TY - JOUR
T1 - Meta-analyses of cognitive functions in early-treated adults with phenylketonuria
AU - Romani, Cristina
AU - Olson, Andrew
AU - Aitkenhead, Lynne
AU - Baker, Lucy
AU - Patel, Dhanesha
AU - Spronsen, Francjan Van
AU - MacDonald, Anita
AU - Wegberg, Annemiek van
AU - Huijbregts, Stephan
N1 - Publisher Copyright:
© 2022 The Authors
PY - 2022/12
Y1 - 2022/12
N2 - Our study estimated size of impairment for different cognitive functions in early-treated adults with PKU (AwPKU) by combining literature results in a meta-analytic way. We analysed a large set of functions (N = 19), each probed by different measures (average = 12). Data were extracted from 26 PKU groups and matched controls, with 757 AwPKU contributing 220 measures. Effect sizes (ESs) were computed using Glass’ ∆ where differences in performance between clinical/PKU and control groups are standardized using the mean and standard deviation of the control groups. Significance was assessed using measures nested within independent PKU groups as a random factor. The weighted Glass’ ∆ was − 0.44 for all functions taken together, and − 0.60 for IQ, both highly significant. Separate, significant impairments were found for most functions, but with great variability (ESs from −1.02 to −0.18). The most severe impairments were in reasoning, visual-spatial attention speed, sustained attention, visuo-motor control, and flexibility. Effect sizes were larger with speed than accuracy measures, and with visuo-spatial than verbal stimuli. Results show a specific PKU profile that needs consideration when monitoring the disease.
AB - Our study estimated size of impairment for different cognitive functions in early-treated adults with PKU (AwPKU) by combining literature results in a meta-analytic way. We analysed a large set of functions (N = 19), each probed by different measures (average = 12). Data were extracted from 26 PKU groups and matched controls, with 757 AwPKU contributing 220 measures. Effect sizes (ESs) were computed using Glass’ ∆ where differences in performance between clinical/PKU and control groups are standardized using the mean and standard deviation of the control groups. Significance was assessed using measures nested within independent PKU groups as a random factor. The weighted Glass’ ∆ was − 0.44 for all functions taken together, and − 0.60 for IQ, both highly significant. Separate, significant impairments were found for most functions, but with great variability (ESs from −1.02 to −0.18). The most severe impairments were in reasoning, visual-spatial attention speed, sustained attention, visuo-motor control, and flexibility. Effect sizes were larger with speed than accuracy measures, and with visuo-spatial than verbal stimuli. Results show a specific PKU profile that needs consideration when monitoring the disease.
KW - Executive functions, memory
KW - Language
KW - Metabolic diseases
KW - Patterns of cognitive impairments
KW - PKU
KW - Speed and accuracy
KW - Verbal vs visual
U2 - 10.1016/j.neubiorev.2022.104925
DO - 10.1016/j.neubiorev.2022.104925
M3 - Review article
C2 - 36283539
AN - SCOPUS:85141461788
SN - 0149-7634
VL - 143
JO - Neuroscience and Biobehavioral Reviews
JF - Neuroscience and Biobehavioral Reviews
M1 - 104925
ER -