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Table 1 Overview of impairments reported in outcome measures of cognitive functioning in ET AwPKU across studies

From: A systematic review of cognitive functioning in early treated adults with phenylketonuria

  Discontinued Treatment (Off-Diet) Mixed Sample (On Diet, Relaxed Diet, Off-Diet) On-Diet (* = Continuously Treated) Total
Reference [#] [37] [36] [38] [35] [48] [34] [33] [47] [49] [11, 17, 46] [55] [56] [52] [37] [45] [39] [38] [40, 41, 43] [42] [44]
N (ET AwPKU) 24 25 56 12 46 49 24 38 14 37 57 25 57 23 20* 25* 21 55–57* 21* 9*  
Attention & processing speed  
 Attentional capacity 0/1 2/2e     0/1 3/4    4/10d 1/1    0/1 1/1 1/2d      11/20 k
 Vigilance/focus 1/1      1/1 0/1    1/1    1/1b 1/1 1/1    2/2    7/8 k
 Processing speed    1/1a 2/2 0/2 0/1 0/2    0/1   1/1 1/1b     0/1    0/1 5/12 k
Executive functions
 Complex EF   0/1   1/2 3/3   1/6   3/13 3/6   0/1    0/2 0/1    1/1f   12/35 k
 Inhibitory control   1/2d     1/1j 1/2    0/3       0/2   2/2 0/4 0/1 5/15 k
 Working memory 0/1 2/2e   0/2 1/1 2/2 2/2    2/3     0/1 1/1 1/2d   4/6g    14/20 k
 Verbal fluency     0/2    2/2    1/2h      1/1       4/8
Language
 Basic language skills: semantic processing   0/1   0/1    1/2 0/9   1/5d       0/1      2/18 k
 Complex language skills        0/2 7/21   3/7      0/1       10/31
Memory & learning     0/1                  
 Immediate recall: verbal or visual     0/3    2/2c    0/3            2/8
 Delayed recall: verbal or visual     0/2    3/3   0/1 0/3      0/2       3/11
 Recognition: verbal or visual     0/1    1/1c    0/1      0/2       1/5
 Motor skills      1/1   0/2    2/2 3/4         1/1f   7/10
 Social-cognitive abilities                   2/2j    2/2
 Visual-spatial abilities        1/4   0/1    1/1    0/1       2/7
  1. aonly for off-diet ET AwPKU; b only for older (> 32) ET AwPKU; c Verbal memory/learning only; d significant differences observed in RT, not accuracy; e accuracy: different to controls, RT only different from on-diet ET AwPKU; f high-high PKU group worse performance than low-high PKU group (no controls included in analysis); g differences mainly observed in RT (only 1 measure of accuracy sign. different); h impairments in semantic, not letter fluency; i 1/2 disappeared when including age as covariate; no impairments observed when including IQ as a covariate; j only difference between < 720 and > 720 umol/L (no normative data available); k where off-diet and on-diet ET AwPKU reported separately, the total represents sum of outcome measures per paper, not row