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: 6220550
• 负责人: Harvey L. Levy
• 金额: $ 0.07万
• 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/6220550
• 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; 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

孕妇苯酮尿症（PKU）是PKU的主要未解决问题，威胁要产生小头畸形，智力低下，先天性心脏病，畸形相和在世界各地许多后代的出生体重低。 怀孕期间苯丙氨酸受限的饮食疗法可能会提供一定程度的胎儿保护，但尚不清楚这种保护的程度和条件。 孕产妇PKU合作研究于1984年开发了这些问题。这项研究的目的是确定：1​​）苯丙氨酸受限饮食是否会减少智力低下，自发流产，低出生体重，先天性畸形，神经系统畸形，神经系统畸形和行为障碍的频率，而在怀孕期间无限制的苯丙氨酸母亲（HPA）母亲的妊娠妊娠妊娠损害？ 2）HPA妇女的怀孕结局是否限制了与非HPA妇女相当的怀孕期间苯丙氨酸摄入量的妊娠结局？ 3）在怀孕期间与母体苯丙氨酸水平有关的HPA妇女的怀孕结局。 4）胎龄在胎儿预后的干预措施开始时是否是吗？ 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因素的水平是多少？如果发现降低水平，对妊娠结局的影响有何影响？ NCRR澄清：研究正在流产