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Table 7 Performance of potentially exceptional participants with good cognition in spite of poor metabolic control or vice-versa poor cognition in spite of good control (see text for further explanation). Childhood Phe results for PKU108 are not available

From: Adult cognitive outcomes in phenylketonuria: explaining causes of variability beyond average Phe levels

 

Good cognition but poor adult control

PKU4

PKU107

PKU108

IT

UK

UK

Age

24

40

27

Education (in years)

13

14

16

Gender (M/F)

M

M

M

Full IQ

109

117

126

Feature search RT

−0.1

−0.3

−1.1

Conjoined search RT

0.1

−0.5

−1.4

Pegboard

0.1

−0.6

1.1

Average Z score

0.1

0.0

−0.2

% z score = > 1.5

4.2

8.3

8.3

Childhood (0–10 y)

 Phe Average Median

498

425

–

 Phe Variation

181

213

–

Adolescence (11–16 y)

 Phe Average Median

634

622

845

 Phe Variation

634

113

150

Adulthood (17 y +)

 Phe Average Median

1067

1115

1000

 Phe Variation

239

114

137

Lifetime

 Phe Average Median

672

978

975

 Phe Variation

169

148

139

Current Phe

1635

1080

685