DOES IMPAIRED GROWTH OF PKU PATIENTS CORRELATE WITH THE STRICTNESS OFDIETARY-TREATMENT

To assess whether growth retardation in patients with phenylketonuria (PKU) is related to the strictness of their dietary treatment, the rel ationship between Z scores for height up to 3 y of age and different i ndices of dietary control in 103 early treated Dutch PKU patients was studied. As indices of dietary control, the mean phenylalanine (Phe) c oncentration, the frequency of plasma Phe concentrations < 200 and < 1 20 mu mol/l, and the standard deviation of the individual plasma Phe c oncentrations were studied. These measures of the dietary control were divided into quartiles. The mean Z score of the studied patients show ed a decrease of 0.18/y (SD 0.36). No statistically significant differ ence between any of the quartiles of the studied indices with growth r etardation was found. None of the used indices of dietary control base d on plasma Phe concentrations showed a relationship between different degrees of the strictness of dietary treatment with growth retardatio n in Dutch PKU patients.