Pre-conceptual dietary management for maternal phenylketonuria.
Experience of preconceptual dietary management of maternal hyperphenylalaninaemia is limited. Two Edinburgh mothers with hyperphenylalaninaemia and borderline IQ have received dietary advice to maintain normal plasma Phe concentration both before conception and during pregnancy, in an attempt to prevent foetal abnormality. Satisfactory compliance was only obtained after intensive in-patient instruction and the diets were maintained for long periods prior to conception. Intellectual and psychological factors were very important and the availability of "same day" biochemical reports useful in encouraging the mothers. Both infants have normal Phe concentrations and satisfactory growth and development parameters at present. Further follow-up continues but these cases provide further evidence in favour of the use of preconceptual dietary advice in the management of maternal hyperphenylalaninaemia.
D000293:Adolescent; D000328:Adult; D003267:Contraception; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D010649:Phenylalanine / Q000097:blood*; D010661:Phenylketonurias / Q000097:blood / Q000178:diet therapy / Q000517:prevention & control*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000517:prevention & control*
D010649:Phenylalanine