EFFECTS OF MATERNAL PHENYLKETONURIA ON PREGNANCY OUTCOME

母亲苯丙酮尿症对妊娠结局的影响

• 批准号: 6120770
• 负责人: Harvey L. Levy
• 金额: $ 3.04万
• 依托单位: BOSTON CHILDREN'S HOSPITAL
• 依托单位国家: 美国
• 财政年份: 1998
• 资助国家: 美国
• 起止时间: 1998-12-01 至 1999-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6120770
• 关键词: clinical research; congenital disorders; diet therapy; dietary restriction; embryo /fetus monitoring; female; gestational age; human pregnant subject; human subject; hyperphenylalaninemia; low birth weight infant human; medical complication; mental retardation; nutrition related tag; outcomes research; phenylalanine; phenylketonurias; pregnancy; spontaneous abortion

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

孕产妇苯酮（PKU）是PKU的主要未解决的问题， 威胁要产生小头畸形，智力低下，先天性 许多后代的心脏病，畸形相和低出生体重 全世界。苯丙氨酸限制饮食疗法 怀孕可能会提供一定程度的胎儿保护，但 和这种保护的条件尚不清楚。母亲PKU 1984年开发了协作研究，以解决这些问题。 这项研究的目标是确定： 1）苯丙氨酸受限饮食是否会降低精神的频率 智障，自发流产，低出生体重，先天性 报告的畸形，神经和行为障碍 不受限制的女性丙氨酸（HPA）母亲的怀孕 怀孕期间苯丙氨酸的摄入？ 2）是限制苯丙氨酸摄入的HPA女性的怀孕结果 在怀孕期间，与非HPA妇女相当？ 3）与母苯丙氨酸有关的HPA女性的怀孕结局 怀孕期间的水平。 4）在胎儿的干预开始时是胎龄 结果？ 5）在受孕之前开始饮食有益影响吗？ 6）怀孕期间酪氨酸和微量元素的水平是多少 如果 发现水平降低了？ NCRR澄清：研究正在流产