Skip to main content

Table 1 Algorithm for phenylalanine (Phe) intake adjustments according to mean Phe concentrations

From: Efficacy, safety and population pharmacokinetics of sapropterin in PKU patients <4 years: results from the SPARK open-label, multicentre, randomized phase IIIb trial

Mean Phe concentration, μmol/L

Phe intake adjustment

At Week 2

 0–300

Increase by 5 mg/kg/day

 301–359

No adjustment required

 360–∞

No adjustment required, but monitor concentration at next visit

Post-Week 2

 0–180

Increase by 15 mg/kg/day

 181–240

Increase by 10 mg/kg/day

 241–300

Increase by 5 mg/kg/day

 301–359

No change in dietary Phe intake

 360–∞

Determine if the subject had one or more previous dietary Phe intake increases

• If so, remove the dietary Phe in the order that it was previously increased, beginning with the amount of the last increase

• If not, no adjustment required

 360–1199

If this is first occasion at this concentration, monitor concentration at the next visit and if second occasion is at this concentration, provide dietary counseling

 1200–∞

If this is first occasion at this concentration, provide dietary counseling and monitor concentration at the next visit and if second occasion is at this concentration, provide dietary counseling and terminate from the trial