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Table 3 Third stage protein substitute transition history of children with PKU

From: Transitioning of protein substitutes in patients with phenylketonuria: evaluation of current practice

Patient No

Gender

Type of second stage protein substitute

Type of third stage protein substitute

The age third stage protein substitute transition commenced (years)

The age third stage protein substitute transition finished (years)

Duration (months)

1

F

Semi-solid

Liquid

3.5

3.7

2

2

M

Semi-solid

Liquid

3.1

3.5

5

3

F

Semi-solid

Liquid

5.5

6.5

12

4

M

Semi-solid

Liquid

3.8

4.3

6

5

M

Semi-solid

Powder + Liquid†

5.2

6.6

17

6

M

Semi-solid

Liquid

5.3

5.3

0

7

F

Semi-solid

Powder

5.4

6.7

16

8

M

Semi-solid

Liquid

3.9

4.0

1

9

F

Semi-solid

Liquid

5.0

5.6

7

10

M

Semi-solid

Liquid

4.2

4.4

2

11

F

Semi-solid

Liquid

3.3

4.7

17

12

F

Semi-solid

Liquid

8.0

8.0

0

13

F

Semi-solid

Liquid

4.7

4.7

0

14

M

Semi-solid

Liquid

4.9

5.3

5

15

M

Semi-solid

Powder + Liquid‡

4.3

6.9

32

16

F

Semi-solid

Liquid

5.3

5.6

4

  1. M: Male; F: Female
  2. †Started transition onto cGMP powder and commenced on three doses of cGMP powder; the remainder were given as liquid amino acid protein substitute to ensure target blood phenylalanine levels were not exceeded
  3. ‡Started transition onto liquid, refused more than one dose of liquid so remaining doses were powdered protein substitutes concentrated in protein equivalent