Journal
EUROPEAN JOURNAL OF PEDIATRICS
Volume 159, Issue -, Pages S156-S160Publisher
SPRINGER-VERLAG
DOI: 10.1007/PL00014383
Keywords
maternal phenylketonuria; phenylalanine; collaborative study
Categories
Funding
- NICHD NIH HHS [N01-HD-2-3155, N01-HD-2-3149, N01-HD-2-3148] Funding Source: Medline
Ask authors/readers for more resources
The Maternal Phenylketonuria Study began in 1984 and during the intervening years, 572 pregnancies in hyperphenylalaninemic women and 99 controls and their outcomes have been evaluated. Among hyperphenylalaninemic women who delivered a live infant, only 15.9% were treated and in metabolic control preconceptually, however, another 18.4% were in control by 10 weeks. Compared to the results reported by Lenke and Levy in 1980, there is a marked improvement in outcome with treatment. Microcephaly was unusual in preconceptually treated pregnancies with well controlled phenylalanine restricted diets. Even in pregnancies that established control after conception but before the 8th week, congenital heart disease did not occur in the offspring, however, it did occur in 12% of pregnancies not achieving control until after 10 weeks of pregnancy. Conclusion The recommended level of blood phenylalanine during pregnancy is 120- 360 mu mol/l. Best results were obtained by close cooperation between the attending obstetrician and a metabolic team experienced in the care of persons with phenylketonuria.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available