van Spronsen F J, Verkerk P H, van Houten M, Smit G P, van der Meer S B, Bakker H D, Sengers R C
Beatrix Children's Hospital, University of Groningen, The Netherlands.
Acta Paediatr. 1997 Aug;86(8):816-8. doi: 10.1111/j.1651-2227.1997.tb08603.x.
To assess whether growth retardation in patients with phenylketonuria (PKU) is related to the strictness of their dietary treatment, the relationship between Z scores for height up to 3 y of age and different indices of dietary control in 103 early treated Dutch PKU patients was studied. As indices of dietary control, the mean phenylalanine (Phe) concentration, the frequency of plasma Phe concentrations < 200 and < 120 micromol/l, and the standard deviation of the individual plasma Phe concentrations were studied. These measures of the dietary control were divided into quartiles. The mean Z score of the studied patients showed a decrease of 0.18/y (SD 0.36). No statistically significant difference between any of the quartiles of the studied indices with growth retardation was found. None of the used indices of dietary control based on plasma Phe concentrations showed a relationship between different degrees of the strictness of dietary treatment with growth retardation in Dutch PKU patients.
为评估苯丙酮尿症（PKU）患者的生长发育迟缓是否与其饮食治疗的严格程度相关，研究了103例早期接受治疗的荷兰PKU患者3岁前身高的Z评分与不同饮食控制指标之间的关系。作为饮食控制指标，研究了苯丙氨酸（Phe）平均浓度、血浆Phe浓度<200和<120微摩尔/升的频率以及个体血浆Phe浓度的标准差。这些饮食控制指标被分为四分位数。研究患者的平均Z评分显示每年下降0.18（标准差0.36）。在所研究指标的任何四分位数与生长发育迟缓之间未发现统计学上的显著差异。基于血浆Phe浓度的所有饮食控制指标均未显示荷兰PKU患者不同程度的饮食治疗严格程度与生长发育迟缓之间存在关联。
