[Effect of dietary non-compliance on development in phenylketonuria--studies of 14-year-old patients].
25 children with early treated PKU were studied at the age of 14 years. The IQ was higher at the age of 6-8, 10 and 14 years if the dietary control was good (75% of the control values up to 10 mg/dl) compared to children with poor control. The IQ however decreased up to the age of 14 years in both groups. Discontinuation of the diet in children with a good dietary control at the age of 6 years because of a normal EEG after phenylalanine loading causes a decrease of the IQ from 100 at 6 years to 90 at 10 years. The IQ remains stable thereafter up to 14 years. The IQ ist lower at the age of 7 or 8 years in those children in whom the discontinuation of diet is delayed because of abnormal EEG after phenylalanine loading at the age of 6 years, but remains stable up to the age of 14. According to these results a discontinuation of the diet at the age of 6 years can not be recommended even if the EEG is normal after phenylalanine load.
Theile
H
H
Abteilung Humangenetik, Bereiches Medizin, Karl-Marx-Universität Leipzig.
Graustein
I
I
Wässer
S
S
Bührdel
P
P
D000293:Adolescent; D004569:Electroencephalography / Q000187:drug effects; D005500:Follow-Up Studies; D006801:Humans; D007360:Intelligence / Q000187:drug effects; D010349:Patient Compliance; D010649:Phenylalanine / Q000008:administration & dosage*; D010661:Phenylketonurias / Q000178:diet therapy* / Q000523:psychology
D010649:Phenylalanine
Einfluss der Diätlockerung auf die Entwicklung bei PKU--Untersuchungen bei 14 Jahre alten Patienten.