INTELLECTUAL-DEVELOPMENT IN 12-YEAR-OLD CHILDREN TREATED FOR PHENYLKETONURIA
INTELLECTUAL-DEVELOPMENT IN 12-YEAR-OLD CHILDREN TREATED FOR PHENYLKETONURIA
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DOI：
10.1001/archpedi.1991.02160010037012
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发表时间：
1991-01-01
影响因子：
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通讯作者：
WARNER, R
中科院分区：
文献类型：
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作者：
AZEN, CG;KOCH, R;WARNER, R
对 95 名在新生儿期开始饮食治疗的 12 岁苯丙酮尿症儿童进行智力和成绩测试分数评估。 23名儿童在10岁以后仍将血液苯丙氨酸饮食控制在900μmol/L以下；另外 72 名儿童的血液苯丙氨酸在 18 个月至 10 岁时持续高于该水平。 12岁时的测试分数与开始饮食的年龄以及4至10岁时的血液苯丙氨酸水平呈负相关，与父母的智商分数和失去饮食控制的年龄呈正相关。 10岁以后苯丙氨酸水平保持在900μmol/L以下的儿童在测试成绩上没有表现出缺陷，但算术除外，在这项研究中，90%的儿童在6岁到12岁之间算术成绩有所下降。这些数据有力地支持了应在整个青春期维持苯丙氨酸饮食限制的建议。
Intelligence and achievement test scores were evaluated for 95 12-year-old children with phenylketonuria who had begun dietary therapy during the neonatal period. Dietary control of blood phenylalanine below 900-mu-mol/L was maintained beyond age 10 years in 23 children; 72 others had blood phenylalanine persistently above that level at ages ranging from 18 months to 10 years. Test scores at age 12 years were negatively correlated with the age at initiation of diet and with blood phenylalanine levels from ages 4 to 10 years, and positively correlated with parent IQ scores and the age at loss of dietary control. Children who maintained phenylalanine levels below 900-mu-mol/L beyond age 10 years showed no deficits in test scores, except for arithmetic, the scores of which declined between ages 6 and 12 years in 90% of the children in this study. These data strongly support a recommendation that dietary restriction of phenylalanine should be maintained through adolescence.