Table 2 Reported definitions of PAH deficiency phenotype based on pre-treatment Phe levelsa
Reported Phe ranges | N of respondents/total N of responded dietitians |
|---|---|
How do you define “Mild HPA” based on blood Phe levels in the newborn before treatment? | |
< =360 μmol/L (<=6 mg/dL) | 1/9 |
< 360 μmol/L (< 6 mg/dL) | 3/9 |
120–360 μmol/L (2–6 mg/dL) | 2/9 |
200–360 μmol/L (3.33–6 mg/dL) | 1/9 |
360–600 μmol/L(6–10 mg/dL) | 1/9 |
< 600 μmol/L (< 10 mg/dL) | 1/9 |
How do you define “Mild PKU” based on blood Phe levels in the newborn before treatment? | |
120–360 μmol/L (2–6 mg/dL) | 1/9 |
360–600 μmol/L (6–10 mg/dL) | 3/9 |
< 600 μmol/L (< 10 mg/dL) | 1/9 |
600–900 μmol/L (10–15 mg/dL) | 2/9 |
360–1200 μmol/L (6–20 mg/dL) | 2/9 |
How do you define “Moderate PKU” based on blood Phe levels in the newborn before treatment? | |
> 360 μmol/L (> 6 mg/dL) | 1/8 |
600–1000 μmol/L (10–16.67 mg/dL) | 1/8 |
600–1200 μmol/L (10–20 mg/dL) | 3/8 |
900–1200 μmol/L (15–20 mg/dL) | 2/8 |
1200–1800 μmol/L (20–30 mg/dL) | 1/8 |
How do you define “Classical PKU” based on blood Phe levels in the newborn before treatment?b | |
> 600 μmol/L (> 10 mg/dL) | 1/12 |
≥ 1000 μmol/L (> 16.67 mg/dL) | 1/12 |
> 1200 μmol/L (> 20 mg/dL) | 7/12 |
> 1200–1500 μmol/L (20–25 mg/dL) | 1/12 |
1200–2400 μmol/L (20–40 mg/dL) | 1/12 |
> 1800 μmol/L (> 30 mg/dL) | 1/12 |