|
Blood Phe level (consistently elevated) to prompt initiation of a Phe-restricted diet (n = 19)
|
|
≥ 360 μmol/L (≥6 mg/dL)
|
14 (74)
|
|
360–420 μmol/L (6–7 mg/dL)
|
1 (5)
|
|
≥ 420 μmol/L (≥7 mg/dL)
|
1 (5)
|
|
≥ 480 μmol/L (≥8 mg/dL)
|
2 (10)
|
|
≥ 600 μmol/L (≥10 mg/dL)
|
1 (5)
|
|
Optimal target range of blood Phe, by age
|
|
0–12 months of age (n = 19)
|
|
120–360 μmol/L (2–6 mg/dL)
|
19 (100)
|
|
> 1–2 years of age (n = 19)
|
|
120–360 μmol/L (2–6 mg/dL)
|
19 (100)
|
|
> 2–10 years of age (n = 19)
|
|
120–360 μmol/L (2–6 mg/dL)
|
19 (100)
|
|
> 10–18 years of age (n = 18)
|
|
120–360 μmol/L (2–6 mg/dL)
|
14 (78)
|
|
120–600 μmol/L (2–10 mg/dL)
|
2 (11)
|
|
320–600 μmol/L (5.33–10 mg/dL)
|
2 (11)
|
|
Lowest acceptable average level of blood Phe as a long-term treatment goal, by age
|
|
0–12 months of age (n = 18)
|
|
100 μmol/L
|
1 (6)
|
|
120 μmol/L
|
17 (94)
|
|
> 1–2 years of age (n = 17)
|
|
120 μmol/L
|
16 (94)
|
|
150 μmol/L
|
1 (6)
|
|
> 2–10 years of age (n = 17)
|
|
120 μmol/L
|
16 (94)
|
|
150 μmol/L
|
1 (6)
|
|
> 10–18 years of age (n = 18)
|
|
100 μmol/L
|
1 (6)
|
|
120 μmol/L
|
16 (89)
|
|
200 μmol/L
|
1 (6)
|
|
Would you be comfortable with a patient’s steady Phe level of < 120 μmol/L (n = 19)
|
|
Yes
|
6 (32) a
|
|
No
|
13(68)
|
|
Recommend maintaining higher-end therapeutic range blood Phe and more liberal natural protein intake (n = 19)
|
|
For most/nearly all patients
|
5 (26)
|
|
For some patients
|
9 (47)
|
|
Rarely/never
|
5 (26)
|
|
Recommend maintaining lower-end therapeutic range blood Phe levels and more restricted natural protein intake (n = 19)
|
|
For most/nearly all patients
|
4 (21)
|
|
For some patients
|
4 (21)
|
|
Rarely/never
|
11 (58)
|
