caa a22 4500 475785649 CHVBK 20180406123657.0 cr unu---uuuuu 170329e20000901xx s 000 0 eng 10.1007/PL00014392 doi (NATIONALLICENCE)springer-10.1007/PL00014392 Behaviour in early treated phenylketonuria: a systematic review [Elektronische Daten] [Isabel Smith, Julie Knowles] A search was made of the literature on behaviour in early treated phenylketonuria (PKU). Some 34 publications were selected for systematic review in order to answer the following questions: (1) does behaviour in early treated subjects with PKU differ from that in the general population?, (2) if so, do the differences show any consistent pattern?, matter in real life?, relate to intellectual status, phenylalanine (Phe) control or social factors? and (3) can behavioural problems be precipitated or reversed by changes in Phe control?. Eleven reports based on four studies of >20 subjects provided the most useful data. There was good evidence of differences between subjects with PKU and their unaffected peers in behaviour and perception of self. The pattern of results was consistent across nations and age groups (higher scores on emotional/neurotic items and lower scores on anti-social/aggressive items) and suggested that subjects with PKU are more prone to depression, anxiety, phobic tendencies, isolation from their peers and a less "masculine” self image. Features of attention deficit hyperactivity disorder and associations between behaviour problems and both lower IQ and a worse quality of Phe control were well documented in several studies. Conclusion The literature did not allow for definite conclusions on the cause of the behavioural problems but, taken together, the findings suggest that both the stressful dietary intervention and a degree of neurobiological impairment are likely to play a part. Springer-Verlag Berlin Heidelberg, 2000 Key words Phenylketonuria nationallicence Behaviour nationallicence Dietary intervention nationallicence Review nationallicence AbbreviationsADHD attention deficit hyperactivity disorder nationallicence FPI Freiburger personlichkeits inventar nationallicence MBI Mannheimer biographisches inventar nationallicence MEI Mannheimer eltern interview nationallicence MI mobility inventary nationallicence MMPI Minnesota multiphasic personality inventory nationallicence PFK personlichkeitsfragebogen für kinder nationallicence Phe phenylalanine nationallicence PKU phenylketonuria nationallicence RBST Rutter behaviour scale for teachers nationallicence Smith Isabel Phenylketonuria Register, Division of Biochemistry and Metabolism, Institute of Child Health, University College, 30 Guilford Street, London WC1N 1EH, UK e-mail: SmithI@gosh-tr.nthames.nhs.uk Tel.: +44-171-2429789, GB aut Knowles Julie Phenylketonuria Register, Division of Biochemistry and Metabolism, Institute of Child Health, University College, 30 Guilford Street, London WC1N 1EH, UK e-mail: SmithI@gosh-tr.nthames.nhs.uk Tel.: +44-171-2429789, GB aut https://doi.org/10.1007/PL00014392 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/PL00014392 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Smith Isabel Phenylketonuria Register, Division of Biochemistry and Metabolism, Institute of Child Health, University College, 30 Guilford Street, London WC1N 1EH, UK e-mail: SmithI@gosh-tr.nthames.nhs.uk Tel.: +44-171-2429789, GB aut NATIONALLICENCE 700 1- Knowles Julie Phenylketonuria Register, Division of Biochemistry and Metabolism, Institute of Child Health, University College, 30 Guilford Street, London WC1N 1EH, UK e-mail: SmithI@gosh-tr.nthames.nhs.uk Tel.: +44-171-2429789, GB aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer