Since classical PKU is rare in Taiwan, further studies including detailed neuropsychological tests will be required to evaluate the effect of treatment in this group of patients.

• 苯丙胺酸去氢酶缺乏而造成的苯酮尿症在台湾地区的发生率相当低，而且是轻型的病人较多，我们的研究指出早期饮食治疗确实可以使大部分患者得到正常的智力发展。