PKU—phenylketonuria; HPA—hyperphenylalaninemia and TPA—total protein adequacy.