Maternal phenylketonuria: case management as a preventive approach to a chronic condition affecting pregnancy.

Women with PKU are at extremely high risk for bearing children with multiple congenital anomalies and mental retardation. PKU related psychoeducational and psychosocial issues can impact reproductive decision making and the ability to resume and/or maintain a restricted phenylalanine diet prior to and during pregnancy. Intensive case management may provide a preventive approach to the current "crisis" in the management of maternal phenylketonuria, as well as facilitate ongoing diet therapy during adolescence and adulthood.