Importance of diet in maternal phenylketonuria

Diet has long been recognized as the primary treatment modality for individ uals with phenylketonuria (PKU) during infancy and childhood. Recent findin gs from the Maternal PKU Collaborative Study clearly indicate that dietary restriction of phenylalanine is also necessary to prevent the adverse effec ts of an elevated plasma phenylalanine concentration during pregnancy, whic h include microcephaly, physical anomalies, and mental retardation.