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. 2023 Nov 22;15(23):4883. doi: 10.3390/nu15234883

Table 6.

Patients’ median [P25–P75] blood Phe metabolic control (µmol/L < 12 years and ≥12 years) and % of blood [Phe] within the Portuguese Consensus Recommendation according to age, PKU severity and type of treatment.

Median µmol/L
[P25–P75]
<12 Years
Median µmol/L
[P25–P75]
≥12 Years
Median % of Blood Phe Levels within Target Range
Age <12 years (n = 18) 294 [234–354] -- 65 [50–83.0]
≥12 years (n = 81) -- 450 [270–600] 61 [15.4–100]
PKU severity HPA (n = 24) 247 [234–279] 217 [186–249] 100 [98.3–100]
Mild (n = 48) 331 [257–360] 462 [313–553] 57 [22.7–92.8]
Classical (n = 27) -- 610 [462–806] 17 [0–55.9]
Type of treatment Diet only (n = 82) 295 [236–344] 462 [240–636] 59 [15.5–100]
Diet + BH4 (n = 17) 296 [266–325] 421 [347–503] 77 [50.9–94.1]

Abbreviations: PKU—phenylketonuria; HPA—hyperphenylalaninemia; BH4—tetrahydrobiopterin; Phe—phenylalanine.