Abstract
Growth was assessed retrospectively in 110 children (49 boys, 61 girls) with HPA. They were 12.22 ± 4.74 years (mean ± SD) at the time of the last evaluation. 87 pts had the classical (type 1), 18 the mild form (type 2), 5 pts had persistent hyperphyenylalaninemia (type 3). Height was compared with the growth of normal children by calculation of the standard deviation score (SDS), using the data of Tanner et al. (1966).
The height-SDS was similar in all three groups with 0.40 ± 1.0 (type 1), 0.42 ± 1.06 (type 2) and 0.69 ± 0.46 (type 3). In 27 pts the dietary regimen was discontinued at the age of 8.81 ± 2.24 years with the height-SDS being 0.18 ± 1.19. At 16.02 ± 1.55 years the height-SDS had not changed significantly with 0.46 ± 0.10 SDS.
10 boys and 28 girls have reached final adult height. Boys measured 178.50 ± 8.53 cm, girls 164.51 ± 5.25 cm, which was 0.85 ± 4.74 and 3.64 ± 7.32 cm above target height with no significant difference between type 1 and 2.
Our data demonstrate that growth is normal in the different forms of HPA and is not affected by discontinuation of the dietary regimen.
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Brämswig, J., Karassalidou, S. & Ullrich, K. A RETROSPECTIVE ANALYSIS OF THE GROWTH PATTERN OF 110 CHILDREN WITH HYPERPHENYLALANINEMIA (HPA). Pediatr Res 22, 235 (1987). https://doi.org/10.1203/00006450-198708000-00134
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DOI: https://doi.org/10.1203/00006450-198708000-00134