caa a22 4500 475785827 CHVBK 20180406123657.0 cr unu---uuuuu 170329e20000901xx s 000 0 eng 10.1007/PL00014388 doi (NATIONALLICENCE)springer-10.1007/PL00014388 Burgard Peter Department of General Paediatrics, University of Heidelberg, Im Neuenheimer Feld 150, 69120 Heidelberg, Germany e-mail: Peter_Burgard@med.uni-heidelberg.de, Tel.: +49-6221-562377; Fax: +49-6221-564624, DE aut Development of intelligence in early treated phenylketonuria [Elektronische Daten] [Peter Burgard] Designs, analyses and results of longitudinal studies of intelligence of patients treated early for phenylketonuria (PKU) are reviewed. All studies converge on the conclusion that after the age of 10 years, IQ development is stable for different degrees of dietary relaxation. On average, for each 300 μmol/l increase in blood phenylalanine (Phe) levels pre-school, IQ decreases by about half a standard deviation. Children with Phe levels below 400 μmol/l in early and middle childhood had the best outcomes which were near normal. PKU seems to suppress the global level of IQ without impairment of domain-specific competencies. For historical reasons there is no research on IQ development of early treated patients in middle or late adulthood, and it remains unclear whether older age groups might carry new risks. Conclusion It is argued that control group designs, meta-analysis, and interdisciplinary studies combining psychology, neurology and neuropathology could increase the understanding of phenylketonuria as well as the scientific basis of its treatment. Springer-Verlag Berlin Heidelberg, 2000 Key words Phenylketonuria nationallicence Intelligence nationallicence Meta-analysis nationallicence AbbreviationsGCSPKU German collaborative study of children treated for phenylketonuria nationallicence IQ intelligence quotient nationallicence Phe phenylalanine nationallicence PKU phenylketonuria nationallicence USCSPKU United States collaborative study of children treated for phenylketonuria nationallicence https://doi.org/10.1007/PL00014388 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/PL00014388 text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Burgard Peter Department of General Paediatrics, University of Heidelberg, Im Neuenheimer Feld 150, 69120 Heidelberg, Germany e-mail: Peter_Burgard@med.uni-heidelberg.de, Tel.: +49-6221-562377; Fax: +49-6221-564624, DE aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer