EFFECTS OF MATERNAL PHENYLKETONURIA ON PREGNANCY OUTCOME

母体苯丙酮尿症(PKU)是PKU尚未解决的主要问题， 有可能导致小头畸形，智力低下，先天性 许多后代患有心脏病、畸形相和低出生体重 在世界各地。苯丙氨酸限制饮食治疗期间 怀孕可能会提供一定程度的胎儿保护，但 而这种保护的条件尚不清楚。母性的北大 协作学习是在1984年发展起来的，旨在解决这些问题。 这项研究的目标是确定： 1)限制苯丙氨酸的饮食是否减少了脑力衰竭的发生 发育迟缓、自然流产、低出生体重、先天性 报告的畸形、神经和行为障碍 无限制服用高苯丙氨酸(HPA)母亲的妊娠情况 怀孕期间苯丙氨酸摄入量？ 2)限制苯丙氨酸摄入量的HPA妇女的妊娠结局 在怀孕期间与非HPA妇女相比？ 3)HPA妇女的妊娠结局是否与母体苯丙氨酸有关 在怀孕期间的水平。 4)干预开始时的胎龄是否预示着胎儿 结果呢？ 5)怀孕前开始节食是否有好处？ 6)怀孕期间的酪氨酸和微量元素水平是多少 补充营养对妊娠结局有什么影响？ 水平被发现降低了吗？ NCRR澄清：关于流产的研究

Maternal phenylketonuria (PKU) is a major unresolved problem in PKU, threatening to produce microcephaly, mental retardation, congenital heart disease, dysmorphic facies, and low birth weight in many offspring throughout the world. Phenylalanine restricted dietary therapy during pregnancy may offer some degree of fetal protection, but the extent of and conditions for this protection are not yet known. The Maternal PKU Collaborative Study was developed in 1984 to address these questions. The objectives of this study are to determine: 1) Does the phenylalanine restricted diet reduce the frequency of mental retardation, spontaneous abortion, low birth weight, congenital malformations, neurological and behavioral impairment reported in pregnancies of hyperphenylalanine (HPA) mothers who were on unrestricted phenylalanine intake during pregnancy? 2) Is pregnancy outcome in HPA women who restrict phenylalanine intake during pregnancy comparable to that of non-HPA women? 3) Is pregnancy outcome in HPA women related to maternal phenylalanine levels during pregnancy. 4) Is gestational age at the onset of intervention predictive of fetal outcome? 5) Are there beneficial effects of starting diet prior to conception? 6) What are the levels of tyrosine and trace elements during pregnancy and what are the effects on pregnancy outcome of supplementation if levels are found to be reduced? NCRR Clarification: research is on miscarriage