Maternal phenylketonuria: successful outcome in four pregnancies treated prior to conception

Eur J Pediatr. 1988 Oct;148(1):72-5. doi: 10.1007/BF00441820.

Abstract

The management of four pregnancies in two phenylketonuric women is described. A successful outcome in these pregnancies is ascribed to the initiation of treatment prior to conception and the maintenance of tight control with serum phenylalanine between 100 and 400 mumol/l throughout the gestation period. A trial of diet is desirable before a decision is made about pregnancy and before contraception is ceased. Close contact must be maintained with female phenylketonurics throughout their reproductive life to ensure that this process is followed.

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Humans
  • Phenylalanine / blood
  • Phenylketonurias / diet therapy*
  • Pregnancy
  • Pregnancy Complications / diet therapy*
  • Tyrosine / blood

Substances

  • Tyrosine
  • Phenylalanine